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Monday, July 25, 2011

7/25/2011 - Sjögren's syndrome, LUPUS,

7/25/2011 - Sjögren's syndrome, LUPUS,

Sjogren’s is an AI disease. Some people call it a syndrome and others call it a disease. In our house, we call it a disease. 90% of people with Sjogren’s are women. The average age of onset is 48 although children can also have Sjogren’s. It is unclear what causes Sjogren’s; if it is environmental or inherited. Usually a virus or an infection ‘triggers’ the disease to advance or flair. Most people go six years with various symptoms, jumping from doctor to doctor, before a proper diagnosis is made. Most people have never heard of Sjogren’s and the doctors who should be screening for it usually don’t.

Symptoms of Sjogren’s include (but aren’t limited to) –

- Neurological problems such as concentration, memory loss, brain fog.
- Dry Nose, recurrent sinusitis, nose bleeds.
- Dry mouth, mouth sores, dental decay: Difficulty with chewing, speech.
- Dry skin, vasculitis, Raynaud’s phenomenon
- Stomach upset, gastroparesis, autoimmune pancreatitis
- Peripheral neuropathy (numb or burning legs and.or arms)
- Dry eyes, corneal ulcerations, and infections
- Difficulty swallowing, heartburn, reflux, esophagitis
- Recurrent bronchitis, pneumonia, interstitial lung disease
- Arthritis, muscle pain.
- Abnormal liver function, chronic active autoimmune hepatitis, cirrhosis
- Severe fatigue
- Hyper sensitivity to medications
- “asthma’ type wheezing and heart rhythm issues

Some people have just one or two symptoms and lead ‘normal’ lives. Others get nailed with many of the symptoms and have a very hard time functioning.

Blood tests, the Schirmer eye test, a lip biopsy of a salivary gland, and salivary scintigraphy are tests that are performed to check for Sjogren’s .

There is no cure. There are medications available that can help people with the symptoms. Not everyone can tolerate the medications. 60% of people with Sjogren’s are hyper drug sensitive and have a higher incidence of getting the ‘side effects’ then the rest of the population.

Sjogren’s is considered ‘not fatal’. However, the complications from Sjogren’s are. Pneumonia, liver failure, kidney failure and other internal organ involvement are very serious. The incidence of lymphomas are significantly higher in people with Sjogren’s. Also, the majority of people with one AI disease will eventually be diagnosed with others. Many people with Sjogren’s end up also having Thyroid problems such as Graves Disease or Lupus.

For the conspiracy angle to make this a ‘legitimate’ thread and not just a public service announcement I present some brief anecdotal evidence. In the many discussions Liz (FlyersFan) has had with other Sjogren’s patients over the past months she has discovered that many of them are wondering about a link between AI disease and vaccinations. The Sjogren’s patients who feel there may be a link have no proof, but in their hearts and souls many feel that there might be a link between vaccinations they received and their AI diseases. In particular, some are looking back at their 1970s Swine Flu vaccinations and are seeing that they, and others they know who received the shots, have AI diseases, whereas those they know who didn’t receive the shots now do not have AI diseases. For the record, Liz did NOT receive a Swine Flu shot in the 1970s, or this time around either. She has had all other vaccinations, including some unusual ones that the military gave her such as the plague. 

I hope it makes people aware of Sjogren’s and that if they think they have some symptoms then they should get tested.

First lupus breakthrough in 50 years 
Professor Mackay was the first to show that the overproduction of BAFF was driving lupus. In a follow up study, elevated levels of BAFF were discovered in patients with a number of autoimmune diseases including lupus, rheumatoid arthritis and Sjögren’s syndrome.

She said this was an exciting discovery as it implied that if BAFF production can be blocked, the entire cascade effect that resulted in autoimmune disease could be prevented. 
(visit the link for the full news article)

 unforunately, Benlysta, only helps 35-40% of lupus patients, at best. 
It does not seem to effect lupus sufferers with organ involvement. Although, in the patients it did help-it helped with joint pain. That's a huge plus. 

The only standard drugs there are for lupus are an anti malarial drug, steroids (often high dose steroids), and a chemotherapy drug. Plus pain killers. I don't know if this the drug lupus sufferers are hoping for...but-at least-it's a step in the right direction.  

Benlysta approved: Which Lupus patients will benefit? 

If it is in its early stages it can be controlled with lifestyle changes: 

Stay out of the sun! If you go out wear a hat and cover up your arms and legs and wear sunglasses. The pain attacks are called flares by sufferers of this condition. I call them solar flares. These attacks are triggered by the sun or florescent lighting. Also she may find that her eyes have become light sensitive during these attacks and she should darken her house for a bit. 

Next she should take at least one aspirin a day (after checking with her doctor) to keep her blood thin. One of the effects of lupus is called sticky blood and the aspirin helps to keep this condition at bay. Also the aspirin helps bring down any inflammation in the body, one of the conditons that usually comes on later in the disease. During major flares I take 4 aspirin a day which is not enough and I just got used to the pain. Also, ice packs on a swollen neck or back or over the spine give a lot of instant relief if she is in the inflammation stage. 

Eventually many sufferers develop high blood pressure which then causes kidney damage, so she might want to go easy on eating a lot of protein, think chinese with lots of veggies or fish and veggies and rices and pastas. Also the net claims that potatoes, tomatoes, eggplant, bean sprouts are trigger foods for attacks, but I have never noted any correlation from these foods and my flares. 

The doctor will probably put her on a course of quinine prescriptions and steroids, these have many side effects which vary greatly between people. After years of this and learning the rules to follow many people go off the medication and try to control it by diet and controlling their environment and with aspirin. 

It's a difficult conditon to describe, it varies and takes many different forms but the triggers are the same, so if her symptoms don't match mine, doesn't matter, she should still stay out of the sun and follow the above. 

The whole point is to avoid the flares. The flares cause the damage. Flares can last 2 days or 2 months, it is mostly inflammation within the body, so stay cool, out of the sun, use ice packs, take aspirin, eat right, and try to exercise. 

We have heard acupuncture can be affective, a lot of 'medical' input has been rather ineffective. We thought it may be advantageous to look into Chinese remedies and such. 


I used acupuncture for awhile and it did help relax the muscles which during a flare are tensed constantly from the pain, for some reason it worked better than massages did (maybe all the rubbing in the massage aggravated the inflammation?). The acupuncturist? also prescribed a course of Chinese herbals that were very potent and honestly they just gave me nausea and headaches to add the the pain. Just the acupuncture alone is what helped. I only visited him during flares and as I learned to avoid them he was not needed as much and also I figured out that the ice packs brought down the interior inflammation which was causing the pain, so now I don't visit him at all during my rare flares.

Also, one more bit, sipping on tonic water which contains quinine seems to shorten the length of the flares, it could be just my knowing that quinine medicine is one of the drug courses doctors use makes it seem as though it is helping. The amount of quinine in it is very small.

How is she doing right now? You can tell her for me that people think you're a movie star when you wear sunglasses, a baseball cap and a large men's dress shirt over your outfit, Lol! They think you're a star incognito! Make sure she buys some fun sunglasses! And also, after covering up for awhile she'll notice that her facial lines are going away and the skin on her shoulders, arms and legs will also improve to a great degree! So, there are side benefits to this as I'm 46 and often mistaken for someone in their late 20s! 


What is lupus?
Lupus is a chronic (long-lasting) autoimmune disease where the immune system, for unknown reasons, becomes hyperactive & attacks normal tissue. This attack results in inflammation & brings about symptoms.

What does autoimmune mean?
'Auto' means 'self', so autoimmune literally means that the immune system fights the body itself. Instead of fighting & attacking the bad tissues, such as viruses, it turns on itself & attacks the good tissues.

What is inflammation?
It is a protective process our body uses when tissues are injured. Inflammation helps to eliminate a foreign body or organism (virus, bacteria) & prevent further injury. Signs of inflammation include- swelling, redness, warmth & pain.

What are antibodies?
Antibodies are proteins produced by white blood cells (B lymphocytes). Their normal function is to glue up bacteria and make them easy for the white blood cells to capture and destroy. When the immune system goes wrong, antibodies can be formed that bind to bits of the body (an auto-antibody). Sometimes infection can cause auto-antibodies to be produced and this may be one of the causes of Lupus. The antibodies circulate in the blood, but some of the body's cells have walls permeable enough to let some antibodies in. These can then attack the DNA in the cell's nucleus. That's why some organs can be attacked during a flare while others aren't.

What are the different kinds of lupus?
Discoid lupus (also known as Cutaneous lupus) affects the skin.
Systemic lupus attacks multiple systems in the body which may include- the skin, joints, blood, lungs, kidneys, heart, brain & nervous system.
Drug-induced lupus may develop after taking certain prescription medications. Symptoms generally disappear after the drug is discontinued.

What are the symptoms of systemic lupus?
The symptoms can include- Arthritis (swelling and pain of the joints), muscle pain and weakness, fatigue, sun-sensitivity, hair loss, "Butterfly" or malar rash (a rash across the nose and cheeks), fever, anaemia, headaches, recurrent miscarriages. For more symptoms & descriptions of symptoms see the symptoms page. Some people will have only a few symptoms, others may have them all.

What are the symptoms of discoid lupus?
They include a variety of different looking skin rashes, photosensitivity, & sometimes mouth or nose ulcers.

How is discoid lupus different to systemic lupus?
Discoid Lupus is confined to the skin, whereas systemic lupus may involve any organ system in the body, as well as the skin.

Can discoid lupus turn into systemic lupus?
In approximately 10% of discoid lupus cases, it evolves & develops into systemic lupus. However, this can't be predicted or prevented from happening.

What is the difference between drug-induced lupus & systemic lupus?
Systemic lupus is irreversible, whereas drug-induced lupus generally is reversible. The symptoms of drug-induced lupus generally do not include- kidney involvement or central nervous system involvement.

What drugs are most commonly associated with drug-induced lupus?
The following medications have been definitely proved to be associated with drug-induced lupus-Procainamide (used for heart rhythm abnormalities), Hydralazine (used for high blood pressure), Isoniazid (used for tuberculosis), Quinidine (used for heart rhythm abnormalities), Phenytoin (used for seizures). There are other drugs which might possibly be associated with drug-induced lupus, but as yet there is no definite proof.

Should people diagnosed with SLE or discoid lupus avoid taking the drugs associated with drug-induced lupus?
Most of the drugs associated with drug-induced lupus can be safely used in people with SLE or discoid lupus if there are no suitable alternatives.

How soon after taking the drug do the symptoms appear, & how long after stopping the drug do they disappear?
Drug-induced lupus requires months to years of frequent use of a drug before symptoms appear. Usually symptoms disappear after six months after stopping the drug, but it could be days r weeks, it varies. The ANA may remain positive for years.

What causes lupus?
The exact cause is unknown, but it is likely to be a combination of factors. A person's genetic make-up & exposure to certain trigger factors may provide the right environment in which lupus can develop.

Is lupus hereditary?
It is suspected that people inherit something from their parents that predisposes them to develop lupus. They are not necessarily pre-destined to develop lupus, but they may be more susceptible. Relatives of lupus patients have an approximate 5-12% greater tendency to get the disease if family members have it.

How common is lupus?
It is not known why, but lupus occurs more often in certain ethnic groups. The incidence in Caucasians is approx. 1:1000. In African-Americans, the incidence is approx. 1:250. In Latinos the incidence is approx. 1:500.

What can trigger lupus?
It is believed that certain things may trigger the onset of lupus or cause lupus to flare, these include- Ultraviolet light, certain prescription drugs & antibiotics, infections or viruses, hormones & stress.

Are there any medications people with lupus should avoid?
There are no absolute contraindications to medications for people with lupus. But, as people with lupus are usually 'allergic' people, your doctor should watch for any connection between flares & medications, especially oral contraceptives, sulfa antibiotics & penicillin.

Is there a test for systemic lupus?
No, there is not a single diagnostic test for SLE.

Why is SLE so difficult to diagnose?
For a number of reasons-
SLE is a multi-system disease, & before a multi-system disease can be diagnosed, there have to be symptoms in many parts of the body & lab work (blood tests) that supports the presence of a multi-system disease.
SLE is also difficult to diagnose because it is a disease that does not typically develop rapidly, but develops slowly & evolves over time. Symptoms come & go, it can take time for the disease to show up in blood tests, which one time can be positive & the next be negative again. It can take months or even years for enough symptoms to show up for the doctor to be able to make an accurate diagnosis.
SLE is known as a great imitator, because it mimics so many other diseases & conditions, which often have to be ruled out.
SLE is difficult to diagnose because there is no one diagnostic test for lupus, the doctor has to do a full examination of the patient & do various tests, before looking at all the evidence & coming to a conclusion.

How is SLE diagnosed?
Physicians have to gather information from a variety of sources- past medical history, lab tests & current symptoms. They use a list of 11 criteria to help diagnose SLE. Generally, a person needs to satisfy at least 4 out of the 11 criteria before a diagnosis can be made. (see the diagnosis page for more info.)

What is the ANA test?
The anti-nuclear antibody (ANA) test is a blood test that measures the antibodies that are directed against various components of the nucleus, so-called anti-nuclear antibodies. The nucleus of living cells contains many chemicals, including the well known DNA & RNA. For reasons which are unclear, patients produce antibodies which are directed against a number of these molecules.Throughout the world, the ANA test has become the screening test for lupus. Patients with active lupus generally have high levels of anti-nuclear antibodies. About 95% of people with SLE will have a positive ANA test at some point during their disease. It is rare to have lupus & have a negative ANA test, however it does happen, it can also take a while for the ANA test to become positive.  It is also possible for the ANA to convert from positive to negative following administration of steroids, cytotoxic drugs or kidney failure. Unfortunately, the ANA test, although a very useful screening test, is not specific to lupus. It can be positive in other connective tissue disorders, & also in healthy people. Therefore, a positive ANA test is not diagnostic of lupus, & is only an indicator. A positive ANA test only satisfies one criterion, a person would need to satisfy at least three additional criteria before a doctor would consider diagnosing lupus.

My ANA test came back 'Borderline Positive', what does this mean?
All lab tests have normal values. If a test comes back & the value is at the upper limit of normal, this is often referred to as being borderline. It is likely that a borderline positive ANA assumes more importance if other criteria are also present.

What doctor should a lupus patient see?
There are no rules here. Lupus patients can be diagnosed & treated by a number of different specialists, or indeed a combination which could include- rheumatologist, dermatologist, nephrologist, immunologist, or they can just be treated by their GP.

Do all lupus patients have the same symptoms?
No, symptoms vary from patient to patient. They even vary within one patient from time to time. Lupus is a disease that can attack different organ systems of the body, & it therefore affects everyone differently.

Can an individual with lupus continue to develop new symptoms?
A patient's symptoms can vary from week to week, even from day to day. However it is uncommon for the affected organ system to change, e.g. it is rare for a patient with kidney disease to develop central nervous system lupus.

Is lupus infectious or contagious?
No, it is neither.

Is there a cure for lupus?
At present there is no cure for lupus, but research is being carried out the world over, to find new treatments for lupus & to find out what causes lupus to develop, so there is hope for the future. However, lupus can be controlled using medications.

How is lupus treated?
The majority of lupus symptoms are due to inflammation & so the treatment is aimed at reducing that inflammation. There are four families of medications used in the treatment of lupus- Nonsteroidal anti-inflammatory drugs (NSAIDs), corticosteroids, antimalarials, & cytotoxic drugs (chemotherapy). For more information see the medications page.

What is a flare?
A flare is a sudden change of disease activity, for example, the development of new symptoms. A patient may suddenly feel weak & have joint & muscle aches. Flares can take on many different forms, indicating that the disease is quite active.

What is an exacerbation?
An exacerbation is a 'worsening' & is a term that is generally synonymous with a flare.

What is remission?
A remission is a period of disease-free activity. Certain cases of lupus have become permanently inactive, or in total remission. Although total remission is rare, partial remission - a definite, but limited, period of inactive disease - is more common.

How long will a flare last? How long will remission last?
There is no way of predicting how long a flare will last when it comes, nor is there any way of predicting how long remission will last when it comes.

What is the connection between lupus & allergies?
An allergic state produces a very specific antibody to substances such as drugs, pollen & grass. People with lupus are often very sensitive to such substances.

Is lupus a fatal disease?
The majority of people living with lupus today can expect to live a normal life span. About 20 or so years ago it was a different matter, many more people died from lupus. This was due to the fact that it was only diagnosed when it was very severe, & treatments weren't as effective. Now, with better diagnostic facilities, increased awareness & effective treatments, at least 90% of people with lupus will lead a normal lifespan. Lupus does vary in intensity & degree, & there are people who have a mild case, there are those who have a moderate case & there are some who have a severe case (severe organ involvement), which tends to be more difficult to treat & bring under control. Recent studies in longterm survival rates: Patients diagnosed with Lupus in 1990/95 have 92% chance of living more than the next 5 years (88% if initial diagnosis was lupus nephritis). This compares with 49% in 1960s. The figure has been steadily rising over the decades. Also remember that the chance of living more than the next 5 years for everybody, lupus patient or not, is less than 100%.

When people die of lupus, what do they usually die of?
Overwhelming infection & kidney failure are the two most common causes of death in people with lupus.

Are people with lupus more likely to develop cancer?
People with lupus are no more likely to develop cancer than people in the general population. However, people who have received certain chemotherapy drugs do carry the added risk of developing cancer sometime in later life.

Is there a connection between lupus & multiple sclerosis?
MS & lupus are both autoimmune diseases, & you can have both together, but they are diagnosed & treated in different ways. Lupus can sometimes mimic the symptoms of MS.

Is lupus like AIDS?
No. In AIDS, the immune system is underactive, but in lupus it is overactive. HIV (which can lead to AIDS) is transmissible, lupus isn't.

Can a lupus patient get another autoimmune disease?
Yes, & it is quite common. Antibodies can develop against a variety of organs, tissues or glands, resulting in many different diseases. Among the most frequently experienced autoimmune diseases for a lupus patient to have are Sjogren's Syndrome, multiple sclerosis, & Hashimoto's thyroiditis.

How can I find out more about lupus?
To start with, read all of the information on this site, & follow the links to other sites. Secondly, read some of the many books available on lupus, start by visiting the lupus bookstores on this site, the books are available at a discount! US customers UK customers. Join the national lupus charity in your country, e.g. Lupus UK, The Lupus Foundation of America, they can provide information & support, & run regional groups in your area where you can meet fellow lupus sufferers.

If you still have any questions that want answering, please visit the Message Boards

Lupus and men

It is estimated that for every 10 people who have lupus, only one will be male.
It is often said that men with lupus will be more seriously affected than women, but is this true? Recent studies1 have shown that men with lupus have an increased frequency of seizures, immune-mediated anaemia (low haemoglobin), & lupus anticoagulant (which can lead to blood clots). On the other hand men seem to have a lower frequency of Sjogren's Syndrome, which causes dry eyes & dry mouth. Although men are more likely to have these more serious manifestations, they show up the same in both sexes, i.e. if looking at a man & a woman who have seizures, the man won't necessarily have them worse than the woman.
Do men cope differently? It may be harder for a man to cope with having lupus, because it is often thought of as a women's disease, they may wonder if they are 'less of a man' because they have it, which is certainly not the case. They may also have difficulty in discussing the illness with others, & because there aren't as many men with lupus, they may find it hard to find another sufferer to talk with. Men are often thought of as the bread winner, & if they may feel pressure if they aren't able to work because of their illness. But worrying about such things & getting stressed can make the lupus worse, which leads to being stuck in a viscious circle. This can also lead to problems in their relationships.
Hormones are thought to play a big part in lupus, especially the female hormone oestrogen. Both males & females produce the hormones oestrogen & androgen, but in different quantities. However, it has been shown that men with lupus do not produce abnormal levels of oestrogen. Men with lupus have normal fertility, muscles, hair patterns, voices, etc, they are no less 'manly' than any 'normal' male. More research is needed to determine the role of hormones in lupus.

Autoimmune Diseases

Sjögren's Syndrome

In 1933, Henrik Sjögren (pronounced show-gren) described some symptoms common to a group of patients - dry eyes (keratoconjunctivitis sicca), dry mouth (xerostomia), & arthritis. In the late 60s, doctors found that many patients with these symptoms had an autoimmune process. Sjögren's syndrome can be part of many autoimmune diseases, including lupus, or may exist by itself (primary Sjögrens). At least 10% of lupus patients have Sjögren's.

The symptoms
The eyes usually gritty & scratchy, & are sometimes very sensitive to light. A dry mouth can make swallowing dry foods difficult, & can cause tooth decay & dental hygiene problems. Other mucous membranes may be dry including the lining of the stomach and vagina. Joint pains can also be present.
Sjögren's patients often have very high levels of autoantibodies, especially the anti-Ro antibody.

Sjögren's can be diagnosed with the Schirmer's test which involves putting a very small strip of blotting paper in each eye, to measure the amount of tears produced. Another test is the Rose Bengal staining test, which stains the cornea to check for pitting or areas of scarring.
Patients who have both lupus & Sjogren's are more likely to have the autoantibodies anti-SSA/Ro & anti-SSB/La.
Sometimes a lip biopsy is necessary to confirm the diagnosis.

The treatment is usually symptomatic. Dry eyes can be treated with artificial tears, dry mouth with artificial saliva, or just chewing gum or drinking fluids. There has been some evidence to show that Plaquenil (hydroxychloroquine) can be helpful.

Antiphospholipid Antibodies / Syndrome

Antiphospholipid syndrome is also known as Hughes' Syndrome (named after the doctor who discovered it - Dr Graham Hughes).
Patients with antiphospholipid antibodies & certain symptoms are usually diagnosed with Antiphospholipid Syndrome. These patients have an increased risk of thrombosis (clotting) in veins & arteries. It can be present in lupus patients & also non-lupus patients.

The main symptoms of the syndrome are-
  • Vein Thrombosis (leg, arm or internal organ)
  • Artery Thrombosis -
      Brain - headaches, neurological features
      Limb - pain and circulation problems
      Heart - chest pain
      Other organs - lungs, kidneys
  • Recurrent abortion or miscarriage (usually in the 2nd or 3rd trimester).
  • Low platelet count (thrombocytopenia)
  • Livedo reticularis (lacy, mottled pattern on skin)
  • Migraine
  • Epilepsy
  • Memory loss
  • Chorea (abnormal motor skills)
  • Stoke

Testing for the antiphospholipid antibody
The anticardiolipin (ACA) test measures the actual antibody levels. The three classes of anticardiolipin that are usually tested for are - IgG, IgM & Iga.
Another test, the lupus anticoagulant is a more complicated test & is far less reliable.

The drugs commonly used to treat it are aspirin, Warfarin & Heparin, which help to thin the blood. Aspirin is often given in small doses (75mgs-100mgs daily), this makes the platelets less sticky, which helps to prevent a thrombosis.
Warfarin is usually given to patients who have a larger thrombosis. Warfarin cannot be given to patients who are pregnant, so Heparin is normally used in these cases.
In some patients, anticoagulant therapy is life-long.

Mixed Connective Tissue Disease

Mixed Connective Tissue Disease resembles lupus, it has features of lupus, but also of scleroderma.
It is characterised by four main features -
Raynaud's Phenomenon - Fingers suddenly become cold & turn white, blue, & finally red when circulation returns. The Raynaud's Phenomenon in MCTD is more prominent than in lupus, and is reminiscent of scleroderma.
Prominent arthritis - In MCTD, arthritis is more like rheumatoid arthritis with swelling, especially in the fingers and the fingers become "sausage-like". Other joints may also be involved.
The absence (or rarity) of many general features of lupus, such as kidney disease.
A specific blood test - the anti-RNP - must be positive for a diagnosis of MCTD
There may also be inflammation of the muscles (myositis), with muscle weakness & pain.

MCTD is treated with steroids (low-moderate doses), & some patients respond well to Methotrexate.


What is Scleroderma?
Scleroderma is a chronic autoimmune disease that was first described in the 18th century. The term scleroderma means "hard skin," which describes thickening of the skin from increased deposits of collagen.
There are two types of scleroderma. Localized scleroderma affects the skin in limited areas and the musculoskeletal system. Systemic sclerosis causes more widespread skin changes and may be associated with internal organ damage in the lungs, heart and kidneys. It can cause arthritis, slow contractions in the gastrointestinal tract, muscle inflammation, dry eyes and dry mouth. Most people with scleroderma have cold-induced spasms of small blood vessels in their hands or feet, known as Raynaud’s phenomenon, which caused the fingers or toes to turn white or blue and may be painful.
In most cases, the cause of scleroderma is unknown. However, in a small minority of cases, scleroderma or scleroderma-like illnesses are associated with exposure to certain toxins or as a complication of bone marrow transplants. Scleroderma is not contagious and is rarely inherited.
Systemic sclerosis is associated with over-activation of the immune system, which normally functions to protect the body against cancers and invading infections. This causes damage to cells that line small blood vessels, which in turn leads to the over-production of scar tissue.
The main symptoms-
  • Hardened, thickened skin
  • Poor circulation (Raynaud's syndrome)
  • Stiff joints
  • Muscle inflammation
  • Hiatus hernia
  • Can also affect the heart, lungs & kidneys.
Health Impact
  • Scleroderma affects women more than men and adults more than children.
  • 10-20 new cases are diagnosed per million people each year.
  • Five-year survival rate is 80 – 85 percent.
  • Lung, heart and kidney damage are the most frequent causes of severe disability and death.
  • Many people have decreased hand function because of joint disfigurement or finger ulcers.
Diagnosis of scleroderma is based on clinical history and physical findings. Diagnosis may be delayed in those without significant skin thickening. Laboratory, X-ray and pulmonary function tests determine the extent and severity of internal organ involvement.

There is no known cure for scleroderma. No treatment has been scientifically proven to alter the overall course of the disease, although d-penicillamine is commonly used for this purpose and may be of some value.
There are a number of effective organ-specific treatments for scleroderma. Raynaud’s phenomenon may be helped by calcium channel blockers. Declining renal function and hypertension are often treated with drugs. Esophageal damage from reflux of stomach contents can be treated with acid-reducing drugs. Antibiotics, special diets and medication can improve absorption of nutrients in people who have abnormalities of their intestines. Musculoskeletal pain may respond to nonsteroidal anti-inflammatory agents. Lung inflammation may be treated with cyclophosphamide.
Physical and occupational therapies are used to minimize joint disability and functional impairment.
The Scleroderma Foundation can be reached at             (800) 722-HOPE      .

From the American College of Rheumatology factsheet


Fibromyalgia is a pain-amplification syndrome characterised by widespread stiffness & aching.
About 20% of lupus patients have fibromyalgia, but it can occur on its own.

Muscle aches
Sleep problems
Irritable bowel syndrome
Cognitive dysfunction (memory problems)

To be diagnosed with fibromyalgia, one must have tender points in at least 11 of 18 designated points on the body. Changes in steroid doses can aggravate fibromyalgia, & also patients who are taking corticosteroids develop increased sensitivity to pressure applied to their skin. Therefore it is important to differentiate active lupus from fibromyalgia.

Treatment consists of getting restful sleep, pacing yourself, moist heat, gentle massage & gentle exercise. Medications such as NSAIDs, muscle relaxants & tricyclic antidepressants can also be used.

Drug-Induced Lupus

Drug-induced lupus comes on suddenly in a small number of people taking certain drugs. It mimics SLE, causing joint pains, skin rashes , fatigue & other SLE symptoms.
The main drugs implicated in drug-induced lupus are-
  • Pronestyl (procainamide) - used to treat arrhythmias (heart irregularities)
  • Apresoline (hydralazine) - used to treat high-blood pressure
  • INH (isoniazid) - used to trear tuberculosis
Other drugs that may cause drug-induced lupus include Quinidine, anticonvulsants, beta blockers, & minocycline.
Drugs such as Septrin, ibuprofen, sulfa drugs, don't cause drug-induced lupus as such, but they can exacerbate true underlying lupus, & cause lupus flares.
Drug-induced lupus is different from true lupus in that blood tests are different, heart, lung & kidney involvement is rare. Most patients don't fulfill the criteria for SLE. The symptoms stop when the drug is discontinued, although it can sometimes take up to a few weeks for them to disappear completely.

Lupus Mimics

Some conditions resembling or accompanying Systemic Lupus Erythematosus -
Hardening of the skin caused by overproduction of collagen

Multiple sclerosis 
Fatigue, heaviness or clumsiness in the arms and legs

Rheumatoid arthritis 
Symmetrical joint pain. Also an autoimmune disease and sometimes occurs with SLE

Sjogren's syndrome
Characterized by dry, scaly skin
Mixed connective tissue disorder 
Very similar to SLE, but milder
Inflammation and degeneration of muscle tissues

Lesions are pus-filled blisters and do not atrophy

Seborrheic dermatitis
Skin lesions on lips and nose

Lichen planus
Affects mucous membranes

Affects mucous membranes

Causes bluish-red skin eruptions on face and upper body, accompanied by swelling

Lupus Facts

 There is currently no single test that can definitely say whether a person has lupus or not. There are three different types of lupus - Discoid(cutaneous) lupus, Systemic lupus & Drug-induced lupus.
  •  In approximately 10% of cases of discoid lupus, it evolves & develops into systemic lupus.

  •  There are various factors thought to trigger the onset of lupus, or cause lupus to flare, these include - UV light, certain prescription drugs, infection, certain antibiotics, hormones, & possibly stress.

  •  Approximately 95% of lupus patients have a positive ANA test.

  •  90% of lupus sufferers are female.

  •  Only about 30% of lupus sufferers actually have the classical 'butterfly' rash that is associated with lupus.

  •  Approximately 10% of lupus patients actually have drug-induced lupus. Drug-induced lupus is usually less severe than SLE & will disappear after the patient stops taking the particular drug.

  •  Drugs that have definite proof of an association with drug-induced lupus include - Procainamide (Procan or Pronestyl), Hydralazine (Apresoline or Apresazide), Isoniazid (INH), Quinidine, & Phenytoin (Dilantin).

  •  The widely used acne drug Minocycline, has been shown to cause drug induced lupus symptoms.

  •  Drugs known to exacerbate lupus or increase the risk of allergic reactions in people with lupus, include some antibiotics (sulfa, tetracycline)

  •  The term 'lupus' was derived from the Latin word for wolf in an effort to describe one of the disease's most recognisable features, the rash on the cheeks that suggests a wolf-like appearance.

  •  The technical name for the disease we know of as lupus was first applied to a skin disorder by a Frenchman, Pierre Cazenave, in 1851, though descriptive articles detailing the condition date back to Hippocrates in ancient Greece.

  •  Between 1895 & 1903, the great physician William Osler clearly identified that internal organs may be involved & that lupus could take on a 'systemic' form.

  •  In 1948, a pathologist named Malcolm Hargreaves discovered the LE cell (Lupus Erythematosus cell), which was the first blood test used to help diagnose lupus. He found that 70-80% of patients with active SLE possessed these cells.

  •  During the 1950s, the LE cell was shown to be part of an antinuclear antibody (or ANA) reaction. This led to the development of other tests for autoantibodies.

  •  80% of lupus patients develop the disease between the ages of 15 & 45.

  •  The treatment of lupus aims to suppress the overactive immune system & diminish any inflammation.

  •  The most commonly used treatments for lupus are NSAIDs (Non-steroidal Anti-Inflammatory Drugs), Anti-Malarials (known as disease modifying agents), & steroids. These drugs can be used on their own or in combination.

  •  Occasionally immuno-suppressive drugs need to be used, these include Cytoxan, Azathioprine & Methotrexate.

  •  The most common sites for skin rashes in lupus patients are the palms, elbows & face. Often the rashes are subtle, eg. a faint pinkiness may appear around the cheeks & tips of the fingers or on the soles of the feet.

  •  Many lupus patients are very sun sensitive, & therefore need to cover up well when in the sun.

  •  Some lupus patients report of being affected by UV light, eg. from flourescent lights.

  •  There is no way of telling how long a flare will last. After the initial flare, some lupus patients go into remission & never have another flare, but some patients can be in a flare for years.

  •  Lupus patients are more likely to contract infections such as salmonella, herpes zoster & candida(yeast). Infections in lupus patients tend to last longer & require a longer course of treatment with antibiotics than infections in people who do not have lupus.

  •  Fatigue, malaise, sleep disturbances, myalgias, cognitive impairment & gastrointestinal symptoms are frequent in patients with lupus, & yet may occur in the absence of an obvious disease flare or abnormal blood tests.

  •  Fatigue, headache & cognitive dysfunction (memory, attention, concentration) are symptoms associated with central nervous system (CNS) involvement.

  • Lupus and infections

    Patients with lupus are more susceptible to infections because they have altered immune systems, and also because many patients are on treatment (steroids & cytotoxics) that suppresses immune system function, leaving them more prone to infection.
    Lupus patients who get infections frequently show worse clinical signs, & require longer treatment than non-lupus patients.
    The most common bacterial infections seen in lupus affect the respiratory tract and the urinary tract. Septic arthritis, tuberculosis, salmonella, cold viruses, & shingles are also more common. The most common fungal infection seen in lupus is candida (thrush).
    It is important to distinguish between a lupus flare and an infection. Fever and decreased energy are symptoms that are associated with both infections and lupus flares. Any lupus patient who exhibits symptoms that could be an infection or flare should contact her physician. Blood tests such as a white cell count can help to distinguish a flare from an infection.
    Patients at high risk of infection should probably take antibiotics before surgical or dental procedures.
    Lupus patients should try to minimise their exposure to people who have colds, 'flu, and other infections, although this is easier said than done!
    Lupus patients should probably avoid the antibiotics penicillin and septrin (sulfa), as they may exacerbate lupus, and many lupus patients are allergic to them.
    It has been previously thought that lupus patients should avoid immunisations because they could exacerbate lupus. However, the vaccine for influenza has now been shown to be safe and effective; the pneumococcal vaccine is also safe, but resultant antibody levels are somewhat lower in patients with SLE. It is not advisable for patients receiving steroids or cytotoxic therapy to have live vaccines, because these drugs cause immune suppression that may promote infection.
    Some patients who receive allergy shots (immunotherapy) will have a flare following this treatment. In 1989, the World Health Organisation recommended that patients with autoimmune diseases should not receive allergy shots. Lupus patients should always consult their rheumatologist before receiving immunotherapy.

    Genetics in Lupus

    In this article, "lupus" will mean systemic lupus erythematosus. The most common symptoms of lupus are fever, rash, and arthritis. Women tend to develop lupus more commonly than men, and people of African descent develop lupus more commonly than people of European descent.
    Lupus is only one of many "auto-immune" diseases. "Auto" means that the body has an immune reaction against itself. Rheumatoid arthritis and scleroderma are also auto-immune diseases.
    Given that lupus, rheumatoid arthritis, and scleroderma are all auto-immune disorders, how do physicians tell them apart? The answer is surprisingly old-fashioned. In the case of lupus, physicians compare the patient's symptoms and blood tests to a list of 11 criteria that experts agreed on in 1982. If the patient's data match 4 or more of the criteria, a diagnosis of lupus can be made.

    Auto-immunity causes some, but not all, symptoms of lupus. Other symptoms are caused by problems cleaning up the remnants of auto-immune attacks. These remnants are called "immune complexes" and they circulate in the blood, where they can irritate the inside wall of blood vessels.
    Thus, lupus is more correctly viewed as arising from problems with control of the immune system. It happens that these control problems show up as auto-immunity.

    Can lupus run in families?
    Yes. This was first observed in the 1950s. More recent studies show that the brother or sister of a lupus patient is 25 times more likely to develop lupus than someone in the general population.
    When lupus runs in families, is the reason genes or environment?
    As in most human disease, the answer appears to be "both." Lupus has strong genetic components. It has environmental components as well.

    Lupus in twins
    Studies of twins provide the clearest insight into the relative importance of genes and environment. For example, in 1992 researchers looked at 107 pairs of twins in which at least one twin had lupus.
    Type of twin pairNumber of twin pairsTwin pairs in which both twins had lupus
    ("concordance rate")
    Identical twins4524%
    (11 of 45 pairs)
    Non-identical twins622%
    (1 of 62 pairs)

    If lupus were governed only by genes, then every time one identical twin has lupus, the other should have it, too. (Because identical twins have the same genes.) In other words, the "concordance rate" should be 100%. The table shows, however, that there is a 24% concordance between identical twins. This shows that lupus has an environmental component.

    If lupus were purely an environmental condition, then genes should make no difference at all. The concordance rate would be the same for identical twins and non-identical twins. The table shows, however, that the concordance rate is more than ten times higher in identical twins (24%) as compared with non-identical twins (2%). This shows that lupus has a genetic component.

    What genes are involved in lupus?
    In 95% of cases, genetic susceptibility to lupus is not caused by a single gene. Multiple genes are involved. Identifying them has been slow because different genes seem to be at work in different ethnic groups.

    HLA Genes

    Because lupus is an auto-immune disease, scientists first studied genes that control the immune system. The HLA family of genes, all located on the short arm of chromosome 6 , are important controllers of the immune system. They are divided into 3 classes:
    • HLA class I genes -- These genes have little to do with lupus.
    • HLA class II genes -- Many genes in this group are linked to lupus:
      • The combination of the DR3 and DQ2 variants, or the DR2 and DQ6 variants raise the risk of lupus by a factor of 2 or 3. These genes account for only a small part of the genetic risk for lupus.
      • Many studies of class II genes show no links with lupus. Scientists, therefore, tried dividing lupus into subtypes, according to the results of various blood tests. When they did this, many links between class II genes and lupus subtypes were seen. This suggests that systemic lupus erythematosus is not one disease, but several similar diseases.
    • HLA class III genes -- Many genes in this group are linked to lupus:
      • The C4A and C2 genes are discussed below, in the section on "complement genes."
      • Certain variants of the TNF genes raise the risk of lupus in some ethnic groups.
    Complement Genes
    Less than 5% of patients with lupus owe their genetic susceptibility to a single gene. Many of these genes relate to the body's "complement system." The complement system is part of the immune system.
    • The C1q genes on chromosome 1 sometimes code for a variant of the C1q complement protein that is less efficient than usual. When this happens, lupus can result, especially in children. The C1q protein has both an "attack" function and a "clean-up" function in the immune system. Scientists believe that lupus can be triggered if the remnants of an immune system attack are not cleaned up efficiently.
    • Deficiencies of other complement proteins also lead to lupus, including deficiencies of the proteins coded by the C4A and C2 genes on chromosome 6 , and the C1r and C1s genes on chromosome 12.
    • The MBL2 gene on chromosome 10 is the blueprint for a protein called mannose binding protein that is similar in shape to C1q. In Spanish and African-American populations, certain variants of this gene are more common in persons with lupus. Combinations of this gene and the C4 gene are more strongly associated with lupus than either gene alone.
    Other Genes
    • Three studies have scanned the entire human genome for linkages with lupus. One part of the short arm of chromosome 1 was positive in all 3 studies. Other parts were positive in two of three studies. These results were reassuring, because other studies had identified suspicious genes in precisely these areas of chromosome 1:
      • The FCGR2A gene influences how the body cleans up the results of immune attacks. Certain variants of this gene raise the risk of kidney disease in African-Americans with lupus.
      • The APT1LG1 and ADPRT genes are part of the body's system that controls the lifespan of cells ("apoptosis"). Similar genes in laboratory mice are linked to lupus, but more studies of humans are needed.
    • Regions on chromosomes 2, 6, 14, 16, and 20 also came up positive in at least two of the whole-genome studies mentioned above.
    • Another powerful genetic effect is gender. Ninety percent of all lupus cases are in women.
    Actually, the C1q protein is not a single protein coded by a single gene. Nature is often more complicated than it first appears.Strictly speaking, C1q is the name for a complex of 3 different types of proteins, called A, B, and C. Each of these 3 proteins is made from its own gene on chromosome 1 . Six copies of A, B, and C group together, meaning that C1q is actually a complex of 18 individual proteins! Scientists are not sure whether the A, B, or C gene causes the problem that leads to lupus.

    Two heart medications, procainamide and hydralazine, can trigger an illness that is similar to systemic lupus erythematosus, but not identical. This illness is called drug-induced lupus. At least two genes can contribute to susceptibility to drug-induced lupus:
    • The N-acetyl-transferase 2 gene on chromosome 8 influences how the body processes toxins. It plays a role in several human diseases. There are 2 major variants of the gene: "fast" and "slow." People with the "slow" variant are more likely to develop drug-induced lupus.
    • The HLA class II genes on chromosome 6 , are also involved: People with the DR4 variant are more likely to develop drug-induced lupus.
    Drug-induced lupus usually goes away when the offending medication is stopped, although it sometimes takes years to resolve completely.

    What environmental factors are involved in lupus?
    It has been difficult to pin down the environmental components of lupus. The following factors are the best known:
    • Medications -- As described above, the cardiac medications procainamide and hydralazine can trigger an illness similar to lupus. Of course, most people who take these medications do not develop an illness. We do not know why.
    • Ultraviolet radiation -- Sunlight can worsen the skin problems of people with lupus.
    • Sex hormones -- Women get lupus more commonly than men. And certain men who have higher-than-normal levels of female sex hormones (due to a medical condition called Klinefelter syndrome) develop lupus at a rate between that of women and other men.
    The evidence for other environmental factors -- including infections, diet, and chemical agents and toxins -- is weak and inconsistent. Once the genetics of lupus is better clarified, it will be easier to determine the environmental factors influencing the disease.

    When lupus runs in families, why don't all family members have it?
    It helps to frame this question a little differently.... All members of a family do not have the same height, weight, and face. So, it makes sense that they don't all have the same conditions and diseases -- or the same susceptibility to various diseases.
    Here again, it's genetic and environmental differences that explain differences in our appearance and health. Some family members will inherit genes that predispose to lupus, and others will not. Some family members will be exposed to environmental agents that trigger disease, and others will not.

    There is no lupus in my family. Does this mean it will never occur in my family?
    No. Anyone can develop lupus. About 90% of people with lupus do not have an immediate family member with lupus. But if someone in your family does have lupus, you are at greater risk.

    How will discoveries about DNA help people and families with lupus?
    Further discoveries about lupus genes will lead to more individualized medicine. Prevention, diagnosis, treatment, and prognosis will be personalized, based largely on the strengths and weaknesses found in a person's genes.
    • Treatment -- better use of existing treatments
    Several medicines have been approved to treat lupus. How does your physician know which is best for you? Part of the answer may be in your genes.
    In this article, we've seen how specific genes influence the development and progression of a complex condition -- lupus. Similarly, specific genes may influence the responses to different treatments. Better genetic information could explain why some drugs work better in some people than others. This will make choosing treatments less hit-and-miss than in the past.
    • Treatment -- discovery of new treatments
    Whenever scientists discover a gene involved in lupus, it's a doorway to designing new treatments. If the gene is over-active, then scientists can look for ways to turn it off or interfere with its activity. If the gene is under-active or broken, then scientists can look for ways to turn it on or increase its activity.
    • Prevention, Diagnosis, Prognosis
    Prevention, diagnosis, and prognosis all improve when our ability to calculate risk improves. Scientists believe genes will tell us a lot about the risk of developing lupus and the progression of lupus.
    Article by DNA Sciences

    Osteoporosis and Lupus

    Many lupus patients take steroids, and one of the major side effects is osteoporosis. This article is based on a talk given to the Lancashire & Cheshire Lupus Group, by Dr Kilmiuk, consultant rheumatologist.
    Osteoporosis is a decrease in bone mass, which causes an increased susceptibility to bone fractures. It is most common in postmenopausal women, but patients who are on steroids are at a greater risk at any age.
    However steroid treatment in lupus is often lifesaving, and shouldn't be stopped just because there is an increased risk of osteoporosis. There are things that can be done to reduce your risk of getting osteoporosis, which we will look at later.
    The definition of osteoporosis is a condition that affects the skeleton, with thinning of the bone, accompanied by an architectural change in bone structure.
    The most common sites of fracture in osteoporosis patients are the vertebrae, hip and wrist. Although any bone in the body can be fractured as a result of osteoporosis. In the UK, there are 250,000 fractures to due osteoporosis annually, 50,000 of which are hip fractures. Hip fractures are the ones with the most severe consequences, and are most associated with mortality. If you have one vertebral fracture, there is a 50% risk that you will have another within a year. If you sustain more than one vertebral fracture, curvature of the spine can occur. 20% of those that suffer hip fractures will die due to complications.

    Corticosteroids are live-saving drugs, and are used widely for many conditions, including lupus. An estimated 140,000 people in the UK are taking steroids, and have double the risk of a hip or vertebral fracture due to osteoporosis. 80% of those people don't take any preventative treatments. You need to take over 7.5mg of prenisone/prednisolone a day for more than six months before it has an adverse affect on the bones.

    Throughout our lives, our bones are alive and have a blood supply, they may stop growing, but they are still living. We replace our whole skeleton every seven years! Cells in the body eat holes in bones, which are usually replaced by new bone.

    However, in osteoporosis more bone is eaten away than is replaced, and we are left with a lower bone density. In our early 20s we stop growing, and the skeleton goes through a resting phase. The in our late 30s, we slowly start to lose bone. When we reach the menopause, our oestrogen levels drop, and there is a rapid decline of bone mass. Then, after 10 years, the rate of bone loss evens out and is equal to the level of bone loss in men.

    Risk factors for osteoporosis include: being female; being Caucasian; a family history of osteoporosis; smoking; heavy alcohol intake.

    So how is osteoporosis diagnosed? X-rays can't measure bone density, so a DEXA machine is used. DEXA machines don't deliver high-dose radiation like x-rays do, and they can measure the bone density. There is no need to scan the whole body, only a small part, as bone density should be the same throughout the body. The most useful areas to scan are the lumbar spine & the hip.
    The key to preventing osteoporosis is to enhance the amount of bone we have in the growing period. We can then afford to lose the bone when we reach the menopause. It is better to start preventative measures when we are children, but later is better than never! You should eat a good balanced diet that includes plenty of calcium-rich foods. Dairy produce is the best source of calcium, and skimmed milk contains as much calcium as whole-fat milk. A good vitamin D intake is also needed, as vitamin D helps the body to absorb calcium. Exercise is also very important, bones need constant weight-bearing exercise.

    HRT can help, as it replaces oestrogen, which in turn stops the bone loss. After 10 years of taking HRT there is an increased risk of breast cancer, although this is thought to be an acceptable risk compared to the high possibility of a fatal bone fracture. Plant derived oestrogens do exist, but have not been proven to provide enough oestrogen.

    The use of HRT in lupus patients is controversial, as oestrogen is thought to play a part in triggering lupus flares. However, all HRT does is replace the natural level of oestrogen in the body that was there prior to the menopause. So you have no more oestrogen in your body than you did before the menopause. There is no scientific evidence to say that HRT makes lupus worse.

    A group of drugs called Bisphosphonates can be used to treat osteoporosis & improve bone mass. One such drug is called alendronate.

    Here is our links page on our website which has more links than below.  Sjogren's guide for patients   Nat. Library of Med.
      Lab results website          Sjogren's Society of Canada   The Eye Digest/Sjogren's    Drug info site        Drugs, including drug interactions    RX Assistance
     Free Emergency Medical ID Wallet Card   Includes a meds term dictionary    National Fibromyalgia Association    Fibromyalgia and Sjogren's   Kidney disease site Survival Tips from Sjogren?s Syndrome Foundation

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