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Sjogren's Articles

SS A Multifaceted Disorder

Seattle WA Symposium by Dr. Elaine Alexander, Dr. Philip. J. Mease & Dr P. Scott Pollock

Note: Our thanks to Mary Anne Saathoff, RN, who prepared this excellent summary of this section of the Seattle Symposium. Other portions of this symposium have been reported in previous issues.

Dr. Mease, the Program Chairman for the Seattle Symposium, is Clinical Assistant Professor of Medicine, University of Washington, School of Medicine, Division of Rheumatology, Minor and James Medical Clinic.

* Reprinted with permission of the Sjögren Syndrome Foundation, Inc. (founded 1983)

"The Moisture Seekers" Newsletter. Vol.9 No.9, September 1992 pages 1-7

Introduction
Case History #1
Case History #2
Case History #3

SS Underrecognized
Definition of SS
Autoantibodies

A Journey Through the Body
Vasculitis
Nervous System Involvement

Multiple Sclerosis or Sjogren's Syndrome?
Chronic Fatigue Syndrome or SS?
Fibromyalgia and Sjögren's Syndrome
Viruses and Retroviruses
AIDS and SS-like Symptoms

Genetics & Sjögren's Syndrome

Conclusion
Why is Sjogren's Syndrome Underrecognized?


Introduction

The fields of rheumatology and immunology were well represented at Sjögren's Syndrome Symposium X in Seattle, Washington. Dr. Mease and Dr. Pollack began this extended session by presenting case histories of three of their patients. Each woman experienced her symptoms in a very different way and needed an individualized treatment program. These case histories illustrate the problems that even experienced physicians can have in diagnosing and treating patients suspected of having this multifaceted disorder, Sjögren's syndrome.

Case History #1

Dr. Mease described a 52 year old woman whose symptoms started fifteen years ago with a gritty sensation in her eyes. Later, she began chewing gum to alleviate a dry mouth. When she noticed redness and burning in her eyes, she was thought to have an allergic condition which artificial tears seemed to help. Gradually she began feeling chronically ill, with fever and chills, aching throughout her body, and severe and debilitating fatigue. Additional problems started to develop, including difficulties with memory and concentration. She had noted flashing lights in the periphery of her vision and prickly, tingly, or numb sensations in her hands and feet. Recently she had noticed numbness in the vaginal and buttock areas.

When she came to see Dr. Mease, he found she had multiple points on her body that were very tender. Along with her widespread pain, these characteristic tender points are frequently characteristic of fibromyalgia syndrome. Fibromyalgia is often helped by low doses of amitriptyline at bedtime. She'd been using this and, although it contributed slightly to the dry-ness of her mouth, she had found it helpful for her sleep and, possibly, her aching. Dr. Mease noted that many people with Sjögren's will have coexistent fibromyalgia.

Her laboratory tests showed that her ANA (antinuclear antibody titer) was only mildly elevated. Although she was not positive for the SS-A and SS-B antibodies, this did not rule out a diagnosis of Sjögren's syndrome, as there is an increasing aware ness that many people with Sjögren's may be negative for these antibody studies. A minor salivary gland biopsy showed mild diffuse chronic inflammation and was judged inconclusive for Sjögren's syndrome. Because of her neurocognitive complaints an MRI scan of the brain was done. It showed three small areas called "UBOs", or "unidentified bright objects". These are brain lesions that have been seen before in patients with Sjögren's syndrome, other types of inflammatory or vascular neurologic syndromes, but also may be seen in "normal" individuals.

Her prior treatment had consisted of artificial tears, fluids for the mouth, and the anti-inflammatory medication, ibuprofen. Because of very severe joint aching and fatigue, Dr. Mease prescribed the medication Plaquenil for this patient. Plaquenil is a less toxic immunomodulatory medication that may be useful in a variety of autoimmune conditions. Her exact diagnosis, however, was difficult. Many of her symptoms were similar to those seen in chronic fatigue syndrome; but this diagnosis, because of various autoimmune disease elements, did not seem right. He didn't feel she had pure, classical Sjögren's syndrome or other clear cut diseases. At this time, Dr. Mease considered her to have possible Sjögren's syndrome or "undifferentiated autoimmune disease", as well as the fibromyalgia syndrome.

Case History #2

Dr. Pollock, Associate Clinical Professor of Medicine, University of Washington, School of Medicine, Division of Rheumatology, Minor and James Medical Clinic, began his section of the program by agreeing that many patients were not easy to diagnose, especially as their symptoms don't appear all at once. His first case history illustrates this. She had come to him as a patient nine years ago when she was thirty years old. She was working and was a very active and athletic person who enjoyed frequent games of volleyball. A year prior to this she'd seen another physician after a flu-like illness. It had progressed into respiratory problems, shortness of breath and a type of chest pain called pleurisy. Chest X-rays showed some scarring in her lungs and breathing tests showed a reduction in her pulmonary function. But no specific lung illnesses such as pneumonia, tuberculosis or sarcoid could be found. In fact, nothing else was abnormal.

Even before this respiratory illness, however, she'd noted dryness of her eyes, mouth, and vaginal area. There had been several episodes of swelling of her cheeks in front of her cars. She'd had aches and pains for many years and had noticed a curious rash on her legs, spots that measured about a quarter of an inch. They looked like small bruises and were painful raised bumps that would come and go, usually after she'd played volley ball. The medical name for this is purpura and eventually these lesions were biopsied. She'd been told they represented vasculitis but since she felt well, no one did anything further about it.

When she came to see Dr. Pollock, a Schirmer's test showed very dry eyes. Her blood tests showed a positive rheumatoid factor. In her case, this didn't mean she had rheumatoid arthritis, but showed there was a lot of autoimmune activity in her system. The blood tests also showed a positive ANA titer and the two specific antibodies for Sjögren's, SS-A and SS-B.

Her parotid glands were mildly enlarged and a biopsy or a minor salivary gland was highly positive, showing much inflammation. Based on her history and test results, a definite diagnosis of Sjögren's syndrome was made. In the nine years since her diagnosis, she's been followed closely. Her treatment has been conservative; it has not needed to be aggressive. There's been very little, if any, progression in her dryness. Aside from the special care she needs to take with her symptoms, she's leading a normal life. She's doing well.

Case History #3

Another patient of Dr. Pollock's demonstrated very different symptoms. This 25 year old woman had been very healthy until, suddenly, she began to notice a peculiar sensation starting in her feet and going up her legs. In a period of only ten days, her legs became very stiff, achy, and heavy and she began having trouble walking. When seen by Dr. Pollock at the end of this short period, she'd lost normal feeling below her thighs and showed reflexes that were too active. She'd also seen a neurologist who felt there might be an abnormal process in her right eye.

Due to the rapidity of onset and the severity of her symptoms, many tests were quickly done. An exam of her spinal fluid was suggestive of possible multiple sclerosis. Blood tests showed that she didn't have the SS-A and SS-B antibodies often found in Sjögren's. However, her ANA test was abnormally high and her sedimentation rate was elevated, indicating that an inflammatory process was going on in her body. When a lip biopsy was done, significant inflammation was found in her salivary glands, consistent with Sjögren's syndrome.

It was clear that she needed to be treated quickly and aggressively. Massive doses of intravenous steroids were given; oral prednisone was started and eventually tapered off over the next 8 weeks. Very quickly, she had a total disappearance of all symptoms. Six months later, she showed no neurologic abnormalities whatsoever. She now needs no medication and is doing very well. No one knows whether or not this young woman will have recurrent episodes of these symptoms.

Elaine Alexander, MD, PhD

SS Underrecognized

Dr. Elaine Alexander is Assistant Professor of Medicine, John Hopkins Medical Institutions, Department of Medicine, Division of Molecular & Clinical Rheumatology

In his introduction of Dr. Alexander, Dr. Mease stated that she has been working with Sjögren's syndrome (SS) patients for many years. He also noted that she has been instrumental in landmark research on the basic science and treatment of SS. Dr. Alexander began her presentation by stating that she feels SS is a common condition, which occurs more frequently than is appreciated, in general, by the population and the medical community. The table on page 8 outlines some potential explanations why SS remains unrecognized and under diagnosed. In fact, although there is little known about the actual prevalence of SS, she feels SS may affect more than 2% of the population and may be the most common rheumatic disease.

Dr. Alexander emphasized that SS should not be considered as a disease of middle-aged to elderly women. Rather than occurring suddenly at middle age, she feels this process begins in the late teens or early adulthood and often progresses slowly and insidiously. SS also occurs in men. Recognition and diagnosis can be very difficult, particularly early in the course of the disease or in the younger patient. As this syndrome potentially can affect so many different organs in the body; the location or organ where it may attack will determine which specialist a patient sees. As the case histories of Dr. Mease and Dr. Pollock illustrated, there is a tremendous diversity of symptoms for which patients may see a physician and recognition of the possible underlying SS can be problematic. Dr. Alexander, like all patients and physicians, wishes there were a test for SS that was 100% accurate. Until then, awareness and careful detective work will be the mainstay of the recognition, diagnosis and therapy of Sjögren's syndrome.

Definition of SS

It was Dr. Norman Talal who defined SS as an autoimmune glandular exocrinopathy. Dr. Alexander explained that this means there is an attack from within your own body on these glands that make secretions. The attack comes from that particular type of white blood cell called lymphocyte. Different glands can be affected in different people and they are located in many different places in addition to the eyes and mouth. These glands make secretions to keep you moist. When they are no longer able to function the way they should, due to this lymphocytic infiltration, they produce abnormal, less, or no secretions. The dryness and resulting symptoms are related to this underlying autoimmune attack. Because SS, by definition, is a dryness syndrome of glands, the presence of dryness symptoms is necessary to establish the diagnosis. It is important to recognize the complications of dryness in the different glands as some are preventable and all can be improved by therapy.

Autoantibodies

Antibodies are developed in our bodies in response to something that may harm us, such as an infection. Our bodies are protected by these antibodies but something else can also happen in autoimmune conditions like SS. Then additional antibodies begin to form against our own tissues. These are called autoantibodies and they can be destructive.

One test that is often done in diagnosing SS is to check for the presence of specific autoantibodies, anti-SS-A (Ro) and SS-B (La), which are present in significant quantities in autoimmune diseases such as SS, classical SLE and other related disorders called the "lupus variants" [i.e. SCLE (subacute lupus erythematosus syndrome), NLE (neonatal lupus syndrome), and SS/LE (Sjögren's/lupus overlap syndrome)], but not in normal individuals. These autoantibodies are of great interest in research studies now and may play a pathogenic role in certain disease manifestations. When they are found on blood tests, they are excellent markers for autoimmune disease, and physicians will feel that the blood tests support their clinical diagnosis. But, as the case histories pointed out, these blood markers are not always present. Many physicians are unaware of this. If the blood tests are negative, they may tell their patients they don't have SS. Dr. Alexander feels the absence of these specific blood markers does not preclude a diagnosis of SS. People with SS often make autoantibodies against organs or tissues, such as the salivary glands, thyroid, liver, stomach, or even reproductive organs. In addition, they can be formed against red blood cells that carry oxygen, and against platelets, another type of blood cell that's important in blood clotting mechanisms and lymphocytes, which are immune cells. These autoantibodies may then play a role in other manifestations of SS.

A Journey Through the Body

In describing the various ways in which SS can manifest itself Dr. Alexander described a journey throughout many different organs of the body. Dr. Talal has divided the different features of SS into two categories: glandular (the dryness component) and extra-glandular (complications which affect other organs or tissues.) While the majority of SS patients do not develop these extra glandular complications, it is important for physicians to recognize the many different aspects of this condition.

Dr. Alexander described the sicca complex (dryness of the eyes and mouth), that is such a source or discomfort for SS patients. The familiar symptoms in these areas were described, as were the tests that are done to reach a diagnosis. Function of the tear glands is evaluated using the Schirmer's test and rose bengal staining. Salivary gland function can be determined through lip biopsy and salivary flow tests. These tests help to establish criteria by which doctors can communicate with each other about the extent of involvement of the mouth and eyes. These tests and a number of others also aid researchers in understanding how this syndrome starts and progresses.

Along with dry eyes and mouth, SS patients will often have chronic sinus or ear infections, a dry and sore throat from pharyngitis, hoarseness from laryngitis, or a dry, nonproductive cough from tracheobronchitis. These manifestations can be caused, once again, by lack of normal secretions in these areas. Other lubricating glands are also affected. It is common for SS patients to have dry skin. Dr., Alexander feels the problem of vaginal dryness in women the resulting potential sexual difficulties are very important and often unrecognized. These can be very effectively treated and will enhance a woman's feeling that she's retaining her femininity.

Musculoskeletal symptoms are common in SS and will be discussed later. Arthritis, swelling of joints, in SS usually is mild and non-progressive, without joint deformities, or evidence of severe damage on X-rays. Coexistent but unrelated osteoarthritis is probably the most common cause of arthritis in SS Primary SS patients may be misdiagnosed as having rheumatic arthritis because they have arthritis and a positive rheumatoid factor. In addition, many patients with true rheumatoid arthritis have secondary SS (i.e. SS associated with another rheumatic disease). SS patients may develop muscle weakness secondary to inflammation of muscle (myopathy, myositis.) Occasionally, SS patients may also develop polymyositis or dermatomyositis.

It is now known that there are many organ-specific autoimmunc diseases that can occur alone (i.e. in isolated form), but also may be associated with SS. For ex ample, autoimmune disease in SS may affect the thyroid gland, the kidneys, or the liver. When such patients are evaluated for underlying SS, Dr. Alexander stated that a certain percentage (approximately half) will be found to have SS.

It is relatively common for a patient with SS to develop thyroid disease and there are two specific conditions in which there is an autoimmune attack on the thyroid gland. Graves' disease, which has been diagnosed in both President Bush and his wife Barbara, will cause too much excretion of the thyroid hormone (i.e. hyperthyroidism). In the other condition, Hashimoto's thyroiditis, causes destruction of the gland and results in too little excretion of the thyroid hormone (i.e hypothyroidism).

Continuing her journey discussing various organs of the body that can be involved in SS, Dr. Alexander stated that the GI tract should be recognized as a frequent target of SS malfunction. Lymphocytes can infiltrate the esophagus and cause problems in the movement of food and fluids from the mouth to the stomach. There is often chronic gastritis, sometimes leading to a condition called pernicious anemia. The small bowel can be affected and cause a malabsorption syndrome resembling celiac disease or sprue. The pancreas, which makes insulin and many digestive enzymes, is a common point of lymphocytic infiltration. Dr. Alexander added that the liver, which is such an important organ, is also part of the GI tract. She explained that there are two kinds autoimmune liver diseases (i.e. chronic reactive hepatitis and primary biliary cirrhosis). Each will cause specific problems. Some research studies have been done in patients with these conditions, more than half of them have been shown to have underlying SS.

The lung is commonly involved in SS although usually the involvement is clinically insignificant. Interstitial pneumonia can occur and result in the type of chest x-ray results and breathing studies that Pollock described in one of his case histories. Rarely, lungs may become involved with other more serious inflammation. Dr. Alexander recommended that SS patients have baseline pulmonary function tests. If lung problems do occur, these conditions are usually very responsive to prompt treatment.

The kidneys and the urinary tract can also be involved. Tubules of the kidney may not function normally, changing the levels in the blood and urine of certain important substances called electrolytes. Kidney problems can even result in bone softening of a type different from osteoporosis. A bladder problem called interstitial cystitis, or inflammation of the bladder lining, can develop. This causes much discomfort and symptoms resembling chronic urinary tract infections (UTI's) or actually predispose to UTI's.

Vasculitis

Inflammation of blood vessels, said Dr. Alexander, is called vasculitis. Researchers have now discovered two distinct types of vasculitis, each one of which will be associated with a different serum antibodies. Vasculitis can occur in SS and may affect different organs of the body, most commonly the skin. This type of vasculitis was described in the patient history given by Dr. Pollock; bumps (i.e. purpura) arose on this young woman's legs whenever she was particularly active, as in playing volleyball. There is a very small number of SS patients with skin vasculitis who will develop inflammation in the blood vessels of other organs or the brain. The latter patients may show signs of central and/or peripheral nervous system disease.

Nervous System Involvement

The many ways in which SS can affect the nervous system has been of particular interest to Dr. Alexander. Although this complication probably occurs in only a minority of SS patients, it is very important to recognize that neurologic disease can occur in SS. Two different parts of the nervous system can be involved (i.e. peripheral or central). The central nervous system, often referred to as the CNS, is comprised of the nervous tissue in the brain and spinal chord. The peripheral nervous system refers to nerves that move away from this central area. Some peripheral nerves have sensory functions allowing us to taste, hear, and feel; other peripheral nerves govern motor functions, allowing us to move. Each of the many different peripheral nerves and parts of the CNS can be affected and give rise to different symptoms/signs in patients with SS.

At times, psychiatric symptoms may develop. Or, there may be symptoms that are diffuse in nature such as ability to think or learn or remember correctly. Dr. Alexander remarked that the case history given by Dr. Mease described this type of problem. His patient noticed difficulty with her memory and concentration Dr. Pollock talked of a second patient of his who showed sudden and progressive weakness and loss of feeling in her legs. Her condition, which would be called transvese myelitis, also pointed to a central nervous system involvement, but this time at the level of the spinal chord. It was of great importance to this woman that there was prompt recognition of her underlying SS. This resulted in immediate, appropriate therapy with a total resolution of her neurologic symptoms.

Multiple Sclerosis or Sjogren's Syndrome?

Nervous system involvement in SS, just by the position and nature of lesions and infiltrates that may develop, can also mimic many other diseases. This has been of special interest to Dr. Alexander and is the focus of much of her research. One neurologic condition that SS may closely mimic is multiple sclerosis. Nervous system involvement in SS closely resemble multiple sclerosis clinically. Not only can symptoms be similar, but test results from brain magnetic resonance imaging (MRIs) scans, brain electrophysiologic studies (evoked response tests), and cerebrospinal fluid exams can be indistinguishable, presenting a real diagnostic dilemma. The patient may be informed that, indeed both diseases are present. It is Dr. Alexander's feeling from her research that, if the patient has SS, the patient may not also have multiple sclerosis, but rather CNS disease secondary to SS. The central nervous system symptoms and test findings may be due to the unique blood vessel inflammation that can occur in SS, rather than to demyelination which occurs in MS.

Chronic Fatigue Syndrome or SS?

Dr. Alexander has always been impressed with the chronic fatigue that so many of her patients have described. Often it is accompanied by recurrent episodes of headaches, low-grade fevers, lymph node swelling, and aches and pains in muscles and joints. Patients feel as it they are always getting the flu. She had assumed this was just part of the systemic manifestations of SS. Then, as years progressed, a disease called chronic fatigue syndrome, once called the "yuppie flu", was described. Dr. Alexander quickly noted that features of this disease were similar to those listed as musculoskeletal or systemic complaints in SS. She became quite interested in this condition.

As research on chronic fatigue syndrome began to be published, she was intrigued to see that these patients were reporting attention and concentration deficits, memory loss, confusion, irritability, sleep disturbances and depression. Many people with chronic fatigue syndrome, just as in SS, were showing abnormal brain MRI scans. Small unidentified bright objects, the UBOs, seen on the brain MRI scans. Dr. Alexander remarked once again on the patient history presented by Dr. Mease. Along with other problems, this patient had severe fatigue and an abnormal brain MRI scan.

The relationship and possible overlap between chronic fatigue syndrome and SS remains very interesting to Dr. Alexander. She again mentioned the problem that physicians can have in diagnosis. A patient with SS may show features resembling another disease such as multiple sclerosis or chronic fatigue syndrome. Do they have both diseases, or do they have only SS? Dr Alexander feels the underlying autoimmune disease responsible for the patient's symptoms complex is SS. Therefore patients would not have both SS and the chronic fatigue syndrome. Nor would they have both SS and multiple sclerosis. Their symptoms, instead, are manifestations of the many features that can occur in SS.

Fibromyalgia and Sjögren's Syndrome

There is another constellation of symptoms, closely related to chronic fatigue syndrome, that Dr. Alexander often sees in patients with SS. They consist of complaints of widespread areas of focal musculoskeletal pain. This pain, which can be debilitating, is in the muscles rather than in the joints. Recently, these symptoms have been described as a specific clinical entity called the fibromyalgia syndrome. Many patients with SS have been told that they also have fibromyalgia. In her own practice, Dr. Alexander has given questionnaires to some SS patients and found that approximately 57% of these patients were judged positive for the fibromyalgia syndrome. Conversely, a Swedish research study has shown that many fibromyalgia patients also had SS.

She has also been impressed with what seems to be a great overlap of symptoms occurring in both the fibromyalgia and chronic fatigue syndromes. The relationship between these two conditions and SS is not yet well understood. She tends to feel, however, that these symptoms of the fibromyalgia and chronic fatigue syndromes may be part of the underlying autoimmune disease, SS.

Viruses and Retroviruses

Many conditions, such as various forms of the "flu", are known to be caused by viruses. SS is associated with an increased frequency of lymphoid malignancy (lymphoma) in which a role for viruses has been suggested. Recently there has been much interest in retroviruses. A retrovirus differs from an ordinary virus as it enters the body at some earlier time and causes no immediate problem. After a period of time, which can often be lengthy, it will begin to manifest itself and the patient's symptoms will begin. Many retroviruses are now being identified. It is possible that viruses or retroviruses, or a combination of each, may play a part in SS and many other autoimmune conditions. Dr. Norman Talal, University of Texas, has pioneered in research suggesting the presence of unique retroviruses in SS, although the exact role of retroviruses in SS is unknown. Research in this area is being actively pursued at several institutions including Johns Hopkins and the CDC, the Center for Disease Control.

AIDS and SS-like Symptoms

Retroviruses have been established as the cause of the serious AIDS condition. Dr. Alexander assured those with SS that this does not mean they will develop AIDS. SS patients are at no greater risk for developing AIDS than are persons in the general population Dr. Alexander reported, however, some very interesting information about people that do have AIDS. It's now becoming apparent that a number of them are developing SS-like symptoms. This syndrome, described by Doctors Silvia Itescu and Robert Winchester, is called DILS (diffuse interstitial lymphocytosis syndrome). Just as those with SS, this group of AIDS patients has developed very dry eyes and mouths and has had enormous swelling of parotid glands. They have had symptoms of arthritis and may also develop similar complications, such as lung, kidney, GI and CNS disease, vasculitis and lymphoma.

This condition now being seen in AIDS, however, is most likely not idiopathic SS. The majority of these AIDS patients will not have the autoantibodies that SS patients show. And when lymphocytes that have infiltrated the glands and blood of the AIDS patients are examined, they show features that are very different from the lymphocytes of SS. As symptoms of SS can also occur in this group of AIDS patients, however, Dr. Alexander suggested that physicians may request a HIV test in patients they see with SS features. She feels this is the practice of good clinical medicine and believes it will help to increase the understanding of each condition.

Genetics & Sjögren's Syndrome

The subject of genetic influence on various diseases has long been of interest in the medical community. In many cases, it has been shown that genes inherited from our parents can reveal specific markers that determine whether or not we are at risk for developing certain conditions. Dr. Alexander stated that these genetic deficits provide information for patients and physicians in conditions such as cystic fibrosis, hemophilia and sickle cell disease.

Investigators are also looking at the immunogenetic control of SS, which is much more complex than other inheritable diseases. Her own laboratory has been interested in three structurally related histo-compatibility (HLA) alloantigens/genes (i.e. HLA-DR3, DR5, Drw6) that tend to be present in almost all American caucasian SS patients. It is very interesting, Dr. Alexander said, that other investigators have shown that one of two of these three genes are usually present in those patients with AIDS who also develop SS-like symptoms and in idiopathic SS in other ethnic groups The presence of these three inherited genes however, is not enough to determine who will develop SS. Most likely it will eventually be determined that many other factors must also be present to cause the emergence of this condition. Infection by a virus or retrovirus is just one possibility.

Dr. Alexander believes that, in general, investigators are making major advances in understanding the multiple genes that influence expression of disease. One of the exciting discoveries of the last several years has concerned those conditions known as presumed "variants" of lupus. This would include subacute cutaneous lupus (SCLE) neonatal lupus syndrome (NLE), (with skin disease or congenital heart block), and SS lupus (SS/LE) syndrome. Dr. Alexander's studies indicate almost all of the presumed lupus variant patients have HLA-Dr3, DR5, DRw6 and do not carry the genes associated with classical lupus (i.e. HLA-DR2). This, their genes are the very same ones that occur in primary SS. Thus, patients with the presumed lupus variants appear to be more closely linked clinically, serologically and genetically to primary SS than to classical lupus. Better definition of primary SS, the related lupus variants and classical SLE will allow more precise recognition and diagnosis of disease and hopefully lead to better and more definitive therapeutic approaches.

Conclusion

As Dr. Alexander completed her presentation, she talked again of reasons why it can be difficult to diagnose SS (see Table). The symptoms are often very subtle, perhaps only aching and fatigue. An examining physician may not ask about features of dryness. And often, patients may have become dryer over a period of years and have come to accept it as just their unfortunate situation in life. It is not uncommon for these problems in women to be dismissed as a normal feature of aging.

Another difficulty in diagnosis is that physicians are usually not able to tell something is wrong just by looking at the patient There will not be large rashes or red blotches and SS patients, unlike SLE or RA, usually don't awaken one day unable to get out of bed, showing swollen, inflamed joints and a high fever. Even physicians who do consider a diagnosis of SS may be unaware of the fact that a number of patients will not show the SS-A and SS-B antibodies when their blood is tested. Symptoms, also, can be misdiagnosed. Patients may be told their condition is due only to fibromyalgia, or to chronic fatigue syndrome. They may be told they have multiple sclerosis, SLE, rheumatoid arthritis, or even another condition.

In conclusion, Dr. Alexander stated, once again, that she believes SS is not a rare condition. She feels it is a very common autoimmune rheumatic disorder, probably far more common than even the many in-formed physicians yet anticipate. She pointed out that Elaine Harris and the SS Foundation have made major contributions in bringing this disorder to the attention of both physicians and patients. Dr. Alexander emphasized that in her experience most people will not have the many complications she has described. Instead, their SS will be in a more limited form and they will do very well. Most importantly, she emphasizes the need for continuing patient education and the development of doctor-patient relationships in the approach to the diagnosis and management of SS. The informed and educated patient is an important and essential partner in the doctor-patient interaction.

TABLE 
Why Is Sjogren's Syndrome Under-Recognized?

Lack of:

Awareness that SS is probably the most common rheumatic disease.
Patient awareness and education about SS.
Physician recognition and appreciation of spectrum of SS disease; knowledge of how to evaluate and diagnose.
Recognition that SS may occur not only in middle-aged to elderly women, but
also in children, teenagers and young adults.
Recognition that SS may also occur in men.

Symptoms may be:

Subtle, often insidious.
Symptoms perceived and dismissed as trivial or nuisance by family and physicians.
Dismissed as secondary to "normal" aging". 
Dismissed as secondary to functional impairment (i.e. depression; neurosis, etc.)

External stigmata characteristic of other rheumatic diseases:

Usually subtle or absent from SS.
Skin manifestations of SLE lacking except in SS/LE overlap syndrome.
Rheumatoid arthritis deformities absent, except in RA patients with SS.
Chronic fluctuating, sometimes progressive course, rather than acute serious exacerbations.
Patient feels worse than she/he looks, "You look wonderful, what's the problem?"

Autoantibodies may be absent:

Many patients are antibody positive: anti-Ro [SS-A], anti-La [SS-B], ANA, or rheumatoid factor.
Some patients, however, may be antibody negative. Absence of antibodies does not exclude diagnosis of SS.
Antibody negative, as well as positive, may develop extraglandular complications.

SS is the "Great Mimicker" of other diseases

SLE
RA
Fibromyalgia/fibrositis
Chronic Fatigue Syndrome
Chronic yeast infection
End-organ specific autoimmune disease
Lymphoma
Multiple Sclerosis
Alzheimer's disease

  SSF Disclaimer

* The articles identified as originally published in The Moisture Seekers Newsletter, are the only materials contained in this website that have been reviewed by the Sjogren's Syndrome Foundation.  The SSF in no way endorses any of the medications or treatments mentioned in these articles.  We strongly advise that you check any drugs, treatments or  products mentioned with your own healthcare provider.

Introduction / Case #1 / Case #2 / Case #3
SS Underrecognized / Definition / Autoantibodies

Journey Through the Body / Vasculitis / Nervous System Involvement
MS or Sjogren's / Chronic Fatigue Syndrome? / Fibromyalgia?

Viruses and Retroviruses / AIDS and SS-like Symptoms / Genetics

Conclusion / Why is Sjogren's Syndrome Underrecognized?

Copyright 2005 Sjogren's World