Lupus Symptoms and Syndromes

What are the symptoms of Lupus?

Listed below are merely some of the possible symptoms of lupus. As you can see, these can be fairly wide ranging. Lupus is a disease with many faces, and rarely do two people have exactly the same symptoms. Moreover, symptoms will vary from day to day. You may have just one symptom one day, none the next day, and several together the next. Often these symptoms are difficult to identify as being specifically related to lupus as many of them are common to other disease conditions or may even be side effects of medication.

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Anti-Phospholipid (Hughes') Syndrome

Anti-phospholipid Syndrome, (APS) also known as Hughes' syndrome or "sticky blood syndrome" is an autoimmune disease that can manifest as a primary illness or as a secondary condition along with other autoimmune diseases like Lupus. It is characterized by thrombosis in both the veins and arteries. The mechanism by which APS hypercoagulation is not fully understood.

APS thrombosis by it's very nature poses risks to various organs. This can result in strokes, transcient ischemic attacks (TIA), major organ damage, and other serious complications. APS poses a special risk to pregnancy and a patient is often discovered and diagnosed as a result of multiple miscarriages.

Tests used to diagnosis APS are the ACA (anti-cardiolipid antibody) test and the LA (lupus anti-coagulant) test.

Treatment of APS can range from a daily aspirin dose to strong anti-coagulants such as coumadin and heparin.

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Discoid Lupus Erythematosus

Discoid lupus is a type of lupus that tends to be confined to the skin - other organs in the body are not involved. ["Discoid" means "coin-shaped".] Discoid lupus occurs in patches across the body. These patches tend to be well defined, thickened and scaly, they are slightly red in colour and can itch. Characteristically, the skin most commonly affected is on the cheeks: there may be a "butterfly" distribution across the nose as in SLE, and the other skin areas commonly affected are the palms and soles (reddish, sometimes peeling lesions), the elbows, the ears and the scalp. The appearance can vary between people and also on different areas of the body on the same person. As the patches heal they tend to leave scars and on darker skins the pigment in the skin can be lost leaving white areas. If discoid lupus occurs on the scalp the hair will be lost as the patches heal leaving permanent bald areas.

Discoid lupus is diagnosed when a patient with a discoid lupus rash (confirmed by skin biopsy) does not fulfil the American College of Rheumatology criteria for systemic lupus. Remember, discoid lesions may be a feature of SLE. A physician cannot determine whether or not a discoid lupus lesion on the skin is occurring in the presence or absence of systemic features just by examining the shape of the lesion. This can only be done by taking a complete history and physical examination and interpreting the results of appropriate blood tests.

What is the relationship between discoid and systemic lupus erythematosus? Lupus erythematosus should be viewed as a spectrum of disease. At the mild end of the spectrum, it is characterized by coin-shaped, scarring, skin lesions which we term discoid lesions. At the other end of the spectrum are those systemic lupus erythematosus patients who have no skin lesions, but have systemic features (eg arthritis or renal disease). People with only discoid lesions and no systemic features commonly have no autoantibodies in their serum (i.e., antinuclear or anti-DNA tests will be negative). On the other hand, people with systemic lupus erythematosus are characterized by the presence of one or more types of autoantibodies in their blood.

If the discoid lesions appear only above the neck, they rarely evolve into systemic lupus and are treated with antimalarial drugs or local remedies. This is sometimes called localised DLE. In other cases many lesions are present on the arms, legs, and trunk as well as the head and neck. This is called generalized or disseminated DLE and has a 10% chance of developing into systemic lupus.

Although the disease may not be life-threatening, there are a number of problems: The skin disease may be scarring and severe; some patients have marked sensitivity to sunlight; arthritis can be prominent, as can migraines. Unlike for most SLE patients, the disease may remain active after the menopause and into later life.

Each patch is called a "plaque". These most often occur on areas exposed to the sun like the face, neck, ears, and forearms. Such lesions generally do not itch. Several plaques may occur at once. Each plaque of DLE begins with redness, slight swelling, and some dilation of blood vessels in the affected area of skin. As the lesion persists inflammation occurring in the skin causes destruction of hair follicles and glands and thinning (atrophy) of the skin with loss of normal colour. In some instances thick scales occur, and with the red colour these may give DLE an appearance similar to psoriasis or skin cancer. A biopsy is the only reliable way to diagnose the true identity of such lesions. As a DLE plaque ages, the inflammation burns out, leaving a pale, white, hairless scar as the end result.

DLE lesions may be induced to begin by various factors, the most important of which is the ultraviolet light in sunlight. Other known inducers are trauma (cuts, scratches), x-rays, and some drugs (e.g. gold). In many cases the DLE lesions also seem to begin spontaneously, without a known triggering factor.

One or several autoimmune phenomena may be present in patients with DLE, even though the chances of development of systemic lupus are only about 5%. This means that a person with DLE can manifest some system involvement, e.g. joint pains, hair loss, fatigue, anemia, positive-ANA, low white blood cell counts, etc. Aching joints and other constitutional symptoms are found in 10 to 20 percent of patients with DLE. Blood testing shows a positive ANA test in about half of the cases; other autoantibodies are seen in less than 10 percent. Anemia may be observed in 20 percent of the patients, and a low white blood cell count in half. Such a patient cannot however be said to have SLE unless enough of these systemic manifestations exist to meet the American Rheumatological Association's criteria for SLE. If only one or two such problems exist and are not severe, the diagnosis in such cases is "DLE with some systemic involvement."

Discoid lupus can appear similar to other skin lesions. For example, rosacea, fungal infections, sarcoidosis, seborrhea, dermatomyositis, and a sun-sensitive rash called polymorphous light eruption can be ruled out by a simple skin biopsy and blood tests before diagnosing DLE.

Only about 5% of patients with DLE eventually develop SLE. This may take months or decades to happen. Conversely, about 20-25% of SLE patients have skin lesions indistinguishable from DLE at some time during their illness, possibly as the first symptom.

What happens to the remainder of DLE cases? DLE may continue from several months to several decades, with new plaques appearing as old one "burn out", leaving the typical white atrophic scars in their wake. In about 50% of cases; however, spontaneous complete remission of DLE occurs after about 20 years. This is only an average figure - how each individual will do is highly unpredictable.

Why treat DLE? For one thing the disease and scarring produced by it can be cosmetically disfiguring. The worst complication however, is the risk of development of skin cancer within the old scars of DLE. This happens rarely, and usually only after many years, but it is quite important to diagnose it early so that spread does not occur. The physician's goals in treating DLE, then, are to monitor the patient for possible development of systemic disease; treat active DLE lesions to quiet down the inflammation so that scarring is prevented; watch for development of skin cancer in old scars and treat them early. One last point: systemic corticosteroid therapy is not the treatment of choice for DLE in the absence of SLE.

The treatments most commonly used are strict protection from the sun; anti-malarials (hydroxychloroquine, mepacrine, etc.) and skin (topical) steroids. Steroids by mouth are generally reserved for cases where there is a flare affecting internal organs. In rare cases, severe resistant lesions may require anti-leprosy drugs, oral corticosteroids, azathioprine, or nitrogen mustard ointment

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Eye Conditions in Lupus

Lupus can cause numerous problems with the eyes, either directly, through medications or due to overlap disorders. Some of these problems are:

  1. Episcleritis: inflammation of the tissues overlying the sclera (sclera: the thick, white tissue of the eyeball)
  2. Scleritis: Inflammation of the sclera.
  3. Uveitis/Iritis: Inflammation of the middle layer of the eye including the iris, lens its muscles. Iritis (a specific form of uveitis) is very painful and typically accompanied by photophobia (abnormal intolerance to light). Untreated uveitis can lead to blindness.
  4. Vasculitis (retinal): Inflammation of the vessels supplying blood/nutrients to the retina
  5. Optic Neuritis: Inflammation of the optic nerve
  6. Other concerns: Scarring and inflammation of the conjunctiva, cotton wool (deposits in the liquid part of the eye), dry eyes (either as a result of lupus or of Sjögren's Syndrome.)

Many medications can cause dry eyes, mouth and other mucous membranes . Any anticholinergic drug can cause this. Examples of such drugs are antihistamines such as Benadryl and Phenergan, pain medications, sleep medications, tricyclic antidepressants, antispasmodics, muscle relaxants and antianxiety medications.

Plaquenil, a popular and very useful immunosuppressive used to treat Lupus, can potentially cause retinal damage. It is rare but happens often enough that any patient on this drug should have a baseline macular check and field test before starting the drug (or soon thereafter) and repeated tests every 6 months.

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Gastro-intestinal Symptoms

There are many gastro-intestinal problems associated with autoimmune disease. The most common is probably IBS or Irritable Bowel Syndrome. Symptoms are varied. Some GI problems may be a result of reduced production by the mucous membranes that can occur due to the disease process or due to many of the medications that lupus patients must take.

Some GI problems associated with lupus may be due to sensitivities and allergies. Allergies are common in lupus patients and such reactions to certain foods can cause pain and GI distress.

Another GI symptom seen in lupus is GI Vasculitis. This condition is difficult to diagnose and is usually diagnosed by eliminating other diagnoses and biopsy of GI tissue. Symptoms do not differ greatly from other GI conditions.

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Lupus Nephritis

The following is summarized from a presentation given by Dr. Momir Macanovic, 9 May 1997

Nephritis is due to the deposition in the kidney of anti-double-stranded DNA (anti-dsDNA) antibodies or immune complexes followed by an inflammatory response typical of the immune system. Nephritis should be characterized by biopsy in patients of abnormal renal function tests to determine treatment.

Nephritis ranges from asymptomatic proteinuria (presence of protein in the urine) to sudden onset of renal insufficiency. Dr. Macanovic states that reduction of creatinine clearance is the most reliable indicator of lupus nephritis. (40-80% of all lupus nephritis patients will show this clinical result.) In addition, autoantibodies, mostly to dsDNA, a rise in anti-dsDNA in the blood, decrease in complements C3 and C4, increase in immune complexes and an elevated ESR are typical lupus nephritis clinical findings.

Nephritis is categorized as Types I through V. Types I and II are "mild glomerulonephritis" and require little or no therapy. Types III - V require aggressive treatment with steroids, chemo and/or plasmapheresis.

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Mixed Connective Tissue Disease (MCTD) and Lupus

Mixed Connective Tissue Disease (MCTD) is the term applied to the presence of two or more specific autoimmune diseases in one patient. These diseases are Lupus, scleroderma, rheumatoid arthritis, and polymyositis/dermatomyositis. A patient need have only two of these conditions to be considered as having MCTD. Treatment for MCTD is generally treatment for the disease symptoms that dominate the individual's condition. That is, if the patient has symptoms that are associated more with RA, the patient's treatment will reflect that. MCTD is often confused with UCTD. See Can a Lupus Patient have a negative ANA titre? and the Glossary for more information on UCTD.

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Raynaud's and Lupus

Raynaud's Phenomenon is common in SLE patients (Secondary Raynaud's) though it can manifest as a distinct syndrome (primary). It can affect the hands, feet, nose, ears and lips. Like most autoimmune conditions it is more common in women than in men.

Raynaud's is characterized by spasms of the small vessels in the affected area. It is usually a response to cold temperature, abrupt changes in temperature, or stress. We all get cold hands and feet when the environment is cold, but Raynaud's is a more extreme response. It is not just poor circulation (as might be seen in individuals that are sedentary or have other medical problems.)

Fingers or toes (and the other susceptible areas mentioned above) may first turn white, then after a time may be blue or purple. During this phase the area is usually numb and cold. Often a bright red line can be seen between the white/blue area and the part of the foot/hand that is getting normal circulation. When the vessels stop spasming and blood flow returns these areas may turn bright red and feel hot or have a stinging sensation. In some cases the blood vessels might not go through the initial shutdown phase but dilate excessively and cause this intense redness and burning.

Raynaud's is usually more of an irritant or impediment to daily activity. However, in severe cases it can lead to skin damage, gangrene and/or loss of tissue.

Diagnosis is by visual evidence and history. Since Raynaud's can be related to autoimmune disease, an elevated ESR or ANA and other antibody tests can sometimes help determine if the condition is primary or secondary. Carpal Tunnel Syndrome and certain medications can cause Raynaud's.

Treatment for Raynaud's is largely behavioral: warming the area gently, maintaining the body's core temperature, reducing stress level, stopping smoking (nicotine affects skin temperature and vaso-constriction) and exercise. Medications for Raynaud's include Calcium channel blockers, alpha blockers and nitroglycerin. In Secondary Raynaud's, treatment can be based on controlling the underlying condition.

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Photosensitivity

Photosensitivity is one of the most common symptoms of Lupus. Patients may experience anything from mild rashes and fever to major organ inflammation in response to exposure to ultra-violet, visible and even infra-red (heat) light. Photosensitivity as a result of disease (vs. medication) affects about 50%-75% of all lupus patients. Medications can exacerbate the condition. Most damaging are UVA and UVB. UVB is the major cause of skin damage to all people and is the wavelength most often screened out by standard sunscreen products. However UVA is not blocked by most of these products and for the lupus patient UVA is equally threatening. It is important then that patients use products that block both UVA and UVB. Some major sources of UV exposure are:

Some patients might experience photosensitivity as one of the first indications that they have a problem. It may show up as a light rash on the cheeks and nose or fatigue and aches after exposure. It isn't always obvious that the exposure and the resulting symptoms are connected. Some patients may not notice the symptoms until several hours or even a day or two after the exposure or they may think that it is just a normal response to "overdoing it." As time goes on, the patient might notice that a walk to the park or a Sunday drive results in increasing fatigue or rashes. While most rashes will show up on exposed areas of the body (scalp, arms, neck) the rashes associated with lupus and aggravated by photoexposure can occur on any part of the body.

Photosensitivity often increases over time and the patient who knows they are photosensitive should take precautions to protect themselves from exposure. There are many excellent products available that mechanically or topically reduce UV and visible light penetration into the skin. See Products That Help for links to some of these products. Most literature recommends a product of 30SPF but there are products with much higher ratings available and a layer of clothing can be extremely helpful as well.

It is important with photosensitivity as with all lupus symptoms to remember that because you do not have the symptom currently, does not mean you will not develop it later. Light exposure is one of the things we have the most control over with this disease. It is the opinion of this author that respecting the sun is one of the most important preventive measures we can take. Learn your limits and stick to them whenever possible but be aware that they might change. Extreme exposure can cause life-threatening flares.

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Scleroderma and Lupus

Yet another autoimmune disease that sometimes manifests with SLE. When scleroderma and SLE are diagnosed in one patient, it is referred to as MCTD. See Mixed Connective Tissue Disease (MCTD) and Lupus

Scleroderma, like other autoimmune diseases, can affect many organs because the tissue it attacks is present in many areas. Most commonly it damages the elastic fibers in the skin and other organs. This results in skin tightness and more serious skin damage, damage to the gastro-intestinal tract, kidneys, heart, and lungs. This damage can be due to vessels damaged by scleroderma or by the formation of scar tissue. Scar tissue in healthy people lacks the elasticity of the original tissue. In the scleroderma patient this scar tissue is formed not due to direct injury such as infection or injury, but due to autoantibody presence.

CREST is a variation of Scleroderma. The name of this condition comes from its symptoms

CREST is considered less severe than typical Scleroderma. Other symptoms of Non-CREST scleroderma are similar to other autoimmune symptoms.

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Sjögren's in Lupus

Yet another overlap autoimmune condition, Sjögren's runs the gamut from dry eyes and mouth, to joint pain and inflammation of parts of the eye that can impair vision.

Sjogren's is an autoimmune attack on the mucous membranes which are present in the eyes, ears, sinuses, mouth, nose, salivary glands, GI tract and vaginal area. Sjögren's is treated much like other autoimmune diseases but with special attention to the alleviation of mucous membrane dryness.

Testing for Sjögren's includes:

  1. Schirmer's tests: A test that determines tearing, first done without ocular anesthesia, then done with ocular anesthesia.
  2. Ocular dyes: these test for scarring and dry areas of the eye.
  3. Autoantibodies: SSA and SSB can be instructive in diagnosing Sjögren's
  4. Lip biopsy

There are many drugs that can mimic the drying effect of Sjögren's. These include pain killers, sleep and anxiety medications, antidepressants, antihistamines, and muscle relaxants. Since many of these are given to patients with autoimmune disease, these should be taken into consideration when trying to diagnose Sjögren's as an overlapping condition.

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Vasculitis

Vasculitis is inflammation of the walls of a blood vessel, and can occur in many different illnesses - or by itself without any obvious associated infection or other illness! Vasculitis in lupus is caused by an immune or "allergic" reaction in the vessel walls. Substances which cause allergic reactions are called "antigens", and the body makes proteins called "antibodies" which bind to the antigen and get rid of it. An antigen and its antibody bound together are called "immune complexes." Unfortunately, some immune complexes remain in the body, where they commonly accumulate in blood vessel walls and cause inflammation. In the vasculitis caused by lupus, the antigens causing the immune complexes are often not known. In some cases, the complexes contain DNA and anti-DNA antigens, or Ro (also called SS-A) and anti-Ro antigens.

Vasculitis can be very mild and of little importance, or very severe and life-threatening - or any degree in between. It can cause many different symptoms, depending upon the tissues are involved and the severity of the tissue damage. Some patients are not ill and notice occasional spots on their skin. Others are very ill with systemic symptoms and major organ damage. The diagnosis of vasculitis is based on a person's medical history, current symptoms, a physical examination, and the results of specialized laboratory tests. These may involve blood or urine tests; examination and tests on the organs, tissues and blood; and biopsies. The choice of treatment for vasculitis depends on the severity of the vasculitis, your general health, and your past reactions (positive and negative) to medications.

A list of the more common symptoms includes:

Useful links on Vasculitis

One of the best is an article by Professor Bevra H. Hahn, U.C.L.A., easiest to read at: http://www.hamline.edu/lupus/articles/vasculitis.html

A more general site with some useful pictures is at: www.dermnetnz.org/pre/dna.vasculitis/vasc.html

Vasculitis in the central nervous system is discussed briefly at: http://www.cnsv.net/index.html

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