Musculoskeletal diseases: Systemic lupus erythematosus

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Systematic lupus erythematosus

Lupus erythematosus is a chronic, rheumatic autoimmune disease. This means that the immune system produces antibodies that attack the body’s own healthy tissues and organs, including the joints, kidneys, heart, lungs, brain, blood, or skin. These antibodies, called autoantibodies, contribute to the inflammation of various parts of the body and can cause damage to organs and tissues. Most people experience symptoms such as skin manifestations, arthritis, mouth ulcers, hair loss and fatigue, but symptoms vary from person to person. Systemic lupus erythematosus (SLE), involving multiple parts of the body (beyond the skin), is the most common type of lupus, and is therefore the focus of the current section.

It is estimated that approximately 15,000 (1 in 2,000) Canadians are affected with SLE.³ It can appear in both men and women of all ages, but develops most often between the ages of 15 and 44. In this age group, SLE occurs about 10 times more frequently in adult women than men. Among those under 15 and older than 45, however, it is equally common in men and women. It is more common among African Americans and Asians. It is also more common and more severe among Aboriginal people.^3,36

Early symptoms of SLE include pain in joints and muscles, skin rash (especially on the face), a persistent low-grade fever and fatigue. Other manifestations of the disease include swollen glands, lack of appetite, sensitivity to sunlight, unusual loss of hair (alopecia), oral ulcers, dry eyes and mouth, arthritis, serositis (inflammation of the lining around the heart, lungs, and/or abdomen, causing pain and difficulty taking a deep breath), nausea, vomiting and diarrhea, weight loss, Raynaud’s phenomenon (the fingers and toes may turn white or blue in the cold due to poor circulation), abnormalities of the blood cells (anemia, low platelet count, which can cause bleeding) or low white blood cell count (which can lead to severe infections), increased risk of clots in the veins of the limbs (deep venous thrombosis) or in the arteries to the brain (stroke). Severe internal organ involvement includes kidney problems (leaking of proteins and cells into the urine, which can lead to renal failure requiring dialysis or kidney transplant), lung problems (inflammation or bleeding in the lungs leading to difficulty breathing), heart problems (destruction of valves in the heart, coronary artery disease causing chest pains due to angina), involvement of the brain or nerves with loss of sensation or muscle weakness and various other neurological manifestations. Symptoms can range from mild to life-threatening; severe involvement of the brain, lungs, heart and kidneys lead to a poor prognosis in terms of overall survival and disability. However, for most people, lupus is a mild disease, affecting only a few organs, although some SLE cases have severe organ involvement.

Researchers do not know what causes lupus, although scientists believe there is a genetic predisposition to the disease: up to 5% of children born to individuals with lupus will develop the disease.^37,38 Environmental factors, which include infections, antibiotics, ultraviolet light, extreme stress, and certain drugs, also play a role in triggering the disease. Furthermore, hormonal factors may explain why lupus occurs more frequently in females.

There is no single test that can identify lupus; it may take months or even years for doctors to accurately diagnose. Clinical diagnosis of SLE requires the presence of at least 4 of the 11 American College of Rheumatology classification criteria. These criteria are: malar (“butterfly”) rash over the cheeks of the face; discoid skin rash (patchy redness on the skin); photosensitivity; oral ulcers; arthritis in two or more joints of the extremities; serositis; renal disorder; neurologic disorder; blood count abnormalities; immunologic disorder; and antinuclear antibodies.³⁹

SLE appears in cycles; the disease tends to wax and wane, with periods of exacerbations of the disease (or acute flare-ups) when the disease becomes more active, with more symptoms and possible involvement of a new internal organ not previously affected, and periods of chronic low grade disease activity, in which some symptoms are present but not as severe as during a flare-up. Flare-ups can last from days to weeks, and occasionally months. Rarely, the disease can go into remission, when there are few or no symptoms. In addition, over time, individuals may be left with some damage that accumulates in different organs, from previous active SLE in these organs. The duration and pattern of these cycles are highly variable from individual to individual. Over time, people can be left with permanent damage in some parts of their bodies from acute exacerbations involving internal organs (such as a stroke or nerve damage, end stage renal disease requiring dialysis, scarring in the lungs causing breathlessness).

There is no cure, but for the vast majority of people with lupus, effective treatment can minimize symptoms, reduce inflammation and maintain normal capabilities of the body and immune system. The primary goal is to protect organs from damage by decreasing inflammation and/or the level of autoimmune activity in the body. Treatment approaches are based on the specific needs and symptoms of each person. The variety of medications commonly used depends on the organ(s) involved and the degree of involvement. Generally, mild symptoms (skin rashes, hair loss, ulcers, chest pain, arthritis) are treated with antimalarial agents and with non-steroidal anti-inflammatories (NSAIDs). More severe internal organ involvement requires the use of immunosuppressants, i.e., medications that suppress the immune system. Corticosteroids are often used in small doses for short periods to control flare-ups and in higher doses for longer periods of time to control internal organ involvement. With treatment, SLE can be controlled. However, it should be noted that long-term treatment of severe SLE with corticosteroids and immunosuppressants can have multiple and sometimes considerable side effects. For example, there is an increased risk of infections and of cancer, especially lymphomas, and long term corticosteroid use often results in osteoporosis (thinning of the bones) and increases the risk of diabetes, hypertension and coronary artery disease.⁴⁰

Systemic lupus erythematosus, acute episode
ICD-9 code: 710.0

Description

SLE is sometimes called “the disease with 1000 faces” because there is no real, “typical” case. Clinical diagnosis, however, requires the presence of at least 4 of 11 criteria developed by the American College of Rheumatology. The most common symptoms of SLE, which include fatigue, skin rashes, intermittently sore and swollen joints, mouth ulcers, hair loss, dry eyes and mouth and chest pain, tend to increase during periods of flare-ups, with prominent fatigue and often low-grade fever. Severe internal organ involvement can also develop during periods of flare-ups. These symptoms result in moderate levels of pain and discomfort and mild limitations in physical functioning, depending on the severity of the flare-up. During active periods, they often contribute to limitations in social relationships (because of prominent fatigue as well as pain). Skin rashes may lead to social withdrawal; arthritis (and fatigue) can limit activities and work. Emotional state is affected as well; often people with lupus experience anxiety, depression and a sense of hopelessness due to the chronic, waxing and waning nature of the condition and its potential for developing complications. Prolonged and extreme fatigue may be experienced; it typically stems from the disease itself but can also be the result of medication-related complications (such as anemia). Reduced attention span and mild cognitive dysfunction is very common, particularly during flare-ups of SLE.

Classification

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Systemic lupus erythematosus, chronic active
ICD-9 code: 710.0

Description

During the chronic active stage (in between periods of flare-ups), the most common symptoms of SLE tend to come and go. The intensity of the symptoms is highly variable, depending on individual disease and potentially varying over time for each individual. Severe internal organ involvement is typically during flare-ups, although occasionally some may be chronically involved. With treatment, most people have mild levels of pain and discomfort, mainly due to ongoing arthritis, mouth ulcers, dry eyes and mouth, and mild limitations in physical functioning. Fatigue is often a symptom that never completely goes away and is an important cause of limitation in function and social relationships (due to the lessened stamina for usual activities). Reduced attention span and mild cognitive dysfunction may persist (even when not in a flare-up).

Classification

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Health State Descriptions for Canadians 82-619-MIE