Rashes are one of the most widely recognized clinical features of lupus. There are three main types of lupus rashes. A malar rash is typically a butterfly shaped rash over the nose and cheeks. It can be flat or raised and may be worsened by sun exposure. A discoid rash, as the name suggests, is a circular lesion (or lesions) approximately the size of a coin. These lesions are generally thicker than the other lupus rashes and more likely to cause scarring. Photosensitive rashes are the third type of lupus rash. These rashes generally occur on sun exposed areas and are usually small, red, raised lesions. Oral or nasopharyngeal (in the nose or throat) ulcers are typically painless unless they become infected with bacteria. Joint pain or swelling may also be a feature of lupus and can affect a variety of joints at any given time. Chest pain worsened with deep breaths, also called pleurisy, can occur with lupus. This is caused by inflammation in the tissue around the lungs. Edema, which is swelling in the soft tissue due to water retention, can be an important clinical sign. It can indicate kidney problems and occurs most commonly in the lower extremities, but may also affect the body or the face. Red pinpoint skin lesions called petechiae can also be noted, usually located below the knees. Seizure activity, abnormal behavior, or a change in character or personality may be observed in lupus patients due to brain involvement. Alopecia, which is diffuse hair loss, is also common in lupus patients. Color changes in the fingers or toes with exposure to cold may affect lupus patients as well. This is called Raynaud's Phenomenon. Other symptoms can include fevers, sweats, weight loss, swollen lymph nodes and generalized body aches. All of the symptoms noted in this section can vary in intensity and frequency and may overlap with each other or occur at different times.

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Many of the physical examination findings of lupus correlate closely with the clinical symptoms previously discussed (see clinical symptoms). Malar, discoid and photosensitive types of rashes are common findings. A petechial rash may also be present on the legs. Painless oral and nasopharyngeal ulcers may be noted on examination of the mouth and nose. Swollen joints can sometimes be observed and can limit joint mobility as does the pain on motion that the patient experiences. Inflammation of the tendons (tendinitis) can be seen and usually occur on the hands or feet. When a physician examines the heart and lungs of a lupus patient with a stethoscope, abnormal sounds may be heard. Abnormal heart sounds may include a rub or abnormal scraping sound. This is due to inflammation in the sac that surrounds the heart. A heart murmur or "whooshing" sound may indicate an abnormal heart valve. Inflammation in the tissues surrounding the lungs may create a rub or scraping sound when the patient takes a breath. Edema or swelling due to water retention can also be seen most commonly in the legs. Examining the patient's muscles may reveal tenderness and weakness. Swollen lymph nodes may be found particularly in the neck area.

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Laboratory evaluation plays a key role in the diagnosis of lupus (see Diagnostic Tests in the Rheumatic Diseases on this web site). An important concept in lupus is autoimmunity. This indicates that the immune system which normally protects us from infection, has mounted an attack on our own tissues instead. Several blood tests used to diagnose lupus are indicators of this autoimmune process. The first of these tests is called an ANA test or (antinuclear antibody test). It is present in a very high percentage (over 90%) of lupus patients. An anti-DNA test is the next autoimmune antibody test. It is more specific for lupus than the ANA test and sometimes may be a predictor of major organ involvement. Another typical autoimmune antibody is called anti-Sm or anti-Smith. There are indicators of inflammation on blood tests that can also be abnormal in lupus. Sed rates (ESR) and C-reactive protein (CRP) levels may be elevated indicating inflammation. Proteins called complement (C3 and C4) which are part of the inflammatory process may be abnormally low in lupus patients. (Please see the glossary and Diagnostic Tests in Rheumatic Diseases or our web site for further description of these terms). Abnormalities of blood counts in lupus are very common. These may include anemia (low red blood cell counts) and leukopenia (low white blood cell counts). Platelets, which are responsible for blood clotting, may also be decreased. This is called thrombocytopenia. Kidney abnormalities can be detected in several ways. A urinalysis may show abnormal amounts of protein, white blood cells and red blood cells. Two blood tests called BUN and creatinine, may be elevated and indicate a decline in kidney function. Other blood tests that can be abnormal may indicate problems related to forming blood clots. These tests measure for anticardiolipin antibodies and the presence of a lupus anticoagulant. Another test that is a useful diagnostic test and may also be associated with clotting problems is called an RPR. This is a screening test for syphilis (a bacterial infection) which is positive in some lupus patients. These patients do not have syphilis, but their lupus causes the test to be falsely positive. X-rays are generally normal but may reveal soft tissue swelling around joints due to inflammation. Rarely, joint deformity or destruction can be seen. Depending on an individual patients' clinical symptoms, other tests may be useful. An MRI may help detect abnormalities of the brain in patients with neurologic complaints. Patients with heart or lung related complaints may need a chest x-ray, pulmonary function testing, or an echocardiogram (an ultrasound of the heart that checks structure and function).

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Making the diagnosis of SLE can be difficult due to a large number of potential signs and symptoms all of which may not be present at one particular point in time. Therefore, it may be necessary to follow a patient's physical exam and laboratory analysis over time to make a definitive diagnosis. The American College of Rheumatology has established diagnostic criteria for lupus. In order to make a diagnosis of lupus, patients must have at least four of the following eleven criteria:

1) A malar (butterfly) rash.
2) A discoid rash.
3) A photosensitive rash.
4) Oral or nasopharyngeal ulcers.
5) Arthritis (joint inflammation - joint pain alone is not diagnostic)
6) Seizures or psychosis.
7) Urine abnormality - either abnormal protein levels or red or white blood cells in the urine.
8) Pleuritis or pericarditis (see section on clinical symptoms and physical examination).
9) Low blood cell counts - this can be white cells, red cells or platelets.
10) Positive ANA.
11) Positive anti-DNA or positive anti-Sm or a false positive test for syphilis.

These findings must be observed by a physician in order to establish a lupus diagnosis. (Refer to sections on clinical symptoms or physical examination).

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The treatment of lupus is tailored to each patient depending on the specific manifestations of the disease. Non-pharmacologic (non-drug) therapy may include physical therapy including strengthening and endurance exercises. Topical sunscreens and sun avoidance can help reduce exposure to harmful ultraviolet light which may cause flares of lupus. Pharmacologic therapies are used in lupus to control a wide variety of problems. Topical steroids such as creams or ointments are very helpful in controlling rashes. Nonsteroidal anti-inflammatory drugs are widely used and can help alleviate several symptoms such as muscle and joint pain, fevers, pleurisy and mild pericarditis. Hydroxychloroquine is a slow-acting medication especially useful at controlling skin manifestations of lupus. It may also be helpful in controlling muscle pain or joint pain from arthritis. Corticosteroids are rapid acting anti-inflammatory drugs generally used to quickly control more severe disease flares. At low dosages they can help dramatically with arthritis and pleuritis or pericarditis (please refer to section on clinical symptoms and physical exam). When there is evidence of major organ involvement particularly in the kidneys or brain, this medicine is used at much higher dosages. Cytotoxic (toxic to cells) drugs like methotrexate and azathioprine are slower acting and can be used for more serious disease manifestations. These medications are generally started after the use of corticosteroids in order to maintain long-term control of inflammation. Finally, for severe major organ involvement, especially kidney involvement, intravenous cyclophosphamide can be used. It is usually given at large dosages every 3-4 weeks. Further description of specific medications can be found on this web site. Accurate diagnosis and appropriate therapy are critical to the well being of lupus patients. With correct treatment many lupus patients can live long, productive lives.

Joel Rutstein M.D.
Matthew Mosbacker, M.D.
Alex DeJesus