Systemic Lupus Erythematosus
Systemic lupus erythematosus (SLE) is a chronic inflammatory autoimmune disease that can affect various organs of the body and has a wide variety of clinical pictures. The woman to man ratio in the young adult population is about 8:1 while the ratio in the child and the elderly populations is closer to 2:1. The cause of SLE remains uncertain.
Risk factors
Risk factors for SLE include: exposure to the sun, fluorescent lights, tanning beds, infections, or stress. Surgery can increase SLE activity. Pregnancy can trigger the first episode of SLE or cause a relapse.
Signs and symptoms:
Constitutional symptoms are a common early complaint and include: fatigue, unintentional weight loss or weight gain, fever, migratory muscle and joint pain. Specific organ-related symptoms include: Joint pain and stiffness, arthritis typically non-deforming. Skin and mucosal manifestations: malar or butterfly rash, discoid lesions (coin-shaped, raised, scaly, inflammatory paches), skin photosensitivity with sunlight or fluorescent light exposure, hair loss (alopecia), mouth ulcers. The Raynaud’s phenomenon consisting of a white/pale then blue discoloration of the fingers and toes that occurs with cold exposure followed by a normal color with warm exposure. Kidney disease is common in SLE and may be completely asymptomatic. Monitoring for changes in kidney function is strongly recommended (blood tests and urine analysis). The gastrointestinal tract is rarely affected by SLE, but may be a result of medication side effects, (steroids and NSAIDs) or the inflammation of the pancreas (pancreatitis), the lining of the abdomen (peritonitis), or the large intestine (colitis) which can induce severe abdominal pain, nausea, and vomiting. Lung and heart disease are frequent from inflammation of the lining covering the lungs (pleurisy) or the heart (pericarditis). Cerebral lupus includes seizures and psychosis. It exhibits a broad spectrum of symptoms: concentration problems, memory loss, depression, anxiety, dry eyes, and dry mouth. Abnormal blood tests: low blood counts (anemia, low white blood cells – leucopenia; or low platelets – thrombocytopenia) are common in patients with SLE. Immunologic tests: positive ANA (antinuclear antibody), anti-dsDNA antibody, anti-Sm antibody, anti-phospholipids antibody, false positive serologic test for syphilis (RPR), anti-SSA antibody, anti-SSB antibody, low complement C3 and C4.
Treatment
Fatigue can often be treated with steroids, Hydroxychloroquine (Plaquenil), combined with exercise. A short course of Prednisone may also help. Fever due to active SLE can usually be treated with non-steroidal anti-inflammatory drugs (NSAIDs) and/or acetaminophen. Steroids are rarely needed for fever. A photosensitive rash can be prevented by avoiding sun exposure and by using sunscreen (at least SPF 30) daily. Other skin rashes may be treated with, Hydroxychloroquine, Methotrexate or Dapsone. Inflammatory arthritis responds to DMARDs like Hydroxychloroquine, Methotrexate, and Azathioprine. Severe disease flares (kidney, nervous system and vasculitis) require high dose IV corticosteroids in conjunction with immunosuppressive drugs like: Azathioprine (Imuran), Methotrexate, Cyclophosphamide (Cytoxan), Mycophenolate Mophetil (Cellcept). Additional specific treatments are required for: hypertension, infection, cerebral lupus, thrombosis and blood disorders. A close follow-up with the rheumatologist and regular blood and urine analyses every three months are essential for the control of the disease and for monitoring the medication side effects.