Lupus

Reference: Current Diagnosis and Treatment-Rheumatology (Imboden et. al.) UpToDate- Overview of clinical manifestations of SLE in adults, Therapy and prognosis of SLE in adults **General:** SLE is usually a multi-organ, multisystem autoimmune disease (different from chronic cutaneous lupus and drug induced lupus [INH, procainamide, hydralazine, minocycline, anti TNFs]) **Epidemiology/risk factors:** Usually occurs in women (9:1) in their 20-30s, more common in African Americans. Prevalence is 130/100,000 white females. Risk factors and triggers include genetic predisposition, sun exposure, sulfa allergies, possibly echinacea, smoking, EBV infection, silica and mercury exposure, higher dose OCP and hormone replacement, possibly elevated prolactin levels. **Symptoms:** huge range of symptoms including fever, weight loss, fatigue, LAD, polyarthralgias/arthritis, myalgias, myositis, malar or discoid rash, livido reticularis, raynaud, alopecia, oral ulcers, dry eyes and mouth, lupus nephritis, eye involvement (episcleritis, retinitis, optic neuritis, uveitis), pleurisy, pleural effusions, peri/myo/endocarditis, pulmonary hemorrhage, coronary arteritis and aneurysm, GI involvement (esophageal dysmotility, hepatosplenomegaly, elevated LFTs, mesenteric vasculitis, colitis, primary biliary cirrhosis, Budd-Chiari syndrome, ascites), neuro involvement (cognitive impairment, seizures, psychosis, TIA, stroke, mononeuritis multiplex, pheripheral neuropathy, cranial neuropathy, chorea, pseudotumor cerebri, transverse myelitis, encephalopathy, coma). **Diagnosis:** SLE diagnosed if 4 or more of the following present: malar rash, discoid rash, photosensitivity, oral ulcers, arthritis, serositis, renal disorder (>0.5 g/day proteinuria, 3+ proteinuria, cellular casts), neurologic disorder (seizures, psychosis without other causes), hematologic disorder (hemolytic anemia, leukopenia, lymphopenia, thrombocytopenia), positive ANA, and immunologic disorder ( antibody positive to double-stranded DNA, anti Sm, or positive antiphospholipid antibodies (anti-cardiolipin, lupus anticoagulant, false-positive RPR).  **Treatment** (brief): Plaquenil helps with cutaneous disease, arthritis, fatigue and to prevent flares (very well tolerated). Topical steroids for discoid lupus. NSAIDs for arthritis (if no renal involvement), low - dose steroid treatment for milder disease (arthritis etc). For more severe disease treatment includes high dose steroids, cyclophosphamide, methotrexate, mycophenolate mofetil (Cellcept), azathioprine, Retuximab. In general should promote sun protection, smoking cessation. **Follow-up:** Depends on disease severity. As a primary care physician important to monitor for the complications of the disease and its treatment. Follow creatinine and UA to watch for renal involvement. Treat hypertension with ACE-I/ARB, treat hyperlipidemia (at risk for accelerated atherosclerosis), stroke prevention, monitor and treat osteopenia, osteoporosis and vitamin D deficiency, appropriate cancer screening (increased malignancies with certain treatments and immunosuppresants), give pneumovax q5years, flu vaccine annually.
 * Brief overview of Systemic Lupus Erythematous**