Systemic lupus erythematous (SLE)
1. Describe the genetic, nongenetic, and immunologic factors in the etiology of SLE.
2. What are criteria for the diagnosis of SLE?
3. List common clinical manifestations of lupus (present in over 50% of patients).
4. Describe the role of antinuclear antibodies and immune complexes in the pathogenesis of renal disease in SLE.
5. What is lupus anticoagulant and what clinical and laboratory features are noted in patients who have this antibody?
6. Briefly describe the pathology in the joints, CNS, serosal surfaces, heart, and lung.
7. What is the long term prognosis of lupus?
8. Define chronic discoid LE, subacute cutaneous LE, and drug-induced LE.
Laboratory diagnosis of connective tissue diseases ( Robbins 5th Ed., Table 6-7, p. 201) or (Robbins 6th Ed., Table 7-8, p. 218)
1. Describe the four ANA patterns, including the significance of nucleolar and centromere patterns. What is the importance of DNA and Sm antibodies in the diagnosis of SLE? How are serum levels of complement affected in SLE? What is the cause and significance of low serum complement levels in SLE? What is the sensitivity of U1-RNP antibodies for mixed connective tissue disease (MCTD)?
2. Select and interpret the appropriate autoantibody and complement tests for SLE, rheumatoid arthritis, scleroderma, inflammatory myopathies, Sjögren's syndrome, and MCTD.
3. Define rheumatoid factor (RF). What is the stimulus for RF production? How specific is this test for rheumatoid arthritis?
4. List the diseases associated with the depression of complement (either C3, C4, or both) and indicate changes in C4 and C3 associated with each of these diseases.
1. (Robbins 5th Ed., p. 199-208) or (Robbins 6th Ed., p.
216-225)
2. Images in Diseases of Immunity Case Studies folder: Case 2
J. W., a 20-year-old female, presented with complaints of fever for two weeks, pleuritic chest pain, and intermittent joint pain in her hands, feet, and knees. On examination, she was free of skin lesions. She had a pleural friction rub, a slightly swollen, tender right knee, and enlarged axillary, cervical, and inguinal lymph nodes. A chest film showed a small right pleural effusion, and joint films were normal except for a small amount of soft tissue swelling in the right knee. Hemoglobin was 10 g/dL and urinalysis revealed 4+ proteinuria with white cells, red cells, and red cell casts in the urine sediment. AVDRL test was positive. A test for anti-treponemal antibodies, however, was negative. The blood urea nitrogen (BUN) and creatinine were slightly elevated and moderate hypoalbuminemia and hypergammaglobulinemia were present. An ANA test was positive at a titer of 1:2560, and was reported as showing a speckled pattern. A dsDNA antibody determination by the Crithidia luciliae assay was positive at 1:320 and an ENA antibody determination was reported as: RNP (U1-RNP) and Sm antibody present. A C4 level was <10 mg/dL (reference range: 12-45 mg/dL) and a C3 < 40 mg/dL (reference range: 88-192 mg/dL). The patient was treated with steroids, 60 mg/day of prednisone. After four weeks the urine protein was 2+, the sediment had cleared, and the BUN and serum creatinine had returned toward normal.