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Case 18

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Immunology Cases 2005Case 18J.P. is a 63-year-old woman who was admitted to CPMC for evaluation of daily fevers to 102°F,rash, and diffuse arthralgias (joint pains). Physical examination was notable for BP 160/95 mmHg and the presence of a tender, palpable, purpuric rash on her lower extremities (Fig. 1). Shehad no lymphadenopathy (enlarged lymph nodes) or joint swelling. Laboratory examinationrevealed Hct 34.5% (low), WBC 7.9 (X109/l) (normal). Serum creatinine was 2.0 mg/dl(elevated). Serum bilirubin, transaminases and alkaline phosphatase (liver function tests) were allslightly elevated. Her ESR (erythrocyte sedimentation rate) was 74 (very high; consistent withinflammation). A urinalysis revealed no protein, minimal microscopic hematuria (3-4 RBC perhigh power field) and occasional WBCs and numerous granular casts. A test for Hepatitis Bsurface antigen was positive. The following tests were negative or normal: Coombs, heterophileagglutinins, anti-streptolysin 0 (ASLO), latex fixation, ANA (anti-nuclear antibodies),cryoglobulins and SPEP (serum protein electrophoresis). All bacterial and fungal cultures werenegative. Skin tests to tuberculin, fungal, mumps were all non-reactive. A chest X-ray wasnormal. During her hospitalization, she developed paresthesias (tingling) of the first three fingerson the right hand followed by intermittent burning pain in the same distribution. She alsocomplained of sciatica which, in retrospect, she noted intermittently in the prior 6 months. Aneurological examination was notable for muscular weakness in the L2, 3 distribution. EMG(electromyogram) of the median nerve and L2, L3 was consistent with a neuropathy. A biopsy ofthe skin is shown below (Fig. 2). She was treated with pulse intravenous methylprednisolone (15mg/kg/dose IV X 3 days), followed by oral prednisone (1 mg/kg/day) and her fever resolvedslowly. She remained afebrile but her neuropathy persisted and Cytoxan (cyclophosphamide)was added to her regimen with resultant improvement in her neurological symptoms.Fig. 1. Anterior tibial surface of J.P.demonstrates a slightly raised, tender,purpuric rash.Immunology Cases 2005Questions for Case 18(1) What antigen was most likely involved in the etiology of this patient's immune vasculardisease?(2) What immunologic mechanisms caused the renal and neurologic abnormalities?(3) What are the similarities and differences between the renal disease in this patient and inpatients with SLE? If a renal angiogram were obtained in J.P., what would the results likely be?(4) What other immunologic and clinical parameters distinguish J.P. from patients with SLE?(5) The classification of vasculitis is complex. Mention three other types of vasculitis anddescribe how they are different from the current case.Fig. 2. H&E stain of a section from askin biopsy reveals lymphocyteinfiltration around a


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