1. Henoch-SchöNlein Purpura in Adults
- Author
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G Nasswetter, Javier A. Cavallasca, María del Rosario Maliandi, and María José López Meiller
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lcsh:R5-920 ,medicine.medical_specialty ,Henoch-Schonlein purpura ,medicine.diagnostic_test ,business.industry ,Complete blood count ,General Medicine ,medicine.disease ,Gastroenterology ,Surgery ,Purpura ,Internal medicine ,Biopsy ,Skin biopsy ,medicine ,medicine.symptom ,lcsh:Medicine (General) ,business ,Vasculitis ,Palpable purpura ,Systemic vasculitis - Abstract
Henoch-Schonlein Purpura (HSP) is a systemic vasculitis mediated by IgA and characterized by the clinical triad of non-thrombocytopenic palpable purpura, abdominal pain, and arthritis. In addition, there may be varying degrees of renal involvement. It is the most frequent vasculitis in children and the incidence in adults varies from 3.4 to 14.3 cases per million, depending upon the experimental series analyzed.1 We report the onset, course and response to treatment of five adult patients who required hospitalization at our institution between 2002 and 2006 (all fulfilled the 1990 American College of Rheumatology (ACR) criteria).2 Case 1 A 24-year-old white male with frequent episodes of pharyngitis was admitted for an eight day course of palpable purpuric lesions on his lower limbs, arthritis in both ankles, and diffuse abdominal pain. A complete blood count with differential analysis, liver function test, coagulation test, chemistry panel, and ERS were normal. A urinary sediment showed dysmorphic hematuria with 30 to 40 red blood cells/high power field and proteinuria of 2.29 g / 24 hr. A skin biopsy of the lower limbs showed leukocytoclastic vasculitis. IgA and C3 deposits were observed upon immunofluorescent assay. The renal biopsy indicated mesangial nephropathy due to IgA deposition (Figure 1). Figure 1 Renal biopsy with mesangial IgA deposition (inmunofluorescence, 200X) The patient began treatment with IV methylprednisolone, 1 g/day for 3 days, and continued with oral prednisone, 40 mg/day. During treatment, the patient presented an increase in proteinuria (3.12 g/day), the persistence of dysmorphic hematuria, and developed hypertension. He was therefore treated with IV cyclophosphamide, 1g/m²/month for six months, and enalapril, 10 mg/day. Both blood and urine laboratory parameters stabilized after the first pulse of cyclophosphamide.
- Published
- 2008
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