2009 Southern Regional Meeting Abstracts

Case Report: INTRODUCTION: Henoch-Schonlein Purpura (HSP) is a vasculitis associated with IgA deposition and is characterized by arthralgia, purpuric rash, abdominal pain and nephropathy. Anti-neutrophil cytoplasmic antibodies (ANCA) are present in many isotypes (IgM, IgG or IgA). Two common patterns, cytoplasmic (c-ANCA) react with proteinase 3 (PR3) is specific for Wegner's granulomatosis and perinuclear (p-ANCA) react with myeloperoxidase (MPO) seen in microscopic polyangiitis. In HSP and IgA nephropathy, IgA ANCAs and IgG p-ANCA have been reported in several cases. But IgG c-ANCA has only been reported twice. We report a case of biopsy proven HSP associated with IgG c-ANCA.
CASE: A 51-year-old white male with unremarkable medical history presented with a two week history of diarrhea, abdominal pain, joint aches and rash in his legs extending to his whole body. On exam he had diffuse palpable purpura, mild epigastric tenderness, edema on the dorsum of his hands and feet. Abnormal laboratory studies included elevated WBC count, transaminases, alkaline phosphatase and bilirubin as well as hematuria and pyuria. 24-hour urine protein was 413mg. He had a positive c-ANCA by immunofluorescence (IFA), confirmed to be IgG anti-PR3 by ELISA at 14U/ml. Rest of the studies including renal functions, blood counts, coagulation tests, p-ANCA, hepatitis serology, complements, cryoglobulins, cultures and heart echo were normal. Gall bladder ultrasound showed acute cholecystitis. Skin biopsy of recent lesions revealed leukocytoclastic vasculitis with IgA and C3 deposition by IFA. He was treated with intravenous antibiotics, ERCP guided papillotomy and oral prednisone 1mg/kg/day. Six weeks later, he had complete resolution of his rash, systemic symptoms and a stable creatinine.
CONCLUSION: Our case highlights that IgG c-ANCA can be found in HSP, despite being more specific for Wegner's graulomatosis. It is important to make a correct diagnosis as the course and treatment are different. Steroids only were used in our patient with a good outcome, while steroids and cyclophosphamide were required in the previous two reported cases. Till the causality link between IgG c-ANCA and IgA related disease gets established with further studies, we suggest closer monitoring of renal functions and urine analysis in the setting of these patients.