ANCA-Negative, Pauci-Immune Crescentic Glomerulonephritis in a Woman Presenting with IgA Vasculitis

Introduction: Pauci immune,the most common type of rapidly progressive glomerulonephritis usually have circulating ANCA antibodies.IgA vasculitis is a small-vessel vasculitis with IgA-dominant immune deposits commonly presents with immune complex glomerulonephritis.This case presents with ANCA negative IgA vasculitis. Case Description: We report a case of a 54-year-old female,Filipino,hypertensive,newly diagnosed diabetic who presented with abdominal pain and purpuric rashes,edema and oliguria.General survey revealed diffuse,palpable,non-blanching purpuric rashes involving the extremities and trunk with signs of fluid retention.Workup showed a normal platelet,elevated creatinine.Urinalysis showed hematuria and nephritic range proteinuria.The patient was managed as a case of RPGN.Immunologic work up showed negative ANA,Anti-dsDNA IgG and IgM,C3,C4,C-ANCA and P-ANCA results.Skin biopsy revealed fibrosis and presence of small vessel capillary vasculitis.The kidney biopsy revealed cellular crescents on LM, IF showed segmental granular staining predominantly IgA.EM showed widespread foot process effacement and no definite electron dense deposits are identified in GBM and the mesangium.Mangement included methylprednisolone, MMF and temporary hemodialysis.Her condition improved and was hemodialysis free after 6 weeks. Discussion: The coexistence of Pauci imung RPGN and IgA vasculitis is rarely reported.Poor renal prognosis is predicted by an acute nephritic presentation,older age,larger amounts of proteinuria and a more severe nephrotic syndrome.Therapy is supportive with immunosuppression, cytotoxic immunosuppressive and even plasma exchange. Early identification and prompt initiation of treatment as well as timely assessment of the need for renal replacement therapy play the major roles for a favorable outcome.