PAUCI-IMMUNE CRESCENTIC GLOMERULONEPHRITIS: A PROSPECTIVE ANALYSIS

Crescentic Glomerulonephritis

Abstract

Objective: To assess clinical presentation and outcome of patients with Pauci-Immune Crescentic Glomerulo Nephritis (PICGN) in our setup.
Design: Case series.
Place and Duration of Study: Armed Forces Institute of Urology (AFIU) and Military Hospital (MH) Rawalpindi from November 2007 to October 2009.
Patients and Methods: In a prospective design, patients diagnosed as pauci-immune glomerulo nephritis, were included in this case series. Detailed history along with physical examination and thorough investigation of all cases, including vasculitic screening and renal biopsy were carried out. Disease activity was evaluated with Birmingham Vasculitis Assessment Score (BVAS) and organ involvement assessed by Disease Extent Index (DEI). The patients were treated with a combination of cyclophosphamide and steroids and were followed up on monthly basis. All data was recorded and analyzed using SPSS version 16.
Results: Twelve patients were included in this case series. Beside renal involvement (100%), constitutional symptoms were present in majority of patients (91.7%), lung involvement (58.3%), upper respiratory tract (41.7%) and musculoskeletal system (33.3%). Six patients were positive for c-ANCA and 4 for p-ANCA. Three patients (25%) developed End Stage Renal Disease (ESRD). There were five deaths (41. 7%) during study period which included these three with ESRD.
Conclusion: Pauci-immune cresentic glomerulo nephritis is an uncommon systemic disease with varied presentation and has high mortality without treatment. Combined treatment with cyclophosphamide and steroids improves outcome but can have life threatening side-effects. Therefore treatment needs to be individualized and benefit to risk ratio weighed before its commencement.