FP157CRESCENTIC IGA NEPHROPATHY- IS THE INDIAN COHORT DIFFERENT?

Abstract

Introduction and Aims: Crescentic IgA nephropathy (IgAN) is defined as IgA disease with >50% of the kidney biopsy with crescents. Crescentic IgAN carries a very poor prognosis and the initial creatinine at presentation predicts long-term outcome. There are very few studies, which have characterized the disease. There is no prospective study of the disease entity. The present prospective study was carried out to evaluate the clinico-pathological correlation and outcome of crescentic IgAN. Methods: : The present prospective study was carried out to from September 2011 to December 2014. Patients >12 yrs with Crescentic IgAN were included in the study. Patients with infection with hepatitis B surface antigen, anti hepatitis C antibody, HIV-I/II and those positive for antinuclear factor were excluded from the study. Patients were followed prospectively monthly for a period of 12 months or till end stage renal (ESRD) or death. Primary objective of the study was to evaluate percentage of patients achieving remission/ESRD and the secondary outcome to see for histological correlation with clinical outcome. Rapidly progressive renal failure (RPRF): Progressive renal dysfunction of < 3 months with active sediments in urine, Chronic kidney disease (CKD)- progressive renal dysfunction of > 3 months or 3.5 gm or >2 gm/day with serum albumin of < 2.5 gm/dl. Results: A total of 60 cases of crescentic IgAN were enrolled in the study. The mean age of the patients was 28.91+/-10.14 (12-60) years. The study included 46 (76.67%) males and 14 (23.33%) females. The mean 24 hour-urine protein (UP), serum creatinine and serum albumin were 2.22+/-1.33 (0.36-5.8) gm, 6.37+/-4.13 (0.78-15.3) mg/ dl and 3.39+/-0.78 (2-5.3) gm/dl respectively. Active sediments were present in 44 (74.57%) patients at presentation. The clinical presentation was RPRF in 34 (56.66%) cases, nephrotic syndrome in 04 (6.66%) and CKD in 20 (33.33%) and 2 (3.33%) cases presented with only subnephrotic proteinuria. Seventeen (28.33%) patients received combination of pulse cyclophosphamide and steroids, 29 (48.33%) received only steroids and 14 (23.34%) cases with tubular atrophy and interstitial fibrosis >70% were not treated with any immunosuppressive. At the end of 12 months 03 (5%) achieved CR, 08 (13.33%) had PR progressive CKD in 09 (15%) and 02 (3.33%) had persistent NS. Thirty-eight (63.33%) cases progressed to ESRD by the end of 12 months. Serum creatinine at presentation and IFTA> 50% and presence of fibrous crescent on biopsy was risk factor for achieving ESRD. Conclusions: Crescentic IgAN carries a very poor prognosis, with majority of the cases progressing to ESRD by 12 months. Serum creatinine at presentation and presence of diffuse IFTA and fibrous crescents on biopsy predicts development of ESRD.