Anti-Gbm Disease One and Half Years Experience in a Tertiary Care Centre

ABSTRACT

BACKGROUND: Anti-glomerular basement membrane (GBM) antibody GN is a rare glomerular disease (0.5-1 per million population) with poor outcome in terms of renal survival. It is caused by auto-antibodies against the non-collagenous domain of the a3 chain of type IV collagen and usually present as a rapidly progressive crescentic GN. Anti-GBM GN may present either as an isolated kidney disease or as a pulmonary-renal syndrome (Goodpasture's syndrome) in 40%-60% of patients. Linear staining of the GBMs for immunoglobulin ( Predominantly IgG & rarely IgA ) in renal biopsy with anti-GBM antibodies in serum is pathognomonic of Anti-GBM disease. Initiating immunosuppression with steroids and cyclophosphamide plus plasmapheresis are the cornerstone of treatment whereas no treatment is recommended if dialysis dependent at presentation 100% crescents or >50% global glomerulosclerosis in an adequate biopsy sample or not having pulmonary hemorrhage. AIM OF THE STUDY To study demographic and clinical profile treatment administered and outcome (in terms of renal survival) in the patients with biopsy-proven anti-GBM disease. METHOD(S) Single-center prospective observational study (January 2021 to June 2022 ) and study population being the admitted patients in Nephrology Department of I.P.G.M.E.R and SSKM hospital Kolkata. RESULT(S) Total 7 patients were diagnosed as having Anti- GBM disease in this time period with median age of 42 yrs ( range from 11 yrs to 68 yrs), and Female Male ratio was 52. Rapidly Progressive Renal Failure with Oliguria (71.4% ) was the most common presentation and 85.7% required Hemodialysis on presentation. 57.1% patients had 100% crescent in renal biopsy. 1 out of 7 patients had overlap with MPO and did not required RRT at presentation. 1 out of 7 patients had associated pulmonary hemorrhage. 28.5% patients received PLEX while others were treated supportively as per protocol. 28.5% patients previously had COVID 19 infection which was diagnosed retrospectively by detecting COVID 19 IgG antibody in serum. 71.4% patients end up in HD requiring renal failure while 1 patient succumbed to death. CONCLUSION(S) Most of the patients aged between 20 yrs to 60 yrs and were female (both 71.4%). Most of the patients (71.4%) received supportive treatment as per protocol. Only 1 patient (with MPO overlap) is dialysis free with discharge creatinine of 3.3 and receiving EUVAS protocol. Most of the patients who presented late with HD requiring renal failure end up in ESRD.