C1q Nephropathy - A profile

AbstractBackground C1q nephropathy is an under diagnosed disease with varied clinical presentation. Diagnostic criteria of C1q nephropathy 1. Dominant or codominat immunoflurescent staining for c1q in kidney biopsy2. Mesangial electron dense deposits 3. No clinical and serological evidence of SLE.Materials and methods 13 patients who satisfied the above criteria were studied. Clinical profile and laboratory parameters including urine analysis, urine spot PCR, blood biochemistry, serum complement and histopathological profile were analyzed. Creatinine clearance was estimated using Cockgraut Gault formula. They were followed followed up for assessment of response to treatment.Results Among the 13 patients 12 were female (92.3). All (100) were hypertensive at the time of presentation. Age ranged from 15 to 48 yrs with mean of 34 yrs. Microscopic haematuria was found in all 13 patients ( (100) Nephrotic proteinuria was found 10 patients (77) and 4 patients (30.7) had GFR60mlmin. Kidney biopsy revealed Diffuse Proliferative Glomerular Nephrits in 12 patients (92.3) one patient had FSGS (7.7). Cellular crescents were found in 2 patients (15.3). One patient was lost for follow-up. 3 patients (25) improved with ACE inhibitors and statins. 9 patients (69.2) were started on steroids of which the 4 patients who had renal failure received cyclophosphamide in addition to steroids. Of the 9 patients, complete remission was found in 2 patients (22) , partial remission in 2 patient(22) and no response to immunosuppressive mediation was found in 5 patients(55.5) ( 1 patient had FSGS and 4 patients had DPGN)Conclusion among the 13 cases with c1q nephropathy1.All patients had hypertension and microscopic hamaturia.2.Nephrotic proteinuria was seen in three fourth of the patients3.Commonest histopathological presentation was diffuse proliferative Glomerulonephrits.4.Half of the patients showed poor response to oral steroids