Clinical Presentation and Outcome of c1q Nephropathy - A Single-Centre Prospective Study
Journal Title: INTERNATIONAL JOURNAL OF SCIENTIFIC STUDY - Year 2018, Vol 6, Issue 6
Abstract
Background: C1q nephropathy is a rare glomerular disease with characteristic mesangial c1q deposition noted on immunofluorescence microscopy. It is histologically defined and poorly understood. Light microscopic features are heterogeneous and comprise minimal change disease, focal segmental glomerulosclerosis (FSGS), and proliferative glomerulonephritis (GN). Aim: This study aims to study the clinical presentation, histopathological profile, and outcomes in patients with c1q nephropathy. Methods: A total of 13 patients who satisfied the above criteria were studied. Clinical profile and laboratory parameters including urine analysis, urine spot protein-creatinine ratio, blood biochemistry, serum complement, and histopathological profile were analyzed. Creatinine clearance was estimated using Cockgraut Gault formula. They were followed up for the 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 year with the mean of 34 years. Microscopic hematuria was found in all 13 patients (100%). Nephrotic proteinuria was found in 10 patients (77%), and 4 patients (30.7%) had GFR <60 mL/min. The kidney biopsy revealed diffuse proliferative glomerular nephritis (DPGN) 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 four patients who had renal failure received cyclophosphamide in addition to steroids. Of the nine patients, complete remission was found in 2 patients (22%), partial remission in 2 patients (22%), and no response to immunosuppressive medication was seen in 5 patients (55.5%) (one patient had FSGS and four patients had DPGN). Conclusion: Of the 13 cases with c1q nephropathy, all patients had hypertension and microscopic hematuria. Nephrotic proteinuria was seen in three-fourths of the patients. The most common histopathological presentation was diffuse proliferative GN. Half of the patients showed poor response to oral steroids.
Authors and Affiliations
V Kannan Bhaba, N Gopalakrishnan, C Ilango, P K Senthil Kumar, R P Senthil, Heber Anandan
Keywords
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