C1q nephropathy first described in 1985 is a rare cause of glomerular diseases, especially in adults, but still not much is known about and lacks specific guidelines. The diagnosis is based on mesangial C1q deposition either dominant or codominant pattern in the absence of systemic lupus erythematosus (SLE). Here, we report a 28-year-old female who presented with anasarca, frank nephrotic syndrome, and hypertension. Also, antinuclear antibodies (ANA) was negative and renal biopsy revealed focal segmental glomerulosclerosis (FSGS) morphological pattern along with mesangial C1q predominant staining with primary podocytopathy and mesangial electron-dense immune deposits on electron microscopy. The diagnosis of C1q Nephropathy was made and oral steroids were started. The minimal change disease (MCD) pattern has better outcomes than FSGS. Focal segmental glomerulosclerosis might respond poorly or gets dependent on oral steroids and often requires second-line immunosuppressive therapy.
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