School of Medicine and Health Sciences Poster Presentations

Title

C1q Nephropathy with a Positive ANA

Document Type

Poster

Abstract Category

Clinical Specialties

Keywords

C1q Nephropathy, Positive ANA, FSGS, nephroptic syndromew

Publication Date

Spring 5-1-2019

Abstract

Abstract Title C1q Nephropathy with a Positive ANA titer Authors Amira H. Elshikh, DO; Jigar J. Patel, MD; Kevin Fu, MD; Farida Izzi, MD; Scott Cohen, MD Introduction: C1q Nephropathy is a histopathologic diagnosis characterized by dominant immunofluorescent staining for C1q electron dense deposits on renal biopsy. There are a variety of light microscopy appearances of C1q nephropathy. The pathology can appear similar to lupus nephritis. However, patients with C1Q nephropathy lack the clinical and serologic markers of systemic lupus erythematosus (SLE). In our case, a 29-year old woman presented with nephrotic syndrome and was found to have C1q nephropathy on renal biopsy. Our patient had a positive ANA titer, which led to a diagnostic conundrum given her biopsy diagnosis. Case description: A 29 year-woman was found to have proteinuria on routine labs that later progressed over the next few months to nephrotic syndrome with worsening lower extremity edema. Her work up revealed a positive ANA titer of 1:640. However, her anti-dsDNA and serum complement levels were normal, and she did not meet clinical criteria for SLE after extensive evaluation. A renal biopsy revealed pathologic features suggestive of C1q nephropathy, without classic features of lupus nephritis. Despite treatment with prednisone and cyclosporine over the next five months, her disease progressed leading to hospital admission for worsening volume overload. A repeat renal biopsy was performed for diagnostic confirmation. The second renal biopsy showed evolution to focal segmental glomerulosclerosis (FSGS) with C1q nephropathy as well as diffuse foot process effacement of the podocytes in more than 90% of capillary loop surfaces. She continues on cyclosporine and is on a slow Prednisone taper with reports of improved symptomatology on outpatient follow-up, though she continues to have evidence of steroid-resistant nephrotic syndrome. Discussion: C1q nephropathy is a rare immune-complex mediated glomerulopathy, thought to be a variant of FSGS, minimal change disease or mesangioproliferative glomerulonephritis. Current diagnostic criteria include 1) C1q electron dense deposits on immunofluorescence microscopy and 2) lack of clinical or laboratory evidence of SLE. There have been no reports of biopsy proven C1q nephropathy with a positive ANA titer. We treated our patient with prednisone and cyclosporine. Lack of clinical improvement lead to a repeat renal biopsy, which demonstrated C1q deposition with progression to FSGS. This patient will be closely monitored for potential development of SLE in the future.

Open Access

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Presented at Research Days 2019.

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C1q Nephropathy with a Positive ANA

Abstract Title C1q Nephropathy with a Positive ANA titer Authors Amira H. Elshikh, DO; Jigar J. Patel, MD; Kevin Fu, MD; Farida Izzi, MD; Scott Cohen, MD Introduction: C1q Nephropathy is a histopathologic diagnosis characterized by dominant immunofluorescent staining for C1q electron dense deposits on renal biopsy. There are a variety of light microscopy appearances of C1q nephropathy. The pathology can appear similar to lupus nephritis. However, patients with C1Q nephropathy lack the clinical and serologic markers of systemic lupus erythematosus (SLE). In our case, a 29-year old woman presented with nephrotic syndrome and was found to have C1q nephropathy on renal biopsy. Our patient had a positive ANA titer, which led to a diagnostic conundrum given her biopsy diagnosis. Case description: A 29 year-woman was found to have proteinuria on routine labs that later progressed over the next few months to nephrotic syndrome with worsening lower extremity edema. Her work up revealed a positive ANA titer of 1:640. However, her anti-dsDNA and serum complement levels were normal, and she did not meet clinical criteria for SLE after extensive evaluation. A renal biopsy revealed pathologic features suggestive of C1q nephropathy, without classic features of lupus nephritis. Despite treatment with prednisone and cyclosporine over the next five months, her disease progressed leading to hospital admission for worsening volume overload. A repeat renal biopsy was performed for diagnostic confirmation. The second renal biopsy showed evolution to focal segmental glomerulosclerosis (FSGS) with C1q nephropathy as well as diffuse foot process effacement of the podocytes in more than 90% of capillary loop surfaces. She continues on cyclosporine and is on a slow Prednisone taper with reports of improved symptomatology on outpatient follow-up, though she continues to have evidence of steroid-resistant nephrotic syndrome. Discussion: C1q nephropathy is a rare immune-complex mediated glomerulopathy, thought to be a variant of FSGS, minimal change disease or mesangioproliferative glomerulonephritis. Current diagnostic criteria include 1) C1q electron dense deposits on immunofluorescence microscopy and 2) lack of clinical or laboratory evidence of SLE. There have been no reports of biopsy proven C1q nephropathy with a positive ANA titer. We treated our patient with prednisone and cyclosporine. Lack of clinical improvement lead to a repeat renal biopsy, which demonstrated C1q deposition with progression to FSGS. This patient will be closely monitored for potential development of SLE in the future.