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Abstract: FR-PO0683

Collapsing Glomerulopathy with an Unusual Twist

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics

Authors

  • Karrothu, Vishnu Vardhan, Baton Rouge General Medical Center, Baton Rouge, Louisiana, United States
  • Ramalingam, Prabhu, Baton Rouge General Medical Center, Baton Rouge, Louisiana, United States
  • Samuel, Jones, Renal Associates of Baton Rouge LLC, Baton Rouge, Louisiana, United States
Introduction

Collapsing glomerulopathy (CG) is a severe form of podocytopathy, usually with a poor prognosis. We present a case of a 20-year-old female with collapsing glomerulopathy in the setting of newly diagnosed Systemic lupus erythematosus (SLE).

Case Description

A 20-year-old African American female presented with Acute Kidney Injury (creatinine 4.49 mg/dl; baseline 0.9 mg/dl 1 month ago), along with cough, diffuse arthralgia, headache, and 8 lb. unintentional weight loss. Urinalysis showed 4+ protein, Urine total protein 2500 mg/dL, and 3+ blood. Initial differentials include post-infectious glomerulonephritis (PIGN), lupus nephritis, and other glomerulopathies. Serology was negative for HIV, RPR, and viral hepatitis panels but positive for ANA and low C3. Due to worsening renal function, a kidney biopsy was done, showing collapsing glomerulopathy with minimal fibrosis. The patient received intravenous Solumedrol 1 g/kg for 3 days. After a few days, serologies were positive for anti-RNP and anti-Smith antibodies. Final biopsy demonstrated glomerular immune complex deposition with a full-house immunofluorescence pattern, confirming lupus nephritis. Treatment with Hydroxychloroquine and mycophenolate led to significant renal recovery. She was discharged on oral prednisone, mycophenolate, and Hydroxychloroquine with outpatient follow-up. Notably, the Apolipoprotein L1 (APOL1) mutation was negative.

Discussion

Lupus nephritis has six histological subclasses, which don't include CG. CG is an aggressive variant of focal segmental glomerulosclerosis (FSGS), characterized by segmental or global collapse of glomerular capillaries and podocyte proliferation. The coexistence of CG and LN is rare but documented in the literature, usually in patients with an APOL1 gene mutation. CG usually has a poor prognosis, but in cases associated with LN, it shows a variable response to immunosuppressive therapy. Our patient showed significant improvement in renal function after immunotherapy, suggesting a more favorable subset of SLE-associated CG. Given the absence of an APOL1 gene mutation and other classical risk factors, we suspect this may represent an atypical presentation of lupus nephritis. This case illustrates the need for broad differential diagnosis in patients with nephrotic-range proteinuria and lupus-like symptoms.