Kidney Week

Abstract: FR-PO0485

Menorrhagia Masking Scleroderma Renal Crisis: A Rare Presentation

Session Information

• AKI: Case Reports - TMA, Vasculitis, Immune-Mediated Injury, and Systemic Disease
  October 23, 2026 | Location: Exhibit Hall A, Convention Center
  Abstract Time: 10:00 AM - 12:00 PM

Category: Acute Kidney Injury

• 102 AKI: Clinical, Outcomes, and Trials

Authors

• Alwan, Iktimal, AdventHealth Tampa, Tampa, Florida, United States

Iktimal Alwan, MD

• Alwan, Abdul Hamid Mohamad, Beirut Arab University Faculty of Medicine, Beirut, Beirut Governorate, Lebanon

Abdul Hamid Mohamad Alwan, MD

• Ramsakal, Asha, AdventHealth Tampa, Tampa, Florida, United States

Asha Ramsakal, DO

• Rothschild, Jason, AdventHealth Tampa, Tampa, Florida, United States

Jason Rothschild, MD

Introduction

Scleroderma renal crisis (SRC) is a life-threatening complication of systemic sclerosis characterized by acute renal failure, severe hypertension, and sometimes thrombotic microangiopathy (TMA). SRC may mimic other TMA syndromes, creating both diagnostic and therapeutic challenges. We describe a rare and diagnostically challenging case in which prolonged menorrhagia obscured the evolving presentation of SRC-associated TMA.

Case Description

A 39-year-old woman presented with two months of menorrhagia. She was hypertensive and had acute kidney injury (AKI), metabolic acidosis, anemia, thrombocytopenia, and hyperbilirubinemia. Initially, AKI was attributed to prerenal disease and acute tubular necrosis secondary to prolonged blood loss. However, despite aggressive intravenous fluid resuscitation, renal function worsened, prompting evaluation.
Further workup revealed elevated LDH, low haptoglobin, and schistocytes on peripheral smear, confirming microangiopathic hemolytic anemia. Urine studies showed nephrotic-range proteinuria. Kidney biopsy confirmed severe TMA. ADAMTS13 activity was not suggestive of thrombotic thrombocytopenic purpura, and absence of diarrheal illness made typical hemolytic uremic syndrome (HUS) unlikely. Given clinical suspicion for atypical HUS, eculizumab was initiated.
As hospitalization progressed, the patient reported hand stiffness. Examination demonstrated sclerodactyly and reduced oral aperture. Serologic testing was positive for ANA and RNA polymerase III antibody. In the setting of hypertension, biopsy-proven TMA, and clinical features including pericardial effusion, SRC-associated TMA was favored, and ACE inhibitor therapy was initiated.
Although hypertension improved following ACE inhibitor therapy, renal function rapidly deteriorated, requiring dialysis and continuation of eculizumab. The patient later demonstrated partial renal recovery with reduction of peritoneal dialysis requirements to twice weekly.

Discussion

This case highlights the diagnostic complexity of SRC presenting with menorrhagia, severe TMA. Initial presentation delayed recognition of systemic sclerosis. Early recognition remains critical, as delayed diagnosis may result in irreversible kidney injury and dialysis dependence. Although ACE inhibition remains the cornerstone of therapy, evidence for eculizumab use in SRC-associated TMA is limited to case reports with variable outcomes, and its role remains uncertain.