Abstract: TH-PO0060
The Clot That Spoke First: Thrombotic Microangiopathy as an Initial Manifestation of Scleroderma Renal Crisis
Session Information
- AKI: Pathogenesis and Disease Mechanisms
November 06, 2025 | Location: Exhibit Hall, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Acute Kidney Injury
- 102 AKI: Clinical, Outcomes, and Trials
Authors
- Khalid, Mahnoor, Texas Health Presbyterian Hospital Plano, Plano, Texas, United States
- Ayoubi, Nawead, Texas Health Presbyterian Hospital Plano, Plano, Texas, United States
- Racheruvu, Mamatha, Texas Health Presbyterian Hospital Plano, Plano, Texas, United States
Introduction
Thrombotic microangiopathy (TMA) is a rare condition marked by microvascular thrombosis and organ ischemia. While typically associated with hemolytic anemia and thrombocytopenia, renal-limited TMA also occurs. We report a case of a healthy young woman initially diagnosed with TMA due to malignant hypertension, later confirmed as scleroderma renal crisis—a rare initial presentation of scleroderma.
Case Description
A 33-year-old woman with recently diagnosed hypertension following preeclampsia in last pregnancy presented with chest pain, facial swelling, and elevated blood pressure for two days. Initial evaluation revealed a systolic BP >200 mmHg and nonspecific EKG changes.
She was on valsartan-hydrochlorothiazide but was off medications for two months after relocating and not yet establishing primary care. Physical exam was notable for a petechial rash. Laboratory tests showed WBC 11 × 10 /L, hemoglobin 10 g/dL, platelets 130 × 10 /µL, creatinine 6.58 mg/dL. Urinalysis was negative for hematuria and proteinuria. Echocardiogram showed normal ejection fraction; renal ultrasound was unremarkable.
Despite blood pressure control, renal function worsened (Cr >7 mg/dL), necessitating hemodialysis.ANA returned positive with a nucleolar pattern; other autoimmune markers including dsDNA, ANCA, anti-GBM, anti-CCP, and RA factor were negative. Given the ANA pattern, anti-Sm/RNP antibodies were checked and returned positive. Skin biopsy of a dorsal rash revealed diminished CD34, consistent with localized scleroderma. Renal biopsy demonstrated acute-on-chronic thrombotic microangiopathy with 60% interstitial fibrosis and tubular atrophy.
The constellation of findings led to a diagnosis of scleroderma renal crisis presenting as thrombotic microangiopathy. The patient was started on urgent Eculizumab therapy. She responded well, with improved renal function, allowing discontinuation of dialysis.
Discussion
TMAs are defined by microangiopathic hemolytic anemia, thrombocytopenia, and microvascular thrombosis due to endothelial injury. SRC presenting as TMA is a life-threatening complication of systemic sclerosis, with ~20% six-month mortality. Complement activation plays a key role in its pathogenesis. Eculizumab, a C5 inhibitor, blocks early complement activation, serving as a rescue in such cases; while long-term management relies on ACE inhibitor therapy.