Abstract: FR-PO0486
The Quiet Crisis: Normotensive Scleroderma Renal Crisis in Newly Diagnosed Systemic Sclerosis-Myositis Overlap
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
- Lakhatariya, Khushboo, UPMC, Pittsburgh, Pennsylvania, United States
- Kaldas, Hoda, UPMC, Pittsburgh, Pennsylvania, United States
Introduction
Scleroderma renal crisis (SRC) is a life-threatening complication of systemic sclerosis (SSc). Normotensive SRC, occurring without overt hypertension, is particularly dangerous due to delayed recognition and worse renal outcomes. High-dose corticosteroids are a known precipitant, while concurrent angiotensin receptor blocker (ARB) therapy may mask blood pressure elevation. We describe normotensive SRC in a patient with newly diagnosed SSc-myositis overlap syndrome.
Case Description
A 63-year-old woman with newly diagnosed SSc-myositis overlap syndrome presented with progressive weakness, dysphagia, Raynaud phenomenon, and interstitial lung disease. Workup demonstrated inflammatory myopathy with anti-TIF1-γ positivity. She received pulse methylprednisolone, IVIG, rituximab, and prednisone 60 mg daily. Baseline blood pressure was chronically low (90–100s/50–60s mmHg), and valsartan was initiated for newly reduced ejection fraction (45%).At rheumatology follow-up, blood pressure was 133/81 mmHg and creatinine had risen from 1.0 to 2.6 mg/dL. Although normotensive by standard criteria, the relative BP increase from baseline raised concern for evolving normotensive SRC. Captopril was initiated outpatient, but worsening renal function prompted admission.On hospitalization, creatinine peaked at 3.9 mg/dL with persistent “normal” systolic pressures in the 120s. Urinalysis was bland. Valsartan was discontinued, captopril aggressively titrated, and prednisone rapidly tapered. Despite BP normalization, renal recovery lagged, prompting renal biopsy. Pathology confirmed SRC with superimposed acute tubular injury, showing endothelial injury, fibrointimal hyperplasia, luminal narrowing, and ischemic glomerular changes without immune deposits.The patient was transitioned to lisinopril and nifedipine ER for discharge. Creatinine stabilized near 3.0 mg/dL with close outpatient follow-up planned.
Discussion
This case highlights the diagnostic difficulty of normotensive SRC in patients with chronically low baseline blood pressure. Relative BP elevation rather than population-defined hypertension thresholds may be the only clue to evolving SRC. High-dose steroids likely precipitated disease, while ARB therapy may have obscured recognition. Renal biopsy proved diagnostically valuable by identifying concurrent acute tubular injury explaining delayed renal recovery.