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Abstract: FR-PO631

A Unique Case of Scleroderma Renal Crisis

Session Information

Category: Glomerular Diseases

  • 1302 Glomerular Diseases: Immunology and Inflammation


  • Coombs, Nickolas, Hartford Hospital, Hartford, Connecticut, United States
  • Anil Kumar, Mythri, Hartford Hospital, Hartford, Connecticut, United States
  • Post, Jarrod B., Hartford Hospital, Hartford, Connecticut, United States

Scleroderma renal crisis (SRC) occurs in 5-20% of patients with diffuse cutaneous systemic sclerosis (SSc). Early initiation of angiotensin converting enzyme inhibitors (ACEi) can promote renal stabilization or recovery. We present a case of SRC in amyopathic dermatomyositis (DM) with overlap syndrome.

Case Description

A 76-year-old female with hypertension (HTN) presented with months of bilateral lower extremity edema, arthralgias, myalgias, and dyspnea. A transthoracic echocardiogram (TTE) was normal and nuclear stress testing revealed two-vessel coronary artery disease. CT chest showed non-specific interstitial pneumonia. Antinuclear antibody (Ab), PL12 Ab and C-Reactive protein were elevated; mycophenolate mofetil (MMF) and methylprednisone were started for DM. She was admitted to the hospital for pulmonary edema secondary to malignant HTN complicated by acute kidney injury (AKI). Her urinalysis, renal ultrasound and TTE were unremarkable. Respiratory status improved with aggressive diuresis and her AKI was thought to be cardiorenal; serum creatinine (SCr) stabilized at 1.8 mg/dl (baseline 0.6). MMF was thought to precipitate pulmonary edema and she was discharged on hydroxychloroquine.

She was readmitted 2 weeks later with similar symptoms and SCr 2.3 mg/dl. Despite diuresis, kidney function declined; right heart catheterization was done and normal. Given a recent diagnosis of DM, accelerated HTN, and worsening AKI this raised suspicion for SRC. Interestingly, sclerodactyly was noted in bilateral fingers as well. Further testing revealed RNA polymerase III Ab positivity; renal biopsy was suggestive of SRC. Hemodialysis (HD) was initiated, and she was discharged on lisinopril, steroid taper, and MMF.


We describe a case of amyopathic DM with overlap of SSc in which the use of steroids and cessation of ACEi precipitated SRC. In 20% of cases, SRC precedes the clinical findings of SSc making diagnosis challenging. As polymyositis and DM commonly overlap with SSc, clinical suspicion for SRC is required when presented with accelerated HTN and AKI. In the absence of skin changes, RNA polymerase Ab positivity should raise suspicion for SRC. Prolonged ACEi in SRC may reconstitute renal function for up to 18 months after onset of SRC; ACEi should continue even in patients on long-term dialysis. However, while ACEi have decreased mortality by >60%, outcomes in SRC remain poor with 42% requiring HD.