SHM Converge Scientific Abstract ePoster Gallery-Puffy Fingers Revealing a Rare Case of Scleroderma Renal Crisis

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This case describes a 62-year-old woman who presented with two weeks of worsening exertional dyspnea, orthopnea, leg edema, headaches, puffy hands, diffuse joint pain, and skin thickening with reduced joint mobility. On exam she was hypertensive, had rales, bilateral edema, and sclerodactyly. Initial studies showed severe heart and kidney involvement, including a markedly elevated BNP, acute kidney injury, pulmonary edema with pleural effusions, and a moderate pericardial effusion.<br /><br />She was initially treated with high-dose steroids for presumed inflammatory arthritis. After sclerodactyly was recognized, autoimmune testing was performed and revealed a high-titer positive ANA and strongly positive anti-RNA polymerase III antibody, with negative anti-Scl-70 and other autoimmune markers. Renal biopsy showed changes consistent with thrombotic microangiopathy and scleroderma renal crisis versus injury from persistent hypertension. Steroids were stopped due to concern that they may have worsened the renal crisis. Despite aggressive diuresis, she developed acute renal failure requiring dialysis. Captopril was started only later, before discharge, because diagnosis was delayed.<br /><br />The discussion emphasizes that the patient’s presentation was most consistent with diffuse cutaneous systemic sclerosis complicated by scleroderma renal crisis. Anti-RNA polymerase III antibodies are associated with diffuse skin disease and a higher risk of renal crisis. Early recognition is critical because scleroderma renal crisis requires prompt blood pressure control and early ACE inhibitor therapy, especially captopril, to preserve renal function. High-dose corticosteroids should generally be avoided in early diffuse systemic sclerosis because they can increase the risk of renal crisis. This case highlights the importance of recognizing classic findings such as sclerodactyly to avoid diagnostic delay and improve outcomes.

Asset Subtitle

Sonia K. Gentile

Meta Tag

Author List Cheryl Peavler, Sonia K. Gentile

Category Clinical Vignettes

Concept Diffuse Systemic Sclerosis

Concept Scleroderma Renal Crisis

Concept Puffy Hands

Concept Sclerodactyly

Concept Skin Thickening

Distinguished Non-Finalist

Presenter Organization Spectrum Health, Michigan State University

Presenting Author Sonia K. Gentile

Track Adult

Keywords

systemic sclerosis

scleroderma renal crisis

anti-RNA polymerase III

sclerodactyly

acute kidney injury

hypertension

corticosteroids

ACE inhibitor

thrombotic microangiopathy

diffuse cutaneous sclerosis

Diffuse Systemic Sclerosis

Scleroderma Renal Crisis

Puffy Hands

Sclerodactyly

Skin Thickening