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Diffuse Cutaneous Systemic Sclerosis With Normotensive Scleroderma Renal Crisis and Myopericarditis: A Case Report
Luís Augusto Barbosa Franco Zörrer1, Lucas Yugi de Souza Terui1, Rodrigo Fanini Balena1
1Internal Medicine, Clinical Hospital Complex of the Federal University of Paraná, Curitiba, BRA.
Cureus
|March 14, 2025
Summary
Systemic sclerosis (SSc) can cause scleroderma renal crisis (SRC), a serious kidney complication. Normotensive SRC, particularly with heart issues like myopericarditis, presents diagnostic and management challenges, worsening patient prognosis.
Area of Science:
- Rheumatology
- Nephrology
- Cardiology
Background:
- Systemic sclerosis (SSc) is a rare autoimmune disease causing multi-organ fibrosis.
- Scleroderma renal crisis (SRC) is a severe complication affecting the kidneys.
- SRC can be hypertensive or normotensive, with normotensive SRC posing diagnostic difficulties.
Observation:
- Normotensive SRC often presents without hypertension, complicating early diagnosis.
- Cardiac complications, such as myopericarditis, worsen the prognosis of normotensive SRC.
- Hypotension in normotensive SRC complicates treatment, including ACE inhibitor use.
Findings:
- This case report details a patient with diffuse cutaneous SSc (dcSSc) experiencing normotensive SRC.
- The patient presented with myopericarditis, leading to acute renal and heart failure.
- The atypical presentation of normotensive SRC without hypertension highlights diagnostic challenges.
Implications:
- Early recognition of normotensive SRC, especially with cardiac involvement, is crucial for timely intervention.
- Identifying risk factors and optimizing management strategies for normotensive SRC are essential.
- Improved understanding and treatment of normotensive SRC can enhance patient outcomes and reduce renal failure risk.
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