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Pre-Capillary Pulmonary Hypertension in a Patient With Idiopathic Inflammatory Myopathy Without Extensive Pulmonary
Steve O Nwokeocha1, Liana Abduova1, Shalini Thiruvarudchelvam1
1Department of Internal Medicine, Bassett Medical Center, Cooperstown, NY, USA.
The American Journal of Case Reports
|July 26, 2026
Summary
Pulmonary hypertension (PH) in dermatomyositis, even without extensive interstitial lung disease (ILD), may be under-recognized. Early idiopathic inflammatory myopathy (IIM) evaluation is crucial for diagnosing PH in rheumatological work-ups.
Area of Science:
- Rheumatology
- Cardiology
- Pulmonology
Background:
- Dermatomyositis, an idiopathic inflammatory myopathy (IIM), commonly involves skin, muscles, and systemic organs.
- Cardiopulmonary complications, often linked to interstitial lung disease (ILD), can lead to pulmonary hypertension (PH) and heart failure.
- PH in dermatomyositis without significant ILD is rare and often overlooked.
Purpose of the Study:
- To highlight the under-recognition of PH in dermatomyositis cases lacking extensive ILD.
- To emphasize the importance of considering IIM in the differential diagnosis of PH.
Main Methods:
- Case report of a 71-year-old woman with dermatomyositis and pre-capillary PH.
- Diagnostic evaluation included right-heart catheterization and imaging to assess for ILD.
- Treatment involved diuretics, corticosteroids, and intravenous immunoglobulins (IVIG).
Main Results:
- The patient was diagnosed with dermatomyositis and pre-capillary PH despite no extensive ILD findings.
- Initial treatment led to clinical improvement.
- The patient unfortunately passed away due to unrelated complications.
Conclusions:
- Pulmonary hypertension in dermatomyositis may occur without extensive ILD and is likely under-diagnosed.
- Idiopathic inflammatory myopathy (IIM) evaluation is recommended for patients with unexplained PH.
- Further research is needed on the pathophysiology, management, and outcomes of PH in IIMs.
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