Pulmonary hypertension in polymyositis

Area of Science:

• Cardiology
• Rheumatology
• Pathology

Background:

• Polymyositis is an idiopathic inflammatory myopathy.
• Pulmonary hypertension (PH) is a known, albeit uncommon, complication of polymyositis.
• Cardiac failure can coexist with polymyositis.

Observation:

• A 69-year-old woman with a 20-month history of polymyositis and cardiac failure presented with severe pulmonary hypertension.
• The patient's severe pulmonary hypertension was noted terminally upon clinic admission.
• She expired within 24 hours of admission.

Findings:

• Autopsy revealed severe plexogenic pulmonary vascular disease.
• This indicates advanced vascular pathology in the pulmonary arteries.
• The findings correlate pulmonary hypertension with polymyositis and cardiac failure.

Implications:

• This case highlights the lethal potential of pulmonary hypertension in polymyositis.
• Early recognition and management of PH in polymyositis patients may be crucial.
• Further research into the pathogenesis of PH in inflammatory myopathies is warranted.