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Sarcoidosis simulating pulmonary veno-occlusive disease
The American Review of Respiratory Disease
|October 1, 1986
Summary
Pulmonary hypertension in a young woman was caused by sarcoidosis affecting her pulmonary veins. This case highlights sarcoidosis mimicking pulmonary veno-occlusive disease.
Area of Science:
- Pulmonary Medicine
- Pathology
- Rheumatology
Background:
- Pulmonary hypertension (PH) is a severe condition characterized by high blood pressure in the pulmonary arteries.
- Sarcoidosis is a multisystem inflammatory disease characterized by noncaseating granulomas.
Observation:
- A young woman presented with a six-month history of progressive pulmonary hypertension.
- Autopsy revealed noncaseating granulomas obliterating pulmonary veins, with no granulomatous involvement of pulmonary arteries.
Findings:
- Noncaseating granulomas were also identified in the liver and hilar lymph nodes.
- The findings confirmed a diagnosis of sarcoidosis.
- Histopathology demonstrated pulmonary vein obstruction by granulomas.
Implications:
- This case is the first to report pulmonary hypertension caused by sarcoidosis.
- Sarcoidosis can present with pulmonary hypertension, simulating pulmonary veno-occlusive disease (PVOD).
- This presentation underscores the importance of considering sarcoidosis in the differential diagnosis of unexplained pulmonary hypertension.
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