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[Sarcoidosis simulating a pulmonary veno-occlusive disease]
F Portier1, G Lerebours-Pigeonniere, L Thiberville
1Service de Pneumologie, Hôpital de Bois-Guillaume.
Revue Des Maladies Respiratoires
|January 1, 1991
Summary
Sarcoidosis can cause pulmonary hypertension and respiratory failure by obstructing pulmonary veins, even without lung scarring. This rare veno-occlusive form led to fatal right heart failure in a 43-year-old man.
Area of Science:
- Pulmonary Medicine
- Cardiology
- Pathology
Background:
- Sarcoidosis is a multisystem inflammatory disease characterized by non-caseating granulomas.
- Pulmonary involvement is common, often presenting with interstitial lung disease.
- Rarely, sarcoidosis can affect pulmonary vasculature, leading to pulmonary hypertension.
Observation:
- A 43-year-old male presented with sarcoidosis causing pulmonary hypertension and respiratory failure.
- Radiological imaging showed no evidence of interstitial fibrosis.
- Despite corticosteroid treatment, the patient experienced progressive respiratory and right heart failure.
Findings:
- Autopsy revealed obliterated pulmonary veins due to non-caseating granulomas.
- Minimal fibrotic changes were noted in the lung parenchyma.
- This case represents a rare instance of sarcoidosis-induced pulmonary veno-occlusive disease.
Implications:
- Highlights a rare but severe manifestation of sarcoidosis affecting pulmonary veins.
- Underscores the importance of considering vascular involvement in sarcoidosis-related pulmonary hypertension.
- Suggests potential for veno-occlusive disease in sarcoidosis, necessitating further investigation and tailored treatment strategies.