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[CREST syndrome presenting as pulmonary hypertension]
D Somme1, M Duterque, M Verdaguer
1Service de Cardiologie, Hôpital Gilles de Corbeil, 59, bd H. Dunant, 91108 Corbeil-Essonnes.
Annales De Cardiologie Et D'Angeiologie
|January 31, 2003
Summary
This case study highlights a rare instance where precapillary pulmonary hypertension (PHT) revealed undiagnosed scleroderma. The patient experienced rapid symptom decline, emphasizing the poor prognosis associated with PHT in scleroderma.
Area of Science:
- Cardiology
- Rheumatology
- Pulmonology
Background:
- Scleroderma is a connective tissue disease characterized by fibrosis and vascular abnormalities.
- Pulmonary hypertension (PHT) is a known complication of systemic sclerosis, but rare in localized scleroderma.
- This case presents PHT as an exceptionally late and revealing manifestation of circumscribed scleroderma.
Observation:
- A 62-year-old patient presented with exertional dyspnea and signs of previously undiagnosed scleroderma.
- Echocardiography confirmed precapillary pulmonary hypertension (PHT) (74/14 mmHg).
- Extensive investigations, including V/Q scan and CT, ruled out pulmonary causes for PHT.
Findings:
- Diagnosis of PHT associated with circumscribed scleroderma was confirmed by hand X-rays, capillaroscopy, esophageal studies, and positive anticentromere antibodies.
- The patient's condition rapidly deteriorated, necessitating treatment with continuous intravenous prostacyclin.
- Anticentromere antibodies were positive, supporting the diagnosis of scleroderma-associated PHT.
Implications:
- This case underscores the potential for precapillary pulmonary hypertension to be a rare, late-onset, and exceptionally revealing sign of circumscribed scleroderma.
- The rapid deterioration and poor prognosis highlight the critical need for early recognition and management of PHT in scleroderma patients.
- Further research into the pathogenesis and optimal treatment strategies for PHT in the context of scleroderma is warranted.