CREST syndrome and pulmonary hypertension: a dark prognosis.

Authors

  • Ana C Carneiro Serviço de Medicina A, Hospital de S. João, Porto.
  • Isabel P Barbosa
  • F Carneiro Chaves

DOI:

https://doi.org/10.20344/amp.1103

Abstract

The CREST syndrome initially described as a limited, more indolent form of diffuse scleroderma, is characterized by calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasias. Subsequent studies showed that visceral abnormalities were not uncommon. Pulmonary abnormalities are frequent especially pulmonary hypertension, this one being a major cause of this syndrome's mortality. The authors present the case of white woman, 61 years old , with dyspnoea, cyanosis and peripheral edema with 12 months of evolution. She was admitted for pulmonary thromboembolism suspicion. After investigation the diagnosis of CREST syndrome was made, associated with severe pulmonary hypertension. The patient was treated with varfarina, nicorandil, nifedipine, furosemide and home oxygen. She died 3 months after. The authors discuss the diagnosis, treatment and follow-up.

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How to Cite

1.
Carneiro AC, Barbosa IP, Chaves FC. CREST syndrome and pulmonary hypertension: a dark prognosis. Acta Med Port [Internet]. 2004 Oct. 31 [cited 2024 Mar. 29];17(5):409-14. Available from: https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/1103

Issue

Section

Arquivo Histórico