Esclerose sistêmica difusa com hipertensão pulmonar isolada: relato de caso

Systemic sclerosis (SS) is an uncommon disease characterized by small blood vessel vasculopathy and increased connective tissue in the skin and in other organs. The pulmonary involvement is common in SS; however, pulmonary hypertension without interstitial fibrosis is rare. The authors present a cas...

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Bibliographic Details
Authors: Zanati, Silméia Garcia [UNESP], Okoshi, Katashi [UNESP], Matsubara, Luiz Shiguero [UNESP], Inoue, Roberto Minoru Tani [UNESP], Okoshi, Marina Politi [UNESP]
Format: article
Status:Published version
Publication Date:2000
Country:Brasil
Institution:Universidade Estadual Paulista (UNESP)
Repository:Repositório Institucional da UNESP
Language:Portuguese
OAI Identifier:oai:repositorio.unesp.br:11449/212677
Online Access:http://dx.doi.org/10.1590/S0102-35862000000600007
http://hdl.handle.net/11449/212677
Access Level:Open access
Keyword:Systemic scleroderma
Nifedipine
Pulmonary hypertension
Vasodilatador agents
Doppler echocardiography
Escleroderma sistêmico
Nifedipina
Ecodopplercardiograma
Hipertensão pulmonar
Vasodilatadores
Ecocardiografia doppler
Description
Summary:Systemic sclerosis (SS) is an uncommon disease characterized by small blood vessel vasculopathy and increased connective tissue in the skin and in other organs. The pulmonary involvement is common in SS; however, pulmonary hypertension without interstitial fibrosis is rare. The authors present a case of the diffuse form of SS with isolated pulmonary hypertension. The diagnosis of pulmonary hypertension was suggested by physical examination and confirmed by doppler-echocardiography which allowed for the estimation of the pulmonary artery systolic pressure in 80 mmHg. The authors started treatment with nifedipine; however, as the patient presented side effects, it was not possible to increase the dose beyond 30 mg/day. In this dosage, nifedipine did not decrease the pulmonary arterial pressure.