To assess progressive pulmonary involvement in patient with scleroderma. And to establish its possible sequence and its predictive value on patient’s prognosisMETHOD: Retrospective review of 33 patients with established diagnosis of scleroderma. Their PFTs, CT chest, assessment of pulomonary pressure with ultrasonography and abgs were reviewed.
Total 33 patients were reviewed, 27 female and 6 male. All patients had radiological evidence of pulmonary fibrosis, pfts were of restrictive pattern in 24 hile 9 patients had normal pfts, dlco was done on 29 patients, 22 patients have decreased dlco, while 7 patients had normal dlco. Abgs were done on 11 patients, 5 had hypoxemia, while 6 had normal blood gases po2 decreased 92%). 32 out of 33 had pulmonary htn. All patients had pulmonary involvement on ct chest. 27% had normal pfts, 22% patients had normal dlco. Around 54% of patient had normal oxygenation (no hypoxemia), while 97% of patients have pulmonary htn.
All patients had pulmonary involvement on ct chest, 27% had normal pfts, 22% patients had normal dlco. Around 54% of patient had normal oxygenation (no hypoxemia), while 97% of patients had pulmonary htn. Phtn was the most common pulmonary manifestation, it was independent of hypoxemia or pulmonary fibrosis and 2% of patients with normal dlco and was present in almost all our patients with or without other asociated pulmonary abnormalities thus suggesting patients had pulmonary htn without significant involvement of pulmonary parenchyma or interstitium.Although we could not established the definite sequence of pulmonary involvement in scleroderma, since most of our patients have advanced on initial presentation. But based on the above data, phtn is most common manifestation of scleroderma and may be the earliest pulmonary involvement and may predict the development of future progressive pulmonary fibrosis.
Further studies are needed to address if early aggressive therapy with pulmonary htn in scleroderma may prevent future progressive pulmonary fibrosis and improved long-term patient’s prognosis.
M. Khalid, None.