Comprometimento do interstício pulmonar em portadores de esclerose sistêmica progressiva: estudo de uma série de 58 casos Interstitial lung disease in patients with progressive systemic sclerosis: a study of 58 cases

OBJETIVO: Estimar a freqüência de doença intersticial pulmonar em um grupo de indivíduos com esclerose sistêmica progressiva e descrever suas características clínicas, funcionais e radiológicas. MÉTODOS: Após confirmação diagnóstica, 58 pacientes com esclerose sistêmica progressiva foram estudados com tomografia computadorizada de alta resolução, provas de função pulmonar, bem como pesquisa do anti-Scl 70. Foram feitas comparações entre os pacientes com e sem comprometimento intersticial pulmonar e pesquisados possíveis fatores preditivos deste acometimento através de análise multivariada. RESULTADOS: Do total, 51,7% apresentaram evidências de doença intersticial pulmonar na tomografia computadorizada de alta resolução. Dispnéia e tosse foram os sintomas mais relatados, (65,5% e 39,7%, respectivamente). Bronquiolectasias e faveolamento foram as anormalidades tomográficas mais comuns (83,3% e 80,0% respectivamente). Quando comparados com os indivíduos sem doença intersticial pulmonar, os pacientes com este acometimento apresentaram freqüência semelhante de sintomas pulmonares e extrapulmonares, porém apresentaram esclerose sistêmica progressiva de maior duração, estertores crepitantes mais freqüentes, maior positividade de anti-Scl 70, e capacidade vital forçada e pulmonar total reduzidas. Somente uma capacidade vital forçada < 80% apresentou tendência a predizer presença de doença intersticial pulmonar. CONCLUSÃO: Doença intersticial pulmonar foi freqüente neste grupo com esclerose sistêmica progressiva. Não ocorreu associação com sintomas. Houve associação entre doença intersticial pulmonar e presença de estertores crepitantes e anti-Scl 70. Contudo, somente redução da capacidade vital forçada foi preditora de doença intersticial pulmonar.OBJECTIVE: To estimate the frequency of interstitial lung disease in a group of patients with progressive systemic sclerosis, and to describe the clinical, functional and radiological characteristics of the patients studied. METHODS: Fifty-eight patients diagnosed with progressive systemic sclerosis were submitted to high-resolution computed tomography of the chest, pulmonary function tests and a blood test for anti-Scl 70 antibodies. Comparisons were drawn between patients with interstitial lung disease and those without. Logistic regression with multivariate analysis was used to identify factors predictive of interstitial lung disease. RESULTS: Of the 58 patients evaluated, 51.7% presented interstitial lung disease on high-resolution computerized tomography scans. Dyspnea and cough were the most common symptoms (seen in 65.5% and 39.7%, respectively). Bronchiolectasis and honeycombing were the most common tomographic abnormalities (observed in 83.3% and 80%, respectively). When compared to individuals without interstitial lung disease, patients with the condition had a comparable frequency of pulmonary and extrapulmonary symptoms but presented progressive systemic sclerosis of longer duration, a higher frequency of crackling rales, higher rates of anti-Scl 70 positivity, lower vital capacity and reduced total lung capacity. Only forced vital capacity < 80% was found to be a predictor of interstitial lung disease. CONCLUSION: Interstitial lung disease was common in this group of patients with progressive systemic sclerosis. No correlation with symptoms was found, although interstitial lung disease was found to correlate with crackling rales and with anti-Scl 70 positivity. Nevertheless, only reduced forced vital capacity was found to be predictive of interstitial lung disease.