Background: Recent studies have shown that quantification of specific histopathologic features found in usual interstitial pneumonia (UIP) are useful in defining a prognosis, suggesting the need of biopsy in all patients. Objectives: This study examines whether UIP-associated mortality is different in patients diagnosed by high-resolution computed tomography (HRCT) features considered definite of UIP and in patients with no definite radiological diagnosis that required histological confirmation of diagnoses. Methods: Forty-five patients were included (30 males, mean age 65.3 ± 10.7 years). Two groups of patients were identified: those with HRCT findings of definite UIP (n = 26) and those whose radiological diagnosis was not definite and required a surgical biopsy to confirm the presence of UIP (n = 19). Forced vital capacity, forced expiratory volume in 1 s and diffusing capacity for carbon monoxide were measured in all patients. All data were obtained from medical records, and the survival status was obtained by telephone or personal interview. All clinical parameters and HRCTs were obtained within 1 month before surgical lung biopsy. Results: Median survival was not different across groups and was similar to that previously reported (35 months). Kaplan-Meier analysis did not show any difference in 5-year survival between both groups. Conclusions: In a clinical context in which the diagnosis of UIP can be obtained as a dynamic process that includes an integrated clinical, radiological and pathologic approach, a reliable diagnosis of UIP can be obtained based on a typical definite HRCT with no risk of including patients with a more benign disease and a more prolonged survival.
Pulmonary involvement is a prominent feature in systemic sclerosis and a significant cause of morbidity and mortality. A restrictive ventilatory defect is typical and static lung volumes are usually reduced in patients with ILDs. The possibility of obstruction of small airways in progressive systemic sclerosis (SSc) has been suggested by widespread bronchiolectasis and peribronchial fibrosis noted at necropsy. A total of 46 patients with a diagnosis of SSc were retrospectively included in this study. Patients were classified according to their smoking status (never smokers, n = 34 and ex or current smokers, n = 12). Patients were also compared on the basis of the presence or not of an obstructive pattern on spirometry. The purpose of this study was to establish if SSc patients who are smokers have a different pattern of pulmonary function involvement. Our hypothesis was that smoking habit was not the only cause of air trapping and that the existence of small airway involvement secondary to SSc itself cannot be excluded.
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