Pulmonary involvement in long-term mixed connective tissue disease: functional trends and image findings after 10 years.

OBJECTIVES: Interstitial lung disease (ILD) is highly prevalent in patients with mixed connective tissue disease (MCTD). However, little is known about the long-term progression of ILD in MCTD. The aims of this study were to describe pulmonary function test (PFT) and high-resolution computed tomography (HRCT) results in long-term MCTD patients, to measure changes in PFT and HRCT results over a 10-year period, and to ascertain correlations in functional and imaging data. METHODS: In this retrospective cohort study, comparison between baseline and follow-up PFT and HRCT data was performed for 39 unselected consecutive MCTD patients. RESULTS: At baseline, 51% of the patients had abnormal PFTs. Forced vital capacity (FVC) was slightly reduced at baseline (77% of predicted), but remained stable after 10 years. A relative decrease of 15% in the diffusion capacity for carbon monoxide (DLCO) was detected (from 84% to 71% of predicted, p<0.001). The median lower lobes ILD-HRCT score progressed from 7.5% at baseline to 11.2% at follow-up (p=0.02), and findings of traction bronchiolectasis and honeycombing increased (p<0.05). A moderate negative correlation was observed between functional parameters and quantification of image findings. CONCLUSIONS: Functional and radiologic alterations suggestive of ILD in long-term MCTD patients are prevalent, mild, and progressed slightly over time. The most sensitive parameters for detecting subtle progression of ILD in MCTD patients are trends in DLCO, quantification of lower-lobes disease by HRCT (lower-lobes %ILD-HRCT score), and qualitative analysis of HRCT imaging.

Transbronchial biopsy in chronic hypersensitivity pneumonitis.

INTRODUCTION: The diagnosis of chronic hypersensitivity pneumonitis (CHP) is based on relevant exposure, tomographic findings and, in some cases, pathological data. The role of bronchoscopy is uncertain, especially in the fibrotic form of CHP. AIM: To analyze the yield of transbronchial biopsy (TBBx) in patients with CHP according to tomographic findings and to evaluate the importance of bronchoalveolar lavage (BAL) in the diagnostic approach. METHODS: This retrospective study analyzed patients with CHP who underwent TBBx in São Paulo, Brazil. The TBBx findings were classified as characteristic (granulomas and/or multinucleated giant cells, associated or not with peribronchiolar infiltration of lymphocytes and plasma cells and bronchiolocentric distribution) and supportive (data indicative of small airway injury: peribronchiolar metaplasia, organizing pneumonia, and intra-alveolar foamy macrophages). RESULTS: One hundred nine patients were included. The presence of characteristic findings of HP was seen in 16 patients (14.7%), and supportive findings were seen in 32 patients (29.4%), with a total yield of 44%. Pathological characteristic findings were more common in cases without fibrosis on high-resolution computed tomography (HRCT) (27.3% vs. 9.2%, p= 0.014), whereas the presence of supportive findings was more common in fibrotic HP (38.1% vs. 9.1%, p= 0.002). Fifty-two patients had differential cytology in BAL fluid. Lymphocytosis (> 20%) was present in 51.9% of the patients. There was no difference in the median lymphocyte count according to nonfibrotic and fibrotic tomographic findings on HRCT. CONCLUSION: TBBx and BAL fluid cytology have a role in CHP diagnosis.

Squawks in interstitial lung disease prevalence and causes in a cohort of one thousand patients.

Squawks are lung adventitious sounds with a mix of both musical and nonmusical components heard during the inspiratory phase. Small series have described squawks in interstitial lung diseases. Hypersensitivity pneumonitis and other diseases involving small airways can result in squawks, but new interstitial lung diseases (ILDs) involving peripheral airways are being described. A retrospective analysis was performed on 1000 consecutive patients from a database of ILD of a tertiary referral center. Squawks were recorded in 49 cases (4.9%), hypersensitivity pneumonitis (23 cases), connective tissue disease (7), microaspiration (4), pleuroparenchymal fibroelastosis (4), fibrosing cryptogenic organizing pneumonia (, 3), familial ILD (2), sarcoidosis (2), idiopathic pulmonary fibrosis (IPF; 1), bronchiolitis (2), and nonspecific interstitial pneumonia (1). One patient had a final diagnosis of IPF. There was a significant association between mosaic pattern and squawks: 20 cases with squawks (40.8%) had mosaic pattern compared with 140 (14.7%) cases without squawks (x = 23.6, P < .001).Findings indicative of fibrosis were described on high-resolution chest tomography (HRCT) in 715 cases (71.5%). Squawks were more common in patients with findings indicative of fibrosis on HRCT: 45 of 715 (6.3%) compared with 4 of 285 (1.4%) of those without findings indicative of fibrosis (x = 10.46, P = .001).In conclusion, squawks are an uncommon finding on physical examination in patients with ILD, but when present suggest fibrosing ILD associated with bronchiolar involvement. However, squawks are rare in IPF.