Severe lung fibrosis is common in MCTD, has an impact on pulmonary function and overall physical capacity and is associated with increased mortality.

Lung disease extent and progression in MCTD are modest. Yet, the extension continues several years after MCTD diagnosis causing lung function decline and increasing the risk of mortality. The study identified male gender, elevated anti-RNP titre, presence of anti-ro52 antibodies and absence of arthritis as the strongest predictors of ILD progression.

Patients with common variable immunodeficiency (CVID) have impaired production of immunoglobulins and hence recurrent airway infections, which in turn may lead to radiological changes and impaired lung function. Uncertainty exists about the nature and frequency of the radiological and the physiological abnormalities, and how they relate to each other. We reassessed high resolution computed tomography (HRCT) images in 65 patients, reported results from previously measured lung function tests, and studied relations between radiology, function and clinical variables. Airway obstruction, ventilatory restriction and impaired gas diffusion was found in 40, 34 and 21% of the patients, respectively. HRCT abnormalities were present in 94% of the subjects, mild changes being the most common. Bronchial wall thickening, found in two thirds of the patients, was related to airway obstruction and impaired gas diffusion. Linear and/or irregular opacities, the most frequent interstitial abnormality, was related to impaired gas diffusion. Bronchiectasis was found in more than half, but only severe bronchiectasis was related to airway obstruction. Since bronchial wall thickening and linear and/or irregular opacities are both frequent and important determinants of impaired pulmonary function, more attention should be given to these features in the follow up of CVID patients.

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To our knowledge, this study is the first to demonstrate a highly significant difference in PFT between patients with antisynthetase syndrome with 6 years of followup and healthy controls. DLCO displayed the highest difference with mean 53% lower value in the patients. FVC and DLCO correlated significantly with ILD extent, indicating these variables as appropriate outcome measures in antisynthetase syndrome-associated ILD.