Interstitial lung disease: are we missing formes frustes of connective tissue disease?

P atients with connective tissue disease (CTD) may encompass a variety of respiratory manifestations, including: pleural disease; pulmonary hypertension; bronchial or bronchiolar disease; interstitial pneumonia; restrictive lung disease secondary to musculo-skeletal changes; and iatrogenic drug-induced lung disease. Patients also have an increased risk of opportunistic infections, often favoured by corticosteroids and/or immunosuppressive or immunomodulatory therapy [1, 2]. The respective frequency of manifestations, their clinical and radiological presentations, response to treatment, and outcome are dependent on the underlying CTD.