INTERSTITIAL PULMONARY FIBROSIS ASSOCIATED CONNECTIVE TISSUE DISEASES – CLINICAL AND IMAGING ASPECTS

Abstract

The lung is a common site of complications in systemic connective tissue diseases (CTD), and its involvement can present in several ways. Although it is generally thought that interstitial lung disease develops later on in CTD, it is often the initial presentation (“lung dominant” CTD). Interstitial lung disease (ILD) can be present in most types of CTD, including rheumatoid arthritis, systemic sclerosis, systemic lupus erythematosus, polymyositis or dermatomyositis, Sjögren’s syndrome and mixed connective tissue disease. Despite similarities in clinical and pathologic presentation, the prognosis and treatment of CTD associated ILD (CTD-ILD) can differ greatly from that of other forms of ILD. Interstitial lung disease must be detected early in the course of collagen disorders by performing high-resolution computed tomography and pulmonary function tests. The pattern described on HRCT is predictive for treatment response and disease progression. Immunosuppression is the mainstay of treatment for ILD, although data from randomized controlled trials (RCTs) to support specific treatments are lacking. The management of patients with CTD-associated ILD is optimized by multidisciplinary collaboration.

Keywords

• interstitial lung disease (ild)
• connective tissue diseases (ctd)
• high-resolution computed tomography (hrct)
• pulmonary function tests