Interstitial lung disease (ILD) may occur in the
connective tissue diseases, with a negative impact on survival. The diagnosis of ILD is established by
crackles of the lung bases at auscultation, and high resolution computed tomography of the chest demonstrating diffuse opacities predominating in the bases (ground glass opacities, reticular opacities, honeycombing, and
traction bronchiectases). Bronchoalveolar lavage mostly contributes to the differential diagnosis. Video-assisted thorascopic lung biospy is seldom required for clinical management. Clinically significant ILD occurs in 25% of patients with
systemic sclerosis, 7-30% in patients with
dermatopolymyositis (especially with antisynthetase
antibodies including anti-Jo-1
antibodies, often with a subacute onset), and 5% of patients with
rheumatoid arthritis. Nonspecific
interstitial pneumonia (with
fibrosis) is the predominating histopathological pattern in
systemic sclerosis and
dermatopolymyositis; a pattern of
usual interstitial pneumonia is frequent in
rheumatoid arthritis (with a clinical and radiological presentation similar to that of
idiopathic pulmonary fibrosis). ILD of various presentations may occur in Sjögren syndrome, possibly associated with thin-walled
cysts; pulmonary
lymphoma must be ruled out. Little information is available regarding treatment of ILD in
connective tissue disease. Clinically modest short-term efficacy of
cyclophosphamide treatment has been shown in
systemic sclerosis, but was not maintained at 2 years.