Imaging Lung Disease in Systemic Sclerosis

Abstract

Interstitial lung disease and pulmonary hypertension (PH) are the most common cardiopulmonary findings in patients with systemic sclerosis (SSc). About two thirds of patients suffering from SSc develop scleroderma interstitial lung disease. PH is present in about 20% of SSc patients and is typically associated with severe lung disease, although it may be an isolated manifestation of SSc. High-resolution CT scanning is a key method for evaluating chest involvement. There are four roles of imaging in scleroderma interstitial lung disease: 1) detection of lung involvement, 2) identification of patients likely to respond to treatment, 3) assessment of treatment efficacy, and 4) exclusion of other significant diseases to include PH and cardiac and esophageal abnormalities.

The Pulmonary Fibrosis-Associated MUC5B Promoter Polymorphism Does Not Influence the Development of Interstitial Pneumonia in Systemic Sclerosis

“…Also, SSc-IP is observed more frequently in women and typically occurs in the third to fi fth decades of life, whereas idiopathic pulmonary fi brosis is more common in men, with a typical age of onset after the fi fth decade. 3,[23][24][25][26] Thus, when considering the genetic factors that increase the risk of developing SSc-IP, it is not surprising that SSc-IP appears to be distinct from the idiopathic forms of IP. In fact, based on our fi ndings, we would predict that the immu nologic causes of IP do not involve the MUC5B promoter polymorphism.…”

Thoracic Manifestations of Connective Tissue Diseases

“…It may lead to aspiration pneumonitis or bronchiolitis. 4,26 The heart is also a frequent target of scleroderma, with possible involvement including myocardial fibrosis, arrhythmias, pericardial disease, and heart failure. 26,27 Mediastinal lymphadenopathy is likewise common, occurring in up to 50% of patients.…”

“…4,26 The heart is also a frequent target of scleroderma, with possible involvement including myocardial fibrosis, arrhythmias, pericardial disease, and heart failure. 26,27 Mediastinal lymphadenopathy is likewise common, occurring in up to 50% of patients. 27 Unlike pericardial disease, pleural disease is uncommon and when present is generally accompanied by parenchymal lung disease.…”

Subclinical Interstitial Lung Disease

“…Chest CT scans have been used to identify occult ILD in patients with scleroderma as pulmonary complications are an important cause of morbidity and mortality (54)(55)(56). In a study of scleroderma patients with normal lung volumes, 56% of subjects had HRCT abnormalities suggesting fibrosing alveolitis (55).…”