Pulmonary Fibrosis In Systemic Sclerosis: From Pathogenesis To A New Diagnostic Alghorithm

Research Article
Angelo De Cata., Michele Inglese., Francesca Molinaro., Marco Bernal., Renato Carignola., Stefano Notarangelo., Salvatore De Cosmoand Marco Sperandeo
DOI: 
xxx-xxxxx-xxxx
Subject: 
science
KeyWords: 
Systemic Sclerosis, Pulmonary Fibrosis, Transthoracic Ultrasonography
Abstract: 

Our experience of thoracic ultrasound (TUS) over three decades tells us that the fervent support of TUS in diagnostics put forward in recent publications is misplaced, potentially engendering misinformation and possibly resulting in misleading recommendations or even poor best-practice guidelines. Although it is tempting to champion diagnostic TUS in patients with fibrosis, misplaced confidence in the accuracy of lung US findings (e.g., “ring down” or B-lines) could lead to misdiagnosis, not to mention legal controversy. The use of thoracic US has been proposed in patients with systemic sclerosis, whose pulmonary function tests do not reliably identify early interstitial lung involvement. Actually, in this as in every interstitial lung disease, high-resolution computed tomography (HRCT) is the golden standard for diagnosis. However, cost and radiation exposure rule out its usual utilization as screening test. Such a high specificity allows to exclude interstitial involvement in patients with systemic sclerosis but without lung disease. The latter characteristic suggests that chest US could be a useful complementary technique in the management of patients with systemic sclerosis, allowing to screen for interstitial lung involvement better than pulmonary function tests. Consequently we believe, that, at least in patients without lung and heart co-morbidities, US could be a valuable tool to select those to refer for HRCT thoracic scan in the follow-up of the disease, with sensible saving in terms of cost and radiation exposure.