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Purpose: This study sought to evaluate, by means of a retrospective analysis, the relationship between pulmonary hypertension (PH) and fibrotic interstitial lung disease (ILD) in a population of 43 patients affected by systemic sclerosis. In addition, we assessed the role of highresolution computed tomography (HRCT) in the diagnostic and therapeutic pathway for such patients. Materials and methods: Forty-three patients affected by progressive systemic sclerosis (PSS) and PH underwent functional, haemodynamic and HRCT evaluations between November 2001 and May 2011. Functional evaluation was performed through respiratory function testing and spirometry. Haemodynamic assessment was done with transthoracic echocardiogram (ECG) and right heart catheterisation. Semiquantitative evaluation of radiological involvement was performed using different measures: visual score, Goh's score, Wells' score and Warrick's score. Results: Of 43 patients, 16 showed fibrotic ILD after a HRCT evaluation. The main pattern of ILD was identified with nonspecific interstitial pneumonia (NSIP). Our study also evaluated the relationship among radiological, functional and haemodynamic indexes; different correlations were found to be significant, in particular, Wells' and visual scores ≥40% correlated significantly with diffusion capacity for carbon monoxide (DLCO); Wells' score also correlated significantly with percentage of predicted forced vital capacity (FVC), percentage of predicted forced expiratory volume in 1 s (FEV1) and composite physiologic index (CPI). If presence of oesophagopathy evaluated with HRCT was included, this sign correlated significantly both with DLCO (p=0.03) and mean pulmonary arterial pressure (mPAP) (p=0.03), considering total population. Conclusions: Our findings confirm the diagnostic role of HRCT in evaluating the extent of lung damage in systemic sclerosis. Furthermore, a significant correlation between mPAP and esophagopathy suggests a possible positive predictive value of this sign in identifying a subphenotypic category of patients affected by systemic sclerosis in whom we can find a more severe visceral impairment and a more frequent vascular involvement

Although honeycombing is one of the key features for the diagnosis of idiopathic pulmonary fibrosis (IPF), its origin and evolution are still poorly understood. The aim of our study was to analyse the natural history of honeycombing in patients treated with single-lung transplantation. We considered seven patients who underwent single-lung transplantation; two of them (28.6%) were excluded from our analysis because they died in the posttransplantation period, whereas the remaining five (71.4%) were evaluated with computed tomography (CT) over 67.6±38.56 months. Each CT scan was assessed for disease extension and cyst size (visual score and size of target cysts); CT scans acquired after 2006 were also assessed for native lung volume. All patients showed disease progression (with a concurrent reduction in lung volume in two, 40%) and a progression of honeycombing, with increased number and size of cysts in four (80%). We observed dimensional changes in all target cysts (enlargement or reduction); three patients (60%) also had radiological evidence of complications, such as spontaneous rupture with pneumothorax and development of mycetomas within the cysts. Honeycombing is a dynamic process in which the overall trend is represented by a dimensional increase in cystic pattern; however, single cysts may have a different evolution (enlargement, reduction or complications). This behaviour could be explained by the variety of the pathogenetic processes underlying honeycombing, with cysts that may present abnormal communication with the airway, including the development of a check-valve mechanism.