Comparative analysis of density histograms and visual scores in incremental and volumetric high-resolution computed tomography of the chest in idiopathic pulmonary fibrosis patients.

BACKGROUND: Volumetric high-resolution computed tomography (HRCT) of the chest has recently replaced incremental CT in the diagnostic workup of idiopathic pulmonary fibrosis (IPF). Concomitantly, visual and quantitative scores have been proposed for disease extent assessment to ameliorate disease management. PURPOSE: To compare the performance of density histograms (mean lung attenuation, skewness, and kurtosis) and visual scores, along with lung function correlations, in IPF patients submitted to incremental or volumetric thorax HRCT. MATERIAL AND METHODS: Clinical data and CT scans of 89 newly diagnosed and therapy-naive IPF patients were retrospectively evaluated. RESULTS: Forty-six incremental and 43 volumetric CT scans were reviewed. No differences of density histograms and visual scores estimates were found by comparing two HRCT techniques, with an optimal inter-operator agreement (concordance correlation coefficient >0.90 in all instances). Single-breath diffusing lung capacity for carbon monoxide (DLCOsb) was inversely related with the Best score (r = -00.416; p = 0.014), the Kazerooni fibrosis extent (r = -0.481; p = 0.004) and the mean lung attenuation (r = -0.382; p = 0.026), while a positive correlation was observed with skewness (r = 0.583; p = 0.001) and kurtosis (r = 0.543; p = 0.001) in the incremental HRCT sub-group. Similarly, in the volumetric CT sub-cohort, DLCOsb was significantly associated with skewness (r = 0.581; p = 0.007) and kurtosis (r = 0.549; p = 0.018). Correlations with visual scores were not confirmed. Forced vital capacity significantly related to all density indices independently on HRCT technique. CONCLUSIONS: Density histograms and visual scores similarly perform in incremental and volumetric HRCT. Density quantification displays an optimal reproducibility and proves to be superior to visual scoring as more strongly correlated with lung function.

First Description of the Hyperpnea-Hypopnea Periodic Breathing in Patients with Interstitial Lung Disease-Obstructive Sleep Apnea: Treatment Implications in a Real-Life Setting.

There is evidence that hypopneas are more common than apneas in obstructive sleep apnea (OSA) related to idiopathic pulmonary fibrosis (IPF). We investigated the frequency distribution of hypopneas in 100 patients with interstitial lung diseases (ILDs) (mean age 69 yrs ± 7.8; 70% males), including 54 IPF cases, screened for OSA by home sleep testing. Fifty age- and sex-matched pure OSA patients were included as controls. In ILD-OSA patients the sleep breathing pattern was characterized by a high prevalence of hypopneas that were preceded by hyperpnea events configuring a sort of periodic pattern. This finding, we arbitrarily defined hyperpnea-hypopnea periodic breathing (HHPB), was likely reflecting a central event and was completely absent in control OSA. Also, the HHPB was highly responsive to oxygen but not to the continuous positive pressure support. Thirty-three ILD-OSA patients (42%) with a HHPB associated with a hypopnea/apnea ratio ≥3 had the best response to oxygen with a median residual AHI of 2.6 (1.8-5.6) vs. 28.3 (20.7-37.8) at baseline (p < 0.0001). ILD-OSA patients with these characteristics were similarly distributed in IPF (54.5%) and no-IPF cases (45.5%), the most of them being affected by moderate-severe OSA (p = 0.027). Future studies addressing the pathogenesis and therapy management of the HHPB should be encouraged in ILD-OSA patients.

Obstructive sleep apnoea and right ventricular function: A combined assessment by speckle tracking and three-dimensional echocardiography.

BACKGROUND: Little is known on right ventricular (RV) involvement in obstructive sleep apnoea (OSA). This study aimed at evaluating early RV dysfunction by standard and advanced echocardiography in OSA. METHODS: Fifty-nine OSA patients without heart failure and 29 age-matched controls underwent standard, speckle tracking and real time 3D echocardiography of right ventricle. OSA patients performed lung function tests and overnight cardio-respiratory monitoring with evaluation of apnea-hypopnea index (AHI). RESULTS: OSA had significantly higher body mass index and systolic blood pressure (BP) than controls. RV diameters and systolic pulmonary arterial pressure (sPAP) were significantly higher in OSA, in presence of comparable tricuspid annular plane systolic excursion (TAPSE). OSA showed marginally lower RV global longitudinal strain (GLS) (p<0.05) and RV lateral wall strain (RV LLS) (p=0.04). Three-dimensional RV ejection fraction did not differ between the two groups. By stratifying patients according to sPAP, 18 OSA patients with sPAP≥30mmHg had lower TAPSE (p<0.05), RV GLS and RV LLS (both p<0.001) than 37 patients with normal sPAP. By separate multivariate analyses, RV GLS and RV LLS were independently associated with sPAP (both p<0.0001), AHI (p=0.035 and p=0.015 respectively) and BMI (p<0.05 and p=0.034) but not with age and systolic BP in OSA. CONCLUSIONS: A subclinical RV dysfunction is detectable by speckle tracking in OSA. The impairment of RV GLS and RV LLS is more prominent than that of TAPSE and is evident when RVEF is still normal. GLS is independently associated with sPAP and OSA severity.

Sarcoidosis with prevalent and severe joint localization: a case report.

BACKGROUND: Sarcoidosis is a systemic granulomatous disease of unknown origin, characterized by the formation of granulomas without central necrosis. Each organ and tissue can be affected by the disease, but in most cases mainly the lungs and mediastinal lymph nodes but also skin, heart, eyes and joints are involved, the latter are mainly the metacarpophalangeal joints and bone lesions are often associated with involvement of the overlying skin. The diagnosis is often of exclusion, based on clinical and radiological suspicion, and should be confirmed by biopsy, although in each case it is necessary to exclude other possible causes of granulomatosis, including infections by mycobacteria. Here it is reported a case of particularly aggressive sarcoidosis with primitive involvement of the small joints of the hands and feet, and mediastinal lymph nodes. CASE PRESENTATION: The subject, a man, 60 years old, born in Morocco but living in Italy for many years, presented important involvement of bone structures and soft periarticular tissue, and was affected by the formation of granulomas without "caseum necrosis". The painful symptoms and the skin ulceration had led to surgical amputation of the distal phalanges of most fingers of his hands and feet, but with subsequent resurgence of lesions in acral locations after surgery. The PET/CT scan showed an amount of radiotracer in mediastinal lymph nodes, while the lymph nodes sampled by TBNA were normal and the CD4/CD8 ratio was less than 3 in the bronchoalveolar lavage. We ruled out any possible infectious cause, including mycobacterial infection (both tubercular and atypical), so the patient was treated with systemic corticosteroids, with an excellent clinical and radiological response. CONCLUSIONS: Such a case shows how the disease can have variable expressions, without primitive lung involvement; therefore, it should be necessary to consider any possible, unpredictable localization of the disease.