Idiopathic Interstitial Pneumonias

BackgroundPulmonary hypertension (PH) is a common finding in patients with chronic fibrosing idiopathic interstitial pneumonias (IIP). Little is known about the response to pulmonary vasodilator therapy in this patient population. COMPERA is an international registry that prospectively captures data from patients with various forms of PH receiving pulmonary vasodilator therapies.MethodsWe retrieved data from COMPERA to compare patient characteristics, treatment patterns, response to therapy and survival in newly diagnosed patients with idiopathic pulmonary arterial hypertension (IPAH) and PH associated with IIP (PH-IIP).ResultsCompared to patients with IPAH (n = 798), patients with PH-IIP (n = 151) were older and predominantly males. Patients with PH-IIP were treated predominantly with phosphodiesterase-5 inhibitors (88% at entry, 87% after 1 year). From baseline to the first follow-up visit, the median improvement in 6MWD was 30 m in patients with IPAH and 24.5 m in patients with PH-IIP (p = 0.457 for the difference between both groups). Improvements in NYHA functional class were observed in 22.4% and 29.5% of these patients, respectively (p = 0.179 for the difference between both groups). Survival rates were significantly worse in PH-IIP than in IPAH (3-year survival 34.0 versus 68.6%; pConclusionsPatients with PH-IIP have a dismal prognosis. Our results suggest that pulmonary vasodilator therapy may be associated with short-term functional improvement in some of these patients but it is unclear whether this treatment affects survival.Trial registrationclinicaltrials.gov NCT01347216.

ObjectiveAlthough a possible association among myeloperoxidase-anti-neutrophil cytoplasmic antibody (MPO-ANCA), microscopic polyangiitis (MPA), and idiopathic pulmonary fibrosis (IPF) has been suggested, the clinical significance of MPO-ANCA in idiopathic interstitial pneumonias (IIPs), including IPF and non-IPF, remains unclear. We aimed to investigate the frequency of MPO-ANCA positivity, as well as MPA incidence and risk factors for development in patients initially diagnosed with IIP.MethodsWe retrospectively analysed 305 consecutive patients who were initially diagnosed as IIP and had MPO-ANCA results available.ResultsOf the 305 patients, 26 (8.5%) were MPO-ANCA-positive. Baseline characteristics were similar between the MPO-ANCA-positive and -negative patients. The cumulative 5-year MPA incidence was 24.3% in the MPO-ANCA-positive patients and 0% in the -negative patients (P ConclusionMPO-ANCA positivity was uncommon, but was associated with subsequent MPA development in patients initially diagnosed with IIP, including both IPF and non-IPF cases. The study suggested that attention should be paid to MPA development in MPO-ANCA-positive IIP patients with UIP pattern on HRCT and those without treatment for IIP.

BackgroundThe aim of this study was to analyze the relative frequency, clinical characteristics, disease onset and progression in f-IPF vs. sporadic IPF (s-IPF).MethodsFamilial IPF index patients and their family members were recruited into the European IPF registry/biobank (eurIPFreg) at the Universities of Giessen and Marburg (UGMLC). Initially, we employed wide range criteria of f-IPF (e.g. relatives who presumably died of some kind of parenchymal lung disease). After narrowing down the search to occurrence of idiopathic interstitial pneumonia (IIP) in at least one first grade relative, 28 index patients were finally identified, prospectively interviewed and examined. Their family members were phenotyped with establishment of pedigree charts.ResultsWithin the 28 IPF families, overall 79 patients with f-IPF were identified. In the same observation period, 286 f-IIP and s-IIP patients were recruited into the eurIPFreg at our UGMLC sites, corresponding to a familial versus s-IPF of 9.8%. The both groups showed no difference in demographics (61 vs. 79% males), smoking history, and exposure to any environmental triggers known to cause lung fibrosis. The f-IPF group differed by an earlier age at the onset of the disease (55.4 vs. 63.2 years; p ConclusionsThe 28 f-IPF index patients presented an earlier onset and more aggressive natural course of the disease. The disease seems to affect consecutive generations at a younger age.Trial registrationNr. NCT02951416 http://www.www.clinicaltrials.gov.

BACKGROUND: Low skeletal muscle mass has been reported to associated with poor prognosis in patients with idiopathic pulmonary fibrosis (IPF). However, such associations have scarcely reported in idiopathic interstitial pneumonias (IIPs) other than IPF. Quantification of muscle mass obtained from chest computed tomography (CT) is used as a simple screening tool for sarcopenia in patients with respiratory diseases. However, the optimal thoracic site for muscle mass quantification is controversial. Moreover, there have been no reports investigating the association between muscle mass and acute exacerbations. This study aimed to evaluate optimal site for muscle mass quantification in chest CT to predict survival and acute exacerbation in IPF and non-IPF idiopathic interstitial pneumonias. METHODS: This study included 528 patients diagnosed with IIP at 29 facilities between September 1, 2013, and April 30, 2016, following multidisciplinary discussions with prospective follow-up over a 5-year period. The cohort was divided into two groups: those with IPF and those with non-IPF IIPs. Skeletal muscle mass was quantified using the erector spinae muscle index (ESMI) and pectoralis muscle index (PMI), defined as the respective muscle area on chest computed tomography (CT) divided by height squared at the time of enrollment. Associations between these indices at baseline and both survival and acute exacerbation were analyzed. RESULTS: In both IPF and non-IPF cohorts, Cox regression analysis revealed that patients with low ESMI had a poorer prognosis compared to those with normal ESMI, even after adjusting for age, sex, % forced vital capacity (FVC), and smoking exposure level. The hazard ratios were 0.62 (95% CI 0.40–0.90; p = 0.013) and 0.46 (95% CI 0.26–0.83; p = 0.009), respectively. In contrast, no significant relationship was identified between PMI and survival. Multivariable Cox regression analysis confirmed that ESMI was an independent predictor of survival in both IPF and non-IPF patients. Additionally, acute exacerbations occurred more frequently in the low ESMI group and low PMI group, particularly among non-IPF patients. CONCLUSIONS: The ESMI obtained from chest CT is associated with survival in not only in IPF patients but also in the non-IPF patients. The ESMI and PMI also associate with acute exacerbations in non-IPF patients.