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IntroductionIdiopathic pulmonary fibrosis (IPF) has an unpredictable course corresponding to various profiles: stability, physiological disease progression and rapid decline. A minority of patients experience acute exacerbations (AEs). A recent study suggested that ozone and nitrogen dioxide might contribute to the occurrence of AE. We hypothesised that outdoor air pollution might influence the natural history of IPF.MethodsPatients were selected from the French cohort COhorte FIbrose (COFI), a national multicentre longitudinal prospective cohort of IPF (n=192). Air pollutant levels were assigned to each patient from the air quality monitoring station closest to the patient’s geocoded residence. Cox proportional hazards model was used to evaluate the impact of air pollution on AE, disease progression and death.ResultsOnset of AEs was significantly associated with an increased mean level of ozone in the six preceding weeks, with an HR of 1.47 (95% CI 1.13 to 1.92) per 10 µg/m3 (p=0.005). Cumulative levels of exposure to particulate matter PM10 and PM2.5 were above WHO recommendations in 34% and 100% of patients, respectively. Mortality was significantly associated with increased levels of exposure to PM10 (HR=2.01, 95% CI 1.07 to 3.77) per 10 µg/m3 (p=0.03), and PM2.5 (HR=7.93, 95% CI 2.93 to 21.33) per 10 µg/m3 (p<0.001).ConclusionThis study suggests that air pollution has a negative impact on IPF outcomes, corroborating the role of ozone on AEs and establishing, for the first time, the potential role of long-term exposure to PM10 and PM2.5 on overall mortality.

Background Light chain deposition disease (LCDD) is a very rare entity. Clinical manifestations of LCDD vary according to the organs involved. Data on pulmonary LCDD are scarce and limited to small series or case reports. This study aimed to describe the characteristics and outcome of diffuse pulmonary non-amyloid LCDD localized to the lungs. Study design and methods A multicenter retrospective cohort study was conducted. Clinical characteristics were collected, and chest CTs were centrally reviewed. The diagnosis of pulmonary non-amyloid LCDD was confirmed by immunohistochemistry. Results Thirty-one cases were identified (68% female), with a median age at diagnosis of 50 years (IQR 20). Baseline FEV1/FVC was < 0.70 in 45% of patients. Mean ( ±  SD) FEV1 and DLCO were 86% ± 26.2 and 52% ± 23.9, respectively. CT revealed peculiar patterns of thin-walled cysts (58%) and thin-walled cystic bronchiectases (27%). Increased serum kappa light chain was found in 87% of patients. Histological analysis showed kappa light chain deposits in all patients, except one with lambda chain deposits. Median annual FEV1 decline was 127 ml (IQR 178) and median DLCO decline was 4.3% (IQR 4.3). Sixteen patients received immunomodulatory treatment or chemotherapy; serum light chain levels decreased in 9 cases (75%), without significant improvement in FEV1 ( p  = 0.173). Overall, 48% of patients underwent bilateral lung transplantation. Transplant-free survival at 5 and 10 years were 70% and 30%, respectively. An annual FEV1 decline greater than 127 ml/year was associated with increased risk of death or transplantation ( p  = 0.005). Conclusions Diffuse pulmonary LCDD is characterised by female predominance, a peculiar imaging pattern with bronchiectasis and/or cysts, progressive airway obstruction and severe DLCO impairment, and poor outcome. Lung transplantation is a treatment of choice.

Background: Idiopathic pulmonary fibrosis (IPF) is characterized by a male predominance. The aim of the study was to explore gender differences in a well-designed French multicentre prospective IPF cohort (COhorte FIbrose, COFI) with a 5-year follow-up. Methods: Between 2007 and 2010, 236 patients with incident IPF were included in COFI. Gender characteristics were compared using a t -test, Chi-squared test and ANOVA, as appropriate. Survival analyses were performed. Results: Fifty-one (22%) females and 185 (78%) males with an average age at diagnosis of 70.1 ± 9.20 and 67.4 ± 10.9 years, respectively, were included in the cohort. Women were significantly less exposed to tobacco smoke [never n = 32 (62.7%) vs. n = 39 (21.1%), p < 0.001] and to occupational exposure [ n = 7 (13.7%) vs. n = 63 (34.1%), p = 0.012]. Baseline forced vital capacity, % of predicted (FVC%) was significantly better in women compare to men (83.0% ± 25.0 v. 75.4% ± 18.7 p = 0.046). At presentation honeycombing and emphysema on CT scan were less common in women [ n = 40 (78.4%) vs. n = 167 (90.3%) p = 0.041] and [ n = 6 (11.8%) vs. n = 48 (25.9%) p = 0.029], respectively. During follow-up fewer women were transplanted compared to men [ n = 1 (1.96%) vs. n = 20 (10.8%) p = 0.039]. Medians of survival were comparable by gender [31 months (CI 95%: 28–40) vs. 40 months (CI 95%: 33–72) p = 0.2]. After adjusting for age and FVC at inclusion, being a woman was not associated to a better survival. Conclusions: Women appear to have less advanced disease at diagnosis, maybe due to less exposure history compare to men. Disease progression and overall survival remains comparable regardless gender, but women have less access to lung transplantation.

Background: Interstitial lung disease (ILD) is a leading cause of death in systemic sclerosis (SSc). Sensitivities and specificities of the current pulmonary function tests (PFTs) for the detection of ILD in SSc are poor. Objective: To determine whether diffusion capacity of the lungs for carbon monoxide (DLCO) partitioned into membrane conductance for CO (DmCO) and alveolar capillary blood volume (Vcap) could provide more sensitive clues to ILD than current PFTs. Methods: DmCO and Vcap were determined in 35 consecutive SSc patients in whom a cardiac and/or pulmonary vascular abnormality had been rejected according to the recommended screening algorithm. ILD was diagnosed with high-resolution computed tomography. Results: Among 35 patients [6 men; median age (first–third quartile) 61.9 years (49.5–67.7)], 22 had no ILD and 13 did. Total lung capacity (TLC), vital capacity and DLCO [percentage of predicted value (%pred)] were lower in patients with ILD [86 (82–103) vs. 106 (98–112), p = 0.01, 96 (88–112) vs. 114 (104–121), p = 0.04, and 67 (59–81) vs. 80 (71–94), p = 0.02, respectively]. DmCO (%pred) and the ratio of DmCO to Vcap were much lower in patients with ILD [54 (48–72) vs. 83 (66–92), p < 0.001, and 0.22 (0.21–0.27) vs. 0.40 (0.35–0.53), p < 0.0001, respectively]. According to receiver operating characteristic analysis, the DmCO:Vcap ratio displayed higher sensitivity and specificity than TLC, vital capacity and DLCO in identifying ILD in our study group (p < 0.01). Conclusions: These results suggest that the partitioning of DLCO might be of interest for identifying ILD in SSc patients.

Hypersensitivity pneumonitis (HP) is the term used to describe a disease previously referred to as extrinsic allergic alveolitis or allergic alveolitis. It is an immunologic lung disease that occurs in pre-sensitized subjects: the chronic or repeated inhalation of finely dispersed antigens from a wide variety provokes a hypersensitivity reaction with granulomatous inflammation in the distal bronchioles and alveoli. The causative antigens can be classified in three broad categories: microorganisms and molds, animal and insect proteins, and chemical compounds. In many cases, causal antigens remain undeterminated. The prevalence and incidence of HP vary considerably, depending on disease definitions, methods of establishing diagnosis, intensity of exposure, environmental conditions and host/genetic risk factors that remain poorly understood.

ELP are uncommon diseases. The most frequent etiology is the chronic use of medicines containing mineral oily substances, mostly paraffin or vaseline, for constipation or rhinopharyngeal diseases. ELP are often asymptomatic. Diagnosis must be considered in case of patient history, with use of oily medicines, and imaging features, particularly hypodense consolidations, suggest it. This diagnosis must be confirmed by various examinations allowing to show presence of intrapulmonary oil, such as bronchoalveolar lavage or histological specimens of which fat stains will be used. Endogenous lipid pneumonia must be eliminated. Early recognition and discontinuation of exposure are essential to prevent the lesions from evolving towards fibrosis and respiratory failure.