RationaleProgressive fibrosing interstitial lung disease (PF-ILD) is characterised by progressive physiologic, symptomatic, and/or radiographic worsening. The real-world prevalence and characteristics of PF-ILD remain uncertain.MethodsPatients were enrolled from the Canadian Registry for Pulmonary Fibrosis between 2015–2020. PF-ILD was defined as a relative forced vital capacity (FVC) decline ≥10%, death, lung transplantation, or any 2 of: relative FVC decline ≥5 and <10%, worsening respiratory symptoms, or worsening fibrosis on computed tomography of the chest, all within 24 months of diagnosis. Time-to-event analysis compared progression between key diagnostic subgroups. Characteristics associated with progression were determined by multivariable regression.ResultsOf 2746 patients with fibrotic ILD (mean age 65±12 years, 51% female), 1376 (50%) met PF-ILD criteria in the first 24 months of follow-up. PF-ILD occurred in 427 (59%) patients with idiopathic pulmonary fibrosis (IPF), 125 (58%) with fibrotic hypersensitivity pneumonitis (HP), 281 (51%) with unclassifiable ILD (U-ILD), and 402 (45%) with connective tissue disease-associated ILD (CTD-ILD). Compared to IPF, time to progression was similar in patients with HP (hazard ratio [HR] 0.96, 95% confidence interval, CI 0.79–1.17), but was delayed in patients with U-ILD (HR 0.82, 95% CI 0.71–0.96) and CTD-ILD (HR 0.65, 95% CI 0.56–0.74). Background treatment varied across diagnostic subtypes with 66% of IPF patients receiving antifibrotic therapy, while immunomodulatory therapy was utilised in 49%, 61%, and 37% of patients with CHP, CTD-ILD, and U-ILD respectively. Increasing age, male sex, gastroesophageal reflux disease, and lower baseline pulmonary function were independently associated with progression.InterpretationProgression is common in patients with fibrotic ILD, and is similarly prevalent in HP and IPF. Routinely collected variables help identify patients at risk for progression and may guide therapeutic strategies.

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RationaleExtracellular vesicles (EVs) are small lipid vesicles, and EV-coupled microRNAs (miRNAs) are important modulators of biological processes. Fibrocytes are circulating bone marrow-derived cells that migrate into the injured lungs and contribute to fibrogenesis. The question of whether EV-coupled miRNAs derived from fibrocytes are able to regulate pulmonary fibrosis has not been addressed yet.MethodsPulmonary fibrosis was induced in rats by intratracheal administration of an adenoviral gene vector encoding active transforming growth factor-β1 (TGF-β1) or control vector. Primary fibrocytes and fibroblasts were cultured from rat lungs and were sorted by anti-CD45 magnetic beads. Human circulating fibrocytes and fibrocytes in bronchoalveolar lavage fluid (BALF) were isolated by fibronectin-coated dishes. Fibrocytes were cultured on different stiffness plates or decellularised lung scaffolds. We also determined the effects of extracellular matrix (ECM) and recombinant TGF-β1 on the cellular and EV-coupled miRNA expression of fibrocytes.ResultsThe EVs of fibrocytes derived from fibrotic lungs significantly upregulated the expression of col1a1 of fibroblasts. Culturing on rigid plates or fibrotic decellularised lung scaffolds increased miR-21-5 p expression compared with soft plates or normal lung scaffolds. Dissolved ECM collected from fibrotic lungs and recombinant TGF-β1 increased miR-21-5 p expression on fibrocytes, and these effects were attenuated on soft plates. Fibrocytes from BALF collected from fibrotic interstitial pneumonia patients showed higher miR-21-5 p expression than those from other patients.ConclusionsOur results indicate that ECM contributes to fibrogenesis through biomechanical and biochemical effects on miRNA expression in fibrocytes.

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Summary Background Sarcoidosis is occasionally seen in association with malignancy, both at the time of cancer diagnosis or during follow up. Aim The purpose of this study is to identify patients with paraneoplastic sarcoid, their associated malignancies and disease characteristics. Methods We identified 289 patients diagnosed histologically with sarcoidosis over a 6-year period in one centre, from 2010 to 2016. Fifty of these patients had a prior or concomitant diagnosis of cancer. Results 17.3% of sarcoid cases had an associated malignancy. The most common malignancies were Gastrointestinal (20%), Haematological (18%), Lung (12%), Gynaecological (12%) and Head and Neck cancer (12%). 74% of sarcoid cases had pulmonary disease with sarcoid diagnostic tissue obtained most frequently via endobronchial ultrasound fine needle aspiration (68%). Most sarcoid cases (66%) were diagnosed within the first year of their malignancy diagnosis. Discussion Careful consideration needs to be given to the possibility that potential cancer recurrences suspected on imaging studies may indeed be sarcoid reactions.

Pseudochylothorax is a very rare form of pleural effusion. It is also called chyliform or cholesterol pleural effusion. It is usually a unilateral process and approximately one-third of patients are asymptomatic at presentation. We report a case of a 60 year old man with a background of rheumatoid arthritis who presented with progressive dyspnea. Chest X-ray revealed a new left pleural effusion and a small persistent right pleural effusion. He presented 5 years prior due to recurrent pleural effusion and no diagnosis was made. Repeat thoracentesis yielded 350 milliliters of thick, milky, tan-colored fluid.