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Background: Hemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening syndrome that can occur either in genetically predisposed individuals (primary HLH) or in particular conditions in immunocompromised patients (secondary HLH). Secondary HLH is very rare among solid organ transplant recipients, especially in lung transplant recipients, for whom its prognosis is dismal. Case Report: We report an exceptional case of HLH occurring unusually late following lung transplantation. At 11 years after transplantation, the patient, aged 67 years, presented with pancytopenia, fever, hyperferritinemia, and hypertriglyceridemia, along with splenomegaly. Exhaustive serological and PCR tests ruled out active infection. Bone marrow aspirates showed signs of hemophagocytosis, and bone marrow biopsy was suggestive of posttransplant lymphoproliferative disorder (PTLD). Timely treatment with etoposide and corticosteroids led to a transient improvement in the patient's clinical condition, and rituximab was initiated as a treatment for PTLD. Unfortunately, pancytopenia persisted for weeks, and the patient died from refractory septic shock, despite appropriate intravenous antibiotics. Autopsy revealed lymphoid infiltration of the mediastinal lymph nodes, liver and bone marrow, with some lymphocytes expressing CD3. A final diagnosis of Ann-Arbor stage IV non-EBVmediated monomorphic T-cell PTLD was established. Conclusions: This case report highlights a very unusual and fatal presentation of HLH in a lung transplant recipient, secondary to a T-cell PTLD. Indeed, HLH is typically seen as infection-related and reported to occur in the initial months following transplantation. To date, no guidelines or consensus exist regarding the management of immunosuppression regimen in solid organ transplantation. [ABSTRACT FROM AUTHOR]

This letter, published in the American Journal of Respiratory & Critical Care Medicine, discusses the dysregulation of the IgA-pIgR system in idiopathic pulmonary fibrosis (IPF). The authors conducted a study to investigate the IgA-pIgR system in IPF, analyzing serum and lung tissue samples from IPF patients and control subjects. They found increased levels of IgA+ memory B cells and autoreactive IgA in IPF, suggesting a dysregulation of the IgA-pIgR system in this disease. The study also suggests a potential role of the IgA-pIgR system in epithelial-mesenchymal transition (EMT) and fibrotic features in IPF. Further research is needed to explore the implications of mucosal immunity in pulmonary fibrosis. [Extracted from the article]

Having a family member with pulmonary fibrosis (PF) impacts the prognosis of sarcoidosis patients, as the majority of patients reporting at least one relative with PF present fibrotic characteristics and one-third develop a progressive phenotype https://bit.ly/40KC7Cr., Competing Interests: Conflict of interest: T. Planté-Bordeneuve reports support for the present manuscript from Bourse de Perfectionnement Fondation Mont Godinne, Fondation Médicale Horlait-Dapsens, and Bourse d'Excellence WBI World; and advisory board meeting fees from Boehringer Ingelheim, outside the submitted work. C.J.M. van Moorsel reports grants from Boehringer Ingelheim outside the submitted work; speaker fees from Boehringer Ingelheim outside the submitted work; and is cochair of the ClinGen ILD gene curation expert panel, disclosure made outside the submitted work. M. Veltkamp reports payment or honoraria for lectures, presentations, speakers’ bureaus, manuscript writing or educational events from Chiesi Pharmaceuticals outside the submitted work; and payment for expert testimony from Boehringer Inghelheim outside the submitted work. W. van Es reports payment or honoraria for lectures, presentations, speakers’ bureaus, manuscript writing or educational events from Boeringher Ingelheim outside the submitted work. M. Terwiel, J.J. van der Vis and J.C. Grutters have nothing to disclose., (Copyright ©The authors 2025.)

Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this article.

Chronic obstructive pulmonary disease (COPD), a devastating and irreversible lung disease, causes structural and functional defects in the bronchial epithelium, the (ir)reversibility of which remains unexplored in vitro. This study aimed to investigate the persistence of COPD-related epithelial defects in long-term airway epithelial cultures derived from non-smokers, smokers, and COPD patients. Barrier function, polarity, cell commitment, epithelial-to-mesenchymal transition, and inflammation were evaluated and compared with native epithelium characteristics. The role of inflammation was explored using cytokines. We show that barrier dysfunction, compromised polarity, and lineage abnormalities observed in smokers and COPD persisted for up to 10 wk. Goblet cell hyperplasia was associated with recent cigarette smoke exposure. Conversely, increased IL-8/CXCL-8 release and abnormal epithelial-to-mesenchymal transition diminished over time. These ex vivo observations matched surgical samples' abnormalities. Cytokine treatment induced COPD-like changes in control cultures and reactivated epithelial-to-mesenchymal transition in COPD cells. In conclusion, these findings suggest that the airway epithelium of smokers and COPD patients retains a multidimensional memory of its original state and previous cigarette smoke-induced injuries, maintaining these abnormalities for extended periods., (© 2023 Carlier et al.)