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Pulmonary arterial hypertension (PAH) is rare but remains fatal in infants and children despite the advance of targeted therapies. Lung transplantation (LTx), first performed in pediatric patients in the 1980s, is, with the Potts shunt, the only potentially life-extending option in patients with end-stage PAH but is possible only in tightly selected patients. Size-matching challenges severely restrict the donor organ pool, resulting-together with peculiarities of PAH in infants-in high waitlist mortality. We aimed to investigate survival when using a high-priority allocation program (HPAP) in children with PAH listed for double-LTx or heart-LTx.We conducted a single-center, retrospective, before-after study of consecutive children with severe Group 1 PAH listed for double-LTx or heart-LTx between 1988 and 2019. The HPAP was implemented in France in 2006 and 2007 for heart-LTx and double-LTx, respectively.Fifty-five children with PAH were listed for transplantation. Mean age at transplantation was 15.8±2.8 years and 72% had heart-lung transplantation. PAH was usually idiopathic (65%) or due to congenital heart disease (25%). HPAP implementation resulted in the following significant benefits: Decreased cumulative incidence of waitlist death within 1 and 2 years (p0.0001); increased cumulative incidence of transplantation within 6 months, from 44% to 67% (p0.01); and improved survival after listing (at 1, 3, and 5 years: 61%, 50%, and 44% vs. 92%, 84%, and 72% before and after HPAP implementation, respectively; p = 0.02).HPAP implementation was associated with significant improvements in access to transplantation and in survival after listing in children with end-stage PAH.

We aimed to review the clinical and biological presentation of granulomatosis associated with immune-checkpoint inhibitors (ICI) in patients with melanoma and to explore its association with classical sarcoidosis as well as with cancer response to ICI. To this end, a retrospective study on 18 melanoma patients with histologically proven ICI-induced granulomatosis over a 12-year period in a single center, as well as on 67 similar cases reported in the literature, was conducted. Results indicate ICI-induced granulomatosis is an early side effect (median time to onset: 2 months). Its clinical presentation, with predominant (90%) thoracic involvement, histopathological appearance and supposed underlying biology (involving the mTOR pathway in immune cells, Th17 polarization and TReg dysfunction) are indistinguishable from those of sarcoidosis. Moreover, it appears to be associated with ICI benefit (>65% objective response rate). Evolution is generally favorable, and symptomatic steroid treatment and/or ICI discontinuation are rarely necessary. ICI-associated granulomatosis is critical to explore for several reasons. Practically, it is essential to differentiate it from cancer progression. Secondly, this “experimental” sarcoidosis brings new elements that may help to address sarcoidosis origin and pathophysiology. Its association with ICI efficacy must be confirmed on a larger scale but could have significant impacts on patient management and biomarker definition.

After tobacco, cannabis is the most widely used drug worldwide, and the move to legalise it is growing in more and more countries. The literature about the involvement of cannabis smoking on the development of lung cancer is scarce and most often reassuring [1–3], even though the concentration of carcinogens in cannabis smoke is unquestionably greater than that in tobacco smoke [4]. Moreover, recent studies on cannabis tend to focus on possible therapeutic effects [5]. Studies of the carcinogenic impact of cannabis are indeed limited by its frequent association with tobacco smoking and by its illegal status in most countries [6]. Footnotes This manuscript has recently been accepted for publication in the European Respiratory Journal . It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article. Conflict of interest: Dr. Betser has nothing to disclose. Conflict of interest: Dr. Glorion has nothing to disclose. Conflict of interest: Dr. Mordan has nothing to disclose. Conflict of interest: Dr. Caramella has nothing to disclose. Conflict of interest: Dr. Ghigna has nothing to disclose. Conflict of interest: Dr. Besse has nothing to disclose. Conflict of interest: Dr. planchard has nothing to disclose. Conflict of interest: Dr. Le Pavec has nothing to disclose. Conflict of interest: Dr. Chapelier has nothing to disclose. Conflict of interest: Dr. Friard has nothing to disclose. Conflict of interest: Dr. Gounant has nothing to disclose. Conflict of interest: Dr. Castier has nothing to disclose. Conflict of interest: Dr. Levy has nothing to disclose. Conflict of interest: Dr. Mercier has nothing to disclose. Conflict of interest: Dr. Fabre has nothing to disclose. Conflict of interest: Dr. Fadel has nothing to disclose. Conflict of interest: Dr. pradere has nothing to disclose.

Introduction Lung cancer is a frequent pathology for which the best curative treatment is pulmonary resection. Pulmonary arterial hypertension is a rare disease but pulmonary hypertension associated with parenchymal disease or left heart disease is frequently observed in these patients. The diagnosis of pulmonary hypertension before lung resection makes the perioperative management of these patients more difficult and sometimes leads to rejecting patients for surgery. Areas covered We performed a review of literature on PubMed on Pulmonary hypertension associated lung resection, preoperative assessment of lung resection and perioperative management of PH patients, including guidelines and clinical trials. In this review, we summarize the current state of knowledge regarding the pre and perioperative management of patients with suspected or confirmed PH who can benefit from surgical treatment of lung cancer. Expert opinion Management of PH patients before lung resection should include a very careful workup including at least right heart catheterization with evaluation of the targeted PH treatment in an expert center and evaluation of other comorbidities. Perioperative management must be carried out in a specialized center.

Objective: There are a number of factors that go into a recommendation to treat non-tuberculous mycobacterial lung infection (NTM-LI). The purpose of the study is to validate consensus recommendations on clinical considerations before treating a NTM-LI. Methods: We engaged respiratory physicians of the "Mycobacterioses" working group, part of the research and teaching group in pulmonology and infectious diseases (GREPI), on behalf of the SPLF (Societe de Pneumologie de Langue Francaise) in a Delphi survey concerning three areas of interest for physicians before they had to decide to treat (Diagnosis, Definition of the level of severity and Clinical assessment before deciding to treat) in order to create a decision algorithm. Each assertion was subjected to rating and free comments by these experts via an online questionnaire. The scores were weighted by the number of patients seen each year by the experts. The assertions having obtained a weighted average greater than 9/10 were validated. Otherwise they were subject to re-evaluation during round 2 until reaching 9/10. Results: A total of 37 experts responded. At first round, 9/38 assertions obtained a strong agreement (median ≥7 and 80% of responses >7). Other assertions from all domains, in particular the definition of the level of severity, were revised and new assertions added for submission to scoring in round 2. Fifty-one assertions were finally assessed. Agreement was strong in 78% of the assertions (40/51); moderate in 22% (11/51). There was no disagreement. Conclusion: We obtained a consensus from physicians about clinical considerations before deciding to treat NTM-LI and we propose a decision algorithm to guide a treatment decision.

The purpose of this study was to compare efficacy and tolerance between radiofrequency ablation (RFA) and surgery for the treatment of oligometastatic lung disease.This retrospective study reviewed patients treated in two institutions for up to 5 pulmonary metastases with a maximal diameter of 4cm and without associated pleural involvement or thoracic lymphadenopathy. Patient demographics, tumor characteristics, treatment outcome, and length of hospital stay were compared between the two groups. Efficacy endpoints were overall survival (OS), progression-free survival (PFS) and pulmonary or local tumor progression rates.Among 204 patients identified, 78 patients (42 men, 36 women; mean age, 53.3±14.9 [SD]; age range: 15-81 years) were treated surgically, while 126 patients (59 men, 67 women; mean age, 62.2±10.8 [SD]; age range: 33-80 years) were treated by RFA. In the RFA cohort, patients were significantly older (P0.0001), with more extra-thoracic localisation (P=0.015) and bilateral tumour burden (P=0.0014). In comparison between surgery and RFA cohorts, respectively, the 1- and 3-year OS were 94.8 and 67.2% vs. 94 and 72.1% (P=0.46), the 1- and 3-year PFS were 49.4% and 26.1% vs. 38.9% and 14.8% (P=0.12), the pulmonary progression rates were 39.1% and 56% vs. 41.2% and 65.3% (P0.99), and the local tumour progression rates were 5.4% and 10.6% vs. 4.8% and 18.6% (P=0.07). Tumour size2cm was associated with a significantly higher local tumor progression in the RFA group (P=0.010). Hospitalisation stay was significantly shorter in the RFA group (median of 3 days; IQR=2 days; range: 2-12 days) than in the surgery group (median of 9 days; IQR=2 days; range: 6-21 days) (P0.01).RFA should be considered a minimally-invasive alternative with similar OS and PFS to surgery in the treatment of solitary or multiple lung metastases measuring less than 4cm in diameter without associated pleural involvement or thoracic lymphadenopathy.

Idiopathic pulmonary fibrosis (IPF) is characterized by relentlessly progressive lung function impairment that is consistently fatal in the absence of lung transplantation, as no curative pharmacological treatment exists. The pace of progression varies across patients, and acute life-threatening exacerbations occur unpredictably, causing further sharp drops in lung function. Recently introduced antifibrotic agents slow the pace of disease progression and may improve survival but fail to stop the fibrotic process. Moreover, the magnitude and kinetics of the response to these drugs cannot be predicted in the individual patient. These characteristics require that lung transplantation be considered early in the course of the disease. However, given the shortage of donor lungs, lung transplantation must be carefully targeted to those patients most likely to benefit. Current guidelines for lung transplantation listing may need reappraisal in the light of recent treatment advances. Patients with IPF often have multiple comorbidities such as coronary heart disease, frailty, and gastro-oesophageal reflux disease (GERD). Consequently, extensive screening for and effective treatment of concomitant conditions is crucial to appropriate candidate selection and outcome optimisation. A multidisciplinary approach is mandatory. Pulmonologists with expertise in IPF must work closely with lung transplant teams. Careful consideration must be given to preoperative optimisation, surgical technique, and pulmonary rehabilitation to produce the best post-transplantation outcomes.

Background. We sought to determine whether invasive aspergillosis (IA) during the first year after lung transplantation increased the risk of chronic lung allograft dysfunction (CLAD). Methods. We retrospectively reviewed the records of 191 patients who underwent lung transplantation at our institution between January 2013 and December 2017. Screening for Aspergillus was with bronchial aspirates, bronchoalveolar lavage if indicated or during surveillance bronchoscopy, radiography, and computed tomography. We used Fine and Gray multivariable regression to identify potential risk factors for CLAD. Results. During the first posttransplant year, 72 patients had at least 1 deep-airway sample positive for Aspergillus; 63 were classified as having IA and were included in the study. Median number of endoscopies per patient during the first year was 9 (range, 1–44). Median time from transplantation to first Aspergillus-positive sample was 121 d. Bronchial aspirate samples and bronchoalveolar lavage fluid were positive in 71 and 44 patients, respectively. Aspergillus fumigatus (n = 36, 50%) predominated; bacterial samples were also positive in 22 (31%) patients. IA within 4 mo after transplantation was independently associated with CLAD development (subdistribution hazard ratio, 3.75; 95% confidence interval [CI], 1.61-8.73; P < 0.01) by regression analysis. Survival at 3 and 5 y conditional on 1-y CLAD-free survival was 37% (95% CI, 24%-58%), and 24% (95% CI, 11%-52%) in the IA

Idiopathic pulmonary fibrosis (IPF) is a deadly disease with a median survival of approximately three years in historical cohorts. Despite increased knowledge of disease pathophysiology and selection of more targeted therapy, main clinical trials yielded negative results. However, two agents, pirfenidone and nintedanib, were recently shown to be effective in IPF and received marketing authorization worldwide. Both drugs significantly reduce functional decline and disease progression with an acceptable safety profile. Yet, none of these drugs actually improves or even stabilizes the disease or the symptoms perceived by the patient. Several other treatments and combinations are currently tested, and many more are ready for clinical trials. Their completion is critical for achieving the ultimate goal of curing patients with IPF.

Objective To describe a new entity characterized by airway-centered fibroelastosis. Methods We identified cases with prominent airway-centered elastosis in lung samples, and little or no pleural involvement identified through a pathologic database at a single institution over an 8-year period. Results Airway-centered fibroelastosis was characterized by (1) extensive airway-centered fibroelastosis of the upper lobes on histopathology and (2) marked bronchial abnormalities with bronchial wall thickening, bronchial wall deformation, and bronchiectasis, along with progressive parenchymal retraction and predominantly subpleural upper-lobe consolidations on high-resolution CT. Pateints were five nonsmoking women aged between 38 and 56 years old. They experienced chronic dyspnea with acute attacks of wheezing and dyspnea. Moderate to severe physiological abnormalities were observed, with an obstructive pattern in three cases and a restriction in two. Despite inhaled and oral corticosteroids, the disease was progressive in all patients and evolved to chronic respiratory failure, requiring lung transplantation in two patients. Four patients had chronic asthma. Conclusions We consider airway-centered fibroelastosis to be a unique and distinct pathological entity in women that needs to be individualized, with a specific clinical, imaging, and pathological presentation. We hypothesize that airway-centered fibroelastosis may be idiopathic or asthma-associated.

Resume Introduction L’omalizumab, un anticorps monoclonal bloquant les immunoglobulines E, a depuis maintenant dix ans une autorisation de mise sur le marche dans l’asthme severe atopique de l’adulte, en cas de mauvais controle malgre de fortes doses de corticoides inhales associes a des bronchodilatateurs de longue duree d’action. Il s’agit de la premiere biotherapie utilisee a large echelle dans l’asthme severe. Etat des connaissances L’omalizumab a prouve un benefice, notamment dans la prevention des exacerbations severes d’asthme et ce, avec un profil de tolerance tres satisfaisant. Il s’agit d’une alternative interessante a considerer, malgre son cout, face aux complications a long terme de la corticotherapie orale continue. Perspectives Ces dix ans d’utilisation sont l’occasion de faire le point sur le mecanisme d’action de l’omalizumab, le benefice a en attendre, les effets secondaires ainsi que le rapport cout–efficacite de cette molecule. On rappelle egalement les modalites pratiques d’utilisation de l’omalizumab, notamment l’importance d’une evaluation rigoureuse de son efficacite a 16 semaines de traitement. Les futures indications de cette therapeutique sont egalement abordees. Conclusion L’omalizumab a prouve aussi bien dans des etudes randomisees de grande ampleur que dans des etudes de « vraie vie » son interet dans l’asthme severe allergique.

International audience; BACKGROUND: Due to its multisystemic nature, scleroderma is considered a relative contraindication to lung transplantation at many centers. However, recent studies suggest similar post-transplant outcomes in patients with scleroderma compared to those with other causes of interstitial lung disease (ILD). Furthermore, it remains unknown whether scleroderma-associated pulmonary arterial hypertension (PAH) influences post-transplant outcomes. Our objective in this study was to assess the indications, survival, and prognostic factors of lung or heart lung transplantation for scleroderma lung disease. METHODS: We retrospectively reviewed the data of 90 patients with scleroderma who underwent lung or heart lung transplantation between 1993 and 2016 at 14 European centers. International criteria were used to diagnose scleroderma. Pulmonary hypertension (PH) was diagnosed during right heart catheterization based on international guidelines. RESULTS: Survival rates after 1, 3, and 5 years were 81%, 68%, and 61%, respectively. By univariate analysis, borderline-significant associations with poorer survival were found for female gender (hazard ratio 2.11; 95% confidence interval [CI] 0.99 to 4.50; p = 0.05) and PAH as the reason for transplantation (hazard ratio 1.90; 95% CI 0.96 to 3.92; p = 0.06). When both these factors were present in combination, the risk of death was 3-fold that in males without PAH. The clinical and histologic presentation resembled veno-occlusive disease in 75% of patients with PAH. CONCLUSIONS: Post-transplant survival rates and freedom from chronic lung allograft dysfunction in patients with scleroderma were similar to those in patients with other reasons for lung transplantation. Female sex and PAH in combination was associated with lower survival. (C) 2018 International Society for Heart and Lung Transplantation. All rights reserved.

The occurrence of pulmonary fibrosis in numerous individuals from the same family suggests a genetic cause for the disease. During the last 10 years, mutations involving proteins from the telomerase complex and from the surfactant system have been identified in association with pulmonary fibrosis. Mutations of TERT, the coding gene for the telomerase reverse transcriptase, are the most frequently identified mutations and are present in 15% of cases of familial pulmonary fibrosis. Other mutations (TERC, surfactant proteins genes) are only rarely evidenced in adults. Patients with mutations involving the telomerase complex may present with pulmonary fibrosis, hematologic, cutaneous or liver diseases. Other genetic variations associated with pulmonary fibrosis such as a polymorphism in the promoter of MUC5B or a polymorphism in TERT have been recently described, and could be considered to be part of a polygenic transmission. Evidence for mutations associated with the development of pulmonary fibrosis raises numerous clinical questions from establishing a diagnosis, providing counselling to deciding on therapy, and requires specific studies. From a pathophysiological point of view, the function of the genes highlights the central role of alveolar epithelium and aging in fibrogenesis.

Aberrant expression of master phenotype regulators or alterations in their downstream pathways in lung fibroblasts may play a central role in idiopathic pulmonary fibrosis (IPF). Interrogating IPF fibroblast transcriptome datasets, we identified Forkhead Box F1 (FOXF1), a DNA-binding protein required for lung development, as a candidate actor in IPF. Thus we determined FOXF1 expression levels in fibroblasts cultured from normal or IPF lungs in vitro, and explored FOXF1 functions in these cells using transient and stable loss-of-function and gain-of-function models. FOXF1 mRNA and protein were expressed at higher levels in IPF fibroblasts compared with normal fibroblasts (mRNA: +44%, protein: +77%). Immunohistochemistry showed FOXF1 expression in nuclei of bronchial smooth muscle cells, endothelial cells, and lung fibroblasts including fibroblastic foci of IPF lungs. In normal lung fibroblasts, FOXF1 repressed cell growth and expression of collagen-1 (COL1) and actin-related protein 2/3 complex, subunit 2 (ARPC2). ARPC2 knockdown inhibited cell growth and COL1 expression, consistent with FOXF1 acting in part through ARPC2 repression. In IPF fibroblasts, COL1 and ARPC2 repression by FOXF1 was blunted, and FOXF1 did not repress growth. FOXF1 expression was induced by the antifibrotic mediator prostaglandin E2 and repressed by the profibrotic cytokine transforming growth factor-β1 in both normal and IPF lung fibroblasts. Ex vivo, FOXF1 knockdown conferred CCL-210 lung fibroblasts the ability to implant in uninjured mouse lungs. In conclusion, FOXF1 functions and regulation were consistent with participation in antifibrotic pathways. Alterations of pathways downstream of FOXF1 may participate to fibrogenesis in IPF fibroblasts.

Presence of anti-human leukocyte antigen donor-specific antibodies (DSAs) is associated with poor outcome after lung transplantation. Currently, DSAs are detected using the Luminex technique, which may be overly sensitive. The new C1q assay allows for the exclusive detection of complement (C1q)-binding antibodies, involved in antibody-mediated rejection. We investigated whether early detection of complement-binding DSAs is associated with chronic lung allograft dysfunction (CLAD) and survival.From 2009 to 2012, lung transplant recipients from three transplantation centres were screened for the presence of DSA and their complement-binding capacity during the 6-12 months post-transplantation in a stable condition.The analysis included 168 patients. The 3-year rates of freedom from CLAD and graft survival were lower for patients with complement-binding DSAs (33.6% and 53.7%, respectively), as compared with patients with non-complement-binding DSAs (61.9% and 77.4%, respectively) and patients without DSA (70% and 84.9%, respectively) (p0.001 and p=0.001, respectively). Detection of complement-binding DSA was associated with a risk of graft loss that was nearly tripled after adjustment for clinical, functional, histological and immunological factors (hazard ratio 2.98, 95% CI 1.33-6.66; p=0.008).Assessment of the C1q-binding capacity of DSA appears to be useful in identifying stable lung transplant recipients at high risk of lung allograft loss.

Background Autoantibodies against lung epithelial antigens are often detected in patients with Idiopathic Pulmonary Fibrosis (IPF). Anti-Parietal Cell Antibodies (APCA) target the H+/K+ATPase (proton pump). APCA prevalence and lung H+/K+ATPase expression was never studied in IPF patients. Methods We retrospectively collected clinical, lung function and imaging data from APCA positive patients (APCA+IPF) and compared them with APCA negative IPF patients matched on the date of diagnostic assessment. H+/K+ATPase expression was assessed with immunohistochemistry and PCR. Results Among 138 IPF patients diagnosed between 2007 and 2014 and tested for APCA, 19 (13.7%) APCA+ patients were identified. APCA+IPF patients were 16 men and 3 women, mean age 71 years. The median titer of APCA was 1:160. A pernicious anemia was present in 5 patients and preceded the fibrosis in 3 cases. With a mean follow up of 31 months, 2 patients had an exacerbation and 7 patients died. As compared with 19 APCA- IPF patients, APCA+IPF patients had a less severe disease with better DLCO (57% vs 43% predicted), preserved PaO2 (85 ± 8 mmHg vs 74 ± 11 mmHg), a lower rate of honeycombing on HRCT (58% vs 89%), but they experienced an accelerated decline of FVC (difference 61.4 ml/year; p = .0002). The H+/K+ATPase was strongly expressed by hyperplastic alveolar epithelial cells in the fibrotic lung. Conclusion Anti-parietal cell autoimmunity is detected in some IPF patients and is associated with an accelerated decline of lung function. Anti-parietal cell autoimmunity may promote lung fibrosis progression.

To describe a new entity characterized by airway-centered fibroelastosis.We identified cases with prominent airway-centered elastosis in lung samples, and little or no pleural involvement identified through a pathologic database at a single institution over an 8-year period.Airway-centered fibroelastosis was characterized by (1) extensive airway-centered fibroelastosis of the upper lobes on histopathology and (2) marked bronchial abnormalities with bronchial wall thickening, bronchial wall deformation, and bronchiectasis, along with progressive parenchymal retraction and predominantly subpleural upper-lobe consolidations on high-resolution CT. Pateints were five nonsmoking women aged between 38 and 56 years old. They experienced chronic dyspnea with acute attacks of wheezing and dyspnea. Moderate to severe physiological abnormalities were observed, with an obstructive pattern in three cases and a restriction in two. Despite inhaled and oral corticosteroids, the disease was progressive in all patients and evolved to chronic respiratory failure, requiring lung transplantation in two patients. Four patients had chronic asthma.We consider airway-centered fibroelastosis to be a unique and distinct pathological entity in women that needs to be individualized, with a specific clinical, imaging, and pathological presentation. We hypothesize that airway-centered fibroelastosis may be idiopathic or asthma-associated.

Lung involvement in connective tissue disorders (CTDs) may present as pleomorphic since any lung compartment may be involved such as airways, exocrine secretory and alveolar epithelia, interstitial lung structure, pulmonary vasculature and pleura as well as, in specific disorders, several tissues of the thoracoabdominal ventilator pump. Any combination of the above anatomic structures may be involved concomitantly although some specific combinations may include a determinant of rheumatic disorders. The diagnosis of a specific CTD requires the fulfilment of clearly defined clinical and laboratory criteria including in most cases positivity for autoantibodies, mostly specific serologic combinations. In this setting, serologic investigation targets mainly, although not exclusively, the detection of antinuclear antibodies. A specific serologic positivity or a combination of autoantibodies constitutes not only a diagnostic criterion for a specific CTD, but may also characterize the pattern of respiratory manifestation in a determinant rheumatic disorder. Therefore, the investigation of lung involvement in CTDs requires adequate skills in the ambit of a multidisciplinary approach and an extended spectrum of diagnostic tools and modalities able to detect both early clinical clues and serologic conversion as well as any pathophysiologic alteration that regards the complexity of respiratory functional status. Although many patients with CTDs suffer from a ‘vicious' combination of lung involvement, lung drug toxicity and infections related to the above two as well as to the ‘mater' disease, for space reasons this review will focus on the established lung manifestations that regard the 7 major CTDs.

Introduction La fibroelastose pleuro-pulmonaire (FEPP) est une nouvelle entite au sein des PID. Nous decrivons 5 cas de fibroelastose avec une atteinte bronchocentrique predominante compliquant un asthme severe. Resultats Il s’agit de 5 femmes (âge 35–56) non fumeuses, qui presentaient un asthme severe, corticodependant. L’asthme etait tardif chez 4 et debutant dans l’enfance pour 1 patiente. Trois etaient atopiques, 2 avaient une maladie de Widal. Trois patientes ont eu une eosinophilie sanguine > 1000/mm 3 . Le VEMS moyen etait a 40,4 % (29–60), avec un declin moyen de 371 mL/an (54–101). Le scanner thoracique revelait des condensations sous pleurales bi-apicales, un epaississement parietal bronchique tres marque avec DDB. Le LBA montrait une alveolite a neutrophiles dans 3 cas. Deux patientes ont beneficie d’une biopsie pulmonaire chirurgicale et 2 ont necessite une transplantation pulmonaire en raison d’une insuffisance respiratoire. L’analyse des explants et des biopsies montrait une fibroelastose essentiellement centrolobulaire remaniant et stenosant les bronches et bronchioles, en association avec des lesions compatibles avec l’asthme (hypercrinie, lame basale epaisse). La plevre et l’espace sous-pleural etaient majoritairement epargnes. Une patiente a eu des biopsies bronchiques, montrant un aspect identique. Conclusion La fibroelastose bronchocentrique est differente de la « fibrose centree sur les voies aeriennes » decrite en 1999 par la predominance de l’elastose. Elle differe de la FEPP par la predominance des lesions bronchiques. Nous proposons d’identifier la fibroelastose bronchocentrique comme une entite compliquant l’inflammation chronique des voies aeriennes observee dans l’asthme.

Introduction Des auto-anticorps diriges contre des antigenes epitheliaux sont souvent detectes chez les patients atteints de fibrose pulmonaire idiopathique (FPI) et posent la question du role d’une auto-immunite muqueuse dans la maladie. Nous decrivons un sous-groupe de patients atteints de pneumopathie interstitielle diffuse idiopathique (PIDI) qui presentent des Ac anti-cellules parietales gastriques (anti-CPG). Methodes Les patients atteints de PIDI vus entre 04/2007 et 12/2014 presentant des anti-CPG positifs sur le bilan diagnostique et sans connectivite definie ont ete identifies. Nous avons recueilli retrospectivement leurs caracteristiques cliniques, biologiques, EFR, tomodensitometriques et evolutives. Resultats Dix-neuf patients ont ete identifies (16 hommes, âge moyen 75 ans, 53 % anciens fumeurs). Le titre median des anti-CPG etait au 1/320e (max > 1280e, min 80e). Douze patients (63 %) presentaient d’autres auto-anticorps a des titres faibles (11 AAN (mediane au 1/80e), 1 p-ANCA anti-MPO, 1 anti-thyroide). Une maladie de Biermer etait presente dans 38 % des cas, et preexistait au diagnostic de fibrose dans 60 % des cas. Lors de l’endoscopie gastrique, 36 % etaient porteurs d’Helicobacter pylori, 85 % presentaient une gastrite atrophique. L’aspect tomodensitometrique etait celui de pneumopathie interstitielle commune certaine (11, 58 %) ou possible (4, 21 %) ou etait inclassable (4, 21 %). Le diagnostic final etait FPI certaine dans 14 cas (73 %) et fibrose inclassable dans 5 cas qui n’avaient pas beneficie de biopsie pulmonaire. Le LBA retrouvait une lymphocytose > 15 % dans 38 % des cas. Avec un suivi moyen de 22 mois, 11 % ont presente une exacerbation et 7 (37 %) sont decedes. Conclusion Nous avons identifie un sous-groupe de patients avec une PIDI (essentiellement FPI) presentant une auto-immunite muqueuse anti-cellules parietales gastriques. La fibrose pulmonaire pourrait favoriser la genese de ces auto-anticorps etant donne l’origine embryologique commune aux voies digestives et respiratoires.