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CT scans of the paranasal sinuses and orbits of 14 patients with Wegener's granulomatosis (WG) are reviewed. Plain film radiographs of the paranasal sinuses demonstrated non specific findings in 13 patients. CT findings showed nonspecific findings including thickened mucosa, sinus opacification in 13 patients. Six patients had also more specific findings, including bony erosion or destruction, ossification, perforation of the nasal septum, retroorbital mass. CT scan is useful in initial staging and treatment planning of patients with Wegener's granulomatosis and ENT involvement, especially in limited forms affecting upper respiratory tract.

Granuloma formation in the lung of patients with sarcoidosis is preceded by the accumulation of large numbers of activated T-lymphocytes, which results, at least in part, from the proliferation of T-lymphocytes within the lung. To determine whether the increased proliferation of lung T-lymphocytes in sarcoidosis results from a failure of mechanisms responsible for limiting their proliferation, we have compared the ability of purified T-lymphocytes present in bronchoalveolar lavage fluid and peripheral blood from normal subjects and patients with sarcoidosis to respond to proliferative signals. The mitogen-induced proliferative response of lavage T-lymphocytes from normal subjects and patients with sarcoidosis was similar, but the response of lavage T-lymphocytes was much less than that observed using normal or sarcoid blood T-lymphocytes. The reduced proliferative response of sarcoid lavage T-lymphocytes could not be overcome by addition of accessory cells or exogenous interleukin-2, and it did not result from the presence of suppressor cells. Furthermore, sarcoid lavage lymphocytes are capable of being activated by "mitogenic" signals, as indicated by the ability of phytohemagglutinin to induce expression of the 4F2 surface antigen and stimulate interleukin-2 production. However, the expression of interleukin-2 receptors was reduced on stimulated sarcoid lavage T-lymphocytes compared with that observed on normal blood T-lymphocytes, which may account in part for their reduced proliferative capacity. Because mechanisms that render lavage T-lymphocytes from normal subjects refractory to proliferative signals appear to operate in sarcoidosis as well, these findings suggest that a defect in the inhibition of T-lymphocyte proliferation is unlikely to be an important cause of lymphocyte accumulation in this disorder.

Neopterin is a metabolite of guanosine-triphosphate, released in vitro by macrophages under the control of gamma-interferon and described as a marker of T cell activation in vivo. We have compared the urinary neopterin/creatinine ratio (mumol/mol) in patients with pulmonary sarcoidosis (n = 66), interstitial lung diseases other than sarcoidosis (nonsarcoid ILD, n = 35), and 45 normal control subjects. For the sarcoid population as a whole, urinary neopterin was higher (496 +/- 52 mumol/mol [mean +/- SEM]) than in control subjects (126 +/- 5 mumol/mol) (p less than 0.001). In patients with nonsarcoid ILD, urinary neopterin was frequently higher in granulomatous and/or lymphoproliferative diseases (hypersensitivity pneumonitis, tuberculosis, primitive Sjögren's syndrome, and malignant lymphomas) (781 +/- 193 mumol/mol, n = 10) but remained normal in other types of nonsarcoid ILD [( 163 +/- 14 mumol/mol, n = 25]: histiocytosis X, idiopathic pulmonary fibrosis, lung collagen-vascular diseases, diffuse neoplasms, pneumoconiosis; p less than 0.001 compared with sarcoidosis). We have also evaluated the relationship between urinary neopterin and the clinical or biologic markers currently used to assess sarcoidosis: alveolar lymphocytosis in lavage fluid (ALY), 67-gallium scan semiquantitative index (67Ga), or serum angiotensin-converting enzyme (SACE). Sarcoid patients with the highest urinary neopterin were those in whom mean values of these markers were the highest (p less than 0.05, all comparisons). Patients with positive markers (i.e., either clinical expression of sarcoidosis-ALY greater than 30%-67Ga greater than 20-SACE greater than 60 U/ml) had significantly higher urinary neopterin levels than did other sarcoid patients (p less than 0.05, all comparisons).

Objective The prognosis of patients with idiopathic pulmonary fibrosis or fibrosing idiopathic nonspecific interstitial pneumonia undergoing invasive mechanical ventilation (MV) for acute respiratory failure is known to be poor. The issue of life support in these patients needs to be reconsidered in light of changes during the past decade in ventilator settings and in the management of acute exacerbation. We therefore aimed to reassess the prognosis of such patients. Methods We retrospectively assessed the outcomes of all medical patients with idiopathic pulmonary fibrosis or fibrosing idiopathic nonspecific interstitial pneumonia who required invasive MV in 3 university hospitals in the Paris area from January 2002 to April 2009. Results In total, 27 patients (mean age, 66 ± 12.8 years) required invasive MV in the intensive care unit: 8 (30%) were successfully weaned from MV, and 6 and 4 were discharged from the intensive care unit and the hospital, respectively. Survivals for patients who did not undergo lung transplant were 22%, 3.7%, and 3.7%, at 30 days, 6 months, and 12 months, respectively. Conclusions We confirm that use of invasive MV for acute respiratory failure in patients with idiopathic pulmonary fibrosis or fibrosing idiopathic nonspecific interstitial pneumonia is associated with a high mortality; however, a subset of patients may be discharged alive from the intensive care unit and hospital, providing an opportunity to consider lung transplant in case of eligibility. Our results suggest that invasive MV should not be systematically denied to these patients but discussed on a case-by-case basis.

Summary Background The present survey coordinated by the French expert centres for rare pulmonary diseases investigated French pulmonologists' current diagnostic and therapeutic practice for idiopathic pulmonary fibrosis (IPF). Methods From December 7, 2011 to February 18, 2012, all French pulmonologists ( n = 2608) were contacted. Those who reported following up at least one IPF patient ( n = 509) were administered a 26-item questionnaire by phone or e-mail. Results 509 pulmonologists (41% of responders, 20% of French pulmonologists) were involved in the management of IPF patients. Of those, 36% discussed the cases with radiologists and pathologists. Out of 406 community pulmonologists practicing outside of reference or competence (e.g. expert) centres, 141 (35%) indicated referring patients to those centres. The 2011 international guidelines for IPF were known by 67% of pulmonologists involved in IPF, 84% of whom considered them appropriate for practice. About 58% of patients were diagnosed with mild to moderate IPF as defined by percentage predicted forced vital capacity ≥50% and percentage predicted diffusing capacity of the lung for carbon monoxide ≥35%. Management resulted from multidisciplinary discussion in 36% of the cases. By the end of December 2011, 49% of patients with mild to moderately severe IPF were treated with oral corticosteroids, and 27% received no treatment. Conclusions Despite correct awareness of international IPF guidelines, modalities of multidisciplinary discussion and of early diagnosis and management need to be improved through the network of expert centres.

Sarcoidosis is frequently attended by hypercalciuria and sometimes by hypercalcaemia. The type of hypercalciuria and its relationships with disease extension and activity have rarely been investigated. In order to clarify these issues we undertook an investigation by a calcium absorption test in 39 patients with untreated thoracic sarcoidosis, and tried to establish correlations with vitamin D3 metabolism and some features of the disease. We found three types of responses. Group I (n = 12) with a normal test had normal 1,25-(OH)2D3 and rare extrathoracic localisations. Group II (n = 14) with absorptive hypercalciuria had higher serum calcium; 1,25-(OH)2D3 (p less than 0.001) and the free 1,25-(OH)2D3 index (p less than 0.05) were raised. Sarcoidosis was more often inflammatory, developing and disseminated. Group III (n = 13) had resorptive hypercalciuria, and hypercalcaemia was frequent. 1,25-(OH)2D3 (p less than 0.01) and the free 1,25-(OH)2D3 index (p less than 0.05) were raised but to the same degree as in Group II. Sarcoidosis was more disseminated and developing than in Groups I and II. In the 39 patients, iPTH and nephrogenous cAMP were low. Post-calcium load urinary calcium/creatinine (Ca/Cr) and 1,25-(OH)2D3 were correlated (p less than 0.05). Extrathoracic extension was associated with higher fasting urinary Ca/Cr (p less than 0.001), and development with higher post-load urinary Ca/Cr (p less than 0.001). Thus, absorptive hypercalciuria is related to the development of sarcoidosis and can be explained by high free 1,25-(OH)2D3, while resorptive hypercalciuria seems to be linked with disease extension. In such a case, the mechanism of osteolysis is not solely accounted for by high 1,25(OH)2 vitamin D3 serum levels, and we postulate that some other factor is at work, related to the extent of the granulomatous process.

Cigarette smoking produces marked alterations in the lung parenchyma and in the population of immune and inflammatory cells present in the lower respiratory tract. These cigarette-induced changes appear to influence the incidence of two different interstitial lung diseases, histiocytosis X and sarcoidosis. Smoking is a strong risk factor for the development of pulmonary histiocytosis X, since the incidence of smoking is very high among patients with histiocytosis X: 90% of the patients with histiocytosis X were smokers; 46% of the controls were smokers (p less than .001). In contrast, smoking appears to reduce the incidence of sarcoidosis: 31% of the patients with sarcoidosis were smokers (p less than .05 compared to controls). In an effort to understand how cigarette smoking influences the incidence of these two disorders, we compared the numbers and types of immune and inflammatory cells recovered by bronchoalveolar lavage from nonsmoking and smoking controls and patients with histiocytosis X and sarcoidosis. Although nonsmoking patients with histiocytosis X did not have a significant increase in the number of alveolar macrophages recovered by lavage (p greater than .2 compared to normals), smoking patients had an increase in the number of alveolar macrophages similar to that observed in the control population. In contrast, the number of macrophages recovered from patients with sarcoidosis who smoked was considerably less than that observed in normal smokers (p less than .05 comparing patients with sarcoidosis and controls who smoked 1-20 cigarettes/day). This difference in the intensity of the cigarette-induced macrophage alveolitis observed in the two patient groups may be important in explaining the opposite effects of cigarette smoking on the incidence of histiocytosis X and sarcoidosis.

To examine the possibility that mast cells have a central role in the pathogenesis of hypersensitivity pneumonitis, 20 patients with this disease were studied with the aim of seeking evidence for mast cell degranulation. The number of mast cells recovered by bronchoalveolar lavage from patients with hypersensitivity pneumonitis was more than 1,000 times greater than those recovered from normal individuals. Furthermore, discontinuation of antigen exposure resulted in an increase in the number of mast cells observed, consistent with the possibility that antigen exposure had induced mast cell degranulation. Cessation of antigen exposure also resulted in a rapid decrease in the number of neutrophils and eosinophils recovered by lavage, followed by an increase in the number of T8+ T lymphocytes present. In each case the time course of the changes was consistent with the possibility that mast cell degranulation had been important in regulating the number of the immune and inflammatory cells present in the lung. Histamine was present in lavage fluid supernatant from patients with hypersensitivity pneumonitis. The amount of histamine present was, however, closely correlated with the number of mast cells present and not with the interval since last antigen exposure. Delay in separating cells from lavage fluid supernatant resulted in an increase in histamine content. These results suggest that the free histamine in lavage fluid resulted from the degranulation of mast cells induced by the lavage procedure as histamine released in vivo has a short half life. We suggest that hypersensitivity pneumonitis results from a "late phase reaction" initiated by antigen induced mast cell degranulation.

Thirty-three cases of sarcoidosis (4.4% of 746 patients) showed an alveolar radiological pattern. A study of pulmonary function was carried out in 25 patients and compared with that of 46 patients with the interstitial radiological type of sarcoidosis. Twenty-two cases have been followed up from one to six years after the initial examination. The radiographic lesions were most often bilateral and included nodules greater than 15 mm with ill-defined margins or diffuse, infiltrative, non-retractile opacities with fluffy margins. Bilateral mediastinal lymph nodes were present in 27 patients. In 20 patients an associated reticulation was found on radiography. In four patients an open lung biopsy was done. The granulomatous nodules were identical to those found in other forms of sarcoidosis, although they were more confluent in the affected areas. Clinical and functional findings did not differ from those in the more common forms of sarcoidosis. Alveolar sarcoidosis has a sudden course. The alveolar radiological patterns always disappeared, with or without steroid treatment, while reticular patterns persisted in four patients. Rapid radiological changes were observed. Some functional abnormalities persisted in cases that were followed. It is concluded that alveolar sarcoidosis is a distinct acute form of sarcoidosis.

In order to clarify the significance of anginal pain and myocardial thallium-201 scan defects in cardiac sarcoidosis, the pharmacologic effect of dipyridamole on myocardial perfusion was assessed by planar thallium-201 myocardial scintigraphy in patients with sarcoidosis.Thallium-201 myocardial scintigraphy was performed at rest and after 0.56 mg/kg intravenous dipyridamole during four minutes in 16 patients with sarcoidosis. The myocardial scan (45-degree and 70-degree left anterior oblique, and anterior views) was divided into 15 segments. Results were evaluated by the number of segmental defects and with a global perfusion score (from 0 to 60) by a semi-quantitative index depending on the size and severity of myocardial thallium-201 defects.Thirteen of the 16 patients showed partial or total reversion of their thallium-201 defects on redistribution scanning either at rest or after dipyridamole. The mean (+/- SD) number of myocardial perfusion defects that were present in all the patients decreased from 5.31 +/- 1.78 at rest to 3.25 +/- 2.52 after redistribution (p less than 0.001) and to 2.19 +/- 2.10 after dipyridamole (p less than 0.001). The mean global perfusion score increased from 53.2 +/- 3.0 at rest to 56.2 +/- 2.9 after redistribution (p less than 0.001) and to 57.2 +/- 2.7 after dipyridamole (p less than 0.001). A significant correlation (r = 0.82, p less than 0.001) was found between the increase of global perfusion score on redistribution and after dipyridamole.The reversibility of myocardial scan defects is a common finding in sarcoidosis. It makes unlikely the role of scar fibrosis or extensive confluent granulomas as a mechanism for such defects. The effect of dipyridamole suggests the presence of reversible disorders lying at the coronary microvascular level.

Abstract 2407 Poster Board II-384 Introduction: Although sarcoidosis is far from being a classical cause of secondary-immune thrombocytopenic purpura (ITP), isolated thrombocytopenia may occur during the course of the disease. To better assess the main characteristics and outcome of patients with a sarcoidosis-associated ITP, a survey was initiated throughout the French study group on sarcoidosis and the national referral center for adult's immune cytopenias. The data of the 20 first cases are reported. Patients and Methods: To be included, all patients had to fulfil the following criteria: 1) Definite diagnosis of sarcoidosis according to the standard international criteria (Am J Respir Crit Care Med 1999; 736-55) and 2) Presence of an immune thrombocytopenia according to the ASH criteria twith platelet count below 100 × 109/L on at least two separate occasions 2 weeks. Drug-induced thrombocytopenia were excluded as well as patients with a hypogammaglobulinemia suggesting a underlying common variable immunodeficiency (CVI) and thrombocytopenia related to hypersplenism. All clinical and biological data were reviewed and analyzed by the same investigator by using a standard form. A complete response (CR) was defined as sustained (> 3months) platelet count > 100 × 109/L with at least a twofold increase for baseline. A partial response (PR) was defined as a platelet count 30×109/L or more and at least twice the initial value. Results: Twenty patients (50% men) were included. Median age at ITP diagnosis was 37.5 years (21 to 83) and median age at sarcoidosis diagnosis was 36 yrs (10 to 83). In 4 patients, ITP preceded the diagnosis of sarcoidosis (median: 67.5 months; 15 to 153). In 11 patients (55%), ITP occurred in the course of prior diagnosed sarcoidosis with a median of 48 mths (6 to 216), and concomitantly with the diagnosis of sarcoidosis in 5 patients (25%). In 13 patients (65%), an active localization of sarcoidosis was present at the diagnosis of ITP. Median platelet count at onset was 11×109/L (3 to 90) and nadir platelet count was 10×109/L (1 to 60). Seven patients (35%) had severe (mucosal) bleeding manifestations and a bleeding score ≥ 8 (described by Khellaf et al). Regarding sarcoidosis, during the follow up, 17 patients (85%) had thoracic involvement, and an extra-thoracic involvement was found in 14 patients (70%) including: eye (n=7), liver (n=5), sinus (n=4), peripheral lymph nodes (n=4), skin (n=3), salivary gland (n=2), spleen (n=1) and kidney (n=1). Nineteen out of the 20 patients were treated specifically for their secondary ITP. After the first line therapy (prednisone at 1mg/kg/day for at least 3 consecutive weeks in all patients associated with IVIg in 10 patients), 12/19 (63%) patients achieved a complete response (CR), 6 a partial response (PR) (32%) and only 1 patient failed to respond. The course of ITP was chronic in 4 patients, among them 2 underwent splenectomy and achieved a CR and 2 were treated with Rituximab (375mg/m2, 4 infusions) and achieved a PR at one year. After a median follow up of 70 months (12 to142), 18 patients (90%) were in CR and 2 in PR with chronic ITP. Eight patients were in remission off therapy whereas 12 patients (60%) were still on corticosteroids at time of analysis with a median dose of 10mg/day (5 to 30). During the follow-up period, 11 patients (55%) had a relapse or flare of sarcoidosis (intra thoracic in 5 patients, extra-thoracic in 6 patients). In 2 patients, a simultaneous relapse of both sarcoidoisis and associated-ITP was observed. Comments: Our preliminary data suggest that the association of sarcoidosis and ITP is not fortuitous and that sarcoidosis should be considered as a potential cause of secondary ITP. ITP in this setting is frequently initially severe and symptomatic and therefore requiring treatment. The overall response rate to treatments commonly used in primary ITP is however good and the long term outcome is favorable suggesting that patients with sarcoidosis-associated ITP should be managed as patients with primary ITP. In contrast, relapse of sarcoidosis is frequent and frequently affect extra-thoracic sites. That a majority of the patients had an active and/or relapsing sarcoidosis during the course of ITP suggests that granuloma may be a predisposing condition for triggering autoimmunity and especially ITP as observed in some cases of CVI-associated immune thrombocytopenia. Disclosures: No relevant conflicts of interest to declare.

Core-needle biopsy guided by ultrasound can be performed for investigating peripheral lymph node (PLN). The aim of this study was to determine the efficacy of this technique in sarcoidosis.Retrospective review of files of all patients in the database of the radiology department of Avicenne university hospital who underwent PLN biopsies guided by ultrasound from January 2008 to June 2011 (n=292). Cases with either granulomas at histology with the procedure or with a final diagnosis of sarcoidosis were included in the study.The histological specimens were adequate in 282 out of 292 cases (96%) showing non-caseating granulomas in 22 cases (n=20 patients with a final diagnosis of sarcoidosis and n=2 patients with tuberculosis). After reviewing clinical files of the 282 patient, 22 were confirmed to have sarcoidosis, at initial presentation (n=19) or later during flare-up or relapse (n=3) with only 2 patients having no granuloma on PLN biopsy. PLN were palpable in 18 cases and only detected by (18F)FDG-PET/CT showing increased PLN uptake in 4 cases. The sensitivity and specificity of adequate biopsy were 91 and 99% and the positive and negative predictive values were 91 and 99%, respectively.Core-needle biopsy guided by ultrasound has a high efficacy for evidencing granulomas in sarcoidosis patients with PLN involvement either clinically palpable or in the presence of (18F)FDG-PET/CT uptake.

Sacroidosis is a multisystemic immune disorder of unknown cause characterized by the formation of epithelioid granuloma in involved organs particularly the lung and the lymphatic system. Consistent recent advances have been made on the clinical, therapeutical and pathogenetic aspects. Clinical aspects : Various clinical phenotypes are better characterized and efforts to grade the severity could facilitate genetic studies and therapeutic trials. Pulmonary hypertension and some infrequent or atypical pulmonary manifestations as well as some rare extrapulmonary localizations have been thoroughly studied. Sarcoidosis-like granulomatosis due to identified causes constitute interesting models and have to be differentiated from sarcoidosis. Therapeutical aspects: Anti-TNFα drugs have been investigated and infliximab has been proved efficient even though some limits to its efficiency have to be taken into account. Pathogenetic aspects: Finally, advances concerning the pathogenesis, particularly genetic predisposing factors and phenotype/genotype correlations have been made.

The aim of this study was to investigate the relationship between the pulmonary vitamin D metabolism in tuberculosis and the calcium metabolism abnormalities and other clinical characteristics of the disease. The metabolism of 25-hydroxyvitamin D3 (25(OH)D3) by alveolar immune cells recovered by bronchoalveolar lavage (BAL) was evaluated in parallel to the results of calcium metabolism, 25(OH) D and 1,25 dihydroxyvitamin D (1,25(OH)2D) plasma levels and other clinical parameters obtained in 14 tuberculosis patients. Whilst predominant metabolites produced by lavage cells in patients and controls were 5(E)--and 5(Z) -19-nor-10-oxo-25(OH)D3, 1,25(OH)2D3 was produced by cells from all tuberculosis patients but not by cells from controls. Calcium metabolism abnormalities were observed in only some patients, but the production of 1,25 (OH)2D3 by lavage cells was found to correlate both with 1,25(OH)2D levels (r = 0.67) and post-load urinary calcium excretion (r = 0.59). 1,25(OH)2D3 production by lavage cells was increased in patients of black origin, and those presenting with hilar adenopathy without pulmonary infiltrates, and was correlated with the number of lymphocytes recovered by lavage (r = 0.87). We conclude that 1,25(OH)2D3 production by alveolar immune cells makes a major contribution to the abnormalities in calcium metabolism seen in tuberculosis patients, and may be partly dependent on the clinical characteristics evaluated here.

We report a case of amyloidosis, restricted to the lower respiratory tract, with prominent tracheobronchial involvement and bilateral hilar lymphadenopathy, associated with a monoclonal serum protein of the immunoglobulin G (IgG) lambda type. The careful search for an extrathoracic site of involvement was negative. This particular association has not been reported previously.

Objectives To assess the value of major histocompatibility complex (MHC) class II antigen (HLA-DR) expression to distinguish anti-synthetase myopathy (ASM) from dermatomyositis (DM). Methods Muscle biopsies from patients with ASM (n = 33), DM without anti-synthetase antibodies (ASAb) (n = 17), and normal muscle biopsy (n = 10) were first reviewed. ASAb included anti-Jo1 (26/33), anti-PL12 (4/33), anti-PL7 (2/33), and anti-EJ (1/33). Immunohistochemistry was performed for MHC-I/HLA-ABC, MHC-II/HLA-DR, membrane attack complex (C5b-9), neural cell adhesion molecule (NCAM)/CD56 expression, and inflammatory cell subsets. Twenty-four ASM and 12 DM patients from another center were added for HLA-DR evaluation. Results Ubiquitous myofiber HLA-ABC expression was equally observed in ASM and DM (93.9% vs 100%, NS). In contrast, myofiber HLA-DR expression was found in 27/33 (81.8%) ASM (anti-Jo1: 23/26, 88.5%; others: 5/7, 71.4%) vs 4/17 (23.5%) DM patients (p

Ectopic formation of secondary lymphoid tissue is initiated by the local attraction of naive T and B cells. In this study, we describe a novel type of organized lymphoid structure in the lung of human idiopathic pulmonary fibrosis, with key features of lymphoid neogenesis, including: 1) recently activated CD40 ligand (CD40L)+ T cells; 2) variable numbers of activated CD40+/CD40L+ B cells, sometimes organized in follicles; 3) fully mature dendritic cells (DC) expressing CD40, CD83, CD86, and DC-lysosome-associated membrane protein; 4) the expression of the chemokine CCL21; 5) the presence of vessels with characteristics of high endothelial venules; and 6) a dense network of follicular DC. Surprisingly, these structures are devoid of CCR7+ naive T cells, proliferating lymphocytes, and germinal centers, suggesting that newly recruited activated DC and Ag-experienced lymphocytes can drive lymphoid neogenesis and that factors present within the lymphoid aggregates, such as CD40L, are essential to induce DC maturation.

A case of sarcoidosis with iterative spontaneous increase and decrease of thoracic lymphadenopathy is described. No condition such as infectious disease, particular environmental exposure or medication could explain this unusual course of thoracic sarcoidosis.

Auteur groupe collaboratif RaDiCo team; International audience; Introduction: Pirfenidone, an antifibrotic medication for idiopathic pulmonary fibrosis (IPF), is now available in France in two formulations: tablets since April 2018, and the initial capsules form. We conducted a cohort study to describe tolerance and acceptability of capsules and/or tablets of pirfenidone in patients with IPF.Methods: This study was nested within the French, non-randomized, multicenter RaDiCo-ILD (Rare Disease Cohort-Interstitial Lung Diseases). Included patients with IPF received at least one dose of pirfenidone tablets or capsules from July 2017 to June 2019 in three populations: the inclusion population (patients treated at least once with pirfenidone during the study period, n = 288); the potential switch population (patients treated with pirfenidone during the switch period starting April 2018, n = 256); the newly treated population (patients who initiated pirfenidone during the study period, n = 162). Each of those last two populations included three subgroups (tablets, capsules, and substitution).Results: In 288 patients treated, 162 newly initiated pirfenidone during the study period: there were no meaningful differences in the baseline characteristics with the 256 patients treated during the potential switch period. In the newly treated population, 30.3% started pirfenidone treatment with tablet formulation. In the potential switch population, 44.9% of patients shifted from capsule to tablet. Half of the patients shifted to tablet formulation within the first 10 months. The mean treatment duration was 21.5 months with a mean dose of 2106.7 mg/day; 46.5% of patients discontinued treatment, mainly because of adverse events. There were fewer discontinuations in the tablets and substitution subgroups than in the capsules-only subgroup. The most reported adverse event was skin rash (11.5%). No new adverse event was identified.Conclusions: This real-life cohort assessing the characteristics of the prescription of pirfenidone tablets and capsules suggests a good acceptability of the tablet formulation by patients with IPF.Trial registration: Clinical trial registered with www.clinicaltrials.gov (NCT04238871).