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Background: Since the previous French guidelines were published in 2017, substantial additional knowledge about idiopathic pulmonary fibrosis has accumulated., Methods: Under the auspices of the French-speaking Learned Society of Pulmonology and at the initiative of the coordinating reference center, practical guidelines for treatment of rare pulmonary diseases have been established. They were elaborated by groups of writers, reviewers and coordinators with the help of the OrphaLung network, as well as pulmonologists with varying practice modalities, radiologists, pathologists, a general practitioner, a head nurse, and a patients' association. The method was developed according to rules entitled "Good clinical practice" in the overall framework of the "Guidelines for clinical practice" of the official French health authority (HAS), taking into account the results of an online vote using a Likert scale., Results: After analysis of the literature, 54 recommendations were formulated, improved, and validated by the working groups. The recommendations covered a wide-ranging aspects of the disease and its treatment: epidemiology, diagnostic modalities, quality criteria and interpretation of chest CT, indication and modalities of lung biopsy, etiologic workup, approach to familial disease entailing indications and modalities of genetic testing, evaluation of possible functional impairments and prognosis, indications for and use of antifibrotic therapy, lung transplantation, symptom management, comorbidities and complications, treatment of chronic respiratory failure, diagnosis and management of acute exacerbations of fibrosis., Conclusion: These evidence-based guidelines are aimed at guiding the diagnosis and the management in clinical practice of idiopathic pulmonary fibrosis., (Copyright © 2022 SPLF. Published by Elsevier Masson SAS. All rights reserved.)

Background: Since the previous French guidelines were published in 2017, substantial additional knowledge about idiopathic pulmonary fibrosis has accumulated., Methods: Under the auspices of the French-speaking Learned Society of Pulmonology and at the initiative of the coordinating reference center, practical guidelines for treatment of rare pulmonary diseases have been established. They were elaborated by groups of writers, reviewers and coordinators with the help of the OrphaLung network, as well as pulmonologists with varying practice modalities, radiologists, pathologists, a general practitioner, a head nurse, and a patients' association. The method was developed according to rules entitled "Good clinical practice" in the overall framework of the "Guidelines for clinical practice" of the official French health authority (HAS), taking into account the results of an online vote using a Likert scale., Results: After analysis of the literature, 54 recommendations were formulated, improved, and validated by the working groups. The recommendations covered a wide-ranging aspects of the disease and its treatment: epidemiology, diagnostic modalities, quality criteria and interpretation of chest CT, indication and modalities of lung biopsy, etiologic workup, approach to familial disease entailing indications and modalities of genetic testing, evaluation of possible functional impairments and prognosis, indications for and use of antifibrotic therapy, lung transplantation, symptom management, comorbidities and complications, treatment of chronic respiratory failure, diagnosis and management of acute exacerbations of fibrosis., Conclusion: These evidence-based guidelines are aimed at guiding the diagnosis and the management in clinical practice of idiopathic pulmonary fibrosis., (Copyright © 2022 SPLF. Published by Elsevier Masson SAS. All rights reserved.)

We report the first description of a Sjögren's syndrome associated with nectrotizing sarcoid-like granulmatosis. A 62-year old woman was admitted for diagnostic exploration of fatigue, weight loss, and fever at 38 degrees C which had progressed for more than 3 months. Chest X-ray showed several pulmonary opacities. There was a history of tuberculosis and thyroiditis at the age of 20 years. Physical examination revealed a sicca syndrome. There was no objective evidence of respiratory disease. Her heart and chest were clear on auscultation. The patient had autoantibodies for SSa and SSb specific antigens. Minor salivary gland biopsy gave a score of 3 in the Chisholm classification. Chest X-ray and CT-scan showed diffuse infiltrative opacities which were dense and peripheral with no retraction. Bronchoalveolar lavage, and bronchial and transbronchial biopsies were non-specific. Surgical lung biopsy showed typical aspects of necrotizing sarcoid-like granulomatosis. After high-dose pulse corticosteroid therapy, the opacities disappeared with no recurrence under oral steroids at three years.

It is well known that silica exposure leads in an experimental model to the development of an acute fibrotic process. In human beings two main observations have already been done: (1) silica exposure is frequently associated with the development of connective tissue disease (CTD), especially progressive systemic sclerosis; (2) 10 to 20% patients with CTD developed pulmonary fibrosis. In this context we report 26 cases of coal miners who presented with clinical, radiological, biological and functional characteristics mimicking idiopathic pulmonary fibrosis (IPF), with or without associated coal worker's pneumoconiosis (CWP). All were men; mean age was 68 +/- 9.2 years. Twenty-three were smokers. Duration of exposure was 28.8 +/- 9.1 years. All the patients had dyspnea (stage III, IV in the NHYA classification) and diffuse crackles. Eleven out of 26 had finger clubbing. Computed tomography showed honeycombing (23 cases), and/or ground glass opacities (6 cases) with bronchiectasis (3 cases) predominant in the lower lobes; 19 had radiological signs of CWP, micronodules (n = 16) and nodules (n = 3) predominant in the upper lobes. BAL exhibited an increased % of neutrophils (11.9 +/- 16.1%). Lung function demonstrated a restrictive pattern (TLC = 73 +/- 15.6% and VC = 80 +/- 18% of predicted values) associated with a decreased DLCO (51.8 +/- 23.6% of predicted values) and hypoxemia (at rest = 66.5 +/- 11.2 mmHg, upon effort = 56 +/- 12 mmHg). Lung biopsies were performed in four cases and demonstrated interstitial fibrosis of intraalveolar septum with an accumulation of immune and inflammatory cells similar to the one described in IPF. The association between IPF and silica exposure with or without associated CWP points out the problem of legal recognition of idiopathic-like pulmonary fibrosis as a complication of the occupational exposure of coal workers.

Purpose: To evaluate the accuracy of spiral CT angiography in the diagnosis of central pulmonary embolism (PE)., Material and Methods: A retrospective study was undertaken in a population of 90 patients who were evaluated with spiral CT angiography (acquisition: 12 to 24 s with or without strict apnea; injection of 90 cm3 of 12, 20 or 30% contrast material at a rate of 4 to 7 cm3, selective pulmonary angiography of each lung (n = 55) and/or ventilation-perfusion (VP) scanning (n = 35)., Results: Among the 55 patients evaluated with both spiral CT and angiography, central pulmonary embolism was excluded in 19 patients (34%), assessed in 29 patients (53%) whereas CT examination was considered as inconclusive in 7 patients (13%) due to interpretive difficulties at the level of obliquely oriented arteries and/or presence of hilar lymph nodes (sensitivity: 90.5%; specificity: 82.6%). With spiral CT, the finding of 95 central emboli (3 main, 61 lobar and 31 segmental) corresponded exactly to the angiographic findings; spiral CT enabled direct visualization of intraluminal filling defects (n = 95) whereas the angiographic recognition of PE was based on direct (n = 57) and indirect (n = 38) signs. Spiral CT angiography was more sensitive and specific than VP scanning., Conclusion: Spiral CT angiography appears as a sensitive and specific noninvasive method for the diagnosis of central PE.

We report a case of a patient of 58 years old who suffered from a left apical opacity occurring in a context of deterioration in general health. Subsequent clinical developments were dominated by the ophthalmic disease and a frontal syndrome. The radiological work-up showed tumoral lesions which had developed from the first rib, from the structures at the base of the cranium and the frontal area and were associated with multiple lacunae of the cranial vault. There was evidence of hyperproteinaemia, and an IgG gamma monoclonal gammopathy, a significant medullary plasmocytosis with morphological anomalies of the plasmocytes leading to a diagnosis of myeloma with a plasmocytoma of the rib and the retro-orbital area. Thoracic disease associated with this pathology is common but mainly present as osteolytic lesions. The occurrence of intrathoracic plasma cell tumours is rarer as are orbital manifestations. In spite of treatment which can frequently lead to an objective response, the prognosis of this disorder remains gloomy.

Spiral scanning (BSV) is a newly acquired scanning technique for all or part of the thoracic volume in a single breath-hold. It offers many advantages when compared to conventional CT scanning. The study of the lungs with a single breath-hold avoids the inconvenience of an anatomical discontinuity while acquiring the information. In a population of patients presenting with multiple, or solitary pulmonary nodules, or the absence of nodule, BSV shows 30 to 40% of supplementary nodules when compared to conventional scanning techniques. BSV enables the optimal use of contrast products in which the iodine is poorly concentrated to study the pulmonary vessels. Certainly this technique will not replace pulmonary angiography for the diagnosis of pulmonary emboli. However, it may be used first in certain situations, dispensing with invasive angiography in patients with an increased risk, or in the initial assessment of patients with pulmonary arterial hypertension of unknown aetiology, or to follow a previously documented pulmonary emboli. The correlation between spiral scanning and conventional angiography for the diagnosis of pulmonary emboli is excellent, to the level of segmental arteries. Finally the continuity, both anatomically and for the lesions obtained by BSV is such that it is now possible to apply to thoracic pathology techniques of multiplanar and three dimensional reconstruction. If all the information is contained in conventional transversal imaging slice by slice, not everything is perceived by the observer because the information is inconveniently presented because the anatomical and lesional picture is deconstructed. The reconstruction of the volume inspected in coronal, sagittal, oblique or three-dimensional viewing, furnishes supplementary information and announces the arrival of trachea-bronchography, and non-invasive angiography three-dimensionally. In parallel to the expected progress in IRM angiography, three-dimensional angio-CT of the thoracic vessels after appropriate evaluation is going to be substituted for more invasive techniques which are currently used.

Systemic diseases such as scleroderma (ScI), rheumatoid polyarthritis (PR), Gougerot-Sjögren Syndrome (GS) have a well known propensity for the lungs. Previous studies have shown evidence of disturbed alveolar cell repair as evidence of a sub-clinical alveolitis. The significance of such cases of latent alveolitis remains to be specified. To determine if latent alveolitis was associated with interstitial chest disease which was undetectable by chest X-ray, 36 consecutive patients had an BAL and a high resolution computered tomographic examination (HRTC) (Scl: n = 21; PR: n = 9; GS: n = 6). The patients had normal respiratory function and chest X-ray was normal. Our results showed 17 out of 36 (47%) with a latent alveolitis (the percentage of lymphocytes and of alveolar polymorpho-nuclear neutrophils was superior or equal to 18 and 4% respectively) (Scl: 12/21; PR: 1/9; GS: 4/6). In the cases of scleroderma a neutrophil alveolitis was predominant (9/12) and was associated in 2 cases with a honeycomb lung and evidence of fibrotic lesions using TDM-HR. Those examinations using HRTC which were normal were equally associated with a latent alveolitis (Scl: 6/12; PR: 1/6; GR: 4/5). These results suggest that the alveolitis can preceed the anatomical damages. These results need to be confirmed in a larger series and the value of early treatment should be evaluated.

The non bronchial systemic arterial circulation of the lung may, for more than one good reason, be considered as a third arterial circulation of the lung, after the bronchial and pulmonary circulation. A perfect knowledge of the anatomy of the circulation and the physiopathological mechanisms leading to its development is a vital prelude to angiographic study. A common point in the majority of situations leading to this non bronchial systemic arterial hypervascularization is the existence of a pleural symphysis allowing the pulmonary penetration of these thoracic parietal vessels. In addition to the contribution of the transpleural non bronchial systemic vessels the role of the arteries of the triangular ligament should be discussed. The pathological significance of this non bronchial systemic hypervascularization is threefold. This circulation can be the source of haemoptysis as shown by angiography and is important for considering therapy when haemoptysis needs treatment by percutaneous embolisation. The duration and the risk of catheterisation may be considerably reduced thanks to the precision of the endoscopic examination at the site of bleeding. There are other types of endothoracic haemorrhage which can be qualified as "haemoptysis like" whose treatment, preventative or curative may also be assured by embolisation of this circulation. Besides a certain number of extracardiac shunts both endo and transthoracic, without any haemorrhagic consequence at the time at which they are discovered show evidence of abnormal communication between the three circulations of the lung; certain of these may need therapy by vaso-occlusion.

Vaso-occlusion of the pulmonary artery is an angiographic technique which involves the voluntary and precise obstruction, temporary or permanent, performed at the end of diagnostic studies or therapeutic studies on one or several branches of the pulmonary artery. The diagnostic indications are touched on briefly at the end of this review because they are older and better known than the therapeutic indications. The latter has developed in less than 10 years and concerns first and foremost two pathologies: hemoptysis and arterio-venous aneurysms of the lung. The erosive pseudo-aneurysm of the pulmonary artery complicating parenchymatous necrosis above all of infections or tumours should be sought in certain situations and treated by vascular occlusion. The pulmonary arteriovenous aneurysms may equally be treated by selective obstruction of their afferent pedicles by metal spirals or detachable balls. This technique, in sacrificing the minimum of pulmonary parenchyma, should be considered according to each case as a possible supplement or replacement for surgery.