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Surgical lung biopsy is essential in the diagnostic algorithm of interstitial lung disease (ILD) of unknown cause. Safety concerns have been recently reiterated. This study prospectively assessed the yield of diagnosis and safety of video-assisted thoracoscopic surgical lung biopsy (VATS-LB) for ILD diagnosis. This prospective study, conducted in 6 ILD-referral Paris hospitals, included 103 patients with ILD. VATS-LB was proposed after initial multidisciplinary discussion. A final diagnosis was made after the procedure, during a second multidisciplinary discussion. The main outcome was to determine the final diagnoses and their proportion after VATS-LB. Other outcomes were the percentage of change in diagnosis and treatment propositions after VATS-LB and adverse events during 3 months after the operation, postoperative pulmonary function, quality of life, and pain. A definite diagnosis was reached in 87 patients (84.4%), and 16 remained unclassifiable (15.6%). After VATS-LB, the hypothesized diagnosis changed in 65 patients (63.1%) and treatment changed in 41 patients (39.8%). One patient died of acute exacerbation. In-hospital complications were predicted by a shorter preoperative 6-minute walking test distance and by forced vital capacity lower than 77%. Postoperative quality of life was not modified at 3 months, whereas forced vital capacity decreased slightly. Postoperative neuropathic pain was revealed in 5% of patients at 1 month and in 2% at 3 months. VATS-LB dramatically changed preoperative hypothetical diagnoses and treatment in ILD of unknown cause, with good patient survival in ILD referral centers. [ABSTRACT FROM AUTHOR]

Résumé L’hémorragie intra-alvéolaire (HIA) est une maladie rare et grave. Le diagnostic est évoqué devant la triade hémoptysie, anémie et infiltrat radiologique. Il est confirmé par la réalisation d’une tomodensitométrie thoracique et d’une fibroscopie bronchique avec lavage broncho-alvéolaire révélant des sécrétions uniformément hémorragiques. Les étiologies en sont nombreuses. Elles sont classiquement différenciées en cause « immune » (vascularites liées aux anticorps anti-cytoplasme des polynucléaires neutrophiles (ANCA), les connectivites/lupus érythémateux systémique (LES) et le syndrome de Goodpasture) et « non immune » (cardiopathie, toxique, infection…). Un bilan étiologique avec la recherche de signes cliniques extrathoraciques, d’auto-anticorps (anticorps anti-nucléaires et anti-membrane basale glomérulaire [ANCA]), la réalisation d’une échographie cardiaque et la recherche d’une imputabilité iatrogénique ou d’un contexte septique évident est urgent. Le LES et le syndrome des antiphopholipides sont les causes immunes les plus fréquentes en rhumatologie avec les vascularites à ANCA. Les autres connectivites sont rarement directement impliquées dans le mécanisme d’HIA, hormis en cas de formes associées à une vascularite et la présence d’ANCA. Une telle situation doit donc faire rechercher une cause « non immune » en priorité. Alveolar hemorrhage (AH) is a rare and serious disease. Diagnosis is suspected with the three following symptoms: hemoptysis, anemia and radiological infiltrate. It is confirmed by performing a thoracic CT scan and bronchoscopy with bronchoalveolar lavage revealing uniformly hemorrhagic secretions. Etiologies are numerous. They are classically differentiated into “immune” causes (vasculitis related to anti-cytoplasmic neutrophil antibodies (ANCA), connective tissue diseases/systemic lupus erythematous (SLE) and Goodpasture's syndrome) and “non-immune” causes (heart disease, toxic, infection…). Etiological assessment with the search for extrathoracic clinical signs, dosage of autoantibodies (ANCA, anti-nuclear antibodies and anti-glomerular basement membrane), the realization of a cardiac ultrasound, elimination of a iatrogenic cause or an obvious septic condition is urgent. SLE and antiphopholipid syndrome are the most common immune causes in rheumatology with ANCA-related vasculitis. Other connective tissue diseases are rarely directly involved in the AH mechanism, except in cases of vasculitis-associated forms and the presence of ANCA. In such a situation, a “non-immune” cause must be sought in priority. [ABSTRACT FROM AUTHOR]

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. [Copyright &y& Elsevier]

To assess the efficacy of inhaled ciclesonide in reducing the risk of adverse outcomes in COVID-19 outpatients at risk of developing severe illness. COVERAGE is an open-label, randomized controlled trial. Outpatients with documented COVID-19, risk factors for aggravation, symptoms for ≤7 days, and absence of criteria for hospitalization are randomly allocated to either a control arm or one of several experimental arms, including inhaled ciclesonide. The primary efficacy endpoint is COVID-19 worsening (hospitalization, oxygen therapy at home, or death) by Day 14. Other endpoints are adverse events, maximal follow-up score on the WHO Ordinal Scale for Clinical Improvement, sustained alleviation of symptoms, cure, and RT-PCR and blood parameter evolution at Day 7. The trial's Safety Monitoring Board reviewed the first interim analysis of the ciclesonide arm and recommended halting it for futility. The results of this analysis are reported here. The analysis involved 217 participants (control 107, ciclesonide 110), including 111 women and 106 men. Their median age was 63 years (interquartile range 59–68), and 157 of 217 (72.4%) had at least one comorbidity. The median time since first symptom was 4 days (interquartile range 3–5). During the 28-day follow-up, 2 participants died (control 2/107 [1.9%], ciclesonide 0), 4 received oxygen therapy at home and were not hospitalized (control 2/107 [1.9%], ciclesonide 2/110 [1.8%]), and 24 were hospitalized (control 10/107 [9.3%], ciclesonide 14/110 [12.7%]). In intent-to-treat analysis of observed data, 26 participants reached the composite primary endpoint by Day 14, including 12 of 106 (11.3%, 95% CI: 6.0%–18.9%) in the control arm and 14 of 106 (13.2%; 95% CI: 7.4–21.2%) in the ciclesonide arm. Secondary outcomes were similar for both arms. Our findings are consistent with the European Medicines Agency's COVID-19 task force statement that there is currently insufficient evidence that inhaled corticosteroids are beneficial for patients with COVID-19. [ABSTRACT FROM AUTHOR]

Background Mutations in the telomerase complex ( TERT and TR ) are associated with pulmonary fibrosis and frequent hematologic manifestations. The aim of this study was to characterize the prognosis of lung transplantation in patients with TERT or TR mutations. Methods Patients with documented TERT or TR mutations who received a lung transplant between 2007 and 2013 in France were identified via an exhaustive search of the lung transplantation network, one expert genetic laboratory, and the clinical research network on rare pulmonary diseases. Results There were 9 patients (7 men) with TERT ( n = 6) or TR ( n = 3) mutations who received a single ( n = 8) or a double ( n = 1) lung transplant for pulmonary fibrosis. Median age was 50 years (range, 35–61 years) at diagnosis and 52 years (range, 37–62 years) at the time of lung transplantation. Thrombocytopenia was present in 7 patients before lung transplantation. After lung transplantation, 6 patients developed myelodysplasia and/or bone marrow failure, directly contributing to death in 4 cases. Anemia was observed in 9 patients, and neutropenia was observed in 3 patients. The median survival after lung transplantation was 214 days (range, 59–1,709 days). Conclusions Patients with mutations of the telomerase complex are at high risk of severe hematologic complications after lung transplantation, in particular, bone marrow failure. Specific recommendations should be developed for appropriate guidance regarding hematologic risk assessment before transplantation and management of the post-transplantation immunosuppressive regimen. [ABSTRACT FROM AUTHOR]

Purpose To describe the initial and follow-up CT features of interstitial lung disease associated with anti-synthetase syndrome (AS-ILD). Materials and methods Two independent thoracic radiologists retrospectively analysed thin-section CT images obtained at diagnosis of AS-ILD in 33 patients (17 positive for anti-Jo1, 13 for anti-PL12, and three for anti-PL7 antibodies). They evaluated the pattern, distribution and extent of the CT abnormalities. They also evaluated the change in findings during follow-up (median 27 months; range 13–167 months) in 26 patients. Results At diagnosis, ground-glass opacities (100%), reticulations (87%) and traction bronchiectasis (76%) were the most common CT findings. Consolidations were present in 45% of patients. A non-specific interstitial pneumonia (NSIP), organizing pneumonia (OP) or mixed NSIP-OP CT pattern were observed in 15 out of 33 (45%), seven out of 33 (21%) and eight out of 33 (24%) patients, respectively, whereas the CT pattern was indeterminate in three patients. During follow-up, consolidations decreased or disappeared in 11 out of 12 patients (92%), among which seven within the first 6 months, but honeycombing progressed or appeared in ten out of 26 patients (38%) and overall disease extent increased in nine out of 26 patients (35%). Conclusion CT features at diagnosis of AS-ILD mainly suggest NSIP and OP, isolated or in combination. Consolidations decrease or disappear in most cases but the disease may progress to fibrosis in more than one third of patients. [ABSTRACT FROM AUTHOR]