Cladribine is effective against cystic pulmonary Langerhans cell histiocytosis.

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Diagnostic investigations.

<p><b>*</b>In 16 instances, CT scan was not performed because of severe hypoxemia precluding transportation to the radiological department.</p><p>In 30 instances, results for BAL were not available either because BAL was not performed due to severe hypoxemia or the BAL results were deemed uninterpretable.</p

Acute Respiratory Failure in Critically Ill Patients with Interstitial Lung Disease

<div><p>Background</p><p>Patients with chronic known or unknown interstitial lung disease (ILD) may present with severe respiratory flares that require intensive management. Outcome data in these patients are scarce.</p><p>Patients and Methods</p><p>Clinical and radiological features were collected in 83 patients with ILD-associated acute respiratory failure (ARF). Determinants of hospital mortality and response to corticosteroid therapy were identified by logistic regression.</p><p>Results</p><p>Hospital and 1-year mortality rates were 41% and 54% respectively. Pulmonary hypertension, computed tomography (CT) fibrosis and acute kidney injury were independently associated with mortality (odds ratio (OR) 4.55; 95% confidence interval (95%CI) (1.20–17.33); OR, 7.68; (1.78–33.22) and OR 10.60; (2.25–49.97) respectively). Response to steroids was higher in patients with shorter time from hospital admission to corticosteroid therapy. Patients with fibrosis on CT had lower response to steroids (OR, 0.03; (0.005–0.21)). In mechanically ventilated patients, overdistension induced by high PEEP settings was associated with CT fibrosis and hospital mortality.</p><p>Conclusion</p><p>Mortality is high in ILD-associated ARF. CT and echocardiography are valuable prognostic tools. Prompt corticosteroid therapy may improve survival.</p></div

Main characteristics of the 83 study patients at ICU admission.

<p>Abbreviations: RV, right ventricular; ILD, interstitial lung disease.</p

Severity, ICU management and outcomes.

<p><b>*</b>ARDS was defined using Berlin criteria <a href="http://www.plosone.org/article/info:doi/10.1371/journal.pone.0104897#pone.0104897-Ranieri1" target="_blank">[2]</a>.</p><p>Pulmonary hypertension (right ventricular dysfunction) was assessed by transthoracic echocardiography (excluding left ventricular dysfunction).</p><p>¥ Responsiveness to corticosteroids was defined as an increase in the ratio of arterial oxygen saturation (PaO₂) over fraction of inspired oxygen (FiO₂) ratio to more than 100 mmHg within 1 week of initiating high-dose corticosteroid therapy.</p><p>£ cyclophophamide or rituximab.</p

Determinants of hospital mortality.

<p>Abbreviations : OR, odds ratio; 95%CI, 95% confidence interval; ILD, interstitial lung disease; ECOG, Eastern Cooperative Oncology Group (the performance score can range from 0 [fully active] to 5 [dead]); CT, computed tomography of the chest; SOFA, Sequential Organ Function Assessment score.</p

Effect of positive end-expiratory pressure (PEEP) titration in patients managed with invasive mechanical ventilation (n = 50).

<p>Right column: correlations linking variations in peak airway pressure (ΔPpeak), plateau pressure (ΔPplat), and PaO₂/FiO₂ (ΔPF) before and after PEEP titration to ICU mortality. Left column: correlations linking variations in peak airway pressure (ΔPpeak), plateau pressure (ΔPplat), and PaO₂/FiO₂ (ΔPF) before and after PEEP titration to pulmonary fibrosis by computed tomography.</p

Univariable and multivariable analyses of factors associated with responsiveness to high-dose corticosteroids.

<p>Abbreviations : OR, odds ratio; 95%CI, 95% confidence interval; ILD, interstitial lung disease; ECOG, Eastern Cooperative Oncology Group (the performance score can range from 0 [fully active] to 5 [dead]); CT, computed tomography of the chest; BAL, broncho-alveolar lavage; SOFA, Sequential Organ Function Assessment score.</p

Correspondence on ‘Glucocorticoid-induced relapse of COVID-19 in a patient with sarcoidosis’

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Phenotypic characterization of interstitial lung disease associated with mutations inSFTPC and ABCA3 in adults

International audienceIntroduction: Mutations in surfactant genes SFTPC or ABCA3 are responsible for interstitial lung disease (ILD) in children and adults. Despite numerous paediatric data, few studies described these entities in adults.Methods: We conducted a multicenter retrospective study of adult patients with ILD associated with mutations in SFTPC or ABCA3 in the French rare pulmonary diseases network OrphaLung.Results: We included 36 patients, 22 in the SFTPC group and 14 in the ABCA3 group. Clinical characteristics were similar between the two groups. Baseline pulmonary function tests reported a median FVC of 59% ([52 – 72]%), a median FEV1 of 63% ([48,3 – 73,3] %), a median TLC of 65 % ([59 – 76] %) and a median DLco of 44 % ([35,4 – 50] %). A pattern of unclassifiable fibrosing ILD was the most frequent on chest computed tomography, found in 85% of patients. Nonspecific interstitial pneumonia and usual interstitial pneumonia were the most common histological patterns in the ABCA3 group and in the SFTPC group, respectively. Median survival before death or lung transplantation was 10 years in the SFTPC group (4 deaths and 8 patients with lung transplantation) and was not reached at the end of follow-up in the ABCA3 group (1 death and 2 patients with lung transplantation).Conclusion: Patients with ILD and mutations in SFTPC or ABCA3 present a distinct phenotype of unclassifiable fibrosing ILD with cysts and ground glass opacities, and their prognosis is often severe. While our results highlight the phenotypical heterogeneity of these patients, they also expand our knowledge of these rare causes of ILD in adult