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ABSTRACTIntroductionAntisynthetase syndrome (ASSD) is a rare auto-immune condition that can present as interstitial lung disease (ILD) and progress into Acute Respiratory Distress Syndrome (ARDS).ImportanceThe purpose of this clinical case is to highlight the importance of considering less prevalent causes of ARDS amid the COVID-19 pandemic.Case reportWe present a 56-year-old Belgian female of African descent without past medical history who demonstrated typical signs of COVID-19 at the start of the pandemic. Based on the disease course as well as CT-scan findings, a diagnosis of COVID-19 was made. She progressed to ARDS for which she got intubated and was started on venovenous membrane oxygenation (VV-ECMO). Despite initial negative screening for antinuclear antibodies, further analysis revealed anti-Jo-antibodies. Diagnosis of ASSD was eventually retained and immunosuppressive therapy was started. However, pulmonary fibrosis had evolved too far and therapy was halted shortly after.

Introduction: The PROOF registry is a prospective, observational study that aimed to monitor disease progression in a real-world cohort of patients with idiopathic pulmonary fibrosis (IPF). Here, longitudinal quality-of-life (QoL) outcomes, healthcare resource use (HCRU), and the association between QoL and mortality in patients enrolled in the PROOF registry are presented. Methods: QoL outcomes (St. George’s Respiratory Questionnaire [SGRQ], EuroQoL-5 dimensions-5 levels Health Questionnaire [EQ-5D-5L], EuroQoL-5 dimensions Health Questionnaire [EQ-5D] visual analogue scale [VAS] and cough VAS) and HCRU were collected for all patients. Associations between baseline QoL and mortality were assessed using univariate and multivariate analyses. During multivariate analyses, individual QoL measures were adjusted for the following covariates: age, sex, percent predicted forced vital capacity, percent predicted diffusing capacity of the lungs for carbon monoxide, smoking status, and supplementary oxygen use at registry inclusion. Results: In total, 277 patients were enrolled in the PROOF registry. During the follow-up period, worsening in cough VAS score, SGRQ symptom score, and SGRQ activity score was observed, while EQ-5D VAS, SGRQ total score, and SGRQ impact score remained stable. During univariate analyses, EQ-5D VAS and all SGRQ sub-scores and total score at baseline were associated with mortality; however, during multivariate analyses, only the SGRQ total score, SGRQ impact score, and SGRQ symptom score at baseline were associated with mortality. During the follow-up period, 261 (94.2%) patients required an outpatient consultation (IPF- or non-IPF-related) and there were 182 hospitalizations in total, most of which were respiratory related (66.5%). Conclusions: The PROOF registry provided valuable, real-world data on the association between baseline QoL and mortality, and longitudinal HCRU and QoL outcomes in patients with IPF over 24 months and identified that SGRQ may be an independent prognostic factor in IPF.

Infections with SARS-CoV-2 continue to cause significant morbidity and mortality. Interleukin (IL)-1 and IL-6 blockade have been proposed as therapeutic strategies in COVID-19, but study outcomes have been conflicting. We sought to study whether blockade of the IL-6 or IL-1 pathway shortened the time to clinical improvement in patients with COVID-19, hypoxic respiratory failure, and signs of systemic cytokine release syndrome.

ABSTRACTPneumocystis jiroveciipneumonia (PJP) can be a severe indicator disease of acquired immunodeficiency syndrome (AIDS). We present two cases of homosexual male patients who came to the emergency unit of a Belgian hospital because of shortness of breath. Both men had been sent back home, initially diagnosed with a benign viral infection. Because of worsening symptoms and gradually evolving hypoxemia, both patients came back and were admitted to the hospital with a diagnosis of (microbiology proven) Pneumocystis jiroveciipneumonia. HIV serology in both men was tested and was clearly positive, indicating a new diagnosis of HIV infection. In this article, we provide an overview of this possibly severe AIDS defining condition. First, we give an introduction of the history of HIV/AIDS and its occurrence in homosexual males in Europe. Secondly, we provide an overview of the diagnosis and treatment of Pneumocystis jiroveciipneumonia. Finally, since the first case reports of Pneumocystis jiroveciipneumonia at the beginning of the AIDS epidemic also included homosexual men, we emphasize the potential importance of a sexual anamnesis in young male patients with an initial complaint of dyspnea.

The advice of a dynamic multidisciplinary discussion (MDD) is believed to be important in the diagnosis of interstitial lung diseases (ILDs). However, to what extent MDD diagnoses differ from the preliminary diagnoses before formal workup and MDD (preMDD diagnoses) is still insufficiently studied.

Eosinopenia was frequently encountered in patients with coronavirus disease 2019 (COVID-19). We describe a case of a 59-year-old man who was treated with high-dose corticosteroids and anti-interleukin 1 receptor antagonist therapy because of severe acute respiratory distress syndrome due to a so-called cytokine storm in COVID-19. He had chronic eosinophilia for many years due to an unknown Strongyloides stercoralisinfection, proven by serology and a positive polymerase chain reaction test on a stool sample. COVID-19 led to a complete resolution of eosinophilia, even before immunosuppressive treatment was started. Eosinophilia returned after recovery from COVID-19 and started to decline under treatment with ivermectin. Our case confirms previous reports of eosinopenia in COVID-19, as it appears even in patients with chronic eosinophilia. Presence of eosinophilia should prompt screening for strongyloidiasis in all patients eligible for immunosuppressive therapy because of the risk of Strongyloideshyperinfection syndrome, especially if this treatment is empirical.

Presumptive coronary artery spasm, with characteristic ST-segment elevation followed by ventricular arrhythmia and cardiac arrest, is a very uncommon manifestation of the carcinoid crisis and has been shown on angiography only once in a patient with co-existing extensive atheromatous disease. We present the history of a 70-year-old man with angiographically documented fatal diffuse coronary spasm in the absence of significant atheromatosis, related to an unprovoked carcinoid crisis caused by a small peripheral atypical carcinoid tumour in the absence of metastasis.

GM-CSF promotes myelopoiesis and inflammation, and GM-CSF blockade is being evaluated as a treatment for COVID-19-associated hyperinflammation. Alveolar GM-CSF is, however, required for monocytes to differentiate into alveolar macrophages (AMs) that control alveolar homeostasis. By mapping cross-species AM development to clinical lung samples, we discovered that COVID-19 is marked by defective GM-CSF-dependent AM instruction and accumulation of pro-inflammatory macrophages. In a multi-center, open-label RCT in 81 non-ventilated COVID-19 patients with respiratory failure, we found that inhalation of rhu-GM-CSF did not improve mean oxygenation parameters compared with standard treatment. However, more patients on GM-CSF had a clinical response, and GM-CSF inhalation induced higher numbers of virus-specific CD8 effector lymphocytes and class-switched B cells, without exacerbating systemic hyperinflammation. This translational proof-of-concept study provides a rationale for further testing of inhaled GM-CSF as a non-invasive treatment to improve alveolar gas exchange and simultaneously boost antiviral immunity in COVID-19. This study is registered at ClinicalTrials.gov (NCT04326920) and EudraCT (2020-001254-22).

AbstractBackground:The size of a pneumothorax (PTX) is usually estimated by the Light index. Treatment strategies of (primary, spontaneous) PTX partially depend upon the size of the PTX. To our knowledge, the Light index has not yet been correlated with the actual volume of the PTX. Objectives:To correlate the estimated size of a primary spontaneous PTX by means of the Light index, with the actual amount of air present in the pleural space. Methods:Actual PTX volumes were measured by means of manual aspiration of air present in the pleural space in 18 patients with primary spontaneous PTX and correlated with the size estimation obtained by the Light index. Results:Light index and volume measurements were strongly correlated (r = 0.84, p < 0.0001). Conclusions:The Light index is a good estimate of the actual size of a (primary spontaneous) PTX.Copyright © 2001 S. Karger AG, Basel

Six patients with the acquired immunodeficiency syndrome (AIDS) who suffered eight spontaneous pneumothoraces between January 1990 and January 1993 underwent videothoracoscopy. The predominant macroscopic findings, found in four patients on five occasions, were white-yellow nodules dispersed throughout the lung surface. In addition, in one patient, these lesions were associated with multiple small apical bullae and, in another, a large reddish nodule with several smaller white satellite nodules was noted on the parietal pleura. Methenamine silver stain of biopsy samples of both the visceral and parietal nodules in these patients showed the presence of Pneumocystis carinii. May-Grunwald-Giemsa stain of brushing samples of visceral lesions revealed P-cariniiin two patients. In one of these patients, previously performed bronchoalveolar lavage (BAL) had not yielded P-carinii. On two occasions, the thoracoscopic findings were unremarkable, although in one of these patients, computed tomography (CT) had shown a large thick-walled cyst near the left hilum and BAL revealed P-carinii. Talc poudrage through the thoracoscopic cannula followed by chest tube drainage was performed in all patients and was successful in treating three of five with proved P-cariniipneumonia-related pneumothorax. The etiology of pneumothorax in AIDS and the diagnostic and therapeutic utility of videothoracoscopy in these patients are discussed.

This is the report of a 38-year-old man with unilateral dermatomal hyperhidrosis documented by a starch-iodine technique; a subsequent diagnosis was made of a generalized pulmonary adenocarcinoma. The association of unilateral hyperhidrosis and a malignant tumor is reviewed.

Patients with HES and pulmonary infiltrates may pose certain diagnostic problems as the infiltrates may be attributed to infection, infarction, congestive heart failure, or HES itself. We report an 87-year-old woman with idiopathic HES presenting with bibasal alveolar infiltrates. Differential cell count in BAL fluid yielded a very high percentage (73 percent) of eosinophils. Other authors previously mentioned the absence of eosinophils in the lavage fluid despite an important peripheral eosinophilia in a patient with the idiopathic HES but without HES-related pulmonary involvement. Thus, BAL fluid eosinophilia may suggest HES-related pulmonary involvement. Therefore, BAL might be an important diagnostic tool in the management of pulmonary infiltrates in idiopathic HES.

We report a case of recurring catamenial pneumothorax with concurrent pelvic endometriosis. Thoracoscopy revealed a blue-like lesion on top of the dome of the right hemidiaphragm. Microscopic examination of biopsy specimens showed endometriosis. The patient was treated with a Gn-RH analogue and remains well without further evidence of pneumothorax after six months.