COVID-19 in patients with pulmonary alveolar proteinosis: a European multicentre study.
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ABSTRACT:
SUBMITTER: Papiris SA
PROVIDER: S-EPMC9271262 | biostudies-literature | 2023 Jan
REPOSITORIES: biostudies-literature
Publications
COVID-19 in patients with pulmonary alveolar proteinosis: a European multicentre study.
Papiris Spyros A SA Campo Ilaria I Mariani Francesca F Kallieri Maria M Kolilekas Lykourgos L Papaioannou Andriana I AI Gonca Chousein Efsun E Cetinkaya Erdogan E Bonella Francesco F Borie Raphael R Kokosi Maria M Pickworth Thomas T Molina-Molina Maria M Gasa Mercè M Radzikowska Elżbieta E Fijolek Justyna J Jouneau Stéphane S Gomez Emmanuel E McCarthy Cormac C Bendstrup Elisabeth E Piotrowski Wojciech J WJ Pabary Rishi R Hadchouel Alice A Coolen-Allou Nathalie N Alfaro Tiago T Robalo Cordeiro Carlos C Antonogiannaki Elvira-Markela EM Tomos Ioannis P IP Papakosta Despoina D Kontakiotis Theodoros T Panagiotou Panagiota P Douros Konstantinos K Schams Andrea A Lettieri Sara S Papaevangelou Vassiliki V Kanaka-Gantenbein Christina C Karakatsani Anna A Loukides Stelios S Costabel Ulrich U Crestani Bruno B Morgan Cliff C Tazawa Ryushi R Bush Andrew A Griese Matthias M Manali Effrosyni D ED
ERJ open research 20230103 1
<b>Adult PAP patients experience similar #COVID19 rates to the general population, and high rates of hospitalisation and deaths, underscoring their vulnerability and the need for measures to prevent infection. The impact of iGM-CSF must be considered.</b> https://bit.ly/3M0wKnZ. ...[more]
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Project description:BackgroundAutoimmune pulmonary alveolar proteinosis (aPAP) is a rare interstitial lung disease. COVID-19 is associated with worse prognosis in previous lung diseases patients. But the prognosis of aPAP patients after infection with COVID-19 is unclear. In December 2022, China experienced a large-scale outbreak of Omicron variant of the SARS-CoV-2. In this study, we aim to explore the clinical outcomes of aPAP patients infected with COVID-19.ResultsA total of 39 aPAP patients were included in this study. 30.77% patients had a decrease in oxygen saturation after COVID-19 infection. We compared the two groups of patients with or without decreased oxygen saturation after COVID-19 infection and found that patients who had previous oxygen therapy (decreased oxygen saturation vs. non decreased oxygen saturation: 6/12 vs. 4/27, P = 0.043), with lower baseline arterial oxygen partial pressure (74.50 ± 13.61 mmHg vs. 86.49 ± 11.92 mmHg, P = 0.009), lower baseline DLCO/VA% [77.0 (74.3, 93.6) % vs. 89.5 (78.2, 97.4) %, P = 0.036], shorter baseline 6MWD [464 (406, 538) m vs. 532 (470, 575) m, P = 0.028], higher disease severity score (P = 0.017), were more likely to have decreased oxygen saturation after COVID-19 infection.ConclusionaPAP patients with poor baseline respiration have a higher probability of hypoxia after COVID-19 infection, but fatal events were rare.
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Project description:Autoimmunity has been extensively established as a characteristic feature of the post-COVID-19 syndrome. There is evolving evidence of immune system dysregulation leading to the development of autoimmune phenomena in patients with COVID-19. This immune dysregulation may range from the production of autoantibodies to the new onset of rheumatic autoimmune diseases. An extensive literature search in databases from December 2019 to date revealed that no cases of autoimmune pulmonary alveolar proteinosis (PAP) were reported in post-COVID patients. In this context, we report a novel case series of two cases of new-onset autoimmune PAP in post-COVID patients, an entity that has not been described before. We recommend further studies to better understand this association between new-onset autoimmune PAP and SARS-CoV-2.
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| S-EPMC3432632 | biostudies-literature
Project description:This case report demonstrates 44-year old man, presenting with recurring clinical pneumonias during a period of over 1 year. The patient was clinically affected with, i.a., weight loss, finger clubbing and severely reduced diffusion capacity. Repetitive chest x-rays showed bilateral and consolidating infiltrates, and a high-resolution computed tomography of the thorax (HRCT) exposed ground glass opacities superimposed on a reticular pattern as the so-called 'crazy paving' pattern. A bronchoscopic alveolar lavage revealed alveolar proteinaceous material compatible with the diagnosis pulmonary alveolar proteinosis (PAP). PAP is a rare syndrome where surfactant is accumulated in the alveoli, causing respiratory disease in typically young to middle-aged patients with male predominance. Both symptoms and prognosis are variable, and range from spontaneous remission to terminal respiratory failure. The standard treatment is whole lung lavage, where surfactant is mechanically rinsed from the lungs. The lack of specific clinical symptoms makes it easy to overlook the diagnosis, as supported by this case report. It serves as a reminder, that the findings of a crazy paving pattern on HRCT in young adults should alert of this rare disease, and advises on the further examinations required to make the diagnosis.
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Project description:BackgroundAutoimmune pulmonary alveolar proteinosis (aPAP) is a rare disease characterized by progressive surfactant accumulation and hypoxemia. It is caused by disruption of granulocyte-macrophage colony-stimulating factor (GM-CSF) signaling, which pulmonary alveolar macrophages require to clear surfactant. Recently, inhaled GM-CSF was shown to improve the partial pressure of arterial oxygen in patients with aPAP.MethodsIn a double-blind, placebo-controlled, three-group trial, we randomly assigned patients with aPAP to receive the recombinant GM-CSF molgramostim (300 μg once daily by inhalation), either continuously or intermittently (every other week), or matching placebo. The 24-week intervention period was followed by an open-label treatment-extension period. The primary end point was the change from baseline in the alveolar-arterial difference in oxygen concentration (A-aDo2) at week 24.ResultsIn total, 138 patients underwent randomization; 46 were assigned to receive continuous molgramostim, 45 to receive intermittent molgramostim, and 47 to receive placebo. Invalid A-aDo2 data for 4 patients (1 in each molgramostim group and 2 in the placebo group) who received nasal oxygen therapy during arterial blood gas measurement were replaced by means of imputation. For the primary end point - the change from baseline in the A-aDo2 at week 24 - improvement was greater among patients receiving continuous molgramostim than among those receiving placebo (-12.8 mm Hg vs. -6.6 mm Hg; estimated treatment difference, -6.2 mm Hg; P = 0.03 by comparison of least-squares means). Patients receiving continuous molgramostim also had greater improvement than those receiving placebo for secondary end points, including the change from baseline in the St. George's Respiratory Questionnaire total score at week 24 (-12.4 points vs. -5.1 points; estimated treatment difference, -7.4 points; P = 0.01 by comparison of least-squares means). For multiple end points, improvement was greater with continuous molgramostim than with intermittent molgramostim. The percentages of patients with adverse events and serious adverse events were similar in the three groups, except for the percentage of patients with chest pain, which was higher in the continuous-molgramostim group.ConclusionsIn patients with aPAP, daily administration of inhaled molgramostim resulted in greater improvements in pulmonary gas transfer and functional health status than placebo, with similar rates of adverse events. (Funded by Savara Pharmaceuticals; IMPALA ClinicalTrials.gov number, NCT02702180.).
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