ABSTRACT
Background: Interruption of GM-CSF signaling leads to Pulmonary Alveolar Proteinosis (PAP), occasionally to lung infections and relates to the impaired ability of lung macrophages to catabolize phagocytized surfactant and handle microbes. COVID-19 is associated with worse prognosis in lung disorders. We hypothesized that PAP patients would be at increased risk for COVID-19 and poor outcome. Aim and objectives: This multi-center, retrospective, European study aimed to investigate prevalence and clinical consequences of COVID-19 in PAP and the impact of iGM-CSF treatment on hospitalization or death. Method(s): All patients with PAP and COVID-19 diagnosed and followed-up in 11 referral European centers from January 24th 2020 to August 31st 2021 were included. Prevalence, clinical course and outcome were investigated. Result(s): COVID-19 developed in 34/255 (13.3%) of patients, mostly adults (91.2%), all with autoimmune (a)PAP;all patients were infected before the preventive option of vaccination was available;11 (35.5%) were hospitalized, of whom almost half were in the ICU;3 (27%) of hospitalized patients either died or underwent lung-transplant;these three patients had worse DLCO% predicted (p=0.019) and had more often arterial hypertension (AH) (p=0.012), and a smoking history (p=0.002). All patients with mild disease treated at home survived. Among children, 3 developed COVID-19 with good outcome. Conclusion(s): PAP patients experienced similar rates of COVID-19 with the general population but increased rates of hospitalizations and deaths, underscoring the vulnerability of this population and the necessity of preventive measures to avoid infection. If infected, secondary prophylaxis with monoclonal antibodies and the impact of iGM-CSF must be considered.
ABSTRACT
P4 Table 1Characteristics of the subjectsCharacteristics Subjects with ILAs on LDCT (n = 39) Age, yr, mean (± SD) 68.8 (± 4.3) Gender, n (%) Female 15 (38.5) Male 24 (61.5) Smoking status, n (%) Current 7 (17.9) Ex 32 (82.1) Respiratory symptoms, n (%) None 19 (48.7) Cough 3 (7.7) Dyspnoea 9 (23.1) Cough & dyspnoea 6 (15.4) N/A 2 (5.1) Physical examination findings, n (%) None 5 (12.8) Crackles 17 (43.6) N/A 17 (43.6) Baseline lung function,%pred, median (range) FEV1,% pred 91 (58 – 130) FVC,% pred 94.8 (65 – 143) TLco,% pred 57.6 (28.4 – 98.8) Kco,% pred 79.5 (36.4 – 94) MDT Diagnosis ILAs, n (%) 8 (20.5) ILD, n (%) IPF 14 (35.9) Smoking-related ILD 6 (15.4) Hypersensitivity pneumonitis 4 (10.3) PPFE 3 (7.7) Sarcoidosis 1 (2.6) Post-COVID ILD 1 (2.6) Vasculitis 1 (2.6) Unclassifiable 1 (2.6) Treatment, n (%) Smoking cessation advice 6 (15.4) Antifibrotic 7 (17.9) Immunomodulatory treatment 2 (5.1) None 23 (59) ResultsILAs of >5% extent on LDCT were identified in 39/1853 (2.1%) subjects screened between August 2018 and April 2021 (table 1). Respiratory symptoms were present in 18/39 (46.1%) and crackles were auscultated in 17 of 22 subjects (77.3%) undergoing physical examination. Past exposure to potential environmental triggers was noted in 21/39 (53.8%). Diagnostic bronchoalveolar lavage was performed in 7/39 (17.9%) and one patient underwent transbronchial lung cryobiopsy. After MDT discussion, ILD was concluded in 31/39 (79.5%) cases, of which 14/31 (45.2%) were diagnosed with IPF. In the IPF subgroup, antifibrotics were initiated in 7/14 (50%) of cases. In those diagnosed with other ILDs, immunomodulatory treatment was initiated in 2/25 (8%) subjects.ConclusionA large proportion of individuals with newly identified ILAs have an abnormal clinical examination and respiratory symptoms, consistent with the widely held suspicion that ILD is underdiagnosed in the community. Lung cancer screening in this demographic provides a unique opportunity to address this unmet health metric. Earlier identification of ILD, specifically IPF, allows institution of antifibrotic therapies proven to modify the natural history of the disease by preserving lung function and extending life. The cost-effectiveness of this approach for ILD screening warrants detailed evaluation.
ABSTRACT
Introduction: Pneumothorax and pneumomediastinum have both been noted to complicate cases of coronavirus disease 2019 (COVID-19) requiring hospital admission. We report the largest case series yet described of patients with both these pathologies (including nonventilated patients). Methods: Cases were collected retrospectively from UK hospitals with inclusion criteria limited to a diagnosis of COVID-19 and the presence of either pneumothorax or pneumomediastinum. Patients included in the study presented between March and June 2020. Details obtained from the medical record included demographics, radiology, laboratory investigations, clinical management and survival. Results: 71 patients from 16 centres were included in the study, of whom 60 had pneumothoraces (six with pneumomediastinum in addition) and 11 had pneumomediastinum alone. Two of these patients had two distinct episodes of pneumothorax, occurring bilaterally in sequential fashion, bringing the total number of pneumothoraces included to 62. Clinical scenarios included patients who had presented to hospital with pneumothorax, patients who had developed pneumothorax or pneumomediastinum during their inpatient admission with COVID-19 and patients who developed their complication while intubated and ventilated, either with or without concurrent extracorporeal membrane oxygenation. Survival at 28 days was not significantly different following pneumothorax (63.1 +/- 6.5%) or isolated pneumomediastinum (53.0 +/- 18.7%;p=0.854). The incidence of pneumothorax was higher in males. 28-day survival was not different between the sexes (males 62.5 +/- 7.7% versus females 68.4 +/- 10.7%;p=0.619). Patients aged >= 70 years had a significantly lower 28-day survival than younger individuals (>= 70 years 41.7 +/- 13.5% survival versus <70 years 70.9 +/- 6.8% survival;p=0.018 log-rank). Conclusion: These cases suggest that pneumothorax is a complication of COVID-19. Pneumothorax does not seem to be an independent marker of poor prognosis and we encourage continuation of active treatment where clinically possible.