Stella Piel’s research while affiliated with Heidelberg University and other places

Background Pulmonary lymphangiomatosis (PL) is an ultrarare disease characterized by diffuse infiltration of the lung, pleura and/or mediastinum by abnormal lymphatic proliferation. Consented diagnostic or treatment approaches are not established. We therefore aimed to collect data on diagnostics and treatments in a cohort of patients with PL from a tertiary center for rare lung diseases. Methods Clinical, radiological and outcome data from PL patients were collected retrospectively. Results 12 patients were diagnosed between 1996 and 2022 in our center. PL was diagnosed more commonly in female (58%), never smokers (75%) and younger patients (mean age 42 years). Main clinical symptoms comprised haem- and chyloptysis (58%) and dyspnea on exertion (83%). Pulmonary function was mostly restrictive (mean VC 59%) with impaired DLCO (mean 65%). Radiological assessment mainly showed mediastinal involvement (83%), and pleural effusion (67%), pleural thickening (67%) and bronchial wall thickening (67%) while interstitial changes were rare. Diagnosis was confirmed by surgical or transbronchial cryobiopsy. 8 patients were treated with sirolimus, 3 of these combined with a surgical intervention and in one case surgical intervention was necessary 9 months after initiation of sirolimus. Clinical and radiological improvement was demonstrated for all patients treated with sirolimus. 1 patient received a lung transplant due disease progression. Survival rates were 90% after a mean follow up of at least 3 months. Conclusion This case series illustrates the variability of the clinical presentation of PL. Among our patients, those treated with sirolimus showed significant clinical, functional and radiological improvement. However, further investigation is needed to understand the pathogenesis of lymphangiomatosis in order to establish therapeutic approaches.

BACKGROUND: Pulmonary lymphangiomatosis (PL) is an ultrarare disease characterized by diffuse infiltration of the lung, pleura and/or mediastinum by abnormal lymphatic proliferation. Consented diagnostic or treatment approaches are not established. We therefore aimed to collect data on diagnostics and treatments in a cohort of patients with PL from a tertiary center for rare lung diseases. METHODS: Clinical, radiological and outcome data from PL patients were collected retrospectively. RESULTS: 12patients were diagnosed between 1996 and 2022 in our center. PL was diagnosed more commonly in female (58%), never smokers (75%) and younger patients (mean age 42 years). Main clinical symptoms comprised haem- and chyloptysis (58%) and dyspnea on exertion (83%). Pulmonary function was mostly restrictive (mean VC 59%) with impaired DLCO (mean 65%). Radiological assessment mainly showed mediastinal involvement (83%), and pleural effusion (67%), pleural thickening (67%) and bronchial wall thickening (67%) while interstitial changes were rare. Diagnosis was confirmed by surgical or transbronchial cryobiopsy. 8 patients were treated with sirolimus, 3 of these combined with a surgical intervention and in one case surgical intervention was necessary 9 month after initiation of sirolimus. Clinical and radiological improvement was demonstrated for all patients treated with sirolimus. 1 patient received a lung transplant due disease progression. Survival rates were 90 % after a mean follow up of at least 3 months. CONCLUSION: This case series illustrates the variability of the clinical presentation of PL. Among our patients, those treated with sirolimus showed significant clinical, functional and radiological improvement. However, further investigation is needed to understand the pathogenesis of lymphangiomatosis in order to establish therapeutic approaches.

Background: Staff shortages pose a major challenge to the health system. Objectives: The objective of this study was to clarify the role of different causative factors we investigated on staff absenteeism during the COVID pandemic. Methods: The prospective multicentre cohort study assessed the private and professional impact of the pandemic on health care workers (HCWs) using a specially developed questionnaire. HCWs from 7 specialist lung clinics throughout Germany were surveyed from December 1 to December 23, 2021. The current analysis addresses pandemic-related absenteeism. Results: 1,134 HCW (55% female; 18.4% male, 26.3% not willing to provide information on age or gender) participated. 72.8% had received at least one vaccination dose at the time of the survey, and 9.4% reported a COVID infection. Of those with positive tests, 98% reported home quarantine for median (IQR) 14 (12-17) days; 10.3% of those who ultimately tested negative also reported quarantine periods of 14 (7-14) days. 32.2% of vaccinated respondents reported absenteeism due to vaccine reactions of 2 (1-3) days. Overall, 37% (n = 420) of HCW reported pandemic-related absenteeism, with 3,524 total days of absenteeism, of which 2,828 were due to illness/quarantine and 696 to vaccination effects. Independent risk factors for COVID-related absenteeism ≥5 days included already having COVID, but also concern about long-term effects of COVID (OR 1,782, p = 0.014); risk factors for vaccine-related absenteeism ≥2 days included concerns of late effects of vaccination (OR 2.2, 95% CI: 1.4-3.1, p < 0.000). Conclusion: Staff shortages due to quarantine or infections and vaccine reactogenicity have put a strain on German respiratory specialists. The fact that staff concerns also contributed to absenteeism may be helpful in managing future pandemic events to minimize staff absenteeism.

Granulome als Zeichen der spezifischen Entzündung im Lungengewebe treten bei vielen Erkrankungen auf. Das radiologische Standardverfahren bei pulmonalen Granulomatosen ist meistens die Dünnschichtcomputertomografie. Bei klinischem Verdacht und entsprechenden Hinweisen in der Thoraxübersicht sind eine Dünnschicht-CT und die interdisziplinäre Diskussion der Befunde zu empfehlen.

CLINICAL/METHODICAL ISSUE Granulomas as signs of specific inflammation of the lungs are found in various diseases with pulmonary manifestations and represent an important imaging finding. STANDARD RADIOLOGICAL METHODS The standard imaging modality for the work-up of granulomatous diseases of the lungs is most often thin-slice computed tomography (CT). There are a few instances, e. g. tuberculosis, sarcoidosis and silicosis, where a chest radiograph still plays an important role. METHODICAL INNOVATIONS Further radiological modalities are usually not needed in the routine work-up of granulomatous diseases of the chest. In special cases magnetic resonance imaging (MRI) and positron emission tomography (PET)-CT scans play an important role, e. g. detecting cardiac sarcoidosis by cardiac MRI or choline C‑11 PET-CT in diagnosing lung carcinoma in scar tissue after tuberculosis. PERFORMANCE The accuracy of thin-slice CT is very high for granulomatous diseases. ACHIEVEMENTS In cases of chronic disease and fibrotic interstitial lung disease it is important to perform thin-slice CT in order to diagnose a specific disease pattern. Thin-slice CT is also highly sensitive in detecting disease complications and comorbidities, such as malignancies. Given these indications thin-slice CT is generally accepted in the routine daily practice. PRACTICAL RECOMMENDATIONS A thin-slice CT and an interdisciplinary discussion are recommended in many cases with a suspected diagnosis of pulmonary granulomatous disease due to clinical or radiographic findings.

Background: Gastrooesophageal reflux (GER) may play a role in the pathogenesis and disease progression of ILDs by recurrent episodes of microaspiration initiating an inflammatory response with subsequent fibrosis. Yet, the prevalence of laryngopharyngeal reflux (LPR) reflecting GER is unknown in ILD patients. Aim: To assess the occurrence and severity of LPR in ILD patients Methods: 11 patients were enrolled (non-specific interstitial pneumonia (n=2), chronic hypersensitivity pneumonitis (n=3), idiopathic pulmonary fibrosis (n=3), desquamative interstitial pneumonia (n=1), connective tissue ILD (n=1), non classifiable ILD (n=1)) PH was measured over 24 hours in the posterior nasopharynx by a Restech Dx pH probe. Reflux symptoms, position and meals were documented on a portable device by the patients. Reflux symptoms were also assessed by a reflux symptom questionnaire (RSI). LPR was defined as pH <5,5 in an upright position and <5 in a lying position using the RYAN-score according to the DeMeester criteria. Results: LPR was diagnosed in 10 out of 11 patients (46 % non-smoker, means: 68 years, forced vital capacity 74%, diffusing capacity of the lung 49 %). Mean pH was 6.2 (min 3.0, max 7.6). 18 % had an exclusively reflux during night, 82 % patients had a combined reflux in upright and lying position. 46 % had a LPR-positive RSI over 13 points. The RYAN-Score was significantly pathologic in 64 % of the cases. Conclusion: LPR seems to be an underestimated phenomenon in asymptomatic ILD patients. Lager numbers and longer observation periods are required to evaluate the impact on disease progression.