Citations

Abstract Background: Birt-Hogg-Dube (BHD) syndrome is a rare genetic condition associated with the development of renal tumors. This study aims to determine typical age ranges for detecting renal abnormalities, risk factors for tumor development, and long-term outcomes based on current surveillance strategies. Methods: A single-center multi-site retrospective cohort study was performed on all patients with BHD diagnosed from 2000 to 2023. Baseline demographics, pulmonary function, laboratory, radiologic, and histopathologic findings were collected. Logistic regression was used to assess predictor variables for the development of renal tumors with survival analysis evaluated from the date of BHD diagnosis to date of death or last known follow-up. Results: The study included 149 patients with BHD, 39 (26%) with diagnosed renal tumors, of which 28 had histopathologic confirmation. Mean age at renal tumor detection was 53.61 years. Older age and male sex were predictive of renal tumor development ((odds ratio 1.05; 95% CI, 1.01-1.08, P = 0.002) and (odds ratio 2.59; 95% CI, 1.17-5.73, P=0.02), respectively). Time to all-cause mortality appeared shorter in those with renal tumors (Log-rank P = 0.02), though no deaths were from cancer or cancer-related complications. Conclusions: Current screening protocols for renal tumors in BHD suggest the most common presenting age range for presentation is late 40s to early 50s, with older age and male sex as risk factors for tumor development.

Abstract Introduction Interstitial lung disease (ILD) is a heterogenous group of parenchymal lung disorders with multiple etiologies. ILD often affects the quality of life and survival of patients. OSA is commonly diagnosed among ILD patients with reported prevalences of 50-90%, and a prevalence of moderate to severe OSA as high as 65%. There is also concern that patients with ILD may be less tolerant of PAP therapies. We aim to identify differences in PAP treatment response and adherence in patients with ILD and OSA compared with a group of matched OSA controls. Methods A retrospective cohort analysis was conducted on patients ≥ 18 years-old that were evaluated at Mayo Clinic Center for Sleep Medicine between July 2012 to June 2022. Inclusion criteria were patients with a diagnosis of ILD per ATS criteria also diagnosed with OSA by HSAT or PSG. Control OSA patients were matched to ILD/OSA patients by age, BMI, and AHI ranges. Exclusion criteria included comorbid conditions of asthma or COPD. Patients with early non-adherence to CPAP or those who didn't have sleep follow up were also excluded. Follow-up sleep visits were assessed to determine CPAP adherence, change in PAP pressure, addition of oxygen, and change in Epworth sleepiness scale. Results We compared 51 patients with ILD/OSA and 51 OSA matched-controls. Twenty-five ILD/OSA patients were diagnosed by PSG and 26 by HSAT. Median treatment follow-up for the ILD group was 7 months (IQR of 3 to 18 months). Baseline characteristics, including oxygen desaturation index (ODI), oxygen saturation, time spent below 89% during overnight oximetry, and baseline Epworth scale, were similar between ILD/OSA and non-ILD OSA patients. Importantly, there was no statistically significant difference in PAP adherence, median PAP levels, PAP pressure change, residual AHI, or change in Epworth after treatment Conclusion There was no significant difference in baseline OSA characteristics, treatment response, and PAP adherence in ILD patients with OSA compared with matched non-ILD OSA controls. Outcome assessment may be limited by sample size and the shorter follow-up period. Future studies should include longer-term follow-up with a larger ILD cohort to assess the impact of ILD progression on PAP adherence over time. Support (if any)

Antifibrotic treatment has been approved for reducing disease progression in fibrotic interstitial lung disease (ILD). As a result of increased bleeding risk, some experts suggest cessation of antifibrotics prior to lung transplantation (LT). However, extensive knowledge regarding the impact of antifibrotic treatment on postoperative complications remains unclear. We performed a comprehensive search of several databases from their inception through to 30 September 2021. Original studies were included in the final analysis if they compared postoperative complications, including surgical wound dehiscence, anastomosis complication, bleeding complications, and primary graft dysfunction, between those with and without antifibrotic treatment undergoing LT. Of 563 retrieved studies, 6 studies were included in the final analysis. A total of 543 ILD patients completing LT were included, with 161 patients continuing antifibrotic treatment up to the time of LT and 382 without prior treatment. Antifibrotic treatment was not significantly associated with surgical wound dehiscence (RR 1.05; 95% CI, 0.31–3.60; I2 = 0%), anastomotic complications (RR 0.88; 95% CI, 0.37–2.12; I2 = 31%), bleeding complications (RR 0.76; 95% CI, 0.33–1.76; I2 = 0%), or primary graft dysfunction (RR 0.87; 95% CI, 0.59–1.29; I2 = 0%). Finally, continuing antifibrotic treatment prior to LT was not significantly associated with decreased 1-year mortality (RR 0.80; 95% CI, 0.41–1.58; I2 = 0%). Our study suggests a similar risk of postoperative complications in ILD patients undergoing LT who received antifibrotic treatment compared to those not on antifibrotic therapy.

Abstract Introduction Previous studies have shown that sleep disordered breathing (SDB) is common in patients with Interstitial Lung Disease (ILD), and oximetry is often used for screening prior to further diagnostic testing. Current guidelines recommend polysomnography (PSG) for diagnosis of SDB in patients with significant pulmonary disease, however, home sleep apnea tests (HSAT) are increasingly used in clinical practice for a variety of reasons despite lack of evidence regarding accuracy in this population. In this study, we evaluate the correlation between screening oximetry, a commercial brand HSAT (WatchPAT®) and PSG to examine the diagnostic accuracy of this HSAT technology in patients with ILD. Methods The institution electronic medical record was screened for patients with a diagnosis code for ILD who underwent screening oximetry followed by PSG or HSAT using peripheral arterial tonometry from July 1, 2012 to present. Clinical review confirmed presence of ILD according to American Thoracic Society guidelines. Among the respective cohorts, Paired Wilcoxon Test was used to compare the oximetry 4% oxygen desaturation index (ODI) to the HSAT ODI and PSG apnea-hypopnea index (AHI) as well as percent time spent below oxyhemoglobin saturation of 89%. Spearman correlation was used to correlate the oximetry ODI and parameters of SDB on HSAT and PSG. Results Data was analyzed for 25 patients who had undergone oximetry/HSAT and for 25 patients who had undergone oximetry/PSG. Oximetry ODI showed no significant difference from PSG AHI (p = 0.2635) or between HSAT ODI (p = 0.0755), and no difference was seen in hypoxic time between oximetry and PSG (p = 0.9789). Hypoxic time on HSAT was significantly longer than that on oximetry (p < 0.001). Using HSAT ODI as the standard, HSAT AHI and respiratory disturbance index (RDI) showed rs of 0.9638 and 0.8913 respectively, while oximetry ODI was 0.3893. Compared to PSG AHI, the PSG RDI and oximetry ODI rs were 0.9759 and 0.7407 respectively. Conclusion Among patients with ILD, screening oximetry appears to correlate more strongly with indices of SDB and hypoxic time on PSG rather than HSAT. Further studies are warranted to evaluate efficacy of additional HSAT testing modalities in this patient population. Support (If Any)

Context.— Studies of lungs in patients with COVID-19 have focused on early findings. Objective.— To systematically study histopathologic and imaging features and presence of SARS-CoV-2 RNA in lung tissue from patients in later stages of COVID-19. Design.— Autopsies, explants, surgical lung biopsies, transbronchial biopsies, cryobiopsies, and needle biopsies from patients with COVID-19 whose onset of symptoms/confirmed diagnosis was more than 28 days before the procedure were studied. Available images were reviewed. Reverse transcription droplet digital polymerase chain reaction for SARS-CoV-2 RNA was performed on lung tissue. Results.— Of 44 specimens (43 patients; median age, 59.3 years; 26 [60.5%] male) features of acute lung injury (ALI) were seen in 39 (88.6%), predominantly organizing pneumonia and diffuse alveolar damage, up to 298 days after onset of COVID-19. Fibrotic changes were found in 33 specimens (75%), most commonly fibrotic diffuse alveolar damage (n = 22) and cicatricial organizing pneumonia (n = 12). Time between acquiring COVID-19 and specimen was shorter in patients with diffuse ALI (median, 61.5 days) compared with patients with focal (140 days) or no ALI (130 days) (P = .009). Sixteen (of 20; 80%) SARS-CoV-2 reverse transcription droplet digital polymerase chain reaction tests were positive, up to 174 days after COVID-19 onset. Time between COVID-19 onset and most recent computed tomography in patients with consolidation on imaging was shorter (median, 43.0 days) versus in patients without consolidation (87.5 days; P = .02). Reticulations were associated with longer time to computed tomography after COVID-19 onset (median, 82 versus 23.5 days; P = .006). Conclusions.— ALI and SARS-CoV-2 RNA can be detected in patients with COVID-19 for many months. ALI may evolve into fibrotic interstitial lung disease.