Interstitial lung disease progression after genomic usual interstitial pneumonia testing.

BACKGROUND: A genomic classifier for usual interstitial pneumonia (gUIP) has been shown to predict histological UIP with high specificity, increasing diagnostic confidence for idiopathic pulmonary fibrosis (IPF). Whether those with positive gUIP classification exhibit a progressive, IPF-like phenotype remains unknown. METHODS: A pooled, retrospective analysis of patients who underwent clinically indicated diagnostic bronchoscopy with gUIP testing at seven academic medical centres across the USA was performed. We assessed the association between gUIP classification and 18-month progression-free survival (PFS) using Cox proportional hazards regression. PFS was defined as the time from gUIP testing to death from any cause, lung transplant, ≥10% relative decline in forced vital capacity (FVC) or censoring at the time of last available FVC measure. Longitudinal change in FVC was then compared between gUIP classification groups using a joint regression model. RESULTS: Of 238 consecutive patients who underwent gUIP testing, 192 had available follow-up data and were included in the analysis, including 104 with positive gUIP classification and 88 with negative classification. In multivariable analysis, positive gUIP classification was associated with reduced PFS (hazard ratio 1.58, 95% CI 0.86-2.92; p=0.14), but this did not reach statistical significance. Mean annual change in FVC was -101.8 mL (95% CI -142.7- -60.9 mL; p<0.001) for those with positive gUIP classification and -73.2 mL (95% CI -115.2- -31.1 mL; p<0.001) for those with negative classification (difference 28.7 mL, 95% CI -83.2-25.9 mL; p=0.30). CONCLUSIONS: gUIP classification was not associated with differential rates of PFS or longitudinal FVC decline in a multicentre interstitial lung disease cohort undergoing bronchoscopy as part of the diagnostic evaluation.

Post-COVID Interstitial Lung Disease and Other Lung Sequelae.

As the world emerges from the COVID-19 pandemic, clinicians and researchers across the world are trying to understand the sequelae in patients recovered from COVID-19 infection. In this article, the authors review post-acute sequelae of SARS-COV-2, interstitial lung disease, and other lung sequelae in patients recovering from COVID-19 infection.

Development and Validation of a Clinical Diagnostic Scoring System for the Diagnosis of Idiopathic Pulmonary Fibrosis.

Rationale: Interpreting the radiologic data in conjunction with an objective clinical score could help to harmonize idiopathic pulmonary fibrosis (IPF) diagnosis and improve accuracy. Objectives: We sought to establish and validate a multivariable objective scoring model based on clinical parameters by stratifying the risk of patients having IPF diagnosed versus having other forms of interstitial lung disease (ILD) diagnosis. Methods: A clinical score was derived from review of patients evaluated at the Inova Fairfax ILD Program and validated in three distinct cohorts. On the basis of known IPF clinical characteristics, a multivariable model was created and assessed by using receiver operating characteristic curves. Results: There were 844 patients with ILD with either IPF (n = 347, 41%) or non-IPF ILD (n = 497, 59%) diagnosis. On the basis of calculated odds ratios, a score was assigned to each of the following clinical parameters: age, sex, smoking history, race or ethnicity, ILD family history, exposures, presence of connective tissue disease signs or symptoms, and velcro crackles. The final Fairfax IPF Clinical Score (FICS) ranged from 1 to 25. The clinical diagnostic score system was accurate in predicting IPF, as measured by the area under the curve (0.88) in the derivation cohort, with similar areas under the curve of 0.91, 0.81, and 0.71 being demonstrated in the respective validation cohorts. Conclusions: The FICS appears to be an accurate tool for estimating the pretest probability of IPF in patients with ILD. How the FICS performs in conjunction with the various high-resolution computed tomographic patterns remains to be determined. This model could ultimately be useful for increasing the degree of confidence in the final diagnosis and could help to obviate the need for lung biopsy in cases with non-usual interstitial pneumonia patterns on high-resolution computed tomographic images.

HRCT evaluation of patients with interstitial lung disease: comparison of the 2018 and 2011 diagnostic guidelines.

BACKGROUND AND AIMS: Chest high-resolution computed tomography (HRCT) is the central diagnostic tool in discerning idiopathic pulmonary fibrosis (IPF) from other interstitial lung disease (ILDs). In 2018, new guidelines were published and the nomenclature for HRCT interpretation was changed. We sought to evaluate how clinicians' interpretation would change based on reading HRCTs under the framework of the old versus new categorization. MATERIALS AND METHODS: We collated HRCTs from 50 random cases evaluated in the Inova Fairfax ILD clinic. Six ILD experts were provided the deidentified HRCTs. They were all instructed to independently provide two reads of each HRCT, based on the old and the new guidelines. RESULTS: The kappa statistic for concordance for HRCT reads under old guidelines was 0.5, while for the new guidelines it was 0.38. Under the framework of the old guidelines, there were 22 HRCTs with unanimous consensus reads, while only 15 with the new guidelines. There were 12 HRCTs read unanimously as usual interstitial pneumonia (UIP) pattern based on both the old and the new guidelines. Ten HRCTs were read as a possible UIP pattern based on the old guidelines and were classified in nine cases as probable UIP and one indeterminate based on the new guidelines. Of the 28 inconsistent UIP HRCTs (old guidelines), 25 were read as alternative diagnosis suggested, two were read as indeterminate and one as probable UIP. CONCLUSION: Implementation of the new guidelines to categorize HRCTs in ILD patients appears to be associated with greater inter-interpreter variability. How or whether new guidelines improve the care and management of ILD patients remains unclear.The reviews of this paper are available via the supplemental material section.

Pleuroparenchymal fibroelastosis (PPFE) treated with lung transplantation and review of the literature.

A 26-year-old woman presented with a 15-year history of non-progressive dyspnoea. Chest imaging showed bilateral apical pleural and parenchymal scarring, pleural thickening and bronchiectasis. Pulmonary function tests showed a moderate restrictive defect. Non-invasive workup was non-revealing; therefore, the patient was referred for video-assisted thoracic surgery and lung biopsy. Histopathology revealed pleural thickening and, subpleural parenchymal fibrosis and elastic tissue deposition. Lung parenchyma further away from the pleura was well preserved. Based on these findings, the patient was diagnosed with pleuroparenchymal fibroelastosis (PPFE). Since PPFE is a progressive disorder without effective medical therapies, and given our patient's worsening symptoms, she underwent bilateral lung transplantation. It has been almost 4 years since the lung transplantation, our patient continues to do well. To the best of our knowledge, to date, this is the longest follow-up reported for a PPFE patient undergoing lung transplantation.

Controlled trial to improve resident sign-out in a medical intensive care unit.

OBJECTIVE: Poor sign-out or handover of care may lead to preventable patient harm. Critically ill patients in intensive care units (ICU) are complex and prone to rapid clinical deterioration. If clinical deterioration occurs, timeliness of appropriate interventions is essential to prevent or reduce adverse outcomes. Therefore sign-outs need to efficiently transmit key information and provide anticipatory guidance. Interventions to improve resident-to-resident ICU sign-outs have not been well described. We conducted a controlled trial to test the effectiveness of a standardised ICU sign-out process to the usual ICU sign-out. DESIGN: Prospective controlled trial. SETTING: A 26-bed medical intensive care unit (MICU) in an urban tertiary academic medical centre. SUBJECTS: Residents rotating through the MICU. INTERVENTIONS: ICU-specific written sign-out template. METHODS: Residents completed postcall surveys assessing satisfaction with verbal and written sign-outs and incidence of non-routine events. Our main outcome of interest was the occurrence of non-routine events. MAIN RESULTS: Compared with the intervention group, on significantly more nights, night float residents in the control group encountered patients who were sicker than sign-out would have suggested (15.94% vs 43.75%; p<0.0001). On significantly fewer nights, night float residents in the intervention group indicated that either something happened to patients that was unexpected (18.84% vs 36.51%; p=0.023) or they were insufficiently prepared for (4.35% vs 35.94%; p<0.0001). Similarly, on fewer nights, residents in the intervention group indicated that they had to perform interventions that were unplanned or unanticipated (15.9% vs 37.7%; p=0.005). CONCLUSION: A structured sign-out process compared with usual sign-out significantly reduced the occurrence of non-routine events in an academic MICU.

Respiratory Complication in Liver Disease.

Cirrhosis, the twelfth leading cause of death, accounts for 1.1% of all deaths in the United States. Although there are multiple pulmonary complications associated with liver disease, the most important complications that cause significant morbidity and mortality are hepatopulmonary syndrome, hepatic hydrothorax, and portopulmonary hypertension. Patients with cirrhosis who complain of dyspnea should be evaluated for these complications. This article reviews these complications.

End-of-life care beliefs among Hindu physicians in the United States.

Several studies from the United States and Europe showed that physicians' religiosity is associated with their approach to end-of-life care beliefs. No such studies have focused exclusively on Hindu physicians practicing in the United States. A 34-item questionnaire was sent to 293 Hindu physicians in the United States. Most participants believed that their religious beliefs do not influence their practice of medicine and do not interfere with withdrawal of life support. The US practice of discussing end-of-life issues with the patient, rather than primarily with the family, seems to have been adopted by Hindu physicians practicing in the United States. It is likely that the ethical, cultural, and patient-centered environment of US health care has influenced the practice of end-of-life care by Hindu physicians in this country.

Intrathoracic rosai-dorfman disease: hemorrhage with routine diagnostic procedure.

We present a case of a 33-year-old female with a slow growing, right peribronchial vascular mass and associated symptoms of progressive cough, dyspnea on exertion, and hemoptysis. On routine diagnostic flexible bronchoscopy with needle biopsy, the lesion hemorrhaged extensively requiring emergent thoracotomy, right lower and middle bilobectomy. The histopathology of the specimen was consistent with the rare and unusually located entity Rosai-Dorfman disease.

Blood Loss through AV Fistula: A Case Report and Literature Review.

Little has been written about acute blood loss from hemodialysis vascular access. We describe a 57-year-old Caucasian male with an approximately 7 gm/dL drop in hemoglobin due to bleeding from a ruptured aneurysm in his right brachiocephalic arteriovenous fistula (AVF). There was no evidence of fistula infection. The patient was successfully managed by blood transfusions and insertion of a tunneled dialysis catheter for dialysis access. Later, the fistula was ligated and a new fistula was constructed in the opposite arm. Aneurysm should be considered in cases of acute vascular access bleeding in chronic dialysis patients.

Malignant transformation of actinic keratoses to squamous cell carcinoma in an albino.

A 25-year-old male, who was a known case of oculocutaneous albinism presented to us with right inguinal swellings of six months' duration. He gave a preceding history of a similar lump in the right thigh, which was excised at the Chennai Government Hospital. He was diagnosed to have oculocutaneous albinism with actinic keratoses, with multiple squamous cell carcinomas (with metastatic deposits in the right inguinal region) and cutaneous horns. The case is reported to highlight preventive aspects in the management of albinos.