Nonspecific Interstitial Pneumonitis Research Articles

S3843 A Rare Case of Non-Specific Interstitial Pneumonitis (NSIP) Secondary to Infliximab in a Patient With Ulcerative Pancolitis and PANCA Positivity

S3843 A Rare Case of Non-Specific Interstitial Pneumonitis (NSIP) Secondary to Infliximab in a Patient With Ulcerative Pancolitis and PANCA Positivity

A study on the prevalence and prognosis of progressive pulmonary fibrosis: A retrospective observational study.

Interstitial lung disease (ILD) encompasses a heterogeneous group of more than 200 diffuse parenchymal lung diseases with various clinical courses. Disease progression is one of the most important prognostic factors, and, the definition of progressive pulmonary fibrosis (PPF) has recently been established. This study aimed to estimate the prevalence, risk factors, and prognosis of PPF among patients with non-idiopathic pulmonary fibrosis (IPF) in real-world practice. A total of 215 patients were retrospectively analyzed between January 2010 and June 2023 at the Haeundae Paik Hospital in the Republic of Korea. According to the criteria proposed in 2022 by Raghu et al, PPF defined as a condition that satisfies 2 or more of the following in the past year: worsening of respiratory symptoms, physiological evidence of disease progression, and radiological evidence of disease progression. The median age of the subjects was 67 years and 63.7% were female. A total of 40% was diagnosed with PPF and connective tissue disease-associated ILD (52.3%) was the most common type, followed by nonspecific interstitial pneumonitis (NSIP) (25.6%) and cryptogenic organizing pneumonitis (16.3%). In multivariate logistic regression for predicting PPF, both the use of steroids and immunosuppressants (OR: 2.57, 95% CI: 1.41-4.67, P = .002) and home oxygen use (OR: 25.17, 95% CI: 3.21-197.24, P = .002) were independent risk factors. During the follow-up period, the mortality rate was significantly higher in the PPF group than in the non-PPF group (24.4% vs 2.3%, P < .001). In the survival analysis using the Cox proportional hazard regression model, disease progression, older age and lower forced vital capacity (FVC) were independent risk factors for mortality. Our study demonstrated that the prevalence of PPF was 40%. Concomitant therapy of steroids with an immunosuppressants and home oxygen use are risk factors for PPF. PPF itself was significantly associated with high mortality rates. Risk factors for mortality were disease progression, older age, and lower FVC.

Clinical features and risk factors for primary Sjögren's syndrome combined with interstitial lung disease: a retrospective study.

To analyze the clinical characteristics of primary Sjögren's syndrome (pSS) combined with interstitial lung disease (ILD), so as to provide a theoretical basis for the early diagnosis, treatment and prevention of PSS-ILD. From October 2017 to January 2022, patients with pSS who were admitted to the Department of Rheumatology at Yueyang Hospital of Integrated Traditional Chinese and Western Medicine, Shanghai University of Traditional Chinese Medicine were included in this retrospective study. Patients were divided into the pSS-ILD (102 cases) and pSS-non-ILD groups (154 cases) based on the presence or absence of ILD on high-resolution computed tomography (HRCT). Demographics information, clinical symptoms, laboratory indicators and HRCT features were compared, and the logistic regression analysis was utilized to identify the risk factors. A total of 256 patients were included. Patients with pSS-ILD were more often female, and their age and disease duration were significantly higher than those in the pSS-non-ILD group (p < 0.05). The HRCT imaging classification included ground glass-like shadow (78.4%) and patchy solid shadow (17.6%), and Non-specific interstitial pneumonitis (NSIP) (72.5%) was the predominant typology. Regarding the laboratory indexes, the positive rates of erythrocyte sedimentation rate, C-reactive protein, white blood cell count, neutrophil/lymphocyte ratio, triglycerides, total cholesterol, and anti-SS-A52 antibodies were significantly higher in the pSS-ILD patients than in the pSS-non-ILD group, while the positive rates of anti-synaptic antibodies were lower than in the pSS-non-ILD group, and the differences between two groups were statistically significant (p < 0.05). Logistic regression showed that age >60years, longer duration of disease, higher triglycerides, and cholesterol were risk factors for pSS-ILD patients. The clinical features of pSS-ILD patients were xerophthalmia, cough and shortness of breath, and HRCT can help to diagnose the disease at an early stage. Age over 60years, chronic course of disease, and elevated lipid levels are risk factors for ILD in pSS patients, and the relationship between autoimmune antibody levels and the occurrence of ILD needs to be further confirmed in follow-up studies with large sample sizes. These findings have the potential to provide useful information for early diagnosis, treatment, and prevention of the development of pSS-ILD.

Abstract 18975: First Reported Case of SLE Associated Recurrent Pericarditis Treated With Rilonacept

Description of case: A 42 year old female presented with a 1 month history of exertional dyspnea and intermittent pleuritic chest pain. She had a 12-year history of systemic lupus erythematosus (SLE) and non-specific interstitial pneumonitis being managed with mycophenolate mofetil. Labs showed an ESR of 76 mm/hr and CRP of 5.5 mg/dL. CT chest ruled out pulmonary embolism but showed a moderate pericardial effusion, also seen on an echocardiogram. A cardiac MRI (cMRI) showed moderate diffuse circumferential pericardial enhancement. She was diagnosed with lupus pericarditis and initially treated with ibuprofen, colchicine, prednisone, and hydroxychloroquine; however, continued to have persistent symptoms over the next few months. Repeat cMRI showed a transition to effusive constrictive physiology. She was offered pericardiectomy but following shared decision making and multi-disciplinary discussion, she was started on Rilonacept. She experienced significant and sustained relief in symptoms, improvement in inflammatory markers and reduction of pericardial enhancement on cMRI. Discussion To the best of our knowledge, this is the first case of Rilonacept used to successfully treat recurrent pericarditis from SLE. Recurrence in chronic idiopathic pericarditis is thought to be driven by the innate immune system and Interleukin-1 (IL-1), while in autoimmune diseases, it is mediated by the adaptive immune system and Type 1 Interferon, which is associated with a poorer prognosis. The RHAPSODY and AIRTRIP trials demonstrating efficacy of IL-1 blockers in recurrent pericarditis excluded patients with autoimmune diseases. Use of IL-1 blockers to treat Lupus pericarditis has only been reported in 2 cases using Anakinra. Our patient’s positive response to Rilonacept suggests the presence of additional target pathways in autoimmune diseases that may mediate pericarditis recurrence. Future prospective studies are needed to elucidate this further.

Plasma extracellular vesicle proteins as promising noninvasive biomarkers for diagnosis of idiopathic pulmonary fibrosis.

High-resolution computed tomography (HRCT) imaging is critical for diagnostic evaluation of Idiopathic Pulmonary Fibrosis (IPF). However, several other interstitial lung diseases (ILDs) often exhibit radiologic pattern similar to IPF on HRCT making the diagnosis of the disease difficult. Therefore, biomarkers that distinguish IPF from other ILDs can be a valuable aid in diagnosis. Using mass spectrometry, we performed proteomic analysis of plasma extracellular vesicles (EVs) in patients diagnosed with IPF, chronic hypersensitivity pneumonitis, nonspecific interstitial pneumonitis, and healthy subjects. A five-protein signature was identified by lasso regression and was validated in an independent cohort using ELISA. The five-protein signature derived from mass spectrometry data showed an area under the receiver operating characteristic curve of 0.915 (95%CI: 0.819-1.011) and 0.958 (95%CI: 0.882-1.034) for differentiating IPF from other ILDs and from healthy subjects, respectively. Stepwise backwards elimination yielded a model with 3 and 2 proteins for discriminating IPF from other ILDs and healthy subjects, respectively, without compromising diagnostic accuracy. In summary, we discovered and validated EV protein biomarkers for differential diagnosis of IPF in independent cohorts. Interestingly, the biomarker panel could also distinguish IPF and healthy subjects with high accuracy. The biomarkers need to be evaluated in large prospective cohorts to establish their clinical utility.

Clinical and Serological Profile of Systemic Sclerosis Patients in a Tertiary Care Center in Kashmir, North India

Abstract Background: Systemic sclerosis (SSc) is an autoimmune disease that varies by ethnicity and geography. India, being a multiethnic country with many different geographical regions, could have such differences. However, due to a lack of data, such differences remain unknown. Aim: The aim of the study was to study clinical manifestations, organ involvement, autoantibodies, and associated autoimmune diseases in subtypes of SSc patients from Jammu and Kashmir. Materials and Methods: This single-center observational study was conducted in two parts, retrospective and prospective. A total of 112 patients were included. Data were compiled from inpatient and outpatient department records of 66 patients for retrospective part from 2012 to 2016. Prospective data were taken from 46 patients from 2016 to 2018. Patient history, clinical examination, pulmonary function test, high-resolution computed tomography (HRCT), and echocardiography findings were recorded. Results: The female: male ratio was 15:1, with a mean age of 38.3 ± 12.51 years. Sixty-five (58%) patients had limited cutaneous SSc (LcSSc), 22 (19.6%) had diffuse cutaneous SSc, 5 (4.4%) had early scleroderma, and 20 (17.8%) patients had overlap with other autoimmune diseases. Antinuclear antibody was present in 87.5% of patients. Interstitial lung disease was seen in 80.41% of the patients by HRCT, with majority having nonspecific interstitial pneumonitis pattern. Pulmonary artery hypertension was present in 14.28% of the study patients. Conclusions: In the current study and the rest of India, disease onset is a decade earlier than in the Caucasian population. There is a significant variation in presentation among patients in India, with patients from Jammu and Kashmir reporting a higher prevalence of Raynaud’s phenomenon and LcSSc.

Descriptive study of the role of ultrasound in the evaluation of patients with interstitial lung disease associated with autoimmune connective tissue disorders

Background and Aim: High-resolution computed tomography (HRCT) of the chest has been the conventionally accepted modality of radiological investigation utilized in the evaluation and management of interstitial lung diseases (ILDs). The aim of this study was to compare the utilization of lung ultrasound (LUS) as a radiological modality versus HRCT scan of the chest, in the diagnosis of cases of ILD in autoimmune connective tissue disease (AICTD) patients at a tertiary care center in Northern India. Methods: In this descriptive study, 42 consecutive diagnosed cases of AICTD with clinical risk of ILD were included, between July 2016 and March 2019, attending the rheumatology outpatient department of CH WC. They were assessed with lung ultrasonography and HRCT chest. Sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) of LUS were estimated considering HRCT as the reference method. Results: Of these 42 patients, 30 (71.4%) had abnormal LUS findings. HRCT findings suggestive of ILD were seen in 31 (73.8%). Considering HRCT as gold standard, LUS resulted in 01 false-positive and 02 false-negative results. Sensitivity and specificity of LUS with respect to HRCT were 93.55% and 90.91%, respectively. Positive predictive value (PPV) and negative predictive value (NPV) of LUS were calculated at 89.38% and 94.51%, respectively. In 91.6% (11/12) patients with nonspecific interstitial pneumonitis pattern on HRCT, B-lines were found to be numerous and compact (<3 mm distance between B lines). On the other hand, compact B lines were seen in only 21.1% (4/19) cases showing HRCT patterns suggestive of usual interstitial pneumonitis (UIP)/likely UIP, with a significant difference between the two (P = 0.0002). Conclusions: LUS is as nearly sensitive and specific as HRCT chest in the detection of ILD and has the distinct advantage of being without radiation exposure.

Frequency of Interstitial Lung Disease in Rheumatoid Arthritis Patients: A Hospital-Based Study.

Among various extra-articular manifestations of rheumatoid arthritis (RA), interstitial lung disease (ILD) is the most frequent and concerning manifestation. The reported frequency of RA-associated ILD (RA-ILD) varies in the literature. The objective of the present study was to determine the frequency of ILD in RA patients at a teaching hospital in Rawalpindi. 175 male and female patients between 18-70 years were enrolled in the study from January 21, 2022, till July 24, 2022. Patients diagnosed with RA were screened for the concomitant presence of ILD (increased pulmonary markings on chest X-rays and total lung capacity ≤ 80%, predicted on pulmonary function tests). In addition, the frequency of RA-ILD was noted and compared across various subgroups of patients based on age, gender, and disease duration. The mean age of the patients was 45.3 ± 11.5 years. The male-to-female ratio was 1:3.1. The mean disease duration was 6.2 ± 3.5 years at the time of presentation. A total of 118 (67.4%) patients were diagnosed with RA-ILD. The frequency of RA-ILD was significantly higher among patients with a prolonged duration of disease, < 5 years vs. ≥ 5 years (59.1% vs. 75.9%; p-value=0.018). Among 118 patients with RA-ILD, usual interstitial pneumonia (UIP) was the most frequent pattern and was noted in 74 (62.7%) patients, followed by nonspecific interstitial pneumonitis (NSIP), which was noted in 44 (37.3%) patients. When compared, there was no statistically significant difference in the frequency of high-resolution CT (HRCT) pattern of RA-ILD across various subgroups of patients based on age (p-value=0.969), gender (p-value=0.934), and duration of disease (p-value=0.881). In the present study, a substantial proportion of RA patients suffered RA-ILD, which warrants routine screening of these patients for undiagnosed pulmonary involvement so that timely identification and anticipated management may improve the outcome of such cases in future clinical practice.

CYSTIC LUNG DISEASE IN A YOUNG MALE WITH SYSTEMIC LUPUS ERYTHEMATOSUS

CYSTIC LUNG DISEASE IN A YOUNG MALE WITH SYSTEMIC LUPUS ERYTHEMATOSUS

OA17 An evolving case of Anti PM/Scl Anti body associated Aggressive Systemic Sclerosis

OA17 An evolving case of Anti PM/Scl Anti body associated Aggressive Systemic Sclerosis

Enfermedad pulmonar intersticial difusa y su relación con el humo de leña estimado por un índice de exposición anual IEAHL

Introduction: lung diseases that affect the interstice have multicausal etiology where exposure to biomass using firewood as a fuel source in food preparation continues to be rooted in our communities. Objective: to describe an index that quantifies the risk of developing diffuse interstitial lung disease, ILD, in relation to the degree of exposure to wood smoke, according to the number of main meals cooked with said method, multiplied by the number of years exposed, IEAHL. Material and Method: study of cases and controls. We analyzed 37 cases of ILD patients and their relationship to wood smoke based on our own proposal for an annual exposure index and 33 controls admitted for surgical treatment in general surgery, with ethnic characteristics, age and sociocultural status similar to the cases studied. For IEAHL, the range 50 to 99 was chosen for moderate degree exposure and more than 100 severe degree. Results: severe (OR=26 95%CI 2.63 – 270) and moderate (OR=9.6795%CI 1.32 – 70.97) IEAHL are associated with ILD type non-specific interstitial pneumonitis (NSIP), while usual interstitial pneumonitis (UIP) only to a severe degree (OR=20 95%CI 2.04 – 196.03). Conclusion: the index of annual exposure to wood smoke IEAHL is presented as an objective and simple alternative to determine the risk of suffering from diffuse interstitial lung diseases and was found to be associated with NSIP and UIP, thus establishing a relationship between severity of exposure and ILD development.

Quantitative CT and machine learning classification of fibrotic interstitial lung diseases.

To evaluate quantitative computed tomography (QCT) features and QCT feature-based machine learning (ML) models in classifying interstitial lung diseases (ILDs). To compare QCT-ML and deep learning (DL) models' performance. We retrospectively identified 1085 patients with pathologically proven usual interstitial pneumonitis (UIP), nonspecific interstitial pneumonitis (NSIP), and chronic hypersensitivity pneumonitis (CHP) who underwent peri-biopsy chest CT. Kruskal-Wallis test evaluated QCT feature associations with each ILD. QCT features, patient demographics, and pulmonary function test (PFT) results trained eXtreme Gradient Boosting (training/validation set n = 911) yielding 3 models: M1 = QCT features only; M2 = M1 plus age and sex; M3 = M2 plus PFT results. A DL model was also developed. ML and DL model areas under the receiver operating characteristic curve (AUC) and 95% confidence intervals (CIs) were compared for multiclass (UIP vs. NSIP vs. CHP) and binary (UIP vs. non-UIP) classification performances. The majority (69/78 [88%]) of QCT features successfully differentiated the 3 ILDs (adjusted p ≤ 0.05). All QCT-ML models achieved higher AUC than the DL model (multiclass AUC micro-averages 0.910, 0.910, 0.925, and 0.798 and macro-averages 0.895, 0.893, 0.925, and 0.779 for M1, M2, M3, and DL respectively; binary AUC 0.880, 0.899, 0.898, and 0.869 for M1, M2, M3, and DL respectively). M3 demonstrated statistically significant better performance compared to M2 (∆AUC: 0.015, CI: [0.002, 0.029]) for multiclass prediction. QCT features successfully differentiated pathologically proven UIP, NSIP, and CHP. While QCT-based ML models outperformed a DL model for classifying ILDs, further investigations are warranted to determine if QCT-ML, DL, or a combination will be superior in ILD classification. • Quantitative CT features successfully differentiated pathologically proven UIP, NSIP, and CHP. • Our quantitative CT-based machine learning models demonstrated high performance in classifying UIP, NSIP, and CHP histopathology, outperforming a deep learning model. • While our quantitative CT-based machine learning models performed better than a DL model, additional investigations are needed to determine whether either or a combination of both approaches delivers superior diagnostic performance.

Bridging to lung transplantation using high frequency oscillatory ventilation in pediatric non-specific interstitial pneumonitis

Bridging to lung transplantation using high frequency oscillatory ventilation in pediatric non-specific interstitial pneumonitis

December 2021 Pulmonary Case of the Month: Interstitial Lung Disease with Red Knuckles

No abstract available. Article truncated after 150 words. History of Present Illness A 56-year-old man was referred for a second opinion on recent onset of diffuse parenchymal lung disease. He had started noting mild dyspnea with yard work approximately in March 2021. His symptoms progressed over the next month with increasing shortness of breath and some fever. He presented to outside emergency department on April 17, 2021 and chest CT showing patchy ground-glass opacities with some areas of irregular consolidation (Figure 1). He was subsequently seen by an outside pulmonologist and started empirically on prednisone (50 mg/day). An outside lung biopsy had been performed which showed nonspecific interstitial pneumonitis. There was some improvement in his symptoms and his prednisone dose was reduced to 20 mg/day; however, his symptoms subsequently worsened with saturations noted to drop to 85% with any ambulation. He also had swelling of his left face and a biopsy of the parotid gland with the findings …

GOOD COP OR BAD COP: USE OF MACROLIDES TO ARREST THE INFLAMMATORY TURMOIL OF CRYPTOGENIC ORGANIZING PNEUMONIA

TOPIC: Diffuse Lung Disease TYPE: Medical Student/Resident Case Reports INTRODUCTION: Cryptogenic organizing pneumonia (COP) represents a rare type of idiopathic interstitial pneumonia that is characterized by the recruitment of inflammatory cells and deposition of fibrinous material in the form of intraluminal plugs within alveolar ducts and surrounding alveoli. Clinical symptoms include subacute shortness of breath, cough, and fever, and radiological findings include peripheral, patchy, and peribronchovascular regions of ground-glass opacities (GGOs), consolidation on chest CT. CASE PRESENTATION: A 67-year-old man presented to the Pulmonology clinic with 3 months history of worsening shortness of breath(SOB) associated with a cough with white sputum. CT chest revealed widespread GGOs suspicious for a diffuse interstitial lung process. The physical exam was unremarkable except for some dry inspiratory crackles with no wheezing. Serum studies, including inflammatory markers such as sedimentation rate and C-reactive protein, were normal. Autoimmune assays and the Quantiferon Gold test for tuberculosis were negative. Pulmonary function testing was consistent with a restrictive pattern. Bronchoscopy with transbronchial biopsies was performed for further evaluation and negative for any malignant process or infectious etiologies. The patient was started empirically on prednisone with little symptomatic and radiologic improvement at a 2-month interval. Hypersensitivity pneumonitis or a nonspecific interstitial pneumonitis variant of interstitial lung disease were considered and, hence, mycophenolate was added to the patient's steroid regimen. Despite this intensive immunosuppressive therapy, the patient returned to the clinic with a worsening SOB and cough with yellowish, mucopurulent sputum. His immunosuppressive agents were stopped and a repeat bronchoscopy with transbronchial biopsies was obtained that was, again, inconclusive. A multidisciplinary discussion with Infectious Disease and Thoracic Surgery ensued and a surgical biopsy was obtained that revealed findings suggestive of chronic COP. Considering the patient's poor clinical response to steroid therapy alone, a trial of azithromycin was initiated following consultation with Pulmonologists at other US facilities. Patient-reported a remarkable improvement in symptoms at 3 months follow-up with a near resolution of GGOs on chest CT. DISCUSSION: COP is remarkably responsive to steroid therapy, but relapse is common. Steroid-sparing agents such as cyclophosphamide and azathioprine have been used as alternatives with inconsistent benefits and poor tolerability. The anti-inflammatory properties of macrolide antibiotics are well known and have been explored in recent times with few case reports showing promising results. CONCLUSIONS: This case report demonstrates the clinical utility of macrolide antibiotics in the management of COP and conveys the importance of exploring this approach further to improve clinical outcomes in COP. REFERENCE #1: Pathak V, Kuhn JM, Durham C, Funkhouser WK, Henke DC. Macrolide use leads to clinical and radiological improvement in patients with cryptogenic organizing pneumonia. Ann Am Thorac Soc. 2014 Jan;11(1):87-91. doi: 10.1513/AnnalsATS.201308-261CR. PMID: 24460438. REFERENCE #2: Stover DE, Mangino D. Macrolides: a treatment alternative for bronchiolitis obliterans organizing pneumonia? Chest. 2005 Nov;128(5):3611-7. doi: 10.1378/chest.128.5.3611. PMID: 16304320. DISCLOSURES: No relevant relationships by Tania Dhawan, source=Web Response No relevant relationships by Faisal Siddiqui, source=Web Response No relevant relationships by Naresh Veerabattini, source=Web Response

Persistence of post-COVID lung parenchymal abnormalities during the three-month follow-up.

COVID-19-associated pulmonary sequalae have been increasingly reported after recovery from acute infection. Therefore, we aim to explore the charactersitics of persistent lung parenchymal abnormalities in patients with COVID-19. An observational study was conducted in patients with post-COVID lung parenchymal abnormalities from April till September 2020. Patients ≥18 years of age with COVID-19 who were diagnosed as post-COVID lung parenchymal abnormality based on respiratory symptoms and HRCT chest imaging after the recovery of acute infection. Data was recorded on a structured pro forma, and descriptive analysis was performed using Stata version 12.1. A total of 30 patients with post-COVID lung parenchymal abnormalities were identified. The mean age of patients was 59.1 (SD 12.6), and 27 (90.0%) were males. Four HRCT patterns of lung parenchymal abnormalities were seen; organizing pneumonia in 10 (33.3%), nonspecific interstitial pneumonitis in 17 (56.7%), usual interstitial pneumonitis in 12 (40.0%) and probable usual interstitial pneumonitis in 14 (46.7%). Diffuse involvement was found in 15 (50.0%) patients, while peripheral predominance in 15 (50.0%), and other significant findings were seen in 8 (26.7%) patients. All individuals were treated with corticosteroids. The case fatality rate was 16.7%. Amongst the survivors, 32.0% recovered completely, 36.0% improved, while 32.0% of the patients had static or progressive disease. This is the first study from Southeast Asia that identified post-COVID lung parenchymal abnormalities in patients who had no pre-existing lung disease highlighting the importance of timely recognition and treatment of this entity that might lead to fatal outcome.

Mechanic\u2019s hand; is it a\xa0prodromic sign of disease relapse of anti-synthetase syndrome; a case report

BackgroundAnti-synthetase syndrome is the collection of myositis and/or interstitial lung disease with the presence of various antibodies directed against an aminoacyl transfer RNA synthetase. Anti Jo − 1 antibody is the commonest of these antibodies and its presence is characteristically associated with the dermatological manifestation of mechanic’s hands. However, in the absence of other features, whether the presence of mechanic’s hands could be considered as a prodromic sign of disease relapse is not proven. We would like to present a patient who developed mechanic’s hands and subsequently went on to have recurrence in her myositis.Case presentationA 45-year-old female initially presented with a progressive proximal muscle weakness. Her muscle enzymes were elevated, EMG and biopsy were also in keeping with an inflammatory myositis. Subsequently she was found to have an interstitial lung disease with a non-specific interstitial pneumonitis pattern radiologically. Her anti Jo-1 was positive. However, she did not have any dermatological manifestations at the time. With immunosuppressive therapy she achieved remission which lasted for about 2 years. Then she developed fissuring and cracking of the palms and fingers suggestive of mechanic’s hands without any muscle pain, weakness and elevation of muscle enzymes. A few months later she did develop muscle pain, weakness and elevation of muscle enzymes heralding a disease relapse.ConclusionThe presence of mechanic’s hands without other features should be considered as a prodromic sign of disease relapse.

National Registry of Interstitial Lung Disease from Pakistan.

IntroductionInterstitial lung disease (ILD) is a heterogeneous group of over 200 parenchymal lung diseases with a myriad of etiologies. Interstitial lung disease registries from around the world show varying prevalence and incidence of these diseases. The aim of this study was to determine the epidemiology and characteristics of ILD in Pakistan.MethodsThis web-based registry, which is the first multicenter registry of ILD from Pakistan, recruited patients from 10 centers of five major cities between January 2016 and March 2019.ResultsA total of 744 patients were enrolled in the registry. The five most frequent ILDs were idiopathic pulmonary fibrosis (IPF) 34.4%, hypersensitivity pneumonitis (HP) - 17.7%, idiopathic nonspecific interstitial pneumonitis (iNSIP) - 16.8%, connective tissue disease-associated ILD (CTD-ILD) - 16.3%, and sarcoidosis - 9.1%.ConclusionIdiopathic pulmonary fibrosis is the most prevalent ILD in Pakistan, followed by HP and iNSIP. An ongoing prospective registry with longitudinal follow-up will help us further elaborate on the clinical characteristics, treatment, and survival outcome of patients with ILD.

Multidisciplinary team obviates biopsy in most patients with diffuse parenchymal lung diseases—A retrospective study from India

The inflammation and fibrosis in diffuse parenchymal lung diseases (DPLDs) in varied proportions give rise to different patterns in radiology and histopathology. The radiological pattern on CT of the thorax most often allows us to make a diagnosis with varying levels of confidence, to optimize management. With a multidisciplinary team bringing the strengths of their individual domains of knowledge, clinical, radiological, histopathological, and in many cases rheumatological, the level of confidence in making this diagnosis increases, often to the stage where the diagnosis is most often right, is concordant with the diagnosis achieved at histopathology and therefore obviates the need for lung biopsy which carries its own costs and risks of complications. Our study emphasizes the role of the multidisciplinary team (MDT) in the management of DPLDs at a tertiary care referral center. Every case of DPLD presenting to our pulmonology department was discussed in an MDT meeting before subjecting them to any diagnostic intervention or therapy. A clinico-radiological diagnosis was made according to the 2002 ATS/ERS guidelines initially. Later an official ATS/ERS/JRS/ALAT statement on idiopathic pulmonary fibrosis and a 2013 ATS/ERS consensus for the classification and diagnosis of idiopathic interstitial pneumonia was used. The concordance in our study was defined as the percentage of histopathological diagnoses that were identical to the clinico-radiological MDT diagnosis prior to the biopsy. A total of 434 patients with DPLDs were evaluated. The MDT suggested biopsy for only 38.7% (168/434) patients since the pattern was very clear in 266 (61.3%) cases. As not all patients consented to undergo the biopsy procedure when recommended, histopathology was obtained in 102 patients. The histological diagnosis was concordant with the initial MDT diagnosis in 80.3% (82/102) of samples. On an individual basis, connective tissue disease-interstitial lung disease and sarcoidosis showed the best concordance (87%). In idiopathic non-specific interstitial pneumonitis (NSIP) cases, the histopathological diagnosis concurred in only 53.3% (8/15), out of which 8 were NSIP, 4 were usual interstitial pneumonia, and 3 were reported as inadequate sampling on histopathology. The MDT plays a crucial role in the diagnosis of DPLDs. Not every pattern requires biopsy confirmation. However, an idiopathic non-specific interstitial pneumonitis diagnosis by the MDT should probably be better confirmed by biopsy.

Weaning Outcomes and Clinical Characteristics of Severe Mechanically Ventilated COVID-19 Patients During the Three Waves of the COVID-19 Pandemic in Israel

Background: Most of severe COVID-19 patients who survive the intensive care remain mechanically ventilated and require weaning and further rehabilitation. Objectives: This study compares the characteristics and outcomes of severe mechanically ventilated COVID-19 patients admitted to a weaning facility. In addition, the study describes patterns of weaning experienced during the three waves of COVID-19 in Israel. Methods: Clinical, demographic and outcome data was gathered retrospectively for 70 severe mechanically ventilated patients. Univariate analysis was performed to explain the variability in outcome variables. Results: Weaning success was 94% with mean weaning duration of 13±17 days. None of the demographic and clinical variables examined influenced the weaning duration, however patients with morbid obesity and/or diabetes were found to have shorter total duration on mechanical ventilation, hereby suggesting the presence of an obesity paradox. Extra corporal membrane oxygenation (ECMO) was used in 29% of the patients, and didn’t influence the weaning duration in spite being associated with higher rate of pleural complication. There were three main weaning patterns seen in different overlapping levels in the cohort patients: low capacity extra pulmonary pattern, fibrotic-bullosic pulmonary injury pattern and nonspecific interstitial pneumonitis pattern. Conclusions: The current study shows a high success rate of weaning, independent on comorbidities and previous ECMO use.