Efficacy of Rituximab Versus Cyclophosphamide and Mycophenolate for the Treatment of Interstitial Lung Disease in Systemic Sclerosis: A Systematic Review.

Interstitial lung disease (ILD) is a common complication of systemic sclerosis (SSc), contributing to significant morbidity and mortality in affected individuals. The optimal treatment approach for SSc-associated ILD remains uncertain, with rituximab, cyclophosphamide, and mycophenolate among potential therapeutic options. This systematic review aims to evaluate and synthesize the existing evidence on the efficacy of rituximab compared to cyclophosphamide and mycophenolate for the treatment of ILD in patients with systemic sclerosis. A comprehensive search of the following electronic databases, PubMed, Science Direct, Google Scholar, and Cochrane Library, has been conducted to identify relevant studies, including randomized controlled trials, systematic review and meta-analysis, prospective cohort studies, and retrospective cohort studies. Data on study characteristics, participant demographics, interventions, outcomes, and key findings have been extracted and synthesized. The risk of bias in the included studies has been assessed using appropriate tools such as the Cochrane Bias assessment tool for randomized controlled trials, the New Castle Ottawa tool for cohort studies, and the AMSTAR checklist for systematic reviews and meta-analysis. The research team ultimately selected 15 high-quality studies for review. Rituximab demonstrated similar efficacy to cyclophosphamide and mycophenolate in improving lung function (forced vital capacity (FVC) and diffusing capacity of the lung for carbon monoxide (DLCO)), with fewer severe adverse events. Cyclophosphamide, while effective, had higher toxicity, leading to more frequent adverse events such as leukopenia and infections. Mycophenolate showed comparable efficacy to cyclophosphamide but with fewer side effects, making it a well-tolerated alternative. The findings of this systematic review will provide valuable insights into the comparative efficacy of rituximab, cyclophosphamide, and mycophenolate in the management of ILD in systemic sclerosis, informing clinical decision-making and guiding future research in this area.

Efficacy and safety of oral versus intravenous cyclophosphamide in treatment of connective tissue disease-related interstitial lung disease.

OBJECTIVE: Interstitial lung disease (ILD) resulting from connective tissue disease (CTD) greatly undermines people's health. Cyclophosphamide (CYC) is a widely used agent in treating CTD-ILD. We compared the efficacy and safety of oral and intravenous CYC in CTD-ILD treatment. METHODS: The retrospectively enrolled CTD-ILD patients were divided into the oral and intravenous CYC groups. The chest high-resolution computed tomography examination, forced vital capacity (FVC), lung carbon monoxide diffusion capacity (Dlco) determinations, and 6 min walk test (6MWT) were performed pre-treatment and at the 3rd, 6th, and 12th months posttreatment. Radiographic ILD severity was assessed using the Warrick score. Krebs Von den Lungen-6, surfactant protein A (SP-A), SP-D, and erythrocyte sedimentation rate (ESR) before and at the 12th month post-treatment were determined. CYC cumulative dose and occurrence of adverse reactions during treatment were recorded. RESULTS: CYC cumulative dose in the intravenous CYC group was reduced. Compared with oral CYC treatment, intravenous CYC caused decreased Warrick score and increased FVC and 6MWT at the 6th month, and elevated DLco at the 3rd and 6th months posttreatment. SP-A, SP-D and ESR levels in both groups were reduced 12 months posttreatment, with a more evident decrease in the intravenous CYC group. Intravenous CYC had lower total adverse reaction incidence. CONCLUSION: Compared with oral CYC, intravenous CYC decreases Warrick score and increases FVC and 6MWT at 6 months posttreatment, and reduces SP-A, SP-D, and ESR levels after 12 months of treatment, which shows low CYC cumulative dose and adverse reaction incidence in treating CTD-ILD.

Stroke in a Patient With Pulmonary Siderosis: An Unusual Association.

Occupational lung diseases are a major hazard, which can lead to severe complications and a worsening quality of life. Out of these diseases, pulmonary siderosis was considered an innocuous disease. We detail the case of a 42-year-old man who had a history of chronic exposure to ferrous fumes due to his occupation. He presented with breathlessness and symptoms of a stroke. CT imaging studies showed an ischemic infarct in the brain and changes suggestive of interstitial lung disease in the chest, which was eventually diagnosed as pulmonary siderosis. In spite of having no comorbidities and significant past history, the patient developed a debilitating condition most likely as a consequence of the underlying lung pathology. We want to highlight the importance of early detection and proper management of interstitial lung diseases.

Idiopathic Inflammatory Myopathies-associated Interstitial Lung Disease in Adults.

Idiopathic inflammatory myopathies (IIM) are a heterogeneous group of autoimmune diseases characterized by muscle involvement and various extramuscular manifestations. Interstitial lung disease (ILD) is one of the most common extramuscular manifestations of IIM and is associated with significant mortality and morbidity. The clinical phenotypes, treatment responses, and prognosis of IIM-ILD are significantly related to myositis-specific antibody (MSA) profiles, with some racial differences. The features associated with MSA in IIM-ILD could also be relevant to cases of ILD where MSA is present but does not meet the criteria for IIM. The anti-melanoma differentiation-associated gene 5 antibody is highly associated with rapidly progressive ILD (RP-ILD), especially in Asian populations, and with characteristic cutaneous manifestations, such as skin ulcers. Radiologically, ground-glass opacities, consolidations, and nonsegmental linear opacities were more predominant than reticular opacities and honeycombing. While the mortality rate is still around 30%, the prognosis can be improved with early intensive therapy with corticosteroids and multiple immunosuppressants. In contrast, anti-aminoacyl-tRNA synthetase (ARS) antibodies are associated with chronic ILD, although RP-ILD is also common. Patients with anti-ARS antibodies often show lung-predominant presentations, with subtle muscle and skin involvement. Radiologically, reticular opacities, with or without consolidation, are predominant and may progress to honeycombing over time. Combination therapy with corticosteroids and a single immunosuppressant is recommended to prevent relapses, which often lead to a decline in lung function and fatal long-term outcomes. Significant advances in immunology and genetics holds promise for fostering more personalized approaches to managing IIM-ILD.

Associations between 6-minute walk distance and physiologic measures and clinical outcomes in myositis-associated interstitial lung disease.

OBJECTIVE: The 6-min walk test (6MWT) is a simple test widely used to assess sub-maximal exercise capacity in chronic respiratory diseases. We explored the relationship of 6-min walk distance (6MWD) with measurements of physiological, clinical, radiographic measures in patients with myositis-associated interstitial lung disease (MA-ILD). METHOD: We analyzed data from the Abatacept in Myositis Associated Interstitial lung disease (Attack My-ILD) study, a 48-week multicentre randomized trial of patients with anti-synthetase antibodies and active MA-ILD. 6MWD, forced vital capacity (FVC), diffusing capacity (DLCO), high resolution CT, and various physician/patient reported outcome measures were obtained during the trial. Spearman's correlations and repeated-measures analysis with linear mixed-effects models were used to estimate the associations between 6MWD and various physiologic, clinical and radiographic parameters both cross-sectionally and longitudinally. RESULTS: Twenty participants with a median age of 57, 55% male and 85% white were analyzed. Baseline 6MWD did not associate with baseline PFTs. Repeated-measures analysis showed 6MWD over time associated with FVC over time, but not with DLCO. 6MWD over time also correlated with UCSD dyspnea score, Borg scores, as well as global disease activity and muscle strength over time. Emotional role functioning, vitality, general health and physical functioning scores by short form 36 also correlated with 6MWD over time. CONCLUSIONS: : Exploratory work in a small cohort of MA-ILD demonstrated 6MWD over time associated with parallel changes in FVC and patient reported outcomes of dyspnea, but not with DLCO. Larger studies are needed to validate the reliability, responsiveness and utility of the 6MWT in MA-ILD. CLINICAL TRIAL REGISTRATION: ClinicalTrials.gov, NCT03215927.

Pathological features of connective tissue disease-associated interstitial lung disease in transbronchial cryobiopsies.

AIM: Transbronchial cryobiopsies are increasingly used for the diagnosis of interstitial lung disease (ILD), but there is a lack of published information on the features of specific ILD in cryobiopsies. Here we attempt to provide pathological guidelines for separating usual interstitial pneumonia (UIP) of idiopathic pulmonary fibrosis (IPF), fibrotic hypersensitivity pneumonitis (FHP) and connective tissue disease-associated ILD (CTD-ILD) in cryobiopsies. METHODS: We examined 120 cryobiopsies from patients with multidisciplinary discussion (MDD)-established CTD-ILD and compared them to a prior series of 121 biopsies from patients with MDD-established IPF or FHP. RESULTS: A non-specific interstitial pneumonia (NSIP) pattern alone was seen in 36 of 120 (30%) CTD-ILD, three of 83 (3.6%) FHP and two of 38 (5.2%) IPF cases, statistically favouring a diagnosis of CTD-ILD. The combination of NSIP + OP was present in 29 of 120 (24%) CTD-ILD, two of 83 (2.4%) FHP and none of 38 (0%) IPF cases, favouring a diagnosis of CTD-ILD. A UIP pattern, defined as fibroblast foci plus any of patchy old fibrosis/fibrosis with architectural distortion/honeycombing, was identified in 28 of 120 (23%) CTD-ILD, 45 of 83 (54%) FHP and 27 of 38 (71%) IPF cases and supported a diagnosis of FHP or IPF. The number of lymphoid aggregates/mm2 and fibroblast foci/mm2 was not different in IPF, CTD-ILD or FHP cases with a UIP pattern. Interstitial giant cells supported a diagnosis of FHP or CTD-ILD over IPF, but were infrequent. CONCLUSIONS: In the correct clinical/radiological context the pathological findings of NSIP, and particularly NSIP plus OP, favour a diagnosis of CTD-ILD in a cryobiopsy, but CTD-ILD with a UIP pattern, FHP with a UIP pattern and IPF generally cannot be distinguished.

Incidence of and predictive factors for lung cancer in patients with rheumatoid arthritis: A retrospective long-term follow-up study.

OBJECTIVE: To determine the incidence and predictive factors of lung cancer in rheumatoid arthritis (RA). METHODS: We conducted a retrospective follow-up study of patients who were diagnosed with RA at our institution between April 2001 and December 2022. Pulmonary complications were evaluated using high-resolution computed tomography at RA diagnosis. Patients were followed until the diagnosis of lung cancer, diagnosis of other malignancies, death, loss to follow-up, or the end of the study. RESULTS: Among 771 RA patients, 3.5% were diagnosed with combined pulmonary fibrosis and emphysema (CPFE), 4.9% with interstitial lung disease (ILD) alone, and 6.0% with emphysema alone. During follow-up (mean of 9.3 years), the crude incidence rates of lung cancer per 1,000 patient-years were 2.9 in all patients, 47.8 in CPFE patients, 10.5 in ILD patients, 11.9 in emphysema patients, and 0.8 in patients without these complications. The standardized incidence ratios (95% confidence intervals [CI]) compared with the general population were 2.53 (1.29-3.77) for male patients and 0.89 (0.57-1.16) for female patients. In Fine-Gray regression analysis, adjusted hazard ratios (95% CI) of lung cancer were 13.48 (3.14-57.85) for CPFE, 6.42 (1.42-29.09) for ILD alone, and 4.65 (1.18-18.30) for emphysema alone versus without these complications, and 1.02 (1.01-1.04) per additional 1 pack-year for smoking history. These factors were not associated with the risk of other malignancies. CONCLUSION: Close monitoring of lung cancer is needed for RA patients with smoking history and pulmonary complications, especially CPFE.

18F-FDG-PET/CT Uptake by Noncancerous Lung as a Predictor of Interstitial Lung Disease Induced by Immune Checkpoint Inhibitors.

RATIONALE AND OBJECTIVES: Immune checkpoint inhibitors (ICIs) have improved lung cancer prognosis; however, ICI-related interstitial lung disease (ILD) is fatal and difficult to predict. Herein, we hypothesized that pre-existing lung inflammation on radiological imaging can be a potential risk factor for ILD onset. Therefore, we investigated the association between high uptake in noncancerous lung (NCL) on 18F- FDG-PET/CT and ICI-ILD in lung cancer. METHODS: Patients with primary lung cancer who underwent FDG-PET/CT within three months prior to ICI therapy were retrospectively included. Artificial intelligence was utilized for extracting the NCL regions (background lung) from the lung contralateral to the primary tumor. FDG uptake by the NCL was assessed via the SUVmax (NCL-SUVmax), SUVmean (NCL-SUVmean), and total glycolytic activity (NCL-TGA)defined as NCL-SUVmean×NCL volume [mL]. NCL-SUVmean and NCL-TGA were calculated using the following four SUV thresholds: 0.5, 1.0, 1.5, and 2.0. RESULTS: Of the 165 patients, 28 (17.0%) developed ILD. Univariate analysis showed that high values of NCL-SUVmax, NCL-SUVmean2.0 (SUV threshold=2.0), and NCL-TGA1.0 (SUV threshold=1.0) were significantly associated with ILD onset (all p = 0.003). Multivariate analysis adjusted for age, tumor FDG uptake, and pre-existing interstitial lung abnormalities revealed that a high NCL-TGA1.0 (≥149.45) was independently associated with ILD onset (odds ratio, 6.588; p = 0.002). Two-year cumulative incidence of ILD was significantly higher in the high NCL-TGA1.0 group than in the low group (58.4% vs. 14.4%; p < 0.001). CONCLUSION: High uptake of NCL on FDG-PET/CT is correlated with ICI-ILD development, which could serve as a risk stratification tool before ICI therapy in primary lung cancer.

A Systematic Review on Biomarkers: Are There Reliable Molecular Biomarkers in Patients With Rheumatoid Arthritis-Associated Interstitial Lung Disease?

Despite advances in the study of rheumatoid arthritis-associated interstitial lung disease (RA-ILD), the pulmonary manifestation remains an important cause of morbidity and mortality. However, there is a lack of biochemical markers for this manifestation in the literature. Therefore, the objective of this study was to carry out a qualitative systematic review on biochemical markers associated with RA-ILD in the PubMed, Web of Science, Embase, Cochrane Library, and Virtual Health Library (VHL) between January 2015 and July 2024, using the following descriptors: #1 "biomarkers" (MeSH) AND #2 "rheumatoid arthritis" (MeSH) AND #3 "Lung Diseases, Interstitial" (MeSH). Of the 1497 articles found, 27 presented eligibility criteria. The findings were divided into three sessions: "Main biomarkers for RA-ILD," "Other biomarkers for RA-ILD activity," and "Other biomarkers for RA-ILD prognosis." Among the evaluated markers, KL-6, RF, ACPA, ESR, and CRP appear to have prognostic value and association with damage in patients with RA-ILD. The association of some molecules such as sPD-1, sCD25, VCAM-1, MCP-1, and ADMA with tissue damage is intriguing. Longitudinal and randomized studies are imperative to comprehensively delineate the history of RA-ILD and evaluate potential serum biomarkers.

Ischaemic Strokes Caused by Spontaneous Cerebral Air Embolism, A Rare Complication of Interstitial Lung Disease.

Air embolism is a rare cause of ischaemic stroke. It is known that air can enter the cerebral arterial circulation from pulmonary venous circulation through a bronchovenous fistula, or in cases of pulmonary barotrauma in deep-sea diving. We describe a case of spontaneous cerebral air embolism against a background of advanced interstitial lung disease (ILD). To our knowledge, this case demonstrates a mechanism of stroke in ILD patients that has not been previously described. LEARNING POINTS: This case demonstrates a mechanism of stroke in patients with severe interstitial lung disease (ILD) that has not been previously described, and we suggest that in cases of advanced ILD, clinicians should consider this as a possible mechanism of stroke. The management of these patients should include transferring them to hyperbaric facilities to prevent further air emboli.

A self-management package for pulmonary fibrosis: A feasibility study.

Background and objective: There is currently no self-management package designed to meet the needs of people with pulmonary fibrosis (PF). This study evaluated the feasibility and acceptability of a PF-specific self-management package. Methods: Adults with PF were randomly allocated (1:1) to either receive the self-management package with healthcare professional (HCP) support or standardised PF information. Primary outcomes were feasibility and acceptability of the intervention. Secondary outcomes included health-related quality of life, self-efficacy, breathlessness, daily steps, use of PF-related treatments, and healthcare utilisation. Participants' experiences of using the package were explored using qualitative interviews. Results: Thirty participants were included. Recruitment rate was 91% and 100% of those recruited were randomised. Eighty-seven percent of participants who received the package read ≥1 module and set a goal. Secondary outcomes were feasible to collect with high assessment completion rates (87%). Most participants reported the package was easy to use and enhanced knowledge, but suggested some improvements, while HCP support was highly valued. Conclusion: A PF-specific self-management package was feasible to deliver and requires further testing in a trial powered to detect changes in clinical outcomes. Innovation: This is the first self-management package designed specifically for people with PF, informed by patient experience and expert consensus.

Comparison of survivals between sublobar resection and lobar resection for patients with clinical stage I non-small cell lung cancer and interstitial lung disease: a propensity score matching analysis.

BACKGROUND: Patients with early-stage lung cancer and interstitial lung disease have a poorer prognosis than those without interstitial lung disease. This study aimed to compare the long-term outcomes of lobar and sublobar resections in these patients. METHODS: We retrospectively analyzed 138 consecutive patients with clinical stage I non-small cell lung cancer and interstitial lung disease who underwent surgical treatment at two institutions between January 2010 and December 2020. Propensity score matching analysis was performed to adjust for baseline characteristics. RESULTS: Thirty-six patients underwent sublobar resection and 102 underwent lobar resection. The median follow-up was 45.7 months. In all patients, 5-year overall survival (OS) rates were 33.2% and 73.2%, and 5-year recurrence-free survival (RFS) rates were 24.2% and 60.1% in the sublobar and lobar resection groups, respectively (p < 0.01, <0.01). Death due to lung cancer and locoregional recurrence were significantly more frequent in the sublobar resection group than in the lobar resection group (p = 0.034, <0.01, respectively). On propensity score matching analysis, the 5-year OS rates of the 19 matched pairs were 46.3% and 73.2%, and the RFS rates were 31.6% and 67.6% in the sublobar and lobar resection groups, respectively (p = 0.036, <0.01). The Cox proportional hazards model demonstrated a significant association between lobar resection and improved survival (p = 0.047). CONCLUSION: The patients in the lobar resection group had better survival rates than those in the sublobar resection group. In terms of long-term prognosis, deliberately limited surgery may not be necessary for patients who tolerate lobectomy.

The 2024 British Society for Rheumatology guideline for management of systemic sclerosis-executive summary.

This guideline was developed according to the British Society for Rheumatology Guidelines Protocol by a Guideline Development Group comprising healthcare professionals with expertise in SSc and people with lived experience, as well as patient organization representatives. It is an update of the previous 2015 SSc guideline. The recommendations were developed and agreed by the group and are underpinned by published evidence, assessed by systematic literature review and reinforced by collective expert opinion of the group. It considers all aspects of SSc including general management, treatment of organ-based complications, including cardiopulmonary, renal and gastrointestinal tract manifestations, as well as broader impact of disease. Whilst it is focused on adults with SSc we expect that the guideline will be relevant to people of all ages and expert input and review by paediatric rheumatologists and other relevant specialists considered where the guideline was, or may not be, applicable to young people with SSc and juvenile-onset disease. In addition to providing guidance on disease assessment and management the full guideline also considers service organization within the National Health Service and future approaches to audit of the guideline. The lay summary that accompanies this abstract can be found in Supplemental information 1.

The 2024 British Society for Rheumatology guideline for management of systemic sclerosis.

This guideline was developed according to the British Society for Rheumatology Guidelines Protocol by a Guideline Development Group comprising healthcare professionals with expertise in SSc and people with lived experience, as well as patient organization representatives. It is an update of the previous 2015 SSc guideline. The recommendations were developed and agreed by the group and are underpinned by published evidence, assessed by systematic literature review and reinforced by collective expert opinion of the group. It considers all aspects of SSc including general management, treatment of organ-based complications, including cardiopulmonary, renal and gastrointestinal tract manifestations, as well as broader impact of disease. Whilst it is focused on adults with SSc we expect that the guideline will be relevant to people of all ages and expert input and review by paediatric rheumatologists and other relevant specialists considered where the guideline was, or may not be, applicable to young people with SSc and juvenile-onset disease. In addition to providing guidance on disease assessment and management the full guideline also considers service organization within the National Health Service and future approaches to audit of the guideline. The lay summary that accompanies this abstract can be found in Supplemental information 1.

Classification of Patients Based on Dyspnea and Desaturation During Exercise in Interstitial Lung Disease.

BACKGROUND: Dyspnea and desaturation during exercise are essential assessment items for pulmonary rehabilitation. Characterizing patients using these 2 factors may be important for providing more effective pulmonary rehabilitation. This study aimed to categorize subjects with interstitial lung disease (ILD) using dyspnea and desaturation at the end of the 6-min walk test (6MWT). METHODS: This was a retrospective study including 230 stable subjects with ILD who underwent 6MWT in our out-patient department at a general hospital in Japan. The modified Borg scale and oxygen saturation determined by SpO2 at the end of the 6MWT were used for cluster analysis using the k-means method with k = 4. RESULTS: Subjects were classified into 4 characteristic clusters. SpO2 at the end of the 6MWT was lower in cluster 4 (80.5 ± 3.0%) than in clusters 1 (94.3 ± 2.0%), 2 (94.3 ± 1.9%), and 3 (87.9 ± 1.8%) and was lower in cluster 3 than in clusters 1 and 2. The modified Borg scale score at the end of the 6MWT was higher in clusters 2 (4 [3-8]), 3 (3 [0-9]), and 4 (4 [0-7]) than in cluster 1 (0.5 [0-2.0]) and was higher in cluster 2 than in cluster 3. CONCLUSIONS: Subjects with ILD were classified into 4 characteristic clusters using dyspnea and SpO2 at the end of the 6MWT. The 4 clusters are characterized as follows: Cluster 1 had mild desaturation and mild dyspnea; cluster 2 had mild desaturation and severe dyspnea; cluster 3 had both moderate desaturation and dyspnea, and cluster 4 had both severe desaturation and dyspnea. These classification data offer insight for individualized pulmonary rehabilitation for patients with ILD.

Screening, diagnosis, and monitoring of interstitial lung disease in autoimmune rheumatic diseases: A narrative review.

Interstitial lung disease (ILD) is a common and serious manifestation of autoimmune rheumatic diseases. While the prevalence of ILD differs among the individual autoimmune rheumatic diseases, ILD remains an important cause of morbidity and mortality in systemic sclerosis, systemic lupus erythematosus, mixed connective tissue disease, primary Sjögren's disease, rheumatoid arthritis, and idiopathic inflammatory myositis. The present review summarizes recent literature on autoimmune-associated ILD with a focus on screening and monitoring for ILD progression. Reflecting on the currently available evidence, the authors propose a guideline for monitoring for progression in patients with newly diagnosed autoimmune-associated ILD. This review also highlights clinical and biological predictors of progressive pulmonary fibrosis and describes opportunity for further study in the rapidly evolving area of rheumatology and pulmonology.

La enfermedad pulmonar intersticial (EPI) es una manifestación común y seria de las enfermedades autoinmunes. Aunque la prevalencia de EPI difiere de acuerdo a cada enfermedad, continúa siendo una causa importante de morbilidad y mortalidad en la esclerosis sistémica, la artritis reumatoide, el síndrome de Sjögren, la enfermedad mixta del tejido conjuntivo y las miopatías inflamatorias. Este artículo de revisión resume la literatura reciente sobre la EPI asociada con autoinmunidad, con enfoque en la búsqueda y el monitoreo de la progresión de la EPI. Con base en la evidencia disponible, los autores proponen una guía para el monitoreo de la progresión en pacientes con la EPI asociada con autoinmunidad de reciente diagnóstico. Esta revisión también aborda los predictores clínicos y biológicos de la fibrosis pulmonar progresiva y resalta la oportunidad para estudios adicionales en áreas de rápida evolución como la reumatología y la neumología.

Hypersensitivity Pneumonitis: An Interesting Case of Acute Shortness of Breath in a Young Patient.

Hypersensitivity pneumonitis (HP) is a rare disease caused by an inflammation of the distal airway caused by an immune response to inhaled allergens. The clinical presentation and radiological and histological findings can overlap with other pulmonary conditions such as idiopathic pulmonary fibrosis. Therefore, it is essential to consider focused assessment for the patient if a diagnosis of HP is suspected. We present a case involving a young female patient who presented with symptoms of cough, flu-like illness, and dyspnea. Subsequent investigations revealed a diagnosis of nonfibrotic HP. The patient experienced acute respiratory failure and was managed with high-flow oxygen therapy. A detailed investigation determined that the patient's prior exposure to pet parrots at home was a significant factor. Following treatment with steroids and counseling regarding the removal of parrots from the home environment, the patient's condition improved, and she was successfully weaned off of oxygen therapy. This case underscores the importance of a comprehensive social history in evaluating common complaints such as dyspnea. The rarity of parrot-induced HP related to the patient's age, and exposure warrants attention.

Reevaluating diagnosis in interstitial lung disease with a second multidisciplinary discussion.

BACKGROUND: The importance of multidisciplinary discussion (MDD) for diagnosing interstitial lung disease (ILD) is emphasized by several international guidelines. While initial diagnoses are often provisional and require periodic re-evaluation, there is a lack of literature regarding the role of follow-up MDD in clinical practice. METHODS: From September 2020 to January 2022, patients underwent an initial MDD (MDD1) based on clinical, radiological, and pathological evaluations. Each diagnosis was assigned a confidence level. One year later, a second MDD (MDD2) was conducted for re-evaluation, based on subsequent clinical and radiological information. Changes in diagnosis and confidence levels between MDD1 and MDD2 were assessed. RESULTS: Among 52 patients enrolled in both MDDs, the diagnosis for 13 (25%) was revised at MDD2. Of these, 10 patients were initially diagnosed with unclassifiable ILD, and 3 received a low confidence diagnosis of either idiopathic pulmonary fibrosis or idiopathic nonspecific interstitial pneumonia. The most common diagnostic revision was due to the deterioration after antigen exposure or improvement after antigen avoidance, which resulted in a revised diagnosis of HP at MDD2. CONCLUSIONS: Our findings underscore the importance of periodic reassessment of MDD to improve the accuracy of ILD diagnosis. This study highlights the significance of longitudinal clinical and radiological evaluation for diagnostic revision, even in situations when rebiopsy is not feasible.

Pressed for Understanding: Interstitial lung disease in Dry-Cleaning Workers.

Interstitial lung disease (ILD) represents a heterogeneous group of disorders characterized by inflammation and fibrosis of the pulmonary interstitium. Risk factors for ILD include various environmental exposures and identifying specific exposures offers a point of intervention for preventing disease. Here, we present several cases of patients who worked in the dry-cleaning business and have ILD or abnormalities consistent with early ILD on chest CT imaging. While this report does not attempt to establish causality, we hypothesize that exposure to the industrial solvent tetrachloroethylene may serve as a contributing factor given its links to epithelial injury, inflammation, redox imbalance and apoptosis. We hope that this report serves to not only inform readers of this possible connection between dry cleaning and ILD but also lay the foundation for additional studies examining the effects of tetrachloroethylene on the lung.