2023 American College of Rheumatology (ACR)/American College of Chest Physicians (CHEST) Guideline for the Screening and Monitoring of Interstitial Lung Disease in People with Systemic Autoimmune Rheumatic Diseases.

OBJECTIVE: We provide evidence-based recommendations regarding screening for interstitial lung disease (ILD) and the monitoring for ILD progression in people with systemic autoimmune rheumatic diseases (SARDs), specifically rheumatoid arthritis, systemic sclerosis, idiopathic inflammatory myopathies, mixed connective tissue disease, and Sjögren disease. METHODS: We developed clinically relevant population, intervention, comparator, and outcomes questions related to screening and monitoring for ILD in patients with SARDs. A systematic literature review was performed, and the available evidence was rated using the Grading of Recommendations, Assessment, Development, and Evaluation methodology. A Voting Panel of interdisciplinary clinician experts and patients achieved consensus on the direction and strength of each recommendation. RESULTS: Fifteen recommendations were developed. For screening people with these SARDs at risk for ILD, we conditionally recommend pulmonary function tests (PFTs) and high-resolution computed tomography of the chest (HRCT chest); conditionally recommend against screening with 6-minute walk test distance (6MWD), chest radiography, ambulatory desaturation testing, or bronchoscopy; and strongly recommend against screening with surgical lung biopsy. We conditionally recommend monitoring ILD with PFTs, HRCT chest, and ambulatory desaturation testing and conditionally recommend against monitoring with 6MWD, chest radiography, or bronchoscopy. We provide guidance on ILD risk factors and suggestions on frequency of testing to evaluate for the development of ILD in people with SARDs. CONCLUSION: This clinical practice guideline presents the first recommendations endorsed by the American College of Rheumatology and American College of Chest Physicians for the screening and monitoring of ILD in people with SARDs.

2023 American College of Rheumatology (ACR)/American College of Chest Physicians (CHEST) Guideline for the Screening and Monitoring of Interstitial Lung Disease in People with Systemic Autoimmune Rheumatic Diseases.

OBJECTIVE: We provide evidence-based recommendations regarding screening for interstitial lung disease (ILD) and the monitoring for ILD progression in people with systemic autoimmune rheumatic diseases (SARDs), specifically rheumatoid arthritis, systemic sclerosis, idiopathic inflammatory myopathies, mixed connective tissue disease, and Sjögren disease. METHODS: We developed clinically relevant population, intervention, comparator, and outcomes questions related to screening and monitoring for ILD in patients with SARDs. A systematic literature review was performed, and the available evidence was rated using the Grading of Recommendations, Assessment, Development, and Evaluation methodology. A Voting Panel of interdisciplinary clinician experts and patients achieved consensus on the direction and strength of each recommendation. RESULTS: Fifteen recommendations were developed. For screening people with these SARDs at risk for ILD, we conditionally recommend pulmonary function tests (PFTs) and high-resolution computed tomography of the chest (HRCT chest); conditionally recommend against screening with 6-minute walk test distance (6MWD), chest radiography, ambulatory desaturation testing, or bronchoscopy; and strongly recommend against screening with surgical lung biopsy. We conditionally recommend monitoring ILD with PFTs, HRCT chest, and ambulatory desaturation testing and conditionally recommend against monitoring with 6MWD, chest radiography, or bronchoscopy. We provide guidance on ILD risk factors and suggestions on frequency of testing to evaluate for the development of ILD in people with SARDs. CONCLUSION: This clinical practice guideline presents the first recommendations endorsed by the American College of Rheumatology and American College of Chest Physicians for the screening and monitoring of ILD in people with SARDs.

2023 American College of Rheumatology (ACR)/American College of Chest Physicians (CHEST) Guideline for the Treatment of Interstitial Lung Disease in People with Systemic Autoimmune Rheumatic Diseases.

OBJECTIVE: We provide evidence-based recommendations regarding the treatment of interstitial lung disease (ILD) in adults with systemic autoimmune rheumatic diseases (SARDs). METHODS: We developed clinically relevant population, intervention, comparator, and outcomes questions. A systematic literature review was then performed, and the available evidence was rated using the Grading of Recommendations, Assessment, Development, and Evaluation methodology. A panel of clinicians and patients reached consensus on the direction and strength of the recommendations. RESULTS: Thirty-five recommendations were generated (including two strong recommendations) for first-line SARD-ILD treatment, treatment of SARD-ILD progression despite first-line ILD therapy, and treatment of rapidly progressive ILD. The strong recommendations were against using glucocorticoids in systemic sclerosis-ILD as a first-line ILD therapy and after ILD progression. Otherwise, glucocorticoids are conditionally recommended for first-line ILD treatment in all other SARDs. CONCLUSION: This clinical practice guideline presents the first recommendations endorsed by the American College of Rheumatology and American College of Chest Physicians for the treatment of ILD in people with SARDs.

High Interleukin-13 level is associated with disease stability in interstitial Lung disease.

Purpose: Cytokines can help predict prognosis in interstitial lung disease (ILD) and to differentiate between ILD subtypes. The objectives of our study were to evaluate association of baseline cytokine levels with time to ILD progression and to compare baseline cytokine levels between ILD subtypes. Methods: We quantified 27 cytokines using a multiplex assay in peripheral blood samples from 77 patients. Cox proportional hazards regression analysis was performed to evaluate cytokine impact on the time to progression in the total cohort and within each ILD type. We evaluated for significant differences in cytokine levels between ILD types using ANOVA, Wilcoxon signed-rank test and Tukey method. Results: Higher IL-13 level was associated with longer time to progression (hazard ratio 0.52 [0.33-0.81], p-value 0.004). FGF-ß, GM-CSF, and IL-17 levels differed significantly between fibrotic and inflammatory ILD subgroups. Conclusion: IL-13 may be a useful biomarker predicting ILD stability.

Low Rates of Reproductive Counseling Documentation in Women With Interstitial Pneumonia With Autoimmune Features.

BACKGROUND/OBJECTIVE: Women with interstitial pneumonia with autoimmune features (IPAFs), a subset of interstitial lung disease (ILD), are at risk for pregnancy complications. Family planning discussions improve pregnancy outcomes in women with ILD. The objective of this study was to evaluate the documentation of reproductive counseling in IPAF female patients of childbearing age by pulmonary and rheumatology providers at an academic medical center. METHODS: We conducted a medical record review study of pulmonary and rheumatology encounters in reproductive-aged women with IPAF to evaluate documentation of family planning discussions and contraceptive use. We used nonparametric measures of association and logistic regression to evaluate the relationship between patient characteristics and the presence of reproductive counseling documentation by providers. RESULTS: Thirty-one women met IPAF classification and were ≤50 years at initial ILD clinic visit. Twenty-five (81%) of these women had risk factors for adverse pregnancy outcomes. Ten women (32%) had a record of reproductive counseling during any visit with their pulmonary provider. Of the 21 patients who also saw a rheumatology provider, 12 (57%) women had a record of reproductive counseling during any visit with their rheumatology provider. No baseline characteristics were associated with odds of reproductive counseling documentation. CONCLUSIONS: Neither pulmonary nor rheumatology providers consistently discussed family planning/contraceptive use with reproductive-aged women with IPAF. There was a trend for rheumatology providers to discuss reproductive issues with IPAF patients more frequently than pulmonary providers. Efforts should focus on educating providers about the need for reproductive counseling in women with IPAF of childbearing age.

Implementing a Nurse-Driven Protocol for Pneumococcal Vaccination in an Academic Rheumatology Clinic.

OBJECTIVE: Rheumatology patients are at high risk for complications from pneumococcal infections. The goal of this study was to assess the feasibility of implementing a nurse-driven pneumococcal vaccination protocol based on the 2012 Advisory Committee on Immunization Practices (ACIP) guidelines within an academic rheumatology clinic. Our aims were to increase (1) pneumococcal conjugate vaccine (PCV13) and pneumococcal polysaccharide vaccine (PPSV23) monthly vaccination rates in immunosuppressed patients aged 19 to 64 years, and (2) the overall proportion of immunosuppressed patients aged 19 to 64 years who have received both PCV13 and PPSV23 vaccinations by ≥ 10% over a 2-year period. METHODS: We identified eligible adults in the electronic medical record using a search protocol based on preset medication group. We obtained baseline pneumococcal vaccination rates in 2019, calculating the proportion of patients who were unvaccinated, partially vaccinated (received either PCV13 or PPSV23), or fully vaccinated. We created a pneumococcal vaccination protocol based on 2012 ACIP guidelines and converted it into a standing medical order to be implemented by the nursing staff. Postintervention vaccination rates were calculated monthly and at the end of the study period. Multiple comparison testing was performed to assess for significant postintervention changes. RESULTS: The average rate of monthly vaccination with either PCV13 or PPSV23 increased from 4.3% in 2019 to 12.6% in 2021. The proportion of patients who were fully vaccinated increased from 14.6% in 2019 to 26.2% in 2021. Both changes were statistically significant. CONCLUSION: It is feasible to employ a nurse-driven protocol for improving pneumococcal vaccination rates in immunosuppressed patients, despite difficulties posed by coronavirus disease 2019 (COVID-19) pandemic disruptions.

Interstitial Pneumonia with Autoimmune Features: What the Rheumatologist Needs to Know.

PURPOSE OF REVIEW: This narrative review will focus on the role of the rheumatologist in evaluating patients with interstitial lung disease (ILD) without a defined rheumatic disease and will outline the current classification criteria for interstitial pneumonia with autoimmune features (IPAF) and describe what is known regarding IPAF pathobiology, natural history, prognosis, and treatment. Lastly, knowledge gaps and opportunities for future research will be discussed. RECENT FINDINGS: IPAF is a recently defined classification of ILD patients who have features suggesting an autoimmune-mediated process, but do not fulfill current rheumatic disease criteria. The goal of the IPAF criteria is to provide a uniform case definition for the study of autoimmune ILD patients who do not currently fit within standard ILD diagnostic categories, ultimately improving diagnosis and therapy. Many of these patients are referred for rheumatologic evaluation to aid the diagnostic process. The care of the IPAF patient is complex and is multidisciplinary with pulmonology, rheumatology, pathology, radiology, physical therapy, primary care, pulmonary transplant providers all serving vital roles. The rheumatologist has several roles which include classification, disease monitoring, and management.

Variables Associated With Response to Therapy in Patients With Interstitial Pneumonia With Autoimmune Features.

BACKGROUND/OBJECTIVE: We have limited knowledge regarding characteristics of patients with interstitial pneumonia with autoimmune features (IPAF) that are associated with response to immunosuppression. In this study, we used published IPAF criteria to characterize features associated with response to treatment. METHODS: We conducted a single-center medical records review study of 63 IPAF patients to evaluate for serological, clinical, and morphological characteristics that are associated with response to immunosuppression. Response was defined as % relative functional vital capacity decline of less than 10% and absence of death or lung transplant within the first year of continuous immunosuppressive therapy. Nonparametric measures of association and multivariate logistic regression were used to evaluate the relationship between baseline characteristics and immunosuppressive response. RESULTS: There was a trend of greater progression among men, ever smokers, those negative for antisynthetase antibodies, and those with usual interstitial pneumonia radiographic pattern, but no statistically significant relationship was found between baseline serological, clinical, or morphological features and response to immunosuppression. Patients on combination therapy with mycophenolate mofetil and prednisone had less disease progression (p = 0.018) than those on regimens that did not include both of these medications. CONCLUSIONS: In our cohort, baseline clinical assessment did not identify which patients with IPAF will respond to immunosuppressive therapy. Combination therapy with mycophenolate mofetil and prednisone was associated with lack of disease progression in our IPAF patients, including in IPAF-usual interstitial pneumonia. Further studies are needed to evaluate which IPAF patients would benefit from immunosuppressive therapy, antifibrotic therapy, or a combination of both.

Utility of Bronchoalveolar Lavage and Transbronchial Biopsy in Patients with Interstitial Lung Disease.

PURPOSE: Bronchoalveolar lavage and transbronchial biopsy can be a useful tool in the evaluation of interstitial lung disease (ILD), but patient selection for this procedure remains poorly defined. Determining clinical characteristics that help with patient selection for bronchoscopy may improve confidence of ILD classification while limiting potential adverse outcomes associated with surgical lung biopsy. The purpose of this study is to identify factors that were associated with change in multidisciplinary ILD diagnosis (MDD) before and after incorporation of BAL and TBBx data. METHODS: We conducted a retrospective cohort study of ILD patients at a single center who underwent bronchoscopy in the diagnostic workup of ILD. We performed sequential MDD both pre- and post-bronchoscopy to calculate the frequency of change in diagnosis after incorporating information from BAL and TBBx and identify features associated with change in diagnosis. RESULTS: 245 patients were included in the study. Bronchoscopy led to a change in diagnosis in 58 patients (23.7%). The addition of TBBx to BAL increased diagnostic yield from 21.8 to 34.1% (p = 0.027). Identification of antigen, HRCT scan inconsistent with UIP, and absence of a pre-bronchoscopy diagnosis of CTD-ILD or IPAF were associated with a change in diagnosis after bronchoscopy. CONCLUSION: Our study suggests clinical features that may assist with patient selection for bronchoscopy. We suggest bronchoscopy in patients with identified antigen or an HRCT that is consistent with a non-IPF diagnosis. Appropriate patient selection for bronchoscopy may improve ILD diagnostic confidence and avoid potential complications from more invasive and higher risk procedures.

Myositis-specific Antibodies Identify A Distinct Interstitial Pneumonia with Autoimmune Features Phenotype.

Interstitial pneumonia with autoimmune features (IPAF) characterises individuals with interstitial lung disease (ILD) and features of connective tissue disease (CTD) who fail to satisfy CTD criteria. Inclusion of myositis-specific antibodies (MSAs) in the IPAF criteria has generated controversy, as these patients also meet proposed criteria for an anti-synthetase syndrome. Whether MSAs and myositis associated antibodies (MAA) identify phenotypically distinct IPAF subgroups remains unclear.A multi-center, retrospective investigation was conducted to assess clinical features and outcomes in patients meeting IPAF criteria stratified by the presence of MSAs and MAAs. IPAF subgroups were compared to cohorts of patients with idiopathic inflammatory myopathy-ILD (IIM-ILD), idiopathic pulmonary fibrosis (IPF) and non-IIM CTD-ILDs. The primary endpoint assessed was three-year transplant-free survival. Two hundred sixty-nine patients met IPAF criteria, including 35 (13%) with MSAs and 65 (24.2%) with MAAs. Survival was highest among patients with IPAF-MSA and closely approximated those with IIM-ILD. Survival did not differ between IPAF-MAA and IPAF without MSA/MAA cohorts. Usual interstitial pneumonia (UIP) morphology was associated with differential outcome risk, with IPAF patients with non-UIP morphology approximating survival observed in non-IIM CTD-ILDs. MSAs, but not MAAs identified a unique IPAF phenotype characterised by clinical features and outcomes similar to IIM-ILD. UIP morphology was a strong predictor of outcome in others meeting IPAF criteria. Because IPAF is a research classification without clear treatment approach, these findings suggest MSAs should be removed from the IPAF criteria and such patients should be managed as an IIM-ILD.

Myopathy in a 61-year-old Hispanic man.

A 61-year-old Hispanic man presented to a county hospital for subacute progressive weakness, heliotrope rash and dysphagia. There was initial suspicion for dermatomyositis (DM) given the history; however, the physical exam was not consistent. An MRI followed by a muscle biopsy revealed necrotising autoimmune myositis and anti-3-hydroxy-3-methylglutary-coenzyme A-reductase antibody titers returned positive; the patient was diagnosed with necrotising autoimmune myositis. He was treated with corticosteroids and intravenous immunoglobulin, which resulted in improvement in his weakness and functional status. This case represents a unique instance in which a cardinal feature of DM, the heliotrope rash, prompted an erroneous initial diagnosis. It highlights the necessity of developing abroad differential diagnosis and subsequent thorough investigation into patients presenting with suspected idiopathic immune-mediated myopathies.