Interstitial Pneumonia with Autoimmune Features: Aiming to Define, Refine, and Treat.

Interstitial pneumonia with autoimmune features (IPAF) was defined for research purposes as interstitial lung disease (ILD) associated with features of autoimmunity without diagnosed rheumatic disease. Since publication of the IPAF criteria in 2015, there have been multiple studies of IPAF. However, much remains unknown regarding pathogenesis, prognosis, and treatment in IPAF.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.