TY - JOUR
T1 - Interstitial Pneumonia With Autoimmune Features
T2 - An Emerging Challenge at the Intersection of Rheumatology and Pulmonology
AU - Wilfong, Erin M.
AU - Lentz, Robert J.
AU - Guttentag, Adam
AU - Tolle, James J.
AU - Johnson, Joyce E.
AU - Kropski, Jonathan A.
AU - Kendall, Peggy L.
AU - Blackwell, Timothy S.
AU - Crofford, Leslie J.
N1 - Funding Information:
Dr. Wilfong’s work was supported by the NIH (award UL1-TR-000445 from the National Center for Advancing Translational Sciences and grant T32-AR-007304 from the National Institute of Arthritis and Musculoskeletal and Skin Diseases). Dr. Kropski’s work was supported by the NIH (grant K08-HL-130595 from the National Heart, Lung, and Blood Institute), the Francis Family Foundation, and the Pulmonary Fibrosis Foundation. Dr. Kendall’s work was supported by the NIH (grant R01-DK-084246 from the National Institute of Diabetes and Digestive and Kidney Diseases) and the Department of Veterans Affairs. Dr. Blackwell’s work was supported by the NIH (grants P01-HL-92870 and R01-HL-085317 from the National Heart, Lung, and Blood Institute) and the Department of Veterans Affairs. Dr. Crofford’s work was supported by the NIH (award UL1-TR-
Publisher Copyright:
© 2018, American College of Rheumatology
PY - 2018/12
Y1 - 2018/12
N2 - Interstitial lung disease (ILD) remains a cause of significant morbidity and mortality in patients with connective tissue disease (CTD)–associated ILD. While some patients meet clear classification criteria for a systemic rheumatic disease, a subset of patients do not meet classification criteria but still benefit from immunosuppressive therapy. In 2015, the American Thoracic Society and European Respiratory Society described classification criteria for interstitial pneumonia with autoimmune features (IPAF) to identify patients with lung-predominant CTD who lack sufficient features of a systemic rheumatic disease to meet classification criteria. Although these criteria are imperfect, they are an important attempt to classify the patient with undifferentiated disease for future study. Rheumatologists play a key role in the evaluation of potential IPAF in patients, especially as many patients with a myositis-spectrum disease (e.g., non–Jo-1 antisynthetase syndrome, anti–melanoma differentiation–associated protein 5 antibody inflammatory myositis, or anti–PM/Scl antibody–associated inflammatory myositis) would be classified under IPAF using the currently available criteria for inflammatory myositis, and would therefore benefit from rheumatologic comanagement. The aim of this review was to describe the historical context that led to the development of these criteria and to discuss the limitations of the current criteria, diagnostic challenges, treatment options, and strategies for disease monitoring.
AB - Interstitial lung disease (ILD) remains a cause of significant morbidity and mortality in patients with connective tissue disease (CTD)–associated ILD. While some patients meet clear classification criteria for a systemic rheumatic disease, a subset of patients do not meet classification criteria but still benefit from immunosuppressive therapy. In 2015, the American Thoracic Society and European Respiratory Society described classification criteria for interstitial pneumonia with autoimmune features (IPAF) to identify patients with lung-predominant CTD who lack sufficient features of a systemic rheumatic disease to meet classification criteria. Although these criteria are imperfect, they are an important attempt to classify the patient with undifferentiated disease for future study. Rheumatologists play a key role in the evaluation of potential IPAF in patients, especially as many patients with a myositis-spectrum disease (e.g., non–Jo-1 antisynthetase syndrome, anti–melanoma differentiation–associated protein 5 antibody inflammatory myositis, or anti–PM/Scl antibody–associated inflammatory myositis) would be classified under IPAF using the currently available criteria for inflammatory myositis, and would therefore benefit from rheumatologic comanagement. The aim of this review was to describe the historical context that led to the development of these criteria and to discuss the limitations of the current criteria, diagnostic challenges, treatment options, and strategies for disease monitoring.
UR - http://www.scopus.com/inward/record.url?scp=85055503159&partnerID=8YFLogxK
U2 - 10.1002/art.40679
DO - 10.1002/art.40679
M3 - Review article
C2 - 30058242
AN - SCOPUS:85055503159
SN - 2326-5191
VL - 70
SP - 1901
EP - 1913
JO - Arthritis and Rheumatology
JF - Arthritis and Rheumatology
IS - 12
ER -