PMID- 31299466 OWN - NLM STAT- MEDLINE DCOM- 20200414 LR - 20200414 IS - 1532-3064 (Electronic) IS - 0954-6111 (Linking) VI - 155 DP - 2019 Aug TI - Clinical significance of autoantibody positivity in idiopathic pulmonary fibrosis. PG - 43-48 LID - S0954-6111(19)30224-0 [pii] LID - 10.1016/j.rmed.2019.07.001 [doi] AB - RATIONALE: The concept of interstitial pneumonia with autoimmune features (IPAF) was recently proposed by the American Thoracic Society. However, the clinical significance of the serologic domain of IPAF has not yet been established in idiopathic pulmonary fibrosis (IPF). OBJECTIVES: We aimed to investigate the clinical significance of autoantibody positivity in IPF. METHODS: We retrospectively reviewed the records of 512 patients diagnosed as IPF from January 2007 through March 2014. The patients were divided into two subgroups: (i) an autoantibody-positive IPF subgroup (n = 138), consisting of patients with anti-neutrophil cytoplasmic antibody (ANCA) or autoantibodies that met the criteria for the IPAF serologic domain; (ii) a lone IPF subgroup (n = 374), consisting of the rest of the IPF patients. MEASUREMENTS AND MAIN RESULTS: Autoantibody-positivity (HR 0.736, p = 0.043) was an independent risk factors for 5-year mortality on multivariable analysis in the overall IPF patients. In the autoantibody-positive IPF patients, use of glucocorticoid (not for management of acute exacerbation, HR 2.121, p = 0.019), use of immunomodulators (HR 0.310, p = 0.002), malignancy (HR 3.359, p = 0.002), baseline forced vital capacity (HR 0.974, p = 0.017), baseline diffusing capacity of the lung for carbon monoxide (HR 0.981, p = 0.041), and baseline 6-min walk test distance (HR 0.996, p = 0.002) were independent risk factors for 5-year mortality. CONCLUSIONS: Presence of ANCA or autoantibodies of the IPAF serologic domain in IPF patients is associated with better survival outcomes, and the use of immunomodulators is associated with superior survival outcomes. CI - Copyright (c) 2019 Elsevier Ltd. All rights reserved. FAU - Ghang, Byeongzu AU - Ghang B AD - Division of Rheumatology, Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, 88, Olympic-ro 43-gil, Songpa-gu, Seoul, 05505, South Korea; Division of Rheumatology, Department of Internal Medicine, Jeju National University School of Medicine, Aran 13 Gil 15 (Ara-1 Dong), Jeju Si, Jeju Special Self-Governing Province, 63241, South Korea. FAU - Lee, Jungsun AU - Lee J AD - Division of Rheumatology, Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, 88, Olympic-ro 43-gil, Songpa-gu, Seoul, 05505, South Korea. FAU - Chan Kwon, Oh AU - Chan Kwon O AD - Division of Rheumatology, Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, 88, Olympic-ro 43-gil, Songpa-gu, Seoul, 05505, South Korea. FAU - Ahn, Soo Min AU - Ahn SM AD - Division of Rheumatology, Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, 88, Olympic-ro 43-gil, Songpa-gu, Seoul, 05505, South Korea. FAU - Oh, Ji Seon AU - Oh JS AD - Division of Rheumatology, Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, 88, Olympic-ro 43-gil, Songpa-gu, Seoul, 05505, South Korea. FAU - Hong, Seokchan AU - Hong S AD - Division of Rheumatology, Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, 88, Olympic-ro 43-gil, Songpa-gu, Seoul, 05505, South Korea. FAU - Kim, Yong-Gil AU - Kim YG AD - Division of Rheumatology, Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, 88, Olympic-ro 43-gil, Songpa-gu, Seoul, 05505, South Korea. FAU - Yoo, Bin AU - Yoo B AD - Division of Rheumatology, Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, 88, Olympic-ro 43-gil, Songpa-gu, Seoul, 05505, South Korea. FAU - Jeong, Woo Seong AU - Jeong WS AD - Division of Rheumatology, Department of Internal Medicine, Jeju National University School of Medicine, Aran 13 Gil 15 (Ara-1 Dong), Jeju Si, Jeju Special Self-Governing Province, 63241, South Korea. FAU - Kim, Jinseok AU - Kim J AD - Division of Rheumatology, Department of Internal Medicine, Jeju National University School of Medicine, Aran 13 Gil 15 (Ara-1 Dong), Jeju Si, Jeju Special Self-Governing Province, 63241, South Korea. FAU - Lee, Chang-Keun AU - Lee CK AD - Division of Rheumatology, Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, 88, Olympic-ro 43-gil, Songpa-gu, Seoul, 05505, South Korea. Electronic address: cklee@amc.seoul.kr. LA - eng PT - Comparative Study PT - Journal Article DEP - 20190705 PL - England TA - Respir Med JT - Respiratory medicine JID - 8908438 RN - 0 (Antibodies, Antineutrophil Cytoplasmic) RN - 0 (Autoantibodies) RN - 0 (Glucocorticoids) RN - 0 (Immunologic Factors) RN - 7U1EE4V452 (Carbon Monoxide) SB - IM MH - Aged MH - Antibodies, Antineutrophil Cytoplasmic/blood MH - Autoantibodies/*blood MH - Carbon Monoxide/metabolism MH - Female MH - Glucocorticoids/therapeutic use MH - Humans MH - Idiopathic Pulmonary Fibrosis/*diagnosis/drug therapy/*immunology/physiopathology MH - Immunologic Factors/therapeutic use MH - Lung Diseases, Interstitial/*diagnosis/immunology MH - Male MH - Middle Aged MH - Neoplasms/epidemiology MH - Pulmonary Diffusing Capacity/physiology MH - Retrospective Studies MH - Risk Factors MH - Vital Capacity/physiology MH - Walk Test/methods OTO - NOTNLM OT - Autoantibodies OT - Autoimmunity OT - DMARDs (synthetic) OT - Pulmonary fibrosis OT - Treatment EDAT- 2019/07/13 06:00 MHDA- 2020/04/15 06:00 CRDT- 2019/07/13 06:00 PHST- 2019/01/10 00:00 [received] PHST- 2019/05/24 00:00 [revised] PHST- 2019/07/01 00:00 [accepted] PHST- 2019/07/13 06:00 [pubmed] PHST- 2020/04/15 06:00 [medline] PHST- 2019/07/13 06:00 [entrez] AID - S0954-6111(19)30224-0 [pii] AID - 10.1016/j.rmed.2019.07.001 [doi] PST - ppublish SO - Respir Med. 2019 Aug;155:43-48. doi: 10.1016/j.rmed.2019.07.001. Epub 2019 Jul 5.