Hironao Hozumi1,2, Tomoyuki Fujisawa3,4, Ran Nakashima3,4, Hideki Yasui3,4, Yuzo Suzuki3,4, Masato Kono3,4, Masato Karayama3,4, Kazuki Furuhashi3,4, Noriyuki Enomoto3,4, Naoki Inui3,4, Yutaro Nakamura3,4, Tsuneyo Mimori3,4, Takafumi Suda3,4. 1. From the Second Division, Department of Internal Medicine, and the Department of Clinical Pharmacology and Therapeutics, Hamamatsu University School of Medicine, Hamamatsu; Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto, Japan. jubilooreoresagi@ybb.ne.jp hozumi@hama-med.ac.jp. 2. H. Hozumi, Research Associate, MD, PhD, Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine; T. Fujisawa, Research Associate, MD, PhD, Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine; R. Nakashima, Research Associate, MD, PhD, Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University; H. Yasui, Research Associate, MD, PhD, Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine; Y. Suzuki, Research Associate, MD, PhD, Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine; M. Kono, MD, PhD, Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine; M. Karayama, Lecturer, MD, PhD, Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine; K. Furuhashi, Research Associate, MD, PhD, Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine; N. Enomoto, Lecturer, MD, PhD, Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine; N. Inui, Assistant Professor, MD, PhD, Department of Clinical Pharmacology and Therapeutics, Hamamatsu University School of Medicine; Y. Nakamura, Lecturer, MD, PhD, Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine; T. Mimori, Professor, MD, PhD, Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University; T. Suda, Professor, MD, PhD, Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine. jubilooreoresagi@ybb.ne.jp hozumi@hama-med.ac.jp. 3. From the Second Division, Department of Internal Medicine, and the Department of Clinical Pharmacology and Therapeutics, Hamamatsu University School of Medicine, Hamamatsu; Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto, Japan. 4. H. Hozumi, Research Associate, MD, PhD, Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine; T. Fujisawa, Research Associate, MD, PhD, Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine; R. Nakashima, Research Associate, MD, PhD, Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University; H. Yasui, Research Associate, MD, PhD, Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine; Y. Suzuki, Research Associate, MD, PhD, Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine; M. Kono, MD, PhD, Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine; M. Karayama, Lecturer, MD, PhD, Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine; K. Furuhashi, Research Associate, MD, PhD, Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine; N. Enomoto, Lecturer, MD, PhD, Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine; N. Inui, Assistant Professor, MD, PhD, Department of Clinical Pharmacology and Therapeutics, Hamamatsu University School of Medicine; Y. Nakamura, Lecturer, MD, PhD, Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine; T. Mimori, Professor, MD, PhD, Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University; T. Suda, Professor, MD, PhD, Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine.
Abstract
OBJECTIVE: The optimal treatment strategy for anti-aminoacyl-tRNA synthetase antibody-positive polymyositis/dermatomyositis-associated interstitial lung disease (anti-ARS-PM/DM-ILD) is yet to be established. We aimed to evaluate the efficacy of glucocorticoids and calcineurin inhibitors (CNI) in patients with ARS-PM/DM-ILD. METHODS: Progression-free survival (PFS) and overall survival rates were retrospectively evaluated in 32 consecutive patients with ARS-PM/DM-ILD. Disease progression was defined as deterioration in PM/DM-ILD (including recurrence). Predictive factors associated with PFS were analyzed by Cox hazards analysis. The efficacy of first-line prednisolone (PSL) plus CNI therapy was compared with that of PSL monotherapy using propensity score-matched analysis. RESULTS: Overall, 20 (62.5%) and 12 (37.5%) patients received first-line therapy with PSL + CNI and PSL, respectively. The 2-year PFS and 5-year survival rates in the overall cohort were 68.8% and 96.9%, respectively. On multivariate analysis, arterial oxygen pressure (HR 0.86) and PSL monotherapy (vs PSL + CNI; HR 7.29) showed an independent association with PFS. Baseline characteristics of propensity score-matched PSL + CNI and PSL groups were similar. The 2-year PFS rate was significantly higher in the matched PSL + CNI group than in the matched PSL group. All patients who experienced disease progression during first-line therapy were subsequently treated with second-line therapies. The 5-year survival rates of both the matched PSL + CNI and PSL groups were favorable. CONCLUSION: Propensity score-matched analysis demonstrated that first-line PSL + CNI therapy for patients with ARS-PM/DM-ILD significantly improved the PFS compared with PSL monotherapy, although there was no significant difference regarding longterm survival.
OBJECTIVE: The optimal treatment strategy for anti-aminoacyl-tRNA synthetase antibody-positive polymyositis/dermatomyositis-associated interstitial lung disease (anti-ARS-PM/DM-ILD) is yet to be established. We aimed to evaluate the efficacy of glucocorticoids and calcineurin inhibitors (CNI) in patients with ARS-PM/DM-ILD. METHODS: Progression-free survival (PFS) and overall survival rates were retrospectively evaluated in 32 consecutive patients with ARS-PM/DM-ILD. Disease progression was defined as deterioration in PM/DM-ILD (including recurrence). Predictive factors associated with PFS were analyzed by Cox hazards analysis. The efficacy of first-line prednisolone (PSL) plus CNI therapy was compared with that of PSL monotherapy using propensity score-matched analysis. RESULTS: Overall, 20 (62.5%) and 12 (37.5%) patients received first-line therapy with PSL + CNI and PSL, respectively. The 2-year PFS and 5-year survival rates in the overall cohort were 68.8% and 96.9%, respectively. On multivariate analysis, arterial oxygen pressure (HR 0.86) and PSL monotherapy (vs PSL + CNI; HR 7.29) showed an independent association with PFS. Baseline characteristics of propensity score-matched PSL + CNI and PSL groups were similar. The 2-year PFS rate was significantly higher in the matched PSL + CNI group than in the matched PSL group. All patients who experienced disease progression during first-line therapy were subsequently treated with second-line therapies. The 5-year survival rates of both the matched PSL + CNI and PSL groups were favorable. CONCLUSION: Propensity score-matched analysis demonstrated that first-line PSL + CNI therapy for patients with ARS-PM/DM-ILD significantly improved the PFS compared with PSL monotherapy, although there was no significant difference regarding longterm survival.
Authors: Caterina Vacchi; Marco Sebastiani; Giulia Cassone; Stefania Cerri; Giovanni Della Casa; Carlo Salvarani; Andreina Manfredi Journal: J Clin Med Date: 2020-02-03 Impact factor: 4.241