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Clinical Features and Prognostic Factors for Dermatomyositis-Associated Interstitial Lung Diseases with Anti-MDA5 Antibody
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A1679 - Clinical Features and Prognostic Factors for Dermatomyositis-Associated Interstitial Lung Diseases with Anti-MDA5 Antibody
Author Block: T. Fujisawa1, H. Hozumi1, H. Yasui1, Y. Suzuki1, M. Karayama1, K. Furuhashi1, N. Enomoto1, Y. Nakamura1, N. Inui2, T. Suda1; 1Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan, 2Deparmtne of Clinical Pharmacology and Therapeutics, Hamamatsu University School of Medicine, Hamamatsu, Japan.
Rationale. Interstitial lung disease (ILD) is a major complication in polymyositis (PM) and dermatomyositis (DM), and is an important cause of mortality. Autoantibodies to melanoma differentiation-associated gene 5 (MAD5) are specifically expressed in patients with dermatomyositis (DM). Former studies have demonstrated that patients with DM-ILD with anti-MDA5 antibody sometimes show rapidly progressive course and have poorer prognosis compared to PM/DM-ILD without anti-MDA5 antibody. However, little is known about clinical features and prognostic factors in patients with DM-ILD with anti-MDA5 antibody. Objective. The aim of this study was to clarify the clinical features and prognostic factors in patients with DM-ILD with anti-MDA5 antibody Methods. Twenty-three patients with DM-ILD with anti-MDA5 antibody were included in the present study. Clinical characteristics, laboratory data, pulmonary function tests and survival were analyzed retrospectively. Logistic regression analysis was performed to assess prognostic factors. Results. Of the 23 patients (17 female, 6 male), 19 patients (83%) had acute/subacute form of ILD. All the patients were treated with combination of corticosteroid and immunosuppressant (e.g. cyclosporine, tacrolimus, cyclophosphamide, mycophenolate mofetil) after initial diagnosis. Nine patients (39%) died during observation period. Of the 9 dead cases, 8 patients died of respiratory failure due to progression of ILD within 90 days after initial diagnosis. One patient died a sudden death after 18 years of diagnosis. Logistic regression analysis revealed that %FVC, PaO2, serum ferritin level and serum chitotriosidase level were significantly associated with 90-day mortality. Conclusions. Major cause of death in DM-ILD with anti-MDA5 antibody is respiratory failure due to progression of ILD in early stage. Lower level of %FVC and PaO2, higher level of serum ferritin and chitotriosidase predict poor outcome in DM-ILD with anti-MDA5 antibody.
Author Block: T. Fujisawa1, H. Hozumi1, H. Yasui1, Y. Suzuki1, M. Karayama1, K. Furuhashi1, N. Enomoto1, Y. Nakamura1, N. Inui2, T. Suda1; 1Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan, 2Deparmtne of Clinical Pharmacology and Therapeutics, Hamamatsu University School of Medicine, Hamamatsu, Japan.
Rationale. Interstitial lung disease (ILD) is a major complication in polymyositis (PM) and dermatomyositis (DM), and is an important cause of mortality. Autoantibodies to melanoma differentiation-associated gene 5 (MAD5) are specifically expressed in patients with dermatomyositis (DM). Former studies have demonstrated that patients with DM-ILD with anti-MDA5 antibody sometimes show rapidly progressive course and have poorer prognosis compared to PM/DM-ILD without anti-MDA5 antibody. However, little is known about clinical features and prognostic factors in patients with DM-ILD with anti-MDA5 antibody. Objective. The aim of this study was to clarify the clinical features and prognostic factors in patients with DM-ILD with anti-MDA5 antibody Methods. Twenty-three patients with DM-ILD with anti-MDA5 antibody were included in the present study. Clinical characteristics, laboratory data, pulmonary function tests and survival were analyzed retrospectively. Logistic regression analysis was performed to assess prognostic factors. Results. Of the 23 patients (17 female, 6 male), 19 patients (83%) had acute/subacute form of ILD. All the patients were treated with combination of corticosteroid and immunosuppressant (e.g. cyclosporine, tacrolimus, cyclophosphamide, mycophenolate mofetil) after initial diagnosis. Nine patients (39%) died during observation period. Of the 9 dead cases, 8 patients died of respiratory failure due to progression of ILD within 90 days after initial diagnosis. One patient died a sudden death after 18 years of diagnosis. Logistic regression analysis revealed that %FVC, PaO2, serum ferritin level and serum chitotriosidase level were significantly associated with 90-day mortality. Conclusions. Major cause of death in DM-ILD with anti-MDA5 antibody is respiratory failure due to progression of ILD in early stage. Lower level of %FVC and PaO2, higher level of serum ferritin and chitotriosidase predict poor outcome in DM-ILD with anti-MDA5 antibody.
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