Clinical manifestation and prognostic factor in anti-melanoma differentiation-associated gene 5 antibody-associated interstitial lung disease as a complication of dermatomyositis
Open Access
- 23 May 2010
- journal article
- research article
- Published by Oxford University Press (OUP) in Rheumatology
- Vol. 49 (9), 1713-1719
- https://doi.org/10.1093/rheumatology/keq149
Abstract
Objective. The aim of this study is to evaluate the clinical manifestation and prognostic factors of anti-melanoma differentiation-associated gene 5 (MDA5) antibody-associated interstitial lung disease (ILD) with DM. Methods. Fourteen patients who presented with anti-MDA5 antibody and 10 patients with anti-aminoacyl-tRNA synthetase (ARS) antibody were enrolled. All patients were diagnosed as having DM with ILD. Clinical manifestations in the patients with anti-MDA5 antibody were compared with those in the patients with anti-ARS antibody. Results. The frequencies of acute/subacute interstitial pneumonia (A/SIP) and fatal outcome were significantly higher in the subset with anti-MDA5 antibody. The creatine kinase (CK) value was significantly lower and the γ-glutamyl transpeptidase and ferritin values were significantly higher in the subset with anti-MDA5 antibody. Significant correlations were found between PaO2/FiO2 and ferritin (rs = −0.59, P = 0.035), alveolar–arterial oxygen difference (A-aDO2) and KL-6 (rs = 0.73, P = 0.016) and A-aDO2 and ferritin (rs = 0.66, P = 0.013) in the subset with anti-MDA5 antibody. The most significant prognostic factor was ferritin. The cumulative survival rate was significantly lower (P < 0.0001) in the subset with ferritin ≥1600 ng/ml than that in the subset with ferritin Conclusion. Both serum ferritin and anti-MDA5 antibody are powerful indicators for the early diagnosis of A/SIP with DM. Ferritin also predicts disease severity and prognosis for patients with anti-MDA5 antibody. Intensive treatment should be administered to cases that have anti-MDA5 antibody-associated ILD with DM showing hyperferritinaemia, especially if the ferritin level is ≥1600 ng/ml.Keywords
This publication has 26 references indexed in Scilit:
- RNA helicase encoded by melanoma differentiation–associated gene 5 is a major autoantigen in patients with clinically amyopathic dermatomyositis: Association with rapidly progressive interstitial lung diseaseArthritis & Rheumatism, 2009
- Interstitial lung disease in polymyositis and dermatomyositis: Longitudinal evaluation by pulmonary function and radiologyArthritis Care & Research, 2008
- A Case of Hypomyopathic Dermatomyositis Associated with Rapid Progressive Interstitial Pneumonia Resistant to Multi-immunosuppressive TherapyThe American Journal of the Medical Sciences, 2007
- Adult clinically amyopathic dermatomyositis with rapid progressive interstitial lung disease: a retrospective cohort studyClinical Rheumatology, 2007
- HLH‐2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosisPediatric Blood & Cancer, 2006
- Interstitial lung diseases associated with amyopathic dermatomyositisEuropean Respiratory Journal, 2006
- A systematic review of adult-onset clinically amyopathic dermatomyositis (dermatomyositis siné myositis): A missing link within the spectrum of the idiopathic inflammatory myopathiesJournal of the American Academy of Dermatology, 2006
- Treatment of antisynthetase‐associated interstitial lung disease with tacrolimusArthritis & Rheumatism, 2005
- Autoantibodies to a 140‐kd polypeptide, CADM‐140, in Japanese patients with clinically amyopathic dermatomyositisArthritis & Rheumatism, 2005
- Interstitial lung disease in polymyositis and dermatomyositisArthritis Care & Research, 2002