jkra The Journal of the Korean Rheumatism Association J Korean Rheum Assoc 1226-8070 2233-4718 The Korean Rheumatism Association 10.4078/jkra.2007.14.4.401 jkra-14-401 Original Article Rapidly Progressive Interstitial Lung Disease Associated with Dermatomyositis; A Case Report YoonJae YoungM.D. MinSun YangM.D. ParkJu YeeM.D. HongSeung GounM.D. KangHyo JongM.D. Department of Internal Medicine, Bundang Jesaeng General Hospital, Seongnam, Korea 122007 23122007 14 4 401 406 07082007 24082007 Copyright © 2007 The Korean Rheumatism Association 2007 This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. Abstract

A previously healthy 44-year-old woman who was diagnosed as having dermatomyositis suddenly developed severe dyspnea while being in the state of improved condition of muscle weakness. Interstitial lung disease was found on high resolution computed tomography (HRCT). In spite of the treatment with the immune-modulating agent (high dose steroid, cyclopho- sphamide, immunoglubulin and cyclosporine), her condition deteriorated further and rapidly, leading to death. More intensive agent such as FK506 would be necessary in those cases of dermatomyositis-related interstitial lung disease that have poor prognostic factors.

 Interstitial lung disease Dermatomyositis REFERENCES Dalakas MC Hohlfeld R Polymyositis and dermatomyositis Lancet 2003 362 97182 Kurasawa K Nawata Y Takabayashi K Kumano K Kita Y Takiguchi Y Activation of pulmonary T cells in corticosteroid-resistant and-sensitive interstitial pneumonitis in dermatomyositis/polymyositis Clin Exp Immunol 2002 129 5418 김현숙: 서수홍: 허지안: 곽승기: 주지현: 윤종현등 염증성근육병증에동반된폐침범의임상적특징 대한류마티스학회지 2007 14 1522 Nawata Y Kurasawa K Takabayashi K Miike S Watanabe N Hiraguri M Corticosteroid resistant interstitial pneumonitis in dermatomyositis/polymyositis: prediction and treatment with cyclosporine J Rheumatol 1999 26 152733 Maeda K Kimura R Komuta K Igarashi T Cyclosporine treatment for polymyositis/dermatomyositis: is it possible to rescue the deteriorating cases with interstitial pneumonitis? Scand J Rheumatol 1997 26 249 Miyazaki E Ando M Muramatsu T Fukami T Matsuno ᄋ Nureki S Early assessment of rapidly progressive interstitial pneumonia associated with amyopathic dermatomyositis Clin Rheumatol 2005 26 4369 Maryam F Ingrid E Interstitial lung disease in polymyositis and dermatomyositis Curr ᄋpin Rheumatol 2005 17 7016 박근민: 최창민: 엄상원: 황용일: 임재준: 이재호등 폐를침범한피부근염/다발성근염의임상적양상 결핵및호흡기질환 2001 51 35463 Marie I Hachulla E Cherin P Dominique S Hatron PY Hellot MF Intersitial lung disease in polymyositis and dermatomyositis Arthritis Rheum 2002 40 61422 Kang EH Lee EB Shin KC Im CH Chung DH Han SK Interstitial lung disease and amyopathic dermatomyositis Rheumatology 2005 44 12826 ᄋddis CV Sciurba FC Elmaqd KA Starzl TE Tacrolimus in refractory polymyositis with interstitial lung disease Lancet 1999 22 17634 Takada K Nagasaka K Miyasaka N Polymyositis/dermatomyositis and interstitial lung disease: a new therapeutic approach with T-cell-specific immunosuppressants Autoimmunity 2005 38 38392 Fathi M Lundberg IE Interstitial lung disease in polymyositis and dermatomyositis Curr ᄋpin Rheumatol 2005 17 7016 Huh JW Kim DS Lee CK Yoo B Seo JB Kitaichi M Two distinct clinical types of interstitial lung disease associated with polymyositis-dermatomyositis Respir Med 2007 101 17619 Figures

Heliotrope rash which is considered pathognomonic cutaneous features of dermatomyositis.

(A) Poikilodermatous lesions on the back at the first presentation. (B) Gottron's papules appeared two months later after initial presentation.

Increased signal intensity in vastus intermedius, vastus lateralis, rectus femoris muscle, semitendinosus sartorius and short head muscle of biceps femoris on transverse (A) and coronal (B) view of fat suppressed T1-weighted MR images.

(A) The chest HRCT shows ground glass opacity along the bronchovascular bundle in both lung fields and small subpleural nodules in right upper segment. (B) Chest x-ray shows ground glass opacity in both lung fields on one day before she expired.