Journal List > J Korean Rheum Assoc > v.14(4) > 1003592

Yoon, Min, Park, Hong, and Kang: Rapidly Progressive Interstitial Lung Disease Associated with Dermatomyositis; A Case Report

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.

REFERENCES

1). Dalakas MC., Hohlfeld R. Polymyositis and dermatomyositis. Lancet. 2003. 362:971–82.
crossref
2). Kurasawa K., Nawata Y., Takabayashi K., Kumano K., Kita Y., Takiguchi Y, et al. Activation of pulmonary T cells in corticosteroid-resistant and-sensitive interstitial pneumonitis in dermatomyositis/polymyositis. Clin Exp Immunol. 2002. 129:541–8.
3). 김현숙: 서수홍: 허지안: 곽승기: 주지현: 윤종현등. 염증성근육병증에동반된폐침범의임상적특징. 대한류마티스학회지. 2007. 14:15–22.
4). Nawata Y., Kurasawa K., Takabayashi K., Miike S., Watanabe N., Hiraguri M, et al. Corticosteroid resistant interstitial pneumonitis in dermatomyositis/polymyositis: prediction and treatment with cyclosporine. J Rheumatol. 1999. 26:1527–33.
5). 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. 26:24–9.
crossref
6). Miyazaki E., Ando M., Muramatsu T., Fukami T., Matsuno ᄋ., Nureki S, et al. Early assessment of rapidly progressive interstitial pneumonia associated with amyopathic dermatomyositis. Clin Rheumatol. 2005. 26:436–9.
crossref
7). Maryam F., Ingrid E. Interstitial lung disease in polymyositis and dermatomyositis. Curr ᄋpin Rheumatol. 2005. 17:701–6.
8). 박근민: 최창민: 엄상원: 황용일: 임재준: 이재호등. 폐를침범한피부근염/다발성근염의임상적양상. 결핵및호흡기질환. 2001. 51:354–63.
9). Marie I., Hachulla E., Cherin P., Dominique S., Hatron PY., Hellot MF, et al. Intersitial lung disease in polymyositis and dermatomyositis. Arthritis Rheum. 2002. 40:614–22.
10). Kang EH., Lee EB., Shin KC., Im CH., Chung DH., Han SK, et al. Interstitial lung disease and amyopathic dermatomyositis. Rheumatology. 2005. 44:1282–6.
11). ᄋddis CV., Sciurba FC., Elmaqd KA., Starzl TE. Tacrolimus in refractory polymyositis with interstitial lung disease. Lancet. 1999. 22:1763–4.
12). Takada K., Nagasaka K., Miyasaka N. Polymyositis/dermatomyositis and interstitial lung disease: a new therapeutic approach with T-cell-specific immunosuppressants. Autoimmunity. 2005. 38:383–92.
crossref
13). Fathi M., Lundberg IE. Interstitial lung disease in polymyositis and dermatomyositis. Curr ᄋpin Rheumatol. 2005. 17:701–6.
crossref
14). Huh JW., Kim DS., Lee CK., Yoo B., Seo JB., Kitaichi M, et al. Two distinct clinical types of interstitial lung disease associated with polymyositis-dermatomyositis. Respir Med. 2007. 101:1761–9.

Fig. 1.
Heliotrope rash which is considered pathognomonic cutaneous features of dermatomyositis.
jkra-14-401f1.tif
Fig. 2.
(A) Poikilodermatous lesions on the back at the first presentation. (B) Gottron's papules appeared two months later after initial presentation.
jkra-14-401f2.tif
Fig. 3.
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.
jkra-14-401f3.tif
Fig. 4.
(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.
jkra-14-401f4.tif
TOOLS
Similar articles