J Korean Rheum Assoc.  2007 Dec;14(4):401-406.

Rapidly Progressive Interstitial Lung Disease Associated with Dermatomyositis; A Case Report

• Yoon JY
• Min SY
• Park JY
• Hong SG
• Kang HJ

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, cyclophosphamide, 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.

Keyword

Interstitial lung disease; Dermatomyositis

MeSH Terms

Adult
Cyclophosphamide
Dermatomyositis*
Dyspnea
Female
Humans
Lung Diseases, Interstitial*
Muscle Weakness
Tacrolimus
Cyclophosphamide
Tacrolimus

Figure

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  Fig. 1. Heliotrope rash which is considered pathognomonic cutaneous features of dermatomyositis.

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  Fig. 2. (A) Poikilodermatous lesions on the back at the first presentation. (B) Gottron's papules appeared two months later after initial presentation.

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  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.

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  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.

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