Cyclosporin-Aにて一旦軽快し, 減量にて再燃死亡した皮膚筋炎に伴う間質性肺炎の一例  [in Japanese] A patient with interstitial pneumonia associated with dermatomyositis who relapsed after reducing cyclosporin-A treatment  [in Japanese]

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  症例は67歳,女性.眼瞼,両手指伸側に紅斑が出現すると共に,労作時呼吸困難を自覚し,2004年7月21日当院に入院.四肢筋力低下,ヘリオトロープ疹,ゴットロン徴候を呈していたが,CKは194 U/lと正常範囲であった.リウマトイド因子以外の自己抗体は全て陰性,低酸素血症,胸部CTにて間質性肺炎を認め,皮膚筋炎に伴う進行性間質性肺炎と診断した.ステロイドパルス,Cyclosporin-A(以下Cy-A)による治療を開始し著明な改善を得た.その際,Cy-Aのトラフは456.4 ng/mlと著明高値であった.サイトメガロウイルス感染症を契機に,Cy-Aを減量したところ,Cy-Aが有効血中濃度であるにも関わらず,間質性肺炎が急速に再燃進行し死亡した.皮膚筋炎合併間質性肺炎におけるCy-Aの減量には,厳重な注意を必要とすることが示唆されると共に,トラフ値が必ずしも有効血中濃度の指標とならない可能性も示唆された.<br>

  A 67-year-old female noticed dyspnea on exertion associated with the development of erythema in the eyelids and the bilateral fingers, and was admitted to our hospital on July 21, 2004. Proximal muscle weakness in the limbs, heliotrope rash, and Gottron's sign were observed, but the CK level was normal (194 U/l). All autoantibodies except for rheumatoid factor were negative. Hypoxemia and interstitial pneumonia on chest CT images were observed. Based on these findings, a diagnosis of advanced interstitial pneumonia associated with dermatomyositis was made. Combination immunosuppressive therapy was initiated with corticosteroid pulse therapy and cyclosporin-A (Cy-A), resulting in marked improvement. The Cy-A trough concentration was markedly high (456.4 ng/ml). When cytomegalovirus infection developed, the dose of Cy-A was reduced. Although the blood trough concentration of Cy-A was maintained at an adequately high level, the patient died of recurrence of rapidly progressive interstitial pneumonia. Careful observation is required when the dose of Cy-A is reduced for a patient with interstitial pneumonia associated with dermatomyositis. Furthermore, it is suggested that the trough concentration level of Cy-A is not always a useful parameter.<br>


  • Japanese Journal of Clinical Immunology

    Japanese Journal of Clinical Immunology 30(2), 139-143, 2007-04-28

    The Japan Society for Clinical Immunology

References:  13

Cited by:  1


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