全身性強皮症および多発性筋炎のオーバーラップ症候群に合併したⅡ型呼吸不全に対して呼吸器リハビリテーション治療が有効であった1例

Type II Respiratory Failure with Systemic Sclerosis/Polymyositis Overlap Syndrome : A Case Report of Successful Respiratory Rehabilitation Therapy

はじめに

　全身性強皮症は皮膚真皮のコラーゲン主体の結合組織増加，すなわち線維化で特徴づけられる．線維化は肺・消化管・血管壁にも及び，臓器機能，皮膚・腎の血行が障害される全身性疾患である．それに対して多発性筋炎は，炎症性筋炎の中でも主に横紋筋を侵す原因不明の特発性炎症性筋疾患の代表である¹⁾．加えて，複数の膠原病の合併としてオーバーラップ症候群がしばしば報告されている．全身性強皮症と多発性筋炎はそれぞれの疾患において呼吸器リハビリテーション治療の報告が認められるが^{2, 3)}，双方のオーバーラップ症候群に対する呼吸器リハビリテーション治療の報告は渉猟し得た限りでは認められなかった．通常では，全身性強皮症や多発性筋炎の増悪ではステロイドパルスや免疫グロブリン投与などの免疫抑制療法が実施される⁴⁾．今回われわれは，全身性強皮症と多発性筋炎の経過中にⅡ型呼吸不全による意識障害により緊急入院となり，呼吸管理と呼吸器リハビリテーション治療を施行し，自宅退院が可能となった症例を経験したので報告する．

Introduction：Respiratory failures are categorized into types I and II. To our knowledge, we report the first case of pulmonary rehabilitation in a patient with systemic sclerosis/polymyositis overlap syndrome who developed type II respiratory failure.

Methods：The patient was a 77-year-old woman who had received treatment for systemic sclerosis and polymyositis at another hospital. When she visited our hospital to obtain a second opinion, she suddenly lost consciousness and underwent trachea intubation because of type Ⅱ respiratory failure. She received physical therapy on the third day of hospitalization and underwent a tracheotomy on the 16th day. As her thoracic movement was markedly restricted, we started physical training. After she was weaned off from the ventilator on the 43rd day, we performed muscular strength training and aerobic exercise. No exacerbation of CO₂ storage was observed even if chest motion training was performed. She was discharged on the 72nd day and advised to wear retina^®．Administration of therapeutic drugs such as steroids was maintained at the same dose.

Conclusion：Physical therapy, such as chest mobilization, was effective for marked restriction of chest movement in a patient who had both polymyositis and systemic sclerosis.