血漿交換により救命できたMPO-ANCA陽性肺腎疾患の1例(<特集>アフェレシスにおけるセイフティーマネジメント)  [in Japanese] A Case of MPO-ANCA Associated Vasculitis with Renal and Pulmonary Involvements Successfully Treated by Plasma Exchange  [in Japanese]

症例は63歳, 男性.平成15年4月, 全身倦怠感と37℃台前半の発熱で受診.Hb ; 9.1g/dlと正球性貧血, Cr ; 3.9mg/dl, BUN ; 47.8mg/dl, 検尿;潜血(3+), 蛋白(+), 沈査;変形赤血球(+)と腎障害, 胸部X-p, CTにて右下肺野の浸潤影と胸水, 心嚢水の貯留を認めた.さらにCRP;(6+) 12.22mg/dl, ANA ; ×160, MPO-ANCA ; 349EUとANCA高値を認めた.腎生検を予定するも, 気道出血, 呼吸不全が出現し人工呼吸器管理となり, 急激な全身状態の悪化のため中止した.検査および臨床所見からMPO-ANCA陽性肺腎疾患と考え, 血漿交換, 持続血液濾過透析, 透析を行うとともにシクロホスファミド, ステロイド剤のパルス投与を行った.本疾患は極めて急激な経過をたどり集中的に治療しても予後不良のことが多いが, 本症例では早期の血漿交換の適用が, 救命のみならず腎障害の進行を抑制し維持透析からの離脱に有用であったと考えられる.

A 63-year-old male visited our hospital because of general malaise and a low grade fever in April 2003. Normocytic anemia to Hb ; 9.1g/dl and moderate azotemia to Cr ; 3.9mg/dl and BUN ; 47.8mg/dl were documented. Microsopic hematuria and proteinuria were associated, and irregular-shaped erythrocytes were observed microscopically in urinary sediments. On chest X-p diffuse but irregular-shaped infiltration was observed in the right lower lung field, and pleural and pericardial effusions were also documented on thoracic CT. Other laboratory findings indicated : CRP ; 6+, 12.22mg/dl, ANA ; ×160 positive, and MPO-ANCA was 349EU. Although renal biopsy had been scheduled, general condition deteriorated rapidly to require artificial respiration because of respiratory failure due to pulmonary hemorrhage, and the patient was diagnosed as having MPO-ANCA associated vasculitis with renal and pulmonary involvements according to the clinical and laboratory findings without the histological examination of the kidney. Not only the systemic administration of cyclophosphamide and the pulse-treatment by methylprednisolone, but plasma exchange, CHDF, and ordinary hemodialysis were also applied in addition to the artificial respiration. With these treatments serum MPO-ANCA level decreased significantly, and the patient was removed from the artificial respiration and the hemodialysis about 3 months later. Although the prognosis of MPO-ANCA vasculitis is quite poor despite of the various treatments, we report here a patient successfully treated by intensive procedures including immediate application of plasma exchange.