長谷川 みどり

BACKGROUND: Myeloperoxidase anti-neutrophil cytoplasmic antibody-related nephritis (MPO-ANCA nephritis) is occasionally accompanied by lung abnormalities such as pulmonary fibrosis. However, the clinical features of pulmonary fibrosis in patients with MPO-ANCA nephritis have not been well documented. This study was performed to compare the prognosis of a usual interstitial pneumonia (UIP) pattern of lung fibrosis in patients with MPO-ANCA nephritis with the prognosis of idiopathic pulmonary fibrosis (IPF). METHODS: We retrospectively reviewed the medical records of 126 patients with MPO-ANCA nephritis and identified 31 with a UIP pattern of lung fibrosis on high-resolution or thin-slice computed tomography (CT). We compared the characteristics and prognosis of these patients with those of 32 patients with IPF. In 18 patients from both groups, we assessed and compared the decline in lung volume over time using three-dimensional (3D) CT images reconstructed from thin-section CT data. RESULTS: The numbers of male and female patients were nearly equal among patients with MPO-ANCA nephritis exhibiting a UIP pattern; in contrast, significant male dominancy was observed among patients with IPF (p = 0.0021). Significantly fewer smokers were present among the patients with MPO-ANCA nephritis with a UIP pattern than among those with IPF (p = 0.0062). There was no significant difference in the median survival time between patients with MPO-ANCA nephritis with a UIP pattern (50.8 months) and IPF (55.8 months; p = 0.65). All patients with IPF in this cohort received antifibrotic therapy (pirfenidone or nintedanib). Almost half of the deaths that occurred in patients with MPO-ANCA nephritis with a UIP pattern were caused by non-respiratory-related events, whereas most deaths in patients with IPF were caused by respiratory failure such as acute exacerbation. In the 3D CT lung volume analyses, the rate of decline in lung volume was equivalent in both groups. CONCLUSIONS: MPO-ANCA nephritis with a UIP pattern on CT may have an unfavorable prognosis equivalent to that of IPF with a UIP pattern treated with antifibrotic agents.

高齢かつ低心機能患者に腹膜透析(PD)を導入し、生活の質(QOL)のみならず心機能が改善した87歳男性の症例を報告した。10年前より腎硬化症による慢性腎不全にて加療中、心エコーでの所見で高度の低心機能を認めていたが、腎機能が不良のため心カテーテル検査による心精査を施行できなかった。うっ血性心不全を発症し入退院を繰り返していた。腎機能は緩徐に増悪したため、PDカテーテルを挿入し、体液量コントロールを目的にPD導入とした。導入後1年の時点で、心機能、SF-36v2によるQOL評価ともに改善を認めた。下腿の浮腫が消失し、経口摂取が改善し体重は増加した。本症例のように重度の低心機能例では、残腎機能が十分に保たれているうちからPD導入が望ましいと考えられた。本症例ではPDによって心機能が改善したが、心機能改善には血液透析(HD)にはない緩徐な除水に加え、さまざまな物質の除去が関与している可能性があり、今後HDと比較した検討が必要であることを指摘した。