肺毛細血管炎の病理組織学的所見を有するびまん性肺胞出血。3例の症例報告

Diffuse alveolar hemorrhage with histopathologic manifestations of pulmonary capillaritis: Three case reports.

• Jun Xie
• Ying-Yue Zhao
• Jing Liu
• Guang-Min Nong

抄録

背景:

びまん性肺胞出血（DAH）とは、肺毛細血管出血や肺血管小血管損傷（主に動脈や静脈を含む毛細血管）が原因で肺胞腔内に肺微小循環血液がたまる多病因性肺胞出血のことである。DAHは組織学的に肺毛細血管炎（PC）の有無で分類され、文献にはほとんど報告されていない。

BACKGROUND: Diffuse alveolar hemorrhage (DAH) is a multicause pulmonary capillary hemorrhage or pulmonary vascular small vessel injury (mainly capillaries, including arteries and veins), causing pulmonary microcirculation blood to accumulate in the alveolar space. DAH is classified by the histological absence or presence of pulmonary capillaritis (PC) and is rarely reported in the literature.

ケースサマリー:

本症例は6～11歳の女児3名のDAHとPCの報告である。2例はヘモグロビン減少，1例は赤血球沈降率上昇を認めた．高分解能CTで両側びまん性肺浸潤を認め，肺生検でPCの診断が確定した．免疫蛍光検査では，肺胞壁に顆粒状IgGと少量の顆粒状IgAの沈着を1例に認めたが，他の2例では陰性であり，孤立性のpauci-immune PCであると考えられた．治療はグルココルチコイド単独または免疫抑制剤との併用で、全例で症状は消失した。

CASE SUMMARY: This is a report of three girls aged 6-11 years with DAH and PC. Two patients had decreased hemoglobin and one had increased erythrocyte sedimentation rate. High-resolution computed tomography showed bilateral diffuse pulmonary infiltrate, and diagnosis of PC was confirmed by lung biopsy. Immunofluorescence test in one case showed granular IgG and a small amount of granular IgA deposit on the alveolar walls, and was negative in the other two cases, describing isolated pauci-immune PC. Treatment was with glucocorticoid alone or combination with immunosuppressants, and the symptoms resolved in all patients.

結論:

PCは、全身疾患に伴う孤立性および免疫介在性PCに分類される。ほとんどの小児では、グルココルチコイド単独または免疫抑制剤との併用でコントロール可能である。

CONCLUSION: PC is classified as isolated and immune-mediated PC associated with systemic disease. It can be controlled in most children with glucocorticoid alone or combined with immunosuppressants.

©The Author(s) 2020. Published by Baishideng Publishing Group Inc. All rights reserved.