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 好酸球性多発血管炎性肉芽腫症の治療レジメンの選択

出典
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1: 厚生労働省 難治性血管炎に関する調査研究班:抗リン脂質抗体症候群・好酸球性多発血管炎性肉芽腫症・結節性多発動脈炎・リウマトイド血管炎の治療の手引き 2020, 診断と治療社、2021. P. xiii.

ACRの好酸球性多発血管炎性肉芽腫症の分類基準(1990)

分類上、6項目中少なくとも4項目以上が認められた場合、好酸球性多発血管炎性肉芽腫症と判定する。項目の種類を問わず4項目以上認めれば、感度85.0%、特異度99.7%である。
出典
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1: The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis).
著者: A T Masi, G G Hunder, J T Lie, B A Michel, D A Bloch, W P Arend, L H Calabrese, S M Edworthy, A S Fauci, R Y Leavitt
雑誌名: Arthritis Rheum. 1990 Aug;33(8):1094-100.
Abstract/Text: Criteria for the classification of Churg-Strauss syndrome (CSS) were developed by comparing 20 patients who had this diagnosis with 787 control patients with other forms of vasculitis. For the traditional format classification, 6 criteria were selected: asthma, eosinophilia greater than 10% on differential white blood cell count, mononeuropathy (including multiplex) or polyneuropathy, non-fixed pulmonary infiltrates on roentgenography, paranasal sinus abnormality, and biopsy containing a blood vessel with extravascular eosinophils. The presence of 4 or more of these 6 criteria yielded a sensitivity of 85% and a specificity of 99.7%. A classification tree was also constructed with 3 selected criteria: asthma, eosinophilia greater than 10% on differential white blood cell count, and history of documented allergy other than asthma or drug sensitivity. If a subject has eosinophilia and a documented history of either asthma or allergy, then that subject is classified as having CSS. For the tree classification, the sensitivity was 95% and the specificity was 99.2%. Advantages of the traditional format compared with the classification tree format, when applied to patients with systemic vasculitis, and their comparison with earlier work on CSS are discussed.
Arthritis Rheum. 1990 Aug;33(8):1094-100.

厚生労働省による好酸球性多発血管炎性肉芽腫症の診断基準(1998)

出典
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1: 「好酸球性多発血管炎性肉芽腫症」(厚生労働省)(http://www.mhlw.go.jp/file/06-Seisakujouhou-10900000-Kenkoukyoku/0000089895.pdf)を加工して作成

厚生労働省による好酸球性多発血管炎性肉芽腫症の重症度分類

患者の状態を把握するために用いる。
出典
img
1: 「好酸球性多発血管炎性肉芽腫症」(厚生労働省)(http://www.mhlw.go.jp/file/06-Seisakujouhou-10900000-Kenkoukyoku/0000089895.pdf)を加工して作成

ACRの好酸球性多発血管炎性肉芽腫症分類基準(2022)

参考文献:
  1. 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis With Polyangiitis
  1. Arthritis Rheumatol. 74:386-392. (2022)
出典
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1: 著者提供

好酸球性多発血管炎性肉芽腫症の生検所見

a:単核球、巨細胞と散在性の好酸球の浸潤が小血管に認められ、一部フィブリン血栓が存在する。
b:肺血管にみられた好酸球性血管炎。
出典
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1: Odze RD & Goldblum JR: Surgical Pathology of the GI Tract, Liver, Biliary Tract, and Pancreas, 3rd ed. Saunders, 2015.

 好酸球性多発血管炎性肉芽腫症の治療レジメンの選択

出典
img
1: 厚生労働省 難治性血管炎に関する調査研究班:抗リン脂質抗体症候群・好酸球性多発血管炎性肉芽腫症・結節性多発動脈炎・リウマトイド血管炎の治療の手引き 2020, 診断と治療社、2021. P. xiii.

ACRの好酸球性多発血管炎性肉芽腫症の分類基準(1990)

分類上、6項目中少なくとも4項目以上が認められた場合、好酸球性多発血管炎性肉芽腫症と判定する。項目の種類を問わず4項目以上認めれば、感度85.0%、特異度99.7%である。
出典
imgimg
1: The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis).
著者: A T Masi, G G Hunder, J T Lie, B A Michel, D A Bloch, W P Arend, L H Calabrese, S M Edworthy, A S Fauci, R Y Leavitt
雑誌名: Arthritis Rheum. 1990 Aug;33(8):1094-100.
Abstract/Text: Criteria for the classification of Churg-Strauss syndrome (CSS) were developed by comparing 20 patients who had this diagnosis with 787 control patients with other forms of vasculitis. For the traditional format classification, 6 criteria were selected: asthma, eosinophilia greater than 10% on differential white blood cell count, mononeuropathy (including multiplex) or polyneuropathy, non-fixed pulmonary infiltrates on roentgenography, paranasal sinus abnormality, and biopsy containing a blood vessel with extravascular eosinophils. The presence of 4 or more of these 6 criteria yielded a sensitivity of 85% and a specificity of 99.7%. A classification tree was also constructed with 3 selected criteria: asthma, eosinophilia greater than 10% on differential white blood cell count, and history of documented allergy other than asthma or drug sensitivity. If a subject has eosinophilia and a documented history of either asthma or allergy, then that subject is classified as having CSS. For the tree classification, the sensitivity was 95% and the specificity was 99.2%. Advantages of the traditional format compared with the classification tree format, when applied to patients with systemic vasculitis, and their comparison with earlier work on CSS are discussed.
Arthritis Rheum. 1990 Aug;33(8):1094-100.