血管炎の分類基準（草案）Vasculitis 2019

New draft classification criteria: International DCVAS Studyより
1. AAV
・どのcriteriaも、small or medium vessel vasculitisと診断した時に用いる
・どの criteria も感度特異度>90%

＜GPA＞
10項目を合計して5点以上でGPAと分類
Clinical
 Bloody nasal discharge, ulcers, crusting, congestion or blockage, or nasal septal defect/perforations: +3
 Cartilaginous involvement: +2
 Constructive or sensorineural hearing loss: +1
Tests
 cANCA or PR3- antibody positive: +5
 Nodules, mass or cavitation on chest imaging: +2
 Granuloma, extravascular granulomatous inflammation or giant cells on biopsy: +2
 Inflammation, consolidation or effusion of the nasal/paranasal sinuses, or mastoiditis on imaging: +1
 Pauci-immune glomerulonephritis on biopsy: +1
 pANCA or MPO-antibody positive: +1
 Eosinophil ocunt > 1 (* 109 /L): -4

<MPA>
6項目を合計して5点以上でMPAと分類
Clinical
 Bloody nasal discharge, ulcers, crusting, congestion or blockage, or nasal septal defect/perforations: -3
Tests
 pANCA or MPO-antibody positive: +6
 Fibrosis or ILD on chest imaging: +3
 Pauci-immune glomerulonephritis on biopsy: +3
 cANCA or PR3- antibody positive: -1
 Eosinophil ocunt > 1 (* 109 /L): -4

<EGPA>
7項目を合計して6点以上でEGPAと分類
Clinical
 Obstructive airway disease: +3
 Nasal polyps: +3
 Mononeuritis multiplex or monor neuropathy: +1
Tests
 Eosinophil ocunt > 1 (* 109 /L): +5
 Extravascular eosinophil predominant inflammation/increased eosinophils in bone marrow: +2
 Microscopic hematuria: -1
 cANCA or PR3-antibody positive: -3

2. LVV
 Primary component analysisを利用して、GCAとTAKを分類：ほぼ年齢に依存
→そのため、Absolute inclusion critiriaは診断時の年齢：TAK 60歳以下、GCA 40歳以上
 GCAにおけるTABの陽性率：427/704人
 TABは以下のうちいずれかで陽性と定義：Giant cells、Granuloma、Mononuclear infiltration
 TAKではTAB陽性はほぼ0
 Halo signもGCAで陽性率半分程度、TAKではほぼ0.

<GCA>
Absolution criteria
 40歳以上
以下の項目で6点以上であればGCAと分類
Clinical features
 Morning stiffness in shoulder or neck: +2
 Sudden visual loss: +2
 Jaw or tongue claudication: +2
 New temporal headache: +2
 Scalo tenderness: +2
Temporal Artery Exam Findings
 Reduced pulse, ‘cord-like’, or tenderness: +1
Laboratory findings
 ESR 50以上 or CRP 10mg/dL以上: +3
Temporal Artery biopsy
 Definite vasculitius: +5
Imaging findings
 Temporal artery halo sign (US) : +5
 Bilateral axillary involvement: +3
 FDG-PET activity throughout aorta: +3

Primary component analysis を用いてGCAとTKAのclusterに分けた後に、GCAの分類に寄与する項目（Development cohort 70%, Validation cohort 30%で検証）
 Definitive vasculitis on TAB: OR 160.3 (37.6-683.4)
 TA halo on US: [cluster後に追加したためORなし]
 FDG-PET activity throughout aorta: OR 26.5 (8.3-84.6)
 ESR 50以上 or CRP 10mg/dL以上: OR 25.2 (10.2-61.8)
 Bilateral axillary involvement on imaging : OR 13.7 (4.7-39.8)
 Morning stiffness in shoulder/neck: OR 11.6 (5.1-26.4)
 Sudden visual loss: OR 8.0 (3.1-21.1)
 New temporal headache: OR 8.7 (3.8-20.3)
 Scalp tenderness: OR 6.8 (4.0-11.6)
 Jaw or tongue claudication: OR 7.0 (3.3-15.1)
 TA abnormality on vascular exam: OR 3.8 (2.0-7.5)

<TAK>
Absolution criteria
 60歳以下 + 画像で明らかなvasculitsあり
以下の項目で5点以上で荒ればTAKと分類
Clinical features
 Female: +1
 Angina or ischemic cardiac pain attributable to vasculitis: +2
 Arm or leg claudication: +2
Vascular Exam Findings
 Arterial bruit: +2
 Reduced pulse in upper extreamity: +2
 Carotid - reduced pulse or tenderness: +2
 SBP difference in arms (20mmHg以上) : +1
Angiographic and ultrasound findings
 Number of affected arteries (select one): one ortery (+1), two artery(+2), three or more arteries(+3)
 Vasculitis affecting paired branch arteries: +1
 Abdominal aorta involvement with renal or mesenteric invovement: +3

Primary component analysis を用いてGCAとTKAのclusterに分けた後に、TAKの分類に寄与する項目（Development cohort 70%, Validation cohort 30%で検証）
 Abdominal aorta + renal/mess on imaging: OR 15.5 (4.7-51.5)
 Three or more affected arteries on imaging: [cluster後に追加したためORなし]
 Angina or ischemic cardiac pain: OR 7.1 (1.6-30.7)
 Reduced pulse in upper extremity: OR 6.8 (3.0-15.1)
 Arm or leg claudication: OR 6.5 (3.3-13.1)
 Carotid – reduced pulse or tenderness: OR 4.8 (2.0-11.4)
 Arterial bruit: OR 4.6 (2.4-8.8)
 Two affected arteries on imaging: [cluster後に追加したためORなし]
 Imaging involvement of paired branch arteries: OR 3.7 (1.8-7.5)
 SBP difference in arms (20mmHg以上) : OR 3.7 (1.4-9.5)
 Female: OR 2.5 (1.4-4.6)
 One affected artery on imaging: [cluster後に追加したためORなし]

<別途の感想>
 Primary component analysiとLasso regression analysisは、臨床情報による患者のクラスター解析やcriteriaの精度を調べるのに必須
 質問いろいろあったけど聞き取れず。。。
 これらをそれぞれの施設で使用してみてどうなのか報告していく必要あり
Tongue claudicationとか確認したことなかった。。。こんな報告もあるんだBMJ Case Rep. 2012; 2012: bcr2012007241.