Wattsの分類

PSV：原発性全身性血管炎、CHCC：チャペルヒルコンセンサス会議
 
※図中の文献
345：Sada KE, Yamamura M, Harigai M, et al. Research Committee on Intractable Vasculitides, the Ministry of Health, Labour and Welfare of Japan. Classification and characteristics of Japanese patients with
antineutrophil cytoplasmic antibody-associated vasculitis in a nationwide, prospective, inception cohort study. Arthritis Res Ther 2014; 16:R101. PMID: 24758294
356：Watts R, Lane S, Hanslik T, et al. Development and validation of a consensus methodology for the classification of the ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studies. Ann Rheum Dis 2007; 66: 222-227. PMID: 16901958

結節性多発動脈炎でみられる症状

頻度が多いのは発熱、倦怠感、体重減少などの非特異的な症状と、神経障害である。
出典
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1: Clinical features and outcomes in 348 patients with polyarteritis nodosa: a systematic retrospective study of patients diagnosed between 1963 and 2005 and entered into the French Vasculitis Study Group Database.
著者: Christian Pagnoux, Raphaèle Seror, Corneliu Henegar, Alfred Mahr, Pascal Cohen, Véronique Le Guern, Boris Bienvenu, Luc Mouthon, Loïc Guillevin, French Vasculitis Study Group
雑誌名: Arthritis Rheum. 2010 Feb;62(2):616-26. doi: 10.1002/art.27240.
Abstract/Text: OBJECTIVE: Previous studies of polyarteritis nodosa (PAN) included patients with microscopic polyangiitis, because these entities were not distinguished prior to the Chapel Hill Consensus Conference (CHCC). This study was undertaken to describe the main characteristics of and long-term outcomes in patients with well-characterized PAN diagnoses.
METHODS: We conducted a systematic retrospective study of 348 patients who were diagnosed as having PAN between March 1963 and October 2005, were registered in the French Vasculitis Study Group database, and satisfied the American College of Rheumatology and CHCC criteria. Patient characteristics and outcomes were analyzed and compared according to hepatitis B virus (HBV) status.
RESULTS: At diagnosis, the mean +/- SD age was 51.2 +/- 17.3 years. The most frequent findings were general symptoms (93.1%), neurologic manifestations (79%), skin involvement (49.7%), abdominal pain (35.6%), and hypertension (34.8%); 66.2% had renal artery microaneurysms; 70.1% had histologically proven PAN. Patients with HBV-related PAN (n = 123) had more frequent peripheral neuropathy, abdominal pain, cardiomyopathy, orchitis, and hypertension compared with patients with non-HBV-related PAN (n = 225). During a mean +/- SD followup of 68.3 +/- 63.5 months, 76 patients (21.8%) relapsed (63 with non-HBV-related PAN [28%] versus 13 with HBV-related PAN [10.6%]; P < 0.001); 86 patients (24.7%) died (44 with non-HBV-related PAN [19.6%] versus 42 with HBV-related PAN [34.1%]; P = 0.003). Five-year relapse-free survival rates were 59.4% (95% confidence interval [95% CI] 52.6-67.0) versus 67.0% (95% CI 58.5-76.8) for non-HBV-related PAN and HBV-related PAN, respectively. Multivariate analysis retained age >65 years, hypertension, and gastrointestinal manifestations requiring surgery or at least consultation with a surgeon as independent predictors of death, whereas patients with cutaneous manifestations or non-HBV-related PAN had a higher risk of relapse.
CONCLUSION: Our findings indicate that the rate of mortality from PAN remains high, especially for the elderly, and relapses do occur, particularly in patients with non-HBV-related PAN with cutaneous manifestations.
Arthritis Rheum. 2010 Feb;62(2):616-26. doi: 10.1002/art.27240.

結節性多発動脈炎の診断基準

厚生労働省特定疾患難治性血管炎班による診断基準（2006年改訂）

顕微鏡的多発血管炎の診断基準

罹患血管サイズによる血管炎の分類

あくまでも血管サイズによる分類である。Variable vessel vasculitis、single organ vasculitis、Vasculitis associated with systemic disease、Vasculitis associated with probable etiologyは血管サイズによる分類ではないためここには含まれていない。
出典
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1: 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides.
Arthritis Rheum. 2013 Jan;65(1):1-11. doi: 10.1002/art.37715.

血管炎の診断の流れ

まずはどの血管炎でもみられる症状（発熱、倦怠感、体重減少）に加え、大血管炎を示唆する症状があるのか、中小血管炎を示唆する所見があるかをみて鑑別を進める。
大血管炎の可能性が高ければ巨細胞性動脈炎か高安病かを考える。若年女性であれば高安病が、高齢者であれば巨細胞性動脈炎が疑われるので確定診断のための検査を行う。
中小血管炎が疑われたら種々の検査を行って、それぞれの診断基準と照らし合わせて診断する。頭痛、認知機能低下、脳卒中様発作、巣症状、一過性脳虚血発作など中枢性血管炎を疑う場合には髄液検査も行う。寒冷刺激で誘発される紫斑や関節痛はクリオグロブリンを疑うので、クリオグロブリン定性、定量を提出する。組織生検は所見や症状がある部分を選択する。
リウマチやSLE合併が疑われる症例ではANA.RF、抗CCP抗体をそれぞれ提出する。
薬剤性の評価のため薬剤歴は必ず確認する。悪性腫瘍関連も疑わしい場合には検索する。
血液培養は感染性心内膜炎除外のため必ず行う。
出典
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1: 著者提供

Wattsの分類

PSV：原発性全身性血管炎、CHCC：チャペルヒルコンセンサス会議
 
※図中の文献
345：Sada KE, Yamamura M, Harigai M, et al. Research Committee on Intractable Vasculitides, the Ministry of Health, Labour and Welfare of Japan. Classification and characteristics of Japanese patients with
antineutrophil cytoplasmic antibody-associated vasculitis in a nationwide, prospective, inception cohort study. Arthritis Res Ther 2014; 16:R101. PMID: 24758294
356：Watts R, Lane S, Hanslik T, et al. Development and validation of a consensus methodology for the classification of the ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studies. Ann Rheum Dis 2007; 66: 222-227. PMID: 16901958

結節性多発動脈炎でみられる症状

頻度が多いのは発熱、倦怠感、体重減少などの非特異的な症状と、神経障害である。
出典
imgimg
1: Clinical features and outcomes in 348 patients with polyarteritis nodosa: a systematic retrospective study of patients diagnosed between 1963 and 2005 and entered into the French Vasculitis Study Group Database.
著者: Christian Pagnoux, Raphaèle Seror, Corneliu Henegar, Alfred Mahr, Pascal Cohen, Véronique Le Guern, Boris Bienvenu, Luc Mouthon, Loïc Guillevin, French Vasculitis Study Group
雑誌名: Arthritis Rheum. 2010 Feb;62(2):616-26. doi: 10.1002/art.27240.
Abstract/Text: OBJECTIVE: Previous studies of polyarteritis nodosa (PAN) included patients with microscopic polyangiitis, because these entities were not distinguished prior to the Chapel Hill Consensus Conference (CHCC). This study was undertaken to describe the main characteristics of and long-term outcomes in patients with well-characterized PAN diagnoses.
METHODS: We conducted a systematic retrospective study of 348 patients who were diagnosed as having PAN between March 1963 and October 2005, were registered in the French Vasculitis Study Group database, and satisfied the American College of Rheumatology and CHCC criteria. Patient characteristics and outcomes were analyzed and compared according to hepatitis B virus (HBV) status.
RESULTS: At diagnosis, the mean +/- SD age was 51.2 +/- 17.3 years. The most frequent findings were general symptoms (93.1%), neurologic manifestations (79%), skin involvement (49.7%), abdominal pain (35.6%), and hypertension (34.8%); 66.2% had renal artery microaneurysms; 70.1% had histologically proven PAN. Patients with HBV-related PAN (n = 123) had more frequent peripheral neuropathy, abdominal pain, cardiomyopathy, orchitis, and hypertension compared with patients with non-HBV-related PAN (n = 225). During a mean +/- SD followup of 68.3 +/- 63.5 months, 76 patients (21.8%) relapsed (63 with non-HBV-related PAN [28%] versus 13 with HBV-related PAN [10.6%]; P < 0.001); 86 patients (24.7%) died (44 with non-HBV-related PAN [19.6%] versus 42 with HBV-related PAN [34.1%]; P = 0.003). Five-year relapse-free survival rates were 59.4% (95% confidence interval [95% CI] 52.6-67.0) versus 67.0% (95% CI 58.5-76.8) for non-HBV-related PAN and HBV-related PAN, respectively. Multivariate analysis retained age >65 years, hypertension, and gastrointestinal manifestations requiring surgery or at least consultation with a surgeon as independent predictors of death, whereas patients with cutaneous manifestations or non-HBV-related PAN had a higher risk of relapse.
CONCLUSION: Our findings indicate that the rate of mortality from PAN remains high, especially for the elderly, and relapses do occur, particularly in patients with non-HBV-related PAN with cutaneous manifestations.
Arthritis Rheum. 2010 Feb;62(2):616-26. doi: 10.1002/art.27240.