今日の臨床サポート

血管炎症候群の診断アプローチ、分類不能の血管炎

著者: 吉見祐輔 名古屋第二赤十字病院 総合内科

監修: 野口善令 豊田地域医療センター 総合診療科

著者校正済:2021/05/26
現在監修レビュー中
患者向け説明資料

概要・推奨   

  1. 結節性多発動脈の診断に上腸間膜動脈、腎動脈、腹腔動脈の動脈瘤、拡張、狭窄を証明するために古典的血管造影が困難な場合にはCTanigoやMRangioが役立つ可能性がある(推奨度2)
  1. ANCAはANCA関連血管炎の診断に重要であるがその感度特異度は100%ではなく注意が必要である。
  1. 抗GBM抗体疾患の診断に血清抗GBM抗体は必須でなく、血液検査のみで否定することは奨められない。
  1. 閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご契約
  1. 閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご契約が必要と
薬剤監修について:
オーダー内の薬剤用量は日本医科大学付属病院 薬剤部 部長 伊勢雄也 以下、林太祐、渡邉裕次、井ノ口岳洋、梅田将光による疑義照会のプロセスを実施、疑義照会の対象については著者の方による再確認を実施しております。
※薬剤中分類、用法、同効薬、診療報酬は、エルゼビアが独自に作成した薬剤情報であり、
著者により作成された情報ではありません。
尚、用法は添付文書より、同効薬は、薬剤師監修のもとで作成しております。
※薬剤情報の(適外/適内/⽤量内/⽤量外/㊜)等の表記は、エルゼビアジャパン編集部によって記載日時にレセプトチェックソフトなどで確認し作成しております。ただし、これらの記載は、実際の保険適用の査定において保険適用及び保険適用外と判断されることを保証するものではありません。また、検査薬、輸液、血液製剤、全身麻酔薬、抗癌剤等の薬剤は保険適用の記載の一部を割愛させていただいています。
(詳細はこちらを参照)
著者のCOI(Conflicts of Interest)開示:
吉見祐輔 : 未申告[2021年]
監修:野口善令 : 特に申告事項無し[2021年]

改訂のポイント:
  1. 定期レビューを行い、以下の点について加筆修正を行った。
  1. ガイドラインは更新なかったものの、前回漏れがあった点について修正した。
  1. 推奨に、血液培養の重要性を追記及び皮膚生検について記載した。
  1. PANの治療をガイドラインにそって若干変更した。
  1. コーガン病などのまれな疾患は変更なし。

病態・疫学・診察

疫学情報・病態・注意事項  
  1. 血管炎症候群の診断アプローチ、分類不能の血管炎の診断には血管炎を分類して考えることが役立つ。
  1. 血管炎とは血管壁に炎症が起こり血管壁の構造が障害された結果、出血もしくは組織虚血を来し、種々の症状を起こす疾患群である。
  1. 血管炎はまず7つのカテゴリーに分類され、①大血管炎、②中血管炎、③小血管炎、④Variable vessel vasculitis、⑤single organ vasculitis、⑥Vasculitis associated with systemic disease、⑦Vasculitis associated with probable etiology、――がそれにあたる。罹患血管による血管炎の分類を下記の図に示す[1]
 
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

img1:  2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides.
 
 Arthritis Rheum. 2013 Jan;65(1):1-11. do・・・
 
  1. 大血管炎は大動脈とその主要な分枝が侵され、高安病、巨細胞性動脈炎が含まれる。高安病は若年女性に多く、巨細胞性動脈炎は高齢者に多く、しばしばリウマチ性多発筋痛症に合併する。
  1. 中血管炎は主要な内蔵動脈とその分枝が侵され、結節性多発動脈炎と川崎病が含まれる。
  1. 小血管炎では臓器実質内の小動脈、細動脈、毛細血管、静脈が侵され、ANCA(抗好中球細胞質抗体)と関連したANCA関連血管炎と、補体や免疫グロブリンと関連したImmune complex vasculitisに分類される。
  1. ANCA関連血管炎には、顕微鏡的多発血管炎と多発血管炎性肉芽腫症(旧Wegener肉芽腫症)と好酸球性多発血管炎性肉芽腫症(旧チャーグストラウス症候群)が含まれる。
  1. Immune complex vasculitisには、IgA血管炎(ヘノッホ・シェーンライン紫斑病)、クリオグロブリン血管炎、Anti-GBM disease、低補体血症性蕁麻疹様血管炎が含まれる。
  1. Variable vessel vasculitisは特定の血管ではなく、大、中、小すべてのサイズの血管と、動脈、静脈、毛細血管すべてのタイプの血管を侵す血管炎であり、ベーチェット病とコーガン病が含まれる。
  1. single organ vasculitisは単一臓器に起きる血管炎であり、どのサイズのどのタイプの血管が侵されてもよい。ただし全身性血管炎が示唆されないものになる。具体的には皮膚白血球破砕血管炎、皮膚動脈炎、原発性中枢神経血管炎、孤発性大動脈炎などがそれにあたる。
  1. Vasculitis associated with systemic diseaseは全身性疾患と関連した血管炎であり、リウマトイド血管炎やループス血管炎、サルコイド血管炎がそれにあたる。
  1. Vasculitis associated with probable etiologyは特定の病因と関連がありそうな血管炎で、現在言われているものとしては梅毒性動脈炎、B型肝炎ウイルス関連血管炎、C型肝炎ウイルス関連クリオグロブリン血管炎、薬剤誘発性免疫複合体血管炎、薬剤誘発性ANCA関連血管炎、悪性腫瘍関連血管炎などがある。
  1. まずは患者の症状、すでに出ている検査結果などから大、中、小血管炎のどれにあたるかを考えて、それぞれ鑑別を進めると良い。
  1. Variable vessel vasculitisについてはベーチェット病患者に血管炎を疑う所見を認めた場合に考えればよい。ベーチェット病の診断がなされていない場合もあるが、その場合も血管炎の症状+ベーチェット病の症状がないか確認すればよい。
  1. Single organ vasculitisは単一臓器の血管炎であり、血管炎が証明された上で全身性血管炎が否定的な場合に考えればよい。
  1. Vasculitis associated with systemic diseaseとVasculitis associated with probable etiologyについては二次性血管炎であり、血管炎が疑われた場合にそれぞれsystemic diseaseもしくはprobable etiologyがないかを確認するとよい。
  1. 実臨床ではそれぞれの診断基準をどれも満たさない血管炎も存在し、その場合には分類不能とするしかない場合もある。
 
  1. ANCAはANCA関連血管炎の診断に重要であるがその感度特異度は100%ではなく注意が必要である。(参考文献:[2]
  1. 例えば顕微鏡的多発血管炎においてp-ANCA60%、c-ANCA40%で陽性になるとの報告がある。つまりANCAのみで血管炎の診断はできないし、血管炎の分類をすることもできない。
問診・診察のポイント  
問診:
  1. 血管炎を鑑別に挙げるための症状として発熱、倦怠感、皮疹、関節痛、体重減少、末梢神経障害を示唆する感覚低下、運動麻痺、異常感覚などがあり、それらの症状を確認する。

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文献 

著者: J C Jennette, R J Falk, P A Bacon, N Basu, M C Cid, F Ferrario, L F Flores-Suarez, W L Gross, L Guillevin, E C Hagen, G S Hoffman, D R Jayne, C G M Kallenberg, P Lamprecht, C A Langford, R A Luqmani, A D Mahr, E L Matteson, P A Merkel, S Ozen, C D Pusey, N Rasmussen, A J Rees, D G I Scott, U Specks, J H Stone, K Takahashi, R A Watts
雑誌名: Arthritis Rheum. 2013 Jan;65(1):1-11. doi: 10.1002/art.37715.
Abstract/Text
PMID 23045170  Arthritis Rheum. 2013 Jan;65(1):1-11. doi: 10.1002/art.・・・
著者: C Geffriaud-Ricouard, L H Noël, D Chauveau, S Houhou, J P Grünfeld, P Lesavre
雑誌名: Clin Nephrol. 1993 Mar;39(3):125-36.
Abstract/Text UNLABELLED: From 1987 to 1991, 2500 sera were tested for presence of anti-neutrophil cytoplasmic antibodies (ANCA) by standard indirect immunofluorescence (IIF) and specific proteinase 3 (PR3) and myeloperoxydase (MPO) ELISA. Clinical and histological data leading to precise diagnosis were retrospectively obtained in 98 patients with ANCA positivity by IIF and then a comparative study based on ANCA specificity was performed. Vasculitis was present in all cases. Among patients with anti-PR3 (n = 38), 19 had Wegener's granulomatosis (WG), 15 microscopic polyarteritis (mPA), 2 idiopathic necrotizing and crescentic glomerulonephritis (NCGN) and 2 relapsing polychondritis (RP). Among patients with anti-MPO (n = 45), 26 had mPA, 3 classical polyarteritis nodosa (PAN), 5 WG, 8 NCGN, 2 systemic lupus erythematosus (SLE) and one Churg-Strauss syndrome (CSS). Negative MPO and PR3 specific ELISA despite positive IIF were observed in 15 patients (13 WG, 1 mPA, 1 PAN). In the PR3 group, males predominated (66%) and the mean age was 49 years (range 13-85); in the MPO group, females predominated (62%) and the mean age was 57 years (range 13-85). These differences were statistically significant (p < 0.05). Renal involvement was present in 92% of patients and renal biopsy showed pauci-immune necrotizing and crescentic glomerulonephritis in nearly all cases. PR3 specificity was associated with frequent eye involvement (32%) and presence of granulomas (45%), but was not associated with other autoantibodies. MPO specificity was associated with a higher prevalence of pulmonary hemorrhage (40%) and various autoimmune disorders, especially antinuclear antibodies. Cholestasis was observed in 50% of WG with negative MPO and PR3 ELISA. Renal and patient survival at the 75th percentile was 15 months with MPO-ANCA and 16 months with PR3, and was similar for patients with WG and mPA. Relapses occurred in 20% of patients with anti-MPO and 36% of patients with anti-PR3. Serological follow-up was obtained in 44 patients. With immunosuppressive treatment, ANCA disappeared in 66% of cases and this disappearance was always associated with absence of disease activity.
IN CONCLUSION: 1. This study confirms that the presence of ANCA is a good marker of vasculitis. 2. Despite some clinical differences, MPO and PR3-associated vasculitis have a similar prognosis. 3. The titer of ANCA determined by ELISA is not correlated with the severity of vasculitis but disappearance of ANCA is always associated with absence of disease activity.

PMID 7681736  Clin Nephrol. 1993 Mar;39(3):125-36.
著者: Laura B Hughes, S Louis Bridges
雑誌名: Curr Rheumatol Rep. 2002 Feb;4(1):75-82.
Abstract/Text Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis of medium-sized vessels with clinical manifestations resulting from ischemia and infarction of affected tissues and organs. Although the cause of most cases of PAN and the related disorder microscopic polyangiitis (MPA) remains largely unknown, there has been significant progress in understanding the pathogenesis of vascular inflammation. The diagnostic approach to PAN and MPA should be individualized and based on specific organ involvement. Because no test or clinical finding reliably indicates the presence or absence of PAN or MPA, diagnosis requires integration of clinical findings, angiography, and biopsy data.

PMID 11798986  Curr Rheumatol Rep. 2002 Feb;4(1):75-82.
著者: A Nover, J Schmitt, S Wende, A Aulich
雑誌名: Klin Monbl Augenheilkd. 1976 Apr;168(4):461-7.
Abstract/Text The computer-aided tomography system (EMI-Scan) represents a new X-ray method in neuroradiology. Some cases of various space occupying retrobulbar orbit processes are presented, and it is demonstrated that this method is also well suited to the diagnosis of orbit diseases. Physicotechnical basis, technique of examination, the indication for computer tomography and the diagnostic possibilities are described.

PMID 966584  Klin Monbl Augenheilkd. 1976 Apr;168(4):461-7.
著者: Dong-sheng Wang, Yu-jun Zhuang, Hong-hui Sun
雑誌名: Zhonghua Shao Shang Za Zhi. 2007 Feb;23(1):52-4.
Abstract/Text OBJECTIVE: To evaluate the clinical effect of external tissue expansion in the repair of massive skin and soft tissue defects.
METHODS: From August 1998 to January 2004, 10 patients with massive skin and soft tissue defects ( the area ranging from 10 cm x 4 cm to 24 cm x 15 cm) , including 7 with wounds in the leg, 2 with wounds in knee region, and 1 with wounds in the forearm, were enrolled in the study. All patients were subjected to external tissue expansion together with external skeletal fixation for 2 -3 weeks, then the wounds were closed with suturing or supplemented with skin flaps.
RESULTS: The defects were closed completely after external expansion in 4 cases, and in other 5 cases the wounds were significantly decreased in area, and the residual wounds were covered with free skin grafting. In I case the wound could only be reduced in size, and the residual wound was closed with a local flap. Follow-up from 1 to 12 months showed that the wounds were closed with normal cutaneous sensation and good appearance.
CONCLUSION: External skin expansion is a simple, economical method for repair of massive skin soft-tissue defect, which can significantly be reduced or entirely closed.

PMID 17605257  Zhonghua Shao Shang Za Zhi. 2007 Feb;23(1):52-4.
著者: Matthias Alexander Hellmann, Thomas Michael Gerhardt, Christian Rabe, Susanne Haas, Tilman Sauerbruch, Rainer Peter Woitas
雑誌名: Nephrol Dial Transplant. 2006 Feb;21(2):526-9. doi: 10.1093/ndt/gfi279. Epub 2005 Nov 22.
Abstract/Text
PMID 16303779  Nephrol Dial Transplant. 2006 Feb;21(2):526-9. doi: 10.・・・
著者: Franco Dammacco, Stefano Battaglia, Loreto Gesualdo, Vito Racanelli
雑誌名: Autoimmun Rev. 2013 Sep;12(11):1101-8. doi: 10.1016/j.autrev.2013.06.014. Epub 2013 Jun 24.
Abstract/Text This review is based on our experience with ten patients diagnosed with Goodpasture's disease (GD). Six of the patients presented with combined renal and pulmonary insufficiencies; in the remaining four patients the clinical findings were limited to renal involvement. Circulating anti-glomerular basement membrane (GBM) autoantibodies were detected at diagnosis in all patients. Two patients were double-positive for anti-GBM and anti-proteinase-3 neutrophil cytoplasmic antibodies (c-ANCA). Another patient was double positive for anti-GBM and anti-myeloperoxidase cytoplasmic antibodies (p-ANCA). Four patients with rapidly progressive glomerulonephritis underwent hemodialysis: two of these patients died 6 and 8months after diagnosis, and the other two required maintenance dialysis. The remaining six patients were administered variable combinations of plasma-exchange, corticosteroids, and immunosuppressive drugs, which resulted in a remarkable and progressive improvement in renal function and one-year renal survival in all of them. Building on these observations, we provide an update on this relatively rare, frequently severe, and sometimes lethal autoimmune disease of unknown etiology. GD patients typically present with rapidly progressive renal insufficiency and pulmonary hemorrhage. Involvement restricted to the kidneys alone, as in our series, is also seen. The unfailing immunological hallmark of the disease is the occurrence of circulating anti-GBM antibodies, whose titer is directly related to the clinical severity of GD. The antibodies are associated with serum ANCAs in 10% to almost 40% of GD patients, with double positivity indicative of a worse renal prognosis. The target antigen of anti-GBM antibodies is a component of the non-collagenous-1 (NC1) domain of the α3 chain of type IV collagen, α345NC1. The prevalent expression of this hexamer on the basement membrane of both the glomeruli and the pulmonary alveoli accounts for the frequently combined renal and pulmonary involvement. A strong positive association of GD with the HLA-DRB1*15:01 allele has been described, but the factor(s) responsible for the loss of self-tolerance to NC1 autoantigen has not yet been identified. A conformational change in the quaternary structure of the α345NC1 likely plays a crucial role in triggering an immune response and justifies the proposed description of GD as an autoimmune "conformeropathy." The function of autoreactive T-cells in GD is poorly defined but may involve a shift from TH2 to TH1 cytokine regulation, such that affinity maturation and the antigen specificity of the antibody response are enhanced. The timely diagnosis of GD and the adoption of a triple therapeutic regimen comprising plasmapheresis, corticosteroids, and immunosuppressive drugs have remarkably improved the previously dismal outcome of these patients, resulting in a one-year survival rate of 70-90%.

Copyright © 2013 Elsevier B.V. All rights reserved.
PMID 23806563  Autoimmun Rev. 2013 Sep;12(11):1101-8. doi: 10.1016/j.a・・・
著者: Alan D Salama, Tammy Dougan, Jeremy B Levy, H Terry Cook, Steve H Morgan, Sarah Naudeer, Geoff Maidment, Andrew J T George, David Evans, Liz Lightstone, Charles D Pusey
雑誌名: Am J Kidney Dis. 2002 Jun;39(6):1162-7. doi: 10.1053/ajkd.2002.33385.
Abstract/Text Goodpasture's disease is characterized by rapidly progressive glomerulonephritis, often accompanied by pulmonary hemorrhage, in association with deposition of antibodies in a linear pattern on the glomerular basement membrane (GBM). The diagnosis of Goodpasture's disease in patients with acute renal failure often relies on the use of immunoassays to detect circulating anti-GBM antibodies in serum samples. We describe three cases of Goodpasture's disease in which no circulating anti-GBM antibodies were detectable in serum by well-established enzyme-linked immunosorbent assay or Western blotting techniques. The diagnosis of Goodpasture's disease was confirmed by renal biopsy, with linear deposition of immunoglobulin along the GBM and crescentic glomerulonephritis. In addition, an alternative method of antibody detection using a highly sensitive biosensor system confirmed that circulating antibodies were present in sera from both patients tested. Because this technique is not routinely available for the detection of anti-GBM antibodies, we suggest that diagnosis always be confirmed with a renal biopsy, and despite negative serological test results using immunoassay, the diagnosis of Goodpasture's disease should still be considered in the correct clinical context.

Copyright 2002 by the National Kidney Foundation, Inc.
PMID 12046026  Am J Kidney Dis. 2002 Jun;39(6):1162-7. doi: 10.1053/aj・・・
著者: J C Brouet, J P Clauvel, F Danon, M Klein, M Seligmann
雑誌名: Am J Med. 1974 Nov;57(5):775-88.
Abstract/Text
PMID 4216269  Am J Med. 1974 Nov;57(5):775-88.
著者: I Goldsmith, G Y Lip, R L Patel
雑誌名: Am J Cardiol. 1999 Apr 1;83(7):1069-74.
Abstract/Text The Sorin bicarbon bileaflet prosthesis was introduced in 1990. To evaluate the clinical performance of this prosthesis, we reviewed 519 prostheses that were implanted in 488 patients (275 men, 213 women; mean age 59 years; SD 10.8, range 19 to 88) from 1993 to 1997. Preoperatively, 82% of patients were in New York Heart Association (NYHA) functional class III or IV. There were 263 aortic valve replacements (AVRs) (54%), 194 mitral valve replacements (MVRs) (40%), and 31 AVRs and MVRs (both) (6%). Concomitant procedures were performed in 82 patients (17%). Follow-up was complete in 471 (97%) with a total cumulative follow-up of 866 patient-years. The 30-day mortality for patients with AVR was 5.7% (95% confidence interval [CI] 2.9 to 8.5), MVR 17.5% (CI 9.9 to 19.7), and both 19% (CI 7.6 to 51.1), with no early valve-related deaths. Patient survival at 55 months was 76% (SE 2.27%), with patients with AVR being 90%, MVR 63%, and both 61%. This was influenced by the following: (1) valve position, which was higher for MVR (p = 0.0001); (2) poor NYHA functional class (p = 0.0006); (3) reoperation (p = 0.02); and (4) age >70 years (p = 0.0001). Valve-related complications (expressed as percentage per patient year and number of events) were major thromboembolism at 0.9% per year (8), with AVR rates being 1.2% per year (6) and MVR 0.7% per year (2); major hemorrhage at 2.3% per year (20) with AVR rates being 2.4% per year (12) and MVR 2.5% per year (7); bacterial endocarditis at 0.2% per year (2); and nonstructural dysfunction rate of 0.7% per year (6). The reoperation rate was 0.9% per year (8) with AVR being 0.6% per year (3) and MVR 1.7% per year (5). At 55 months, actuarial freedom from major thromboembolism was 97% (SE 1.1%) with AVR being 96% and MVR 98%; major hemorrhage 89% (SE 3.1%) with AVR being 88.6% and MVR 91%; structural valve dysfunction 100% (SE 0.0%); and reoperation 97.1% (SE 1.10%) with AVR being 98.5% and MVR 94.6%. At follow-up, 88% of survivors were in NYHA class I or II. In this series, hospital mortality and overall survival in patients were influenced by the patients' clinical characteristics. There were no early valve-related deaths. Valve-related complications were similar to previously reported series with no episode of structural failure. Our experience with the Sorin bicarbon bileaflet prosthesis suggests that it has a satisfactory clinical performance, with low complication rates.

PMID 10190522  Am J Cardiol. 1999 Apr 1;83(7):1069-74.
著者: Carlo Salvarani, Robert D Brown, Kenneth T Calamia, Teresa J H Christianson, Stephen D Weigand, Dylan V Miller, Caterina Giannini, James F Meschia, John Huston, Gene G Hunder
雑誌名: Ann Neurol. 2007 Nov;62(5):442-51. doi: 10.1002/ana.21226.
Abstract/Text OBJECTIVE: To analyze the clinical findings, response to therapy, outcome, and incidence of primary central nervous system vasculitis (PCNSV) in a large cohort from a single center.
METHODS: We retrospectively studied 101 patients with PCNSV, selected by predetermined diagnostic criteria, who were seen during a 21-year period. This was a collaborative study by five departments at a large multispecialty clinic. Clinical findings and outcomes were compared among patients categorized by method of diagnosis, response to therapy, survival, and degree of disability. An annual incidence rate was calculated.
RESULTS: Seventy patients were diagnosed by angiography and 31 by central nervous system biopsy. Three histological patterns were observed during biopsy. Although most patients responded to therapy, an increased mortality rate was observed. Relapses occurred in one fourth of patients. Mortality rate and disability at last follow-up were greater in those who presented with a focal neurological deficit, cognitive impairment, cerebral infarctions, and angiographic large-vessel involvement but were lower in those with prominent gadolinium-enhanced lesions when evaluated by magnetic resonance imaging. The annual incidence rate of PCNSV was 2.4 cases per 1,000,000 person-years.
INTERPRETATION: PCNSV is a rare disease that may result in serious neurological outcomes or death. Angiography and brain biopsy may complement each other when determining the diagnosis. Early recognition and treatment may reduce poor outcomes. PCNSV is a variable syndrome that appears to consist of several subsets of heterogeneous diseases.

PMID 17924545  Ann Neurol. 2007 Nov;62(5):442-51. doi: 10.1002/ana.212・・・
著者: L Barile-Fabris, M F Hernández-Cabrera, J A Barragan-Garfias
雑誌名: Curr Rheumatol Rep. 2014;16(9):440. doi: 10.1007/s11926-014-0440-9.
Abstract/Text Systemic lupus erythematosus (SLE) is a complex heterogeneous autoimmune disease with a wide variety of clinical and serological manifestations that may affect any organ. Vasculitis prevalence in SLE is reported to be between 11% and 36%. A diverse clinical spectrum, due to inflammatory involvement of vessels of all sizes, is present. Even though cutaneous lesions, representing small vessel involvement, are the most frequent, medium and large vessel vasculitis may present with visceral affection, with life-threatening manifestations such as mesenteric vasculitis, pulmonary hemorrhage, or mononeuritis multiplex, with detrimental consequences. Early recognition and an appropriate treatment are crucial. Recent studies have shown that vasculitis in patients with SLE may present different clinical forms based on the organ involved and the size of the affected vessel. It is noteworthy that the episodes of vasculitis are not always accompanied by high disease activity. Recent articles on this topic have focused on new treatments for the control of vascular disease, such as biological therapies such as Rituximab and Belimumab, among others.

PMID 25023725  Curr Rheumatol Rep. 2014;16(9):440. doi: 10.1007/s11926・・・
著者: Ashima Makol, Cynthia S Crowson, David A Wetter, Olayemi Sokumbi, Eric L Matteson, Kenneth J Warrington
雑誌名: Rheumatology (Oxford). 2014 May;53(5):890-9. doi: 10.1093/rheumatology/ket475. Epub 2014 Jan 17.
Abstract/Text OBJECTIVE: The aim of this study was to determine the clinical correlates and predictors of rheumatoid vasculitis (RV).
METHODS: A retrospective cohort of patients with RV evaluated at a tertiary referral centre between 1 January 2000 and 1 January 2010 was identified. RV cases were compared in a 1:2 ratio to controls (RA without vasculitis) to identify risk factors for developing RV.
RESULTS: Eighty-six RV cases (58% women, 88% white) were identified. Histopathological confirmation was available for 58% of patients. Cutaneous vasculitis was the most common presentation, followed by vasculitic neuropathy. The median age at presentation was 63 years and the median duration of RA was 10.8 years. One third were current smokers. The majority were seropositive and had elevated inflammatory markers. Treatment was with a range of immunomodulating agents. At 6 months, 38% of patients achieved complete remission, 52% had partial improvement and 10% noted no clinical improvement. Thirty-six per cent relapsed by 5 years and 26% died. After adjusting for age and disease duration, current smoking at RA diagnosis [odds ratio (OR) 1.98], coexistent peripheral vascular disease (OR 3.98), cerebrovascular disease (OR 6.48), severe RA (OR 2.02) (characterized by radiographic erosions, nodulosis on clinical examination or requirement of joint surgery) and the use of biologics (OR 2.80) were found to increase the odds for developing RV; the use of HCQ (OR 0.54, CI 0.31, 0.94) and low-dose aspirin (OR 0.42, CI 0.21, 0.85) was associated with decreased odds for developing RV.
CONCLUSION: This largest single-centre series of patients with RV suggests that even in recent years, RV remains a serious complication of RA and is associated with significant mortality.

PMID 24441152  Rheumatology (Oxford). 2014 May;53(5):890-9. doi: 10.10・・・
著者: M Radić, D Martinović Kaliterna, J Radić
雑誌名: Neth J Med. 2012 Jan;70(1):12-7.
Abstract/Text Drug-induced vasculitis is an inflammation of blood vessels caused by the use of various pharmaceutical agents. Vasculitis causes changes in the walls of blood vessels, including thickening, weakening, narrowing and scarring. Inflammation can be short-term (acute) or long-term (chronic) and can be so severe that the tissues and organs supplied by the affected vessels do not get enough blood. The shortage of blood can result in organ and tissue damage, even death. Drug-induced vasculitis is the most common form of vasculitis. The differential diagnosis between drug-induced and idiopathic vasculitic conditions may be difficult in the individual patient. Withdrawal may be helpful to distinguish between these syndromes. Withdrawal of the offending agent alone is often sufficient to induce prompt resolution of clinical manifestations, obviating the need for immunosuppressive and anti-inflammatory drugs. Increasing understanding of the pathophysiological characteristics of all inflammatory vasculitides should lead to better diagnostic and therapeutic approaches to drug-induced vasculitis.

PMID 22271809  Neth J Med. 2012 Jan;70(1):12-7.
著者: Olivier Fain, Mohamed Hamidou, Patrice Cacoub, Bertrand Godeau, Bertrand Wechsler, Jacques Pariès, Jérôme Stirnemann, Anne-Sophie Morin, Marc Gatfosse, Thomas Hanslik, Nadia Belmatoug, Olivier Blétry, Ramiro Cevallos, Isabelle Delevaux, Evelyne Fisher, Gilles Hayem, Gérard Kaplan, Claire Le Hello, Luc Mouthon, Claire Larroche, Véra Lemaire, Anne-Marie Piette, Jean-Charles Piette, Thierry Ponge, Xavier Puechal, Jérôme Rossert, Françoise Sarrot-Reynauld, Didier Sicard, Jean-Marc Ziza, Marcel-Francis Kahn, Loïc Guillevin
雑誌名: Arthritis Rheum. 2007 Dec 15;57(8):1473-80. doi: 10.1002/art.23085.
Abstract/Text OBJECTIVE: To describe characteristics and outcomes of vasculitides associated with malignancies.
METHODS: The requirement for inclusion in this retrospective, 10-year study was development of vasculitis in patients with a progressing malignancy. Malignancies secondary to immunosuppressants used to treat vasculitis were excluded. The main characteristics of vasculitides were analyzed and compared according to the type of malignancy.
RESULTS: Sixty patients were included (male/female sex ratio 2.53, mean age 62.4 years). Mean followup duration was 45.2 months. Vasculitides were cutaneous leukocytoclastic (45%), polyarteritis nodosa (36.7%), Wegener's granulomatosis (6.7%), microscopic polyangiitis (5%), and Henoch-Schönlein purpura (5%). Malignancies were distributed as follows: hematologic in 63.1%, myelodysplastic syndrome (MDS) in 32.3%, lymphoid in 29.2%, and solid tumor in 36.9%. Vasculitides were diagnosed concurrently with malignancy in 38% of the cases. Manifestations of vasculitides were fever (41.7%), cutaneous involvement (78.3%), arthralgias (46.7%), peripheral neuropathy (31.7%), renal involvement (23.3%; 11.7% glomerulonephritis, 11.7% microaneurysms, 6.7% renal insufficiency), and antineutrophil cytoplasmic antibody (20.4%). Vasculitis treatments were corticosteroids (78.3%) and immunosuppressant(s) (41.7%). Vasculitis was cured in 65% of patients, but 58.3% died, with 1 death secondary to vasculitis. Independent of subtype, patients with vasculitides associated with MDS more frequently had renal manifestations (P = 0.02) and steroid dependence (P = 0.04) and achieved complete remission less often (P = 0.04) than patients with vasculitides associated with other malignancies. Patients with vasculitides associated with a solid tumor more frequently had peripheral neurologic involvement (P = 0.05). Patients with vasculitides associated with lymphoid malignancy had less frequent arthralgias (P = 0.01) and renal involvement (P = 0.02).
CONCLUSION: Vasculitides occurring during malignancies present distinctive features according to the vasculitis subtype and nature of the malignancy.

PMID 18050165  Arthritis Rheum. 2007 Dec 15;57(8):1473-80. doi: 10.100・・・
著者: I van der Sluis
雑誌名: Ned Tijdschr Geneeskd. 1975 Jun 14;119(24):975.
Abstract/Text
PMID 1161081  Ned Tijdschr Geneeskd. 1975 Jun 14;119(24):975.

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