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SLEの病態別治療アルゴリズム(わが国のSLE診療ガイドライン2019より)

まずSLEを診断し、さらに疾患活動性を評価して治療を選択する。ヒドロキシクロロキンは臓器病変、病態を問わず全例で投与を考慮する。
さらに臓器病変に応じて治療を選択し、寛解導入を目指す。ループス腎炎では生検病理所見に応じてグルココルチコイド大量投与に加えてミコフェノール酸モフェチル(MMF)またはシクロホスファミド大量静注(IVCY)併用を考慮する。精神神経ループス(NPSLE)ではグルココルチコイド大量投与にIVCYを併用し、難治例ではリツキシマブを考慮する。その他の臓器病変については、主体となる病態に応じた治療法を考慮する。
寛解導入が得られればグルココルチコイドを減量し、適切な免疫抑制薬併用により、寛解維持療法に移行する。初期療法で寛解が得られない場合は、3rd line治療法として血漿交換療法、リツキシマブ、既存の免疫抑制薬の併用や変更などを考慮するが、これらには十分なエビデンスがない。
出典
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1: 厚生労働科学研究費補助金難治性疾患等政策研究事業 自己免疫疾患に関する調査研究(自己免疫班)/日本リウマチ学会 編:全身性エリテマトーデス診療ガイドライン2019.南山堂、2019

1997年版ACR改訂分類基準

SLEの分類基準は米国リウマチ学会(ACR)が1971年に予備基準を発表し、1982年に改訂、1994年に再改訂された。この分類基準の感度と特異度はいずれも約90%である。分類基準は臨床研究のために策定され、個々の症例の診断を目的としてはいないが、限界(感度と特異度)を理解したうえで診断に用いることには問題はない。
出典
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1: Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus.
Arthritis Rheum. 1997 Sep;40(9):1725. doi: 10.1002/art.1780400928.

米国リウマチ学会によるSLEの精神神経症状の分類

SLEにおける中枢神経症状は重大なSLEの難治性臓器病変であり、多彩な精神神経症状がみられるが、特に精神症状とけいれんは頻度が高い。1999年に米国リウマチ学会はSLEの神経症状(中枢神経症状および末梢神経症状)の分類基準を提唱し、NPSLE(neuropsychiatric SLE)と総称される。
出典
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1: The American College of Rheumatology nomenclature and case definitions for neuropsychiatric lupus syndromes.
著者: .
雑誌名: Arthritis Rheum. 1999 Apr;42(4):599-608. doi: 10.1002/1529-0131(199904)42:4<599::AID-ANR2>3.0.CO;2-F.
Abstract/Text: OBJECTIVE: To develop a standardized nomenclature system for the neuropsychiatric syndromes of systemic lupus erythematosus (NPSLE).
METHODS: An international, multidisciplinary committee representing rheumatology, neurology, psychiatry, neuropsychology, and hematology developed case definitions, reporting standards, and diagnostic testing recommendations. Before and after the meeting, clinician committee members assigned diagnoses to sets of vignettes randomly generated from a pool of 108 NPSLE patients. To assess whether the nomenclature system improved diagnostic agreement, a consensus index was developed and pre- and postmeeting scores were compared by t-tests.
RESULTS: Case definitions including diagnostic criteria, important exclusions, and methods of ascertainment were developed for 19 NPSLE syndromes. Recommendations for standard reporting requirements, minimum laboratory evaluation, and imaging techniques were formulated. A short neuropsychological test battery for the diagnosis of cognitive deficits was proposed. In the postmeeting exercise, a statistically significant improvement in diagnostic agreement was observed.
CONCLUSION: The American College of Rheumatology (ACR) Nomenclature for NPSLE provides case definitions for 19 neuropsychiatric syndromes seen in SLE, with reporting standards and recommendations for laboratory and imaging tests. It is intended to facilitate and enhance clinical research, particularly multicenter studies, and reporting. In clinical settings, consultation with other specialists may be required. It should be useful for didactic purposes but should not be used uncritically or as a substitute for a clinical diagnosis. The complete case definitions are available on the ACR World Wide Web site: http://www.rheumatology .org/ar/ar.html.
Arthritis Rheum. 1999 Apr;42(4):599-608. doi: 10.1002/1529-0131(199904...

ループス腎炎の組織学的分類(ISN/RPS分類、2004年)

SLEでは約80%に腎病変が認められ、そのうち25~30%が難治性で、10%程度が腎不全に移行する。ループス腎炎の組織学的分類は予後と治療反応性を反映し、治療方針決定に重要である。ループス腎炎の組織学的分類は2004年に国際腎臓病学会(ISN)と国際病理学会(RPS)により改訂された。IV型が最も腎不全に移行しやすく、III型もそれに次いで予後が悪い。慢性病変(C)があるとグルココルチコイドによる治療の効果が期待できない。
出典
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1: The classification of glomerulonephritis in systemic lupus erythematosus revisited.
著者: Weening JJ, D'Agati VD, Schwartz MM, Seshan SV, Alpers CE, Appel GB, Balow JE, Bruijn JA, Cook T, Ferrario F, Fogo AB, Ginzler EM, Hebert L, Hill G, Hill P, Jennette JC, Kong NC, Lesavre P, Lockshin M, Looi LM, Makino H, Moura LA, Nagata M.
雑誌名: J Am Soc Nephrol. 2004 Feb;15(2):241-50. doi: 10.1097/01.asn.0000108969.21691.5d.
Abstract/Text: The currently used classification reflects our understanding of the pathogenesis of the various forms of lupus nephritis, but clinicopathologic studies have revealed the need for improved categorization and terminology. Based on the 1982 classification published under the auspices of the World Health Organization (WHO) and subsequent clinicopathologic data, we propose that class I and II be used for purely mesangial involvement (I, mesangial immune deposits without mesangial hypercellularity; II, mesangial immune deposits with mesangial hypercellularity); class III for focal glomerulonephritis (involving <50% of total number of glomeruli) with subdivisions for active and sclerotic lesions; class IV for diffuse glomerulonephritis (involving > or = 50% of total number of glomeruli) either with segmental (class IV-S) or global (class IV-G) involvement, and also with subdivisions for active and sclerotic lesions; class V for membranous lupus nephritis; and class VI for advanced sclerosing lesions]. Combinations of membranous and proliferative glomerulonephritis (i.e., class III and V or class IV and V) should be reported individually in the diagnostic line. The diagnosis should also include entries for any concomitant vascular or tubulointerstitial lesions. One of the main advantages of the current revised classification is that it provides a clear and unequivocal description of the various lesions and classes of lupus nephritis, allowing a better standardization and lending a basis for further clinicopathologic studies. We hope that this revision, which evolved under the auspices of the International Society of Nephrology and the Renal Pathology Society, will contribute to further advancement of the WHO classification.
J Am Soc Nephrol. 2004 Feb;15(2):241-50. doi: 10.1097/01.asn.000010896...

頬部紅斑(蝶型紅斑)

両頬部から鼻梁にかけて盛り上がりのある紅斑を認める。紅斑は鼻唇溝を越えない。
出典
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1: 著者提供

ディスコイド疹(円板状ループス)

境界が比較的明瞭な紅斑で、中心部の皮膚萎縮と落屑形成を認める。
出典
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1: 著者提供

口腔粘膜潰瘍

硬口蓋に形成された口腔潰瘍。無痛性が特徴である。
出典
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1: 著者提供

SLEに見いだされる自己抗体と関連する病態

SLEをはじめとする膠原病では、自己の細胞核・細胞質成分に対する多種類の自己抗体が検出され、疾患や病態と密接に関連する。SLEの疾患特異的自己抗体には抗dsDNA抗体と抗Sm抗体があり、陽性であればSLEの可能性は高い。ほかにSLEでの頻度は高いが特異性の低い自己抗体があるが、これらは特定の症状や病態と関連するものが多い。SLEが疑われる場合にはこれらの自己抗体の測定が強く勧められる。
出典
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1: 三森経世:全身性エリテマトーデス.高久史麿ほか監修.新臨床内科学第9版:医学書院,2009;1442. (改変あり)

SLE新分類基準(Systemic Lupus International Collaborating Clinics: SLICC)(2012)

ACR分類基準の感度の低さを改善する目的で策定された第2の分類基準である。高感度の反面、特異度に劣るとされる。
出典
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1: Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus.
著者: Petri M, Orbai AM, Alarcón GS, Gordon C, Merrill JT, Fortin PR, Bruce IN, Isenberg D, Wallace DJ, Nived O, Sturfelt G, Ramsey-Goldman R, Bae SC, Hanly JG, Sánchez-Guerrero J, Clarke A, Aranow C, Manzi S, Urowitz M, Gladman D, Kalunian K, Costner M, Werth VP, Zoma A, Bernatsky S, Ruiz-Irastorza G, Khamashta MA, Jacobsen S, Buyon JP, Maddison P, Dooley MA, van Vollenhoven RF, Ginzler E, Stoll T, Peschken C, Jorizzo JL, Callen JP, Lim SS, Fessler BJ, Inanc M, Kamen DL, Rahman A, Steinsson K, Franks AG Jr, Sigler L, Hameed S, Fang H, Pham N, Brey R, Weisman MH, McGwin G Jr, Magder LS.
雑誌名: Arthritis Rheum. 2012 Aug;64(8):2677-86. doi: 10.1002/art.34473.
Abstract/Text: OBJECTIVE: The Systemic Lupus International Collaborating Clinics (SLICC) group revised and validated the American College of Rheumatology (ACR) systemic lupus erythematosus (SLE) classification criteria in order to improve clinical relevance, meet stringent methodology requirements, and incorporate new knowledge regarding the immunology of SLE.
METHODS: The classification criteria were derived from a set of 702 expert-rated patient scenarios. Recursive partitioning was used to derive an initial rule that was simplified and refined based on SLICC physician consensus. The SLICC group validated the classification criteria in a new validation sample of 690 new expert-rated patient scenarios.
RESULTS: Seventeen criteria were identified. In the derivation set, the SLICC classification criteria resulted in fewer misclassifications compared with the current ACR classification criteria (49 versus 70; P = 0.0082) and had greater sensitivity (94% versus 86%; P < 0.0001) and equal specificity (92% versus 93%; P = 0.39). In the validation set, the SLICC classification criteria resulted in fewer misclassifications compared with the current ACR classification criteria (62 versus 74; P = 0.24) and had greater sensitivity (97% versus 83%; P < 0.0001) but lower specificity (84% versus 96%; P < 0.0001).
CONCLUSION: The new SLICC classification criteria performed well in a large set of patient scenarios rated by experts. According to the SLICC rule for the classification of SLE, the patient must satisfy at least 4 criteria, including at least one clinical criterion and one immunologic criterion OR the patient must have biopsy-proven lupus nephritis in the presence of antinuclear antibodies or anti-double-stranded DNA antibodies.

Copyright © 2012 by the American College of Rheumatology.
Arthritis Rheum. 2012 Aug;64(8):2677-86. doi: 10.1002/art.34473.

EULAR/ACRによるSLE分類基準(2019)

抗核抗体が80倍以上で他疾患では説明できない臨床症状が少なくとも1項目以上ある症例を対象とする。臨床所見7領域と免疫学的検査3領域の計10領域から構成され、各症状・所見に重みづけがなされ、スコアの合計が10点以上の場合にSLEと分類する。
出典
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1: 2019 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Systemic Lupus Erythematosus.
著者: Aringer M, Costenbader K, Daikh D, Brinks R, Mosca M, Ramsey-Goldman R, Smolen JS, Wofsy D, Boumpas DT, Kamen DL, Jayne D, Cervera R, Costedoat-Chalumeau N, Diamond B, Gladman DD, Hahn B, Hiepe F, Jacobsen S, Khanna D, Lerstrøm K, Massarotti E, McCune J, Ruiz-Irastorza G, Sanchez-Guerrero J, Schneider M, Urowitz M, Bertsias G, Hoyer BF, Leuchten N, Tani C, Tedeschi SK, Touma Z, Schmajuk G, Anic B, Assan F, Chan TM, Clarke AE, Crow MK, Czirják L, Doria A, Graninger W, Halda-Kiss B, Hasni S, Izmirly PM, Jung M, Kumánovics G, Mariette X, Padjen I, Pego-Reigosa JM, Romero-Diaz J, Rúa-Figueroa Fernández Í, Seror R, Stummvoll GH, Tanaka Y, Tektonidou MG, Vasconcelos C, Vital EM, Wallace DJ, Yavuz S, Meroni PL, Fritzler MJ, Naden R, Dörner T, Johnson SR.
雑誌名: Arthritis Rheumatol. 2019 Sep;71(9):1400-1412. doi: 10.1002/art.40930. Epub 2019 Aug 6.
Abstract/Text: OBJECTIVE: To develop new classification criteria for systemic lupus erythematosus (SLE) jointly supported by the European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR).
METHODS: This international initiative had four phases. 1) Evaluation of antinuclear antibody (ANA) as an entry criterion through systematic review and meta-regression of the literature and criteria generation through an international Delphi exercise, an early patient cohort, and a patient survey. 2) Criteria reduction by Delphi and nominal group technique exercises. 3) Criteria definition and weighting based on criterion performance and on results of a multi-criteria decision analysis. 4) Refinement of weights and threshold scores in a new derivation cohort of 1,001 subjects and validation compared with previous criteria in a new validation cohort of 1,270 subjects.
RESULTS: The 2019 EULAR/ACR classification criteria for SLE include positive ANA at least once as obligatory entry criterion; followed by additive weighted criteria grouped in 7 clinical (constitutional, hematologic, neuropsychiatric, mucocutaneous, serosal, musculoskeletal, renal) and 3 immunologic (antiphospholipid antibodies, complement proteins, SLE-specific antibodies) domains, and weighted from 2 to 10. Patients accumulating ≥10 points are classified. In the validation cohort, the new criteria had a sensitivity of 96.1% and specificity of 93.4%, compared with 82.8% sensitivity and 93.4% specificity of the ACR 1997 and 96.7% sensitivity and 83.7% specificity of the Systemic Lupus International Collaborating Clinics 2012 criteria.
CONCLUSION: These new classification criteria were developed using rigorous methodology with multidisciplinary and international input, and have excellent sensitivity and specificity. Use of ANA entry criterion, hierarchically clustered, and weighted criteria reflects current thinking about SLE and provides an improved foundation for SLE research.

© 2019, American College of Rheumatology.
Arthritis Rheumatol. 2019 Sep;71(9):1400-1412. doi: 10.1002/art.40930....

非腎性SLEの治療

上から下への配列は、好ましい順序を意味するものではない(例えば、MTX、AZAおよびMMFは、軽症における2次治療または中等症における第1選択治療で同等の選択肢となる)。
*軽症:体質的な症状;軽度の関節炎;発疹≤体表面積(BSA)の9%;血小板数(PLT)50~100×109 /L; SLEDAI≤6; BILAG Cまたは ≤1 BILAG Bの症状。
*中等症:中等度から重度の関節炎(「RA様」);発疹9%~18%BSA;PLTは20~50×109 /L;漿膜炎;SLEDAI 7–12; ≥2 BILAG Bの症状。
*重症:主要な臓器を脅かす疾患(脳炎、脊髄炎、肺炎、腸間膜血管炎);PLT<20×109 /Lの血小板減少症;TTP様疾患または急性血球貪食症候群;発疹>18%BSA; スSLEDAI>12; ≥1 BILAG Aの症状。
†重症化に対するベリムマブおよびアニフロルマブの第1選択治療としての推奨は、主要な臓器病変ではないが、皮膚や関節などから広範な疾患を有する腎外SLEの症例を指す。重症疾患における追加療法としてのアニフロルマブの使用は、主に重度の皮膚疾患を指す。重度の精神神経疾患の患者には、アニフロルマブとベリムマブは推奨されない。
ANI:アニフロルマブ、aPL:抗リン脂質抗体、APS:抗リン脂質症候群、AZA:アザチオプリン、BEL:ベリムマブ、BILAG:イギリス諸島ループス評価グループ、CNI:カルシニューリン阻害薬、CYC:シクロホスファミド、GC:グルココルチコイド、HCQ:ヒドロキシクロロキン、IV:静脈内、MMF:ミコフェノール酸モフェチル、MTX:メトトレキサート、PO:経口、RTX:リツキシマブ、SLEDAI:SLE疾患活動性指数、VKA:ビタミンK拮抗薬。
筆者注:わが国ではMTXは保険適用外、MMFとRTXはループス腎炎のみに適用
出典
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1: EULAR recommendations for the management of systemic lupus erythematosus: 2023 update.
著者: Fanouriakis A, Kostopoulou M, Andersen J, Aringer M, Arnaud L, Bae SC, Boletis J, Bruce IN, Cervera R, Doria A, Dörner T, Furie RA, Gladman DD, Houssiau FA, Inês LS, Jayne D, Kouloumas M, Kovács L, Mok CC, Morand EF, Moroni G, Mosca M, Mucke J, Mukhtyar CB, Nagy G, Navarra S, Parodis I, Pego-Reigosa JM, Petri M, Pons-Estel BA, Schneider M, Smolen JS, Svenungsson E, Tanaka Y, Tektonidou MG, Teng YO, Tincani A, Vital EM, van Vollenhoven RF, Wincup C, Bertsias G, Boumpas DT.
雑誌名: Ann Rheum Dis. 2024 Jan 2;83(1):15-29. doi: 10.1136/ard-2023-224762. Epub 2024 Jan 2.
Abstract/Text: OBJECTIVES: To update the EULAR recommendations for the management of systemic lupus erythematosus (SLE) based on emerging new evidence.
METHODS: An international Task Force formed the questions for the systematic literature reviews (January 2018-December 2022), followed by formulation and finalisation of the statements after a series of meetings. A predefined voting process was applied to each overarching principle and recommendation. Levels of evidence and strengths of recommendation were assigned, and participants finally provided their level of agreement with each item.
RESULTS: The Task Force agreed on 5 overarching principles and 13 recommendations, concerning the use of hydroxychloroquine (HCQ), glucocorticoids (GC), immunosuppressive drugs (ISDs) (including methotrexate, mycophenolate, azathioprine, cyclophosphamide (CYC)), calcineurin inhibitors (CNIs, cyclosporine, tacrolimus, voclosporin) and biologics (belimumab, anifrolumab, rituximab). Advice is also provided on treatment strategies and targets of therapy, assessment of response, combination and sequential therapies, and tapering of therapy. HCQ is recommended for all patients with lupus at a target dose 5 mg/kg real body weight/day, considering the individual's risk for flares and retinal toxicity. GC are used as 'bridging therapy' during periods of disease activity; for maintenance treatment, they should be minimised to equal or less than 5 mg/day (prednisone equivalent) and, when possible, withdrawn. Prompt initiation of ISDs (methotrexate, azathioprine, mycophenolate) and/or biological agents (anifrolumab, belimumab) should be considered to control the disease and facilitate GC tapering/discontinuation. CYC and rituximab should be considered in organ-threatening and refractory disease, respectively. For active lupus nephritis, GC, mycophenolate or low-dose intravenous CYC are recommended as anchor drugs, and add-on therapy with belimumab or CNIs (voclosporin or tacrolimus) should be considered. Updated specific recommendations are also provided for cutaneous, neuropsychiatric and haematological disease, SLE-associated antiphospholipid syndrome, kidney protection, as well as preventative measures for infections, osteoporosis, cardiovascular disease.
CONCLUSION: The updated recommendations provide consensus guidance on the management of SLE, combining evidence and expert opinion.

© Author(s) (or their employer(s)) 2024. No commercial re-use. See rights and permissions. Published by BMJ.
Ann Rheum Dis. 2024 Jan 2;83(1):15-29. doi: 10.1136/ard-2023-224762. E...

ループス腎炎の治療

上から下への順序は、好ましい順序を意味するものではない([ID0701]と同様)。
[ID0701]で概説した一般的な保護対策に加えて、
§BELは、初期治療としてMMFまたは低用量CYCと組み合わせて、維持療法としてMMFまたはAZAと組み合わせて常に投与する必要がある。
^CNIはMMFと組み合わせて投与する必要がある。
*予後不良の要因がある場合に特に推奨される:eGFRの低下、細胞性半月体またはフィブリノイド壊死の組織学的存在、または重度の間質性炎症。
¶高用量CYCの後療法への延長は、重度のLN症例において、6カ月のパルス療法の後に隔月または四半期ごとのCYCパルスで行われる。
†再発性/難治性疾患、特にCYCベースのレジメンに失敗した後。
ACEi:アンジオテンシン変換酵素阻害薬、APS:抗リン脂質症候群、ARB:アンジオテンシン受容体拮抗薬、AZA:アザチオプリン、BEL:ベリムマブ、CNI:カルシニューリン阻害薬、CYC:シクロホスファミド、eGFR:推定糸球体濾過率、GC:グルココルトコイド、HCQ:ヒドロキシクロロキン、IV:静脈内、MMF:ミコフェノール酸モフェチル、MP:メチルプレドニゾロン、PO:経口、RTX:リツキシマブ、SGLT2i:ナトリウムグルコーストランスポーター2阻害薬、TAC:タクロリムス、UPR:尿タンパク質、VKA:ビタミンK拮抗薬、VOC:ボクロスポリン。
出典
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1: EULAR recommendations for the management of systemic lupus erythematosus: 2023 update.
著者: Fanouriakis A, Kostopoulou M, Andersen J, Aringer M, Arnaud L, Bae SC, Boletis J, Bruce IN, Cervera R, Doria A, Dörner T, Furie RA, Gladman DD, Houssiau FA, Inês LS, Jayne D, Kouloumas M, Kovács L, Mok CC, Morand EF, Moroni G, Mosca M, Mucke J, Mukhtyar CB, Nagy G, Navarra S, Parodis I, Pego-Reigosa JM, Petri M, Pons-Estel BA, Schneider M, Smolen JS, Svenungsson E, Tanaka Y, Tektonidou MG, Teng YO, Tincani A, Vital EM, van Vollenhoven RF, Wincup C, Bertsias G, Boumpas DT.
雑誌名: Ann Rheum Dis. 2024 Jan 2;83(1):15-29. doi: 10.1136/ard-2023-224762. Epub 2024 Jan 2.
Abstract/Text: OBJECTIVES: To update the EULAR recommendations for the management of systemic lupus erythematosus (SLE) based on emerging new evidence.
METHODS: An international Task Force formed the questions for the systematic literature reviews (January 2018-December 2022), followed by formulation and finalisation of the statements after a series of meetings. A predefined voting process was applied to each overarching principle and recommendation. Levels of evidence and strengths of recommendation were assigned, and participants finally provided their level of agreement with each item.
RESULTS: The Task Force agreed on 5 overarching principles and 13 recommendations, concerning the use of hydroxychloroquine (HCQ), glucocorticoids (GC), immunosuppressive drugs (ISDs) (including methotrexate, mycophenolate, azathioprine, cyclophosphamide (CYC)), calcineurin inhibitors (CNIs, cyclosporine, tacrolimus, voclosporin) and biologics (belimumab, anifrolumab, rituximab). Advice is also provided on treatment strategies and targets of therapy, assessment of response, combination and sequential therapies, and tapering of therapy. HCQ is recommended for all patients with lupus at a target dose 5 mg/kg real body weight/day, considering the individual's risk for flares and retinal toxicity. GC are used as 'bridging therapy' during periods of disease activity; for maintenance treatment, they should be minimised to equal or less than 5 mg/day (prednisone equivalent) and, when possible, withdrawn. Prompt initiation of ISDs (methotrexate, azathioprine, mycophenolate) and/or biological agents (anifrolumab, belimumab) should be considered to control the disease and facilitate GC tapering/discontinuation. CYC and rituximab should be considered in organ-threatening and refractory disease, respectively. For active lupus nephritis, GC, mycophenolate or low-dose intravenous CYC are recommended as anchor drugs, and add-on therapy with belimumab or CNIs (voclosporin or tacrolimus) should be considered. Updated specific recommendations are also provided for cutaneous, neuropsychiatric and haematological disease, SLE-associated antiphospholipid syndrome, kidney protection, as well as preventative measures for infections, osteoporosis, cardiovascular disease.
CONCLUSION: The updated recommendations provide consensus guidance on the management of SLE, combining evidence and expert opinion.

© Author(s) (or their employer(s)) 2024. No commercial re-use. See rights and permissions. Published by BMJ.
Ann Rheum Dis. 2024 Jan 2;83(1):15-29. doi: 10.1136/ard-2023-224762. E...

SLEの病態別治療アルゴリズム(わが国のSLE診療ガイドライン2019より)

まずSLEを診断し、さらに疾患活動性を評価して治療を選択する。ヒドロキシクロロキンは臓器病変、病態を問わず全例で投与を考慮する。
さらに臓器病変に応じて治療を選択し、寛解導入を目指す。ループス腎炎では生検病理所見に応じてグルココルチコイド大量投与に加えてミコフェノール酸モフェチル(MMF)またはシクロホスファミド大量静注(IVCY)併用を考慮する。精神神経ループス(NPSLE)ではグルココルチコイド大量投与にIVCYを併用し、難治例ではリツキシマブを考慮する。その他の臓器病変については、主体となる病態に応じた治療法を考慮する。
寛解導入が得られればグルココルチコイドを減量し、適切な免疫抑制薬併用により、寛解維持療法に移行する。初期療法で寛解が得られない場合は、3rd line治療法として血漿交換療法、リツキシマブ、既存の免疫抑制薬の併用や変更などを考慮するが、これらには十分なエビデンスがない。
出典
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1: 厚生労働科学研究費補助金難治性疾患等政策研究事業 自己免疫疾患に関する調査研究(自己免疫班)/日本リウマチ学会 編:全身性エリテマトーデス診療ガイドライン2019.南山堂、2019

1997年版ACR改訂分類基準

SLEの分類基準は米国リウマチ学会(ACR)が1971年に予備基準を発表し、1982年に改訂、1994年に再改訂された。この分類基準の感度と特異度はいずれも約90%である。分類基準は臨床研究のために策定され、個々の症例の診断を目的としてはいないが、限界(感度と特異度)を理解したうえで診断に用いることには問題はない。
出典
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1: Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus.
Arthritis Rheum. 1997 Sep;40(9):1725. doi: 10.1002/art.1780400928.