北京大学学报(医学版) ›› 2021, Vol. 53 ›› Issue (5): 933-937. doi: 10.19723/j.issn.1671-167X.2021.05.020

• 论著 • 上一篇    下一篇

Rhupus综合征的临床特点

李正芳,吴雪,武丽君(),罗采南,石亚妹,钟岩,陈晓梅,孟新艳   

  1. 新疆维吾尔自治区人民医院风湿免疫科,乌鲁木齐 830001
  • 收稿日期:2019-09-09 出版日期:2021-10-18 发布日期:2021-10-11
  • 通讯作者: 武丽君 E-mail:wwlj330@126.com

Clinical features of patients with Rhupus syndrome

LI Zheng-fang,WU Xue,WU Li-jun(),LUO Cai-nan,SHI Ya-mei,ZHONG Yan,CHEN Xiao-mei,MENG Xin-yan   

  1. Department of Rheumatology and Immunology, People’s Hospital of Xinjiang Uygur Autonomous Region, Urumqi 830001, China
  • Received:2019-09-09 Online:2021-10-18 Published:2021-10-11
  • Contact: Li-jun WU E-mail:wwlj330@126.com

摘要:

目的: 探讨类风湿关节炎(rheumatoid arthritis,RA)和系统性红斑狼疮(systemic lupus erythematosus,SLE)重叠综合征(Rhupus综合征)的临床和血清学特征。方法: 回顾性分析2010年1月至2018年1月于新疆维吾尔自治区人民医院风湿免疫科就诊的21例Rhupus综合征患者的临床资料,另选取同期81例单纯RA及51例单纯SLE作为对照,比较Rhupus综合征与单纯RA组、单纯SLE组关节受累、自身抗体及临床特点。结果: 21例Rhupus综合征患者中男性3例,女性18例,平均年龄(49.43±11.66)岁,高于单纯SLE组患者 的(40.59±12.73)岁(P=0.008)。以RA起病的Rhupus综合征患者的发病年龄小于单纯RA组[(32.58±11.14)岁 vs. (43.11±11.83),P=0.010], 差异有统计学意义。21例Rhupus综合征患者中,以RA起病者12例,除2例为男性外,其余10例女性患者在出现SLE表现时均处于围绝经期或已绝经,平均闭经或绝经年龄(44.30±5.33)岁(36~50岁);出现SLE表现与RA起病的间隔时间为10.83年。以SLE起病者2例。就诊时同时存在RA与SLE表现者7例,除1例为男性外,其余6例为女性(3例处于围绝经期)。Rhupus综合征组特异性抗体的阳性率与单纯RA组相似,SLE相关表现中肾损害相对少见,但间质性肺病的发生率较高。Rhupus综合征和单纯SLE患者补体C3、补体C4、抗核抗体、抗双链DNA抗体、抗SSA抗体和抗SSB抗体的阳性率差异无统计学意义。结论: Rhupus综合征是一种RA与SLE表现共存的重叠综合征,多以RA起病且RA表现较SLE表现严重。Rhupus综合征的发病可能与性激素水平的变化有关。

关键词: 关节炎, 类风湿, 红斑狼疮, 系统性, Rhupus综合征

Abstract:

Objective: To investigate the clinical and serological features of rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE) overlap syndrome (Rhupus syndrome). Methods: We retrospectively reviewed the medical records of 21 patients with Rhupus syndrome who were hospitalized at Department of Rheumatology and Immunology, People’s Hospital of Xinjiang Uygur Autonomous Region between January 2010 and January 2018. We compared the joint involvement, autoantibodies and clinical manifestations of Rhupus syndrome with 81 cases of RA-alone and 51 cases of SLE-alone. Results: In 21 patients with Rhupus syndrome, there are 3 males and 18 females. Compared with the SLE-alone group, the patients with Rhupus syndrome were older [(49.43±11.66) vs. (40.59±12.73), P=0.008]. The median age of the patients with Rhupus syndrome at RA onset was significantly younger than that of the RA-alone patients [(32.58±11.14) vs. (43.11±11.83), P=0.010]. Of the 21 patients with Rhupus syndrome, the initial diagnosis was RA in 57% (12/21), except 2 male patients, the other 10 patients with SLE manifestations were menopause, the mean age of amenorrhea or menopause was (44.30±5.33) (36-50) years. The mean interval between the onset of SLE and RA was 10.83 years. Two patients started with SLE manifestations. Moreover, both diseases simultaneously developed in 33.3% of the patients. Except one male patient, 3 patients were in menopause stage when RA and SLE appeared. The positive rate of specific antibody Rhupus syndrome was similar to that of RA. Renal damage was relatively rare in SLE related manifestations, but the incidence of interstitial lung disease was higher. There were no significant differences in the prevalence of complements C3 and C4, antinuclear antibody (ANA), anti-double-stranded DNA (anti-dsDNA), anti-SSA or anti-SSB antibody between the Rhupus syndrome and SLE-alone group. Conclusion: Rhupus syndrome is an overlapping syndrome in which RA and SLE coexist. Most of the diseases occur in RA and the related manifestations of RA are more serious than those of SLE. The incidence of Rhupus syndrome may be related to the change of sex hormone levels.

Key words: Arthritis, rheumatoid, Lupus Erythematosus, systemic, Rhupus syndrome

中图分类号: 

  • R593.2

表1

三组患者的人口学特征"

Items Rhupus syndrome (n=21) RA-alone (n=81) SLE-alone (n=51)
Age/years, x ?±s 49.43±11.66 52.21±10.96 40.59±12.73*
Female/% 85.7 84.0 96.1
Age at SLE onset/years, x ?±s 25.56±6.36 33.71±12.94
Age at RA onset/years, x ?±s 32.58±11.14 43.11±11.83*
Duration of SLE/years, x ?±s 4.00±1.41 6.92±6.15
Duration of RA/years, x ?±s 10.83±8.44 8.94±7.47

表2

Rhupus综合征组与单纯RA组患者的临床和免疫学特征"

Items Rhupus syndrome (n=21) RA-alone (n=81)
Clinical features, n(%)
Morning stiffness 18 (85.7) 56 (69.1)
Polyarthritis 20 (95.2) 77 (95.1)
Rheumatoid nodule 1 (4.7) 8 (9.9)
Laboratory features, n(%)
RF 19 (90.5) 68 (84.0)
Anti-CCP 14 (66.7) 75 (92.6)*
AKA 7 (33.3) 27 (33.3)
APF 8 (38.1) 11 (13.6)*
Erosions on radiography, n(%) 20 (95.2) 65 (80.2)

表3

Rhupus综合征和单纯SLE患者的临床和免疫学特征"

Items Rhupus syndrome (n=21) SLE-alone (n=51)
Clinical features, n(%)
Malar or discoid lupus 5 (23.8) 28 (54.9)*
Oral ulcers 3 (14.2) 11 (21.6)
Raynaud’s phenomena 5 (23.8) 8 (15.6)
Renal disorder (proteinuria) 5 (23.8) 26 (50.9)*
Interstitial pulmonary diseases 10 (47.6) 5 (9.8)
Haematological disorder 12 (57.1) 25 (49.0)
Laboratory features, n(%)
ESR 21 (100.0) 39 (76.4)*
CRP 16 (76.2) 20 (39.2)#
Decreased complement C3 16 (76.2) 37 (72.5)
Decreased complement C4 16 (76.2) 43 (84.3)
Anti-dsDNA 9 (42.8) 34 (66.7)
SSA 12 (57.1) 34 (66.7)
SSB 2 (9.5) 11 (21.6)
RF 19 (90.5) 8 (15.6)
Anti-CCP 14 (66.7) 0
[1] Amezcua-Guerra LM, Springall R, Marquez-Velasco R, et al. Presence of antibodies against cyclic citrullinated peptides in patients with “Rhupus”: A cross-sectional study [J]. Arthritis Res Ther, 2006, 8(5):R144.
doi: 10.1186/ar2036
[2] Toone EC Jr, Irby R, Pierce EL. The L.E. cell in rheumatoid arthritis [J]. Am J Med Sci, 1960, 240(5):599-608.
[3] Schur PH. Systemic lupus erythematosus [M]//Beeson PB, McDermott W. Cecil-loeb textbook of medicine. 13th ed. Philadelphia, PA: WB Saunders, 1971: 821.
[4] Arnett FC, Edworthy SM, Bloch DA, et al. The American Rheumatism Association 1987 revised criteria for the classification of rheumatoid arthritis [J]. Arthritis Rheum, 1988, 31(3):315-324.
doi: 10.1002/(ISSN)1529-0131
[5] Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus [J]. Arthritis Rheum, 1997, 40(9):1725.
[6] Weening JJ, D’Agati VD, Schwartz MM, et al. The classification of glomerulonephritis in systemic lupus erythematosus revisited [J]. J Am Soc Nephrol, 2004, 15(2):241-250.
pmid: 14747370
[7] 穆荣, 叶华, 陈适, 等. Rhupus综合征的回顾性临床分析 [J]. 中华内科杂志, 2006, 45(7):540-543.
[8] Hayakawa S, Komine-Aizawa S, Osaka S, et al. Rembrandt’s Maria Bockenolle has a butterfly rash and digital deformities: Overlapping syndrome of rheumatoid arthritis and systemic lupus erythematosus [J]. Med Hypotheses, 2007, 68(4):906-909.
pmid: 17113236
[9] Fernandez A, Quintana G, Matteson EL, et al. Lupus arthropathy: Historical evolution from deforming arthritis to rhupus [J]. Clin Rheumatol, 2004, 23(6):523-526.
doi: 10.1007/s10067-004-0951-2
[10] Simon JA, Granados J, Cabiedes J, et al. Clinical and immunogenetic characterization of Mexican patients with “Rhupus” [J]. Lupus, 2002, 11(5):287-292.
doi: 10.1191/0961203302lu189oa
[11] Tani C, D’Aniello D, Sedie AD, et al. Rhupus syndrome: Assessment of its prevalence and its clinical and instrumental characteristics in a prospective cohort of 103 SLE patients [J]. Autoimmun Rev, 2013, 12(4):537-541.
doi: 10.1016/j.autrev.2012.09.004 pmid: 23063507
[12] Liu T, Li G, Mu R, et al. Clinical and laboratory profiles of rhupus syndrome in a Chinese population: A single-centre study of 51 patients [J]. Lupus, 2014, 23(9):958-963.
doi: 10.1177/0961203314526439 pmid: 24608963
[13] Li J, Wu H, Huang X, et al. Clinical analysis of 56 patients with rhupus syndrome: Manifestations and comparisons with systemic lupus erythematosus: A retrospective case-control study [J]. Me-dicine, 2014, 93(10):e49.
[14] Ceccarelli F, Perricone C, Cipriano E, et al. Joint involvement in systemic lupus erythematosus: From pathogenesis to clinical assessment [J]. Semin Arthritis Rheum, 2017, 47(1):53-64.
doi: 10.1016/j.semarthrit.2017.03.022
[15] Sundaramurthy SA, Karsevar MP, van Vollenhoven RV. Influence of hormonal events on disease expression in patients with the combination of systemic lupus erythematosus and rheumatoid arthritis [J]. J Clin Rheumatol, 1999, 5(1):9-16.
pmid: 19078342
[16] Piga M, Gabba A, Cauli A, et al. Rituximab treatment for “rhupus syndrome”: Clinical and power-Doppler ultrasonographic monitoring of response. A longitudinal pilot study [J]. Lupus, 2013, 22(6):624-628.
doi: 10.1177/0961203313482741 pmid: 23559669
[17] Ikeda K, Sanayama Y, Makita S, et al. Efficacy of abatacept for arthritis in patients with an overlap syndrome between rheumatoid arthritis and systemic lupus erythematosus [J]. Clin Dev Immunol, 2013, 2013:697525.
[18] Iaccarino L, Gatto M, Bettio S, et al. Overlap connective tissue disease syndromes [J]. Autoimmun Rev, 2013, 12(3):363-373.
doi: 10.1016/j.autrev.2012.06.004 pmid: 22743033
[19] Danion F, Sparsa L, Arnaud L, et al. Long-term efficacy and safety of antitumour necrosis factor alpha treatment in rhupus: An open-label study of 15 patients [J]. RMD Open, 2017, 3(2):e000555.
doi: 10.1136/rmdopen-2017-000555
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