利妥昔单抗治疗IgG4相关腹膜后纤维化引发的髂血管闭塞：一例报告及文献复习

doi:10.3877/cma.j.issn.2095-3216.2024.01.008

IgG4相关性疾病(IgG4-related disease，IgG4-RD)是一组由免疫介导的慢性炎症伴纤维化的罕见疾病^[1]，可累及全身多器官和系统，包括胰腺、胆管、唾液腺、泪腺、颌下腺、甲状腺、垂体、肺、肾、腹膜后软组织等，显著升高的IgG4血清水平和肿块样病变是其主要的临床表现。组织病理学表现以浆细胞浸润、席纹状纤维化、闭塞性静脉炎、IgG4阳性浆细胞浸润为特点^[2]。相关研究发现^[3]，约10%左右的IgG4-RD患者可出现腹膜后纤维化(retroperitoneal fibrosis，RPF)。RPF是一种以腹膜后软组织慢性非特异性炎症和纤维化为特征的疾病^[4]，最常见于腹主动脉周围，其次是髂动脉、尿道和肾动脉周围，增生的纤维组织包裹腹主动脉、髂动脉和尿道，就会出现一系列临床表现，并对周围组织造成压迫和不可逆的损伤^[3,4,5]。同时，IgG4相关的RPF经常被误诊为腹膜后恶性肿瘤而行手术切除治疗^[3]，因此早期规范化诊断、综合管理、个体化治疗显得尤为重要。IgG4相关RPF易复发，其治疗具有巨大挑战性。糖皮质激素为该病的一线治疗方案，但可导致明显副作用及治疗不耐受^[6,7,8,9]，联合其他免疫抑制剂的疗效相关循证医学证据并不充分^[10,11,12]，更个性化，副作用尽可能小的治疗方案一直在探索中。利妥昔单抗(rituximab，RTX)清除B细胞疗法的机制为IgG4相关的RPF的治疗提供了新思路。我们报道了1例IgG4相关RPF引发髂总静脉闭塞为首发表现的病例，采用RTX作为激素助减治疗策略，完成RTX疗程一个月后，其血清学指标明显下降，RPF肿块明显消退、髂血管重新畅通，疾病进入临床缓解期。

图1 患者治疗前后髂血管超声对比注：A、B和C：为入院时髂血管超声，可见下腔静脉远端、双侧髂总静脉管壁不均匀性增厚，致下腔静脉远端及右侧髂总静脉狭窄、左侧髂总静脉闭塞；双侧髂外静脉腔内血流瘀滞缓慢，呈云雾状；D、E和F：为RTX治疗后1月复查髂血管超声，可见双侧髂总、髂外动静脉内径正常，血流通畅，未见明显狭窄及血栓形成；下腔静脉远心段后壁稍增厚、血流速度未见明显增快

图2 患者治疗前后腹部影像学检查对比注：A：入院时腹部CT：腹膜后主动脉旁-骶前见团片状软组织密度影，包埋邻近血管；B：入院时腹部MRI：T1加权上可见腹膜后腹主动脉旁-骶前区域见团片状、不规则稍长信号，边界不清；C：RTX治疗后腹部CT：腹膜后主动脉旁-骶前区包块较前明显消退；D：RTX治疗后2月复查腹部CT：腹膜后主动脉旁-骶前区软组织量显著减少，纤维化包块几乎完全消失

[1]	Wallace ZS, Naden RP, Chari S, et al. The 2019 American College of Rheumatology/European League Against Rheumatism classification criteria for IgG4-related disease [J]. Ann Rheum Dis, 2020, 79(1): 77-87.
[2]	Perugino CA, Stone JH. IgG4-related disease: an update on pathophysiology and implications for clinical care [J]. Nat Rev Rheumatol, 2020, 16(12): 702-714.
[3]	Lian L, Wang C, Tian JL. IgG4-related retroperitoneal fibrosis: a newly characterized disease [J]. Int J Rheum Dis, 2016, 19(11): 1049-1055.
[4]	Raglianti V, Rossi GM, Vaglio A. Idiopathic retroperitoneal fibrosis: an update for nephrologists [J]. Nephrol Dial Transplant, 2021, 36(10): 1773-1781.
[5]	Vaglio A, Salvarani C, Buzio C. Retroperitoneal fibrosis [J]. Lancet, 2006, 367(9506): 241-251.
[6]	Sasaki T, Akiyama M, Kaneko Y, et al. Risk factors of relapse following glucocorticoid tapering in IgG4-related disease [J]. Clin Exp Rheumatol, 2018, 36 Suppl 112(3): 186-189.
[7]	Khosroshahi A, Wallace ZS, Crowe JL, et al. International consensus guidance statement on the management and treatment of IgG4-related disease [J]. Arthritis Rheumatol, 2015, 67(7): 1688-1699.
[8]	Kamisawa T, Okazaki K, Kawa S, et al. Amendment of the Japanese consensus guidelines for autoimmune pancreatitis, 2013 III. Treatment and prognosis of autoimmune pancreatitis [J]. J Gastroenterol, 2014, 49(6): 961-970.
[9]	Hart PA, Kamisawa T, Brugge WR, et al. Long-term outcomes of autoimmune pancreatitis: a multicentre, international analysis [J]. Gut, 2013, 62(12): 1771-1776.
[10]	Mahajan VS, Mattoo H, Deshpande V, et al. IgG4-related disease [J]. Ann Rev Pathol, 2014, 9: 315-347.
[11]	Lanzillotta M, Fernàndez-codina A, Culver E, et al. Emerging therapy options for IgG4-related disease [J]. Expert Rev Clin Immunol, 2021, 17(5): 471-483.
[12]	Omar D, Chen Y, Cong Y, et al. Glucocorticoids and steroid sparing medications monotherapies or in combination for IgG4-RD: a systematic review and network meta-analysis [J]. Rheumatology (Oxford), 2020, 59(4): 718-726.
[13]	Nikiphorou E, Galloway J, Fragoulis GE. Overview of IgG4-related aortitis and periaortitis. a decade since their first description [J]. Autoimmun Rev, 2020, 19(12): 102694.
[14]	Khosroshahi A, Carruthers MN, Stone JH, et al. Rethinking Ormond′s disease: "idiopathic" retroperitoneal fibrosis in the era of IgG4-related disease [J]. Medicine (Baltimore), 2013, 92(2): 82-91.
[15]	张文，董凌莉，朱剑，等. IgG4相关性疾病诊治中国专家共识[J]. 中华内科杂志，2021, 60(3): 192-206.
[16]	Wallwork R, Wallace Z, Perugino C, et al. Rituximab for idiopathic and IgG4-related retroperitoneal fibrosis [J]. Medicine (Baltimore), 2018, 97(42): e12631.
[17]	Wang Y, Guan Z, Gao D, et al. The value of ¹⁸F-FDG PET/CT in the distinction between retroperitoneal fibrosis and its malignant mimics [J]. Semin Arthritis Rheum, 2018, 47(4): 593-600.
[18]	Morin G, Mageau A, Benali K, et al. Persistent FDG/PET CT uptake in idiopathic retroperitoneal fibrosis helps identifying patients at a higher risk for relapse [J]. Eur J Intern Med, 2019, 62: 67-71.
[19]	Wallace ZS, Mattoo H, Carruthers M, et al. Plasmablasts as a biomarker for IgG4-related disease, independent of serum IgG4 concentrations [J]. Ann Rheum Dis, 2015, 74(1): 190-195.
[20]	Mattoo H, Mahajan VS, Della-Torre E, et al. De novo oligoclonal expansions of circulating plasmablasts in active and relapsing IgG4-related disease [J]. J Allergy Clin Immunol, 2014, 134(3): 679-687.
[21]	Sakamoto A, Ishizaka N, Imai Y, et al. Serum levels of IgG4 and soluble interleukin-2 receptor in patients with abdominal and thoracic aortic aneurysm who undergo coronary angiography [J]. Atherosclerosis, 2012, 221(2): 602-603.
[22]	Lanzillotta M, Campochiaro C, Mancuso G, et al. Clinical phenotypes of IgG4-related disease reflect different prognostic outcomes [J]. Rheumatology (Oxford), 2020, 59(9): 2435-2442.
[23]	Wallace ZS, Zhang Y, Perugino CA, et al. Clinical phenotypes of IgG4-related disease: an analysis of two international cross-sectional cohorts [J]. Ann Rheum Dis, 2019, 78(3): 406-412.
[24]	Khosroshahi A, Carruthers MN, Deshpande V, et al. Rituximab for the treatment of IgG4-related disease: lessons from 10 consecutive patients [J]. Medicine (Baltimore), 2012, 91(1): 57-66.
[25]	Perugino CA, Stone JH. IgG4-related disease: an update on pathophysiology and implications for clinical care [J]. Nat Rev Rheumtol, 2020, 16(12): 702-714.
[26]	Lanzillotta M, Stone JH, Della-Torre E. B-cell depletion therapy in IgG4-related disease: state of the art and future perspectives [J]. Mod Rheumatol, 2023, 33(2): 258-265.
[27]	Quattrocchio G, Barreca A, Demarchi A, et al. Long-term effects of intensive B cell depletion therapy in severe cases of IgG4-related disease with renal involvement [J]. Immunol Res, 2020, 68(6): 340-352.
[28]	Lanzillotta M, Della-Torre E, Wallace ZS, et al. Efficacy and safety of rituximab for IgG4-related pancreato-biliary disease: a systematic review and meta-analysis [J]. Pancreatology, 2021, 21(7): 1395-1401.
[29]	Hamdan A, Moeen Z, Tariq H, et al. An interesting case of immunoglobulin G4-related retroperitoneal fibrosis treated with rituximab [J]. Cureus, 2021, 13(9): e17940.
[30]	Ebbo M, Grados A, Samson M, et al. Long-term efficacy and safety of rituximab in IgG4-related disease: data from a French nationwide study of thirty-three patients [J]. PLoS One, 2017, 12(9): e0183844.
[31]	Carruthers MN, Topazian MD, Khosroshahi A, et al. Rituximab for IgG4-related disease: a prospective, open-label trial [J]. Ann Rheum Dis, 2015, 74(6): 1171-1177.
[32]	Boyeva V, Alabsi H, Seidman MA, et al. Use of rituximab in idiopathic retroperitoneal fibrosis [J]. BMC Rheumatol, 2020, 4: 40.
[33]	Almeqdadi M, Al-Dulaimi M, Perepletchikov A, et al. Rituximab for retroperitoneal fibrosis due to IgG4-related disease: a case report and literature review [J]. Clin Nephrol Case Stud, 2018, 6: 4-10.
[34]	Dong L, Cong Y, Chen Y, et al. Glucocorticoids and steroid sparing medications monotherapies or in combination for IgG4-RD: a systematic review and network meta-analysis [J]. Rheumatology (Oxford), 2020, 59(4): 718-726.

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Rituximab treatment of iliac vascular occlusion in IgG4related retroperitoneal fibrosis: a case report with literature review

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Rituximab treatment of iliac vascular occlusion in IgG4­related retroperitoneal fibrosis: a case report with literature review

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