原发性干燥综合征中枢神经系统受累
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摘要
原发性干燥综合征(p SS)是一种主要累及外分泌腺的自身免疫性疾病,常常导致患者黏膜干燥,进而引起相关临床症状或并发症。随着p SS流行病学、病理生理学和组织病理学研究的逐渐深入,p SS患者周围神经系统(PNS-SS)受累的研究较多,而中枢神经系统(CNS)表现由于缺乏明确的定义而争议较大。近年来p SS患者中枢神经系统损害(CNS-SS)逐渐受到关注,由于CNS-SS的发病机制复杂、临床表现多样而容易引起误诊误治。本文就近年来关于CNS-SS的病理机制、临床表现、诊断和鉴别诊断及其治疗方法研究进展进行综述,以期为今后临床工作提供参考。
Primary Sjgren Syndrome( p SS) is an autoimmune disease mainly involving the exocrine glands,leading to sicca symptoms and other complications. With studies on epidemiology,pathophysiology,and histopathology of p SS,peripheral nervous system( PNS) involvement has been widely described,but central nervous system( CNS) manifestations are still unknown. Nevertheless,CNS involvement in p SS( CNS-SS) is getting more and more consern recently. Moreover,CNS-SS is easily misdiagnosed for its complexity of pathogenesis and diversity of manifestations. Herein,we review and summarize the progresses in pathological mechanism,clinical manifestations,differential diagnosis,and treatment of CNS-SS.
Primary Sjgren Syndrome( p SS) is an autoimmune disease mainly involving the exocrine glands,leading to sicca symptoms and other complications. With studies on epidemiology,pathophysiology,and histopathology of p SS,peripheral nervous system( PNS) involvement has been widely described,but central nervous system( CNS) manifestations are still unknown. Nevertheless,CNS involvement in p SS( CNS-SS) is getting more and more consern recently. Moreover,CNS-SS is easily misdiagnosed for its complexity of pathogenesis and diversity of manifestations. Herein,we review and summarize the progresses in pathological mechanism,clinical manifestations,differential diagnosis,and treatment of CNS-SS.
引文
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[2] Anagnostopoulos I,Zinzaras E,Alexiou I,et al.The prevalence of rheumatic diseases in central Greece:a population survey[J].BMC Musculoskeletal Disord,2010,11:98.
[3] Birlik M,Akar S,Gurler O,et al. Prevalence of primary Sj9gren’s syndrome in Turkey:a population-based epidemiological study[J]. Int J Clin Pract,2010,63:954-961.
[4] Granssonl G,Haldorsen K,Brun JG,et al. The point prevalence of clinically relevant primary Sj9gren’s syndrome in two Norwegian counties[J]. Scand J Rheumatol,2011,40:221-224.
[5] Brito-Zerón P,Ramos-Casals M, Bove A, et al. Predicting adverse outcomes in primary Sj9gren’s syndrome:identification of prognostic factors[J]. Rheumatology,2007,46:1359-1362.
[6] Yaguchi H,Houzen H,Kikuchi K,et al. An autopsy case of Sj9gren’s syndrome with acute encephalomyelopathy[J]. Int Med,2008,47:1675.
[7] Massara A,Bonazza S,Castellino G,et al. Central nervous system involvement in Sj9gren’s syndrome:unusual,but not unremarkable—clinical,serological characteristics and outcomes in a large cohort of Italian patients[J]. Rheumatology, 2010, 49:1540-1549.
[8] Morreale M,Marchione P,Giacomini P,et al. Neurological Involvement in primary Sj9gren syndrome:A focus on central nervous system[J/OL]. Plos One,2014,9:e84605[2017-10-21].http://journals. plos. org/plosone/article? id=10. 1371/journal.pone.0084605.
[9] Delalande S,De SJ,Fauchais AL,et al. Neurologic manifestations in primary Sj9gren syndrome:a study of 82 patients[J].Medicine,2004,83:280-291.
[10] Molina R,Provost TT,Alexander EL. Two types of inflammatory vascular disease in Sj9gren’s syndrome. Differential association with seroreactivity to rheumatoid factor and antibodies to Ro(SSA)and with hypocomplementemia[J]. Arthritis Rheumat,1985,28:1251-1258.
[11] Asai Y,Nakayasu H,Fusayasu E,et al. Moyamoya disease presenting as thalamic hemorrhage in a patient with neuromyelitis optica and Sj9gren’s syndrome[J]. J Stroke Cerebrovasc Dis,2012,21:7-9.
[12] Yanagawa Y,Sugiura T,Suzuki K,et al. Moyamoya disease associated with positive findings for rheumatoid factor and myeloperoxidase-anti-neutrophil cytoplasmic antibody[J]. West Indian Med J,2007,56:282-284.
[13] Hayashi K,Morofuji Y,Suyama K,et al. Recurrence of subarachnoid hemorrhage due to the rupture of cerebral aneurysms in a patient with Sj9gren’s syndrome. Case report[J]. Neurol Med Chir,2010,50:658-661.
[14] Gono T, Kawaguchi Y, Katsumata Y, et al. Clinical manifestations of neurological involvement in primary Sj9gren’s syndrome[J]. Clin Rheumatol,2011,30:485-490.
[15] Sanahuja J,Ordo1ez-Palau S,BeguéR,et al. Primary Sj9gren syndrome with tumefactive central nervous system involvement[J].Ajnr Am J Neuroradiol,2008,29:1878-1879.
[16] Gono T,Takarada T,Fukumori R,et al. NR2-reactive antibody decreases cell viability through augmentation of Ca2+influx in systemic lupus erythematosus[J]. Arthritis Rheum, 2011,63:3952-3959.
[17] De Giorgio LA,Konstantinov KN,Lee SC,et al. A subset of lupus anti-DNA antibodies cross-reacts with the NR2 glutamate receptor in systemic lupus erythematosus[J]. Nat Med, 2001,7:1189-1193.
[18] Faust TW,Chang EH,Kowal C,et al. Neurotoxic lupus autoantibodies alter brain function through two distinct mechanisms[J].Proc Natl Acad Sci USA,2010,107:18569-18574.
[19] Streifler JY,Molad Y. Connective tissue disorders:systemic lupus erythematosus,Sj9gren’s syndrome,and scleroderma[J]. Handb Clin Neurol,2014,119:463-473.
[20] Teixeira F,Moreira I,Silva AM,et al. Neurological involvement in primary Sj9gren syndrome[J]. Acta Reumatol Port,2013,38:29-36.
[21]费允云,张奉春.原发性干燥综合征中枢神经系统病变21例临床分析[J].中华风湿病学杂志,2007,11:479-482.
[22] Wandinger KP,Stangel M,Witte T,et al. Autoantibodies against aquaporin-4 in patients with neuropsychiatric systemic lupus erythematosus and primary Sj9gren’s syndrome[J]. Arthritis Rheum,2010,62:1198-1200.
[23] Qiao L,Wang Q,Fei Y,et al. The clinical characteristics of primary Sj9gren’s syndrome with neuromyelitis optica spectrum disorder in China:A STROBE-compliant article[J/OL]. Medicine,2015,94:e1145[2017-10-16]. http://insights. ovid. com/pubmed? pmid=26181553
[24]陈静炯,许丹,赵玉武.原发性干燥综合征合并视神经脊髓炎谱系疾病临床分析及文献复习[J].中国现代神经疾病杂志,2013,13:441-444.
[25]贾楠,景筠,李清晨,等.伴干燥综合征的视神经脊髓炎谱系疾病预后分析[J].中华医学杂志,2017,97:827-832.
[26]杨丹,乔琳,赵丽丹.原发干燥综合征伴发脑梗塞1例报告附文献复习[J].北京大学学报:医学版,2016,48:1077-1080.
[27] Arinuma Y,Yanagida T,Hirohata S. Association of cerebrospinal fluid anti-NR2 glutamate receptor antibodies with diffuse neuropsychiatric systemic lupus erythematosus[J]. Arthritis Rheum,2008,58:1130-1135.
[28] Pars K,Pul R,Schwenkenbecher P,et al. Cerebrospinal fluid findings in neurological diseases associated with Sj9gren’s syndrome[J]. Eur Neurol,2017,77:91-102.
[29]杨娉婷,徐枫,肖卫国.干燥综合征神经系统损害临床表现及诊治[J].中国实用内科杂志,2017,37:503-505.
[30] Niu B,Zou Z,Shen Y,et al. A case report of Sj9gren syndrome manifesting bilateral basal ganglia lesions[J/OL]. Medicine(Baltimore),2017,96:e6715[2017-10-12]. http://insights. ovid.com/pubmed? pmid=28445282.
[31]刘静,王玉平.电生理检查在原发干燥综合征合并中枢及周围神经系统损害的应用[J].脑与神经疾病杂志,2016,24:199-201.
[32]屈继波.原发性干燥综合征并发中枢神经脱髓鞘与多发性硬化的鉴别研究进展[J].神经疾病与精神卫生,2012,12:210-213.
[33] Eckstein C,Saidha S,Levy M. A differential diagnosis of central nervous system demyelination:beyond multiple sclerosis[J]. J Neurol,2012,259:801-816.
[34] Ozgocmen S,Gur A. Treatment of central nervous system involvement associated with primary Sj9gren’s syndrome[J]. Curr Pharm Des,2008,14:1270-1273.
[35] Jayarangaiah A,Sehgal R,Epperla N. Sj9grens syndrome and neuromyelitis optica spectrum disorders(NMOSD)a case report and review of literature[J]. BMC Neurol,2014,14:200.
[36] Takahashi Y,Takata T,Hoshino M,et al. Benefit of IVIg for long-standing ataxic sensory neuronopathy with Sj9gren’s syndrome[J]. Neurology,2003,60:503-505.
[2] Anagnostopoulos I,Zinzaras E,Alexiou I,et al.The prevalence of rheumatic diseases in central Greece:a population survey[J].BMC Musculoskeletal Disord,2010,11:98.
[3] Birlik M,Akar S,Gurler O,et al. Prevalence of primary Sj9gren’s syndrome in Turkey:a population-based epidemiological study[J]. Int J Clin Pract,2010,63:954-961.
[4] Granssonl G,Haldorsen K,Brun JG,et al. The point prevalence of clinically relevant primary Sj9gren’s syndrome in two Norwegian counties[J]. Scand J Rheumatol,2011,40:221-224.
[5] Brito-Zerón P,Ramos-Casals M, Bove A, et al. Predicting adverse outcomes in primary Sj9gren’s syndrome:identification of prognostic factors[J]. Rheumatology,2007,46:1359-1362.
[6] Yaguchi H,Houzen H,Kikuchi K,et al. An autopsy case of Sj9gren’s syndrome with acute encephalomyelopathy[J]. Int Med,2008,47:1675.
[7] Massara A,Bonazza S,Castellino G,et al. Central nervous system involvement in Sj9gren’s syndrome:unusual,but not unremarkable—clinical,serological characteristics and outcomes in a large cohort of Italian patients[J]. Rheumatology, 2010, 49:1540-1549.
[8] Morreale M,Marchione P,Giacomini P,et al. Neurological Involvement in primary Sj9gren syndrome:A focus on central nervous system[J/OL]. Plos One,2014,9:e84605[2017-10-21].http://journals. plos. org/plosone/article? id=10. 1371/journal.pone.0084605.
[9] Delalande S,De SJ,Fauchais AL,et al. Neurologic manifestations in primary Sj9gren syndrome:a study of 82 patients[J].Medicine,2004,83:280-291.
[10] Molina R,Provost TT,Alexander EL. Two types of inflammatory vascular disease in Sj9gren’s syndrome. Differential association with seroreactivity to rheumatoid factor and antibodies to Ro(SSA)and with hypocomplementemia[J]. Arthritis Rheumat,1985,28:1251-1258.
[11] Asai Y,Nakayasu H,Fusayasu E,et al. Moyamoya disease presenting as thalamic hemorrhage in a patient with neuromyelitis optica and Sj9gren’s syndrome[J]. J Stroke Cerebrovasc Dis,2012,21:7-9.
[12] Yanagawa Y,Sugiura T,Suzuki K,et al. Moyamoya disease associated with positive findings for rheumatoid factor and myeloperoxidase-anti-neutrophil cytoplasmic antibody[J]. West Indian Med J,2007,56:282-284.
[13] Hayashi K,Morofuji Y,Suyama K,et al. Recurrence of subarachnoid hemorrhage due to the rupture of cerebral aneurysms in a patient with Sj9gren’s syndrome. Case report[J]. Neurol Med Chir,2010,50:658-661.
[14] Gono T, Kawaguchi Y, Katsumata Y, et al. Clinical manifestations of neurological involvement in primary Sj9gren’s syndrome[J]. Clin Rheumatol,2011,30:485-490.
[15] Sanahuja J,Ordo1ez-Palau S,BeguéR,et al. Primary Sj9gren syndrome with tumefactive central nervous system involvement[J].Ajnr Am J Neuroradiol,2008,29:1878-1879.
[16] Gono T,Takarada T,Fukumori R,et al. NR2-reactive antibody decreases cell viability through augmentation of Ca2+influx in systemic lupus erythematosus[J]. Arthritis Rheum, 2011,63:3952-3959.
[17] De Giorgio LA,Konstantinov KN,Lee SC,et al. A subset of lupus anti-DNA antibodies cross-reacts with the NR2 glutamate receptor in systemic lupus erythematosus[J]. Nat Med, 2001,7:1189-1193.
[18] Faust TW,Chang EH,Kowal C,et al. Neurotoxic lupus autoantibodies alter brain function through two distinct mechanisms[J].Proc Natl Acad Sci USA,2010,107:18569-18574.
[19] Streifler JY,Molad Y. Connective tissue disorders:systemic lupus erythematosus,Sj9gren’s syndrome,and scleroderma[J]. Handb Clin Neurol,2014,119:463-473.
[20] Teixeira F,Moreira I,Silva AM,et al. Neurological involvement in primary Sj9gren syndrome[J]. Acta Reumatol Port,2013,38:29-36.
[21]费允云,张奉春.原发性干燥综合征中枢神经系统病变21例临床分析[J].中华风湿病学杂志,2007,11:479-482.
[22] Wandinger KP,Stangel M,Witte T,et al. Autoantibodies against aquaporin-4 in patients with neuropsychiatric systemic lupus erythematosus and primary Sj9gren’s syndrome[J]. Arthritis Rheum,2010,62:1198-1200.
[23] Qiao L,Wang Q,Fei Y,et al. The clinical characteristics of primary Sj9gren’s syndrome with neuromyelitis optica spectrum disorder in China:A STROBE-compliant article[J/OL]. Medicine,2015,94:e1145[2017-10-16]. http://insights. ovid. com/pubmed? pmid=26181553
[24]陈静炯,许丹,赵玉武.原发性干燥综合征合并视神经脊髓炎谱系疾病临床分析及文献复习[J].中国现代神经疾病杂志,2013,13:441-444.
[25]贾楠,景筠,李清晨,等.伴干燥综合征的视神经脊髓炎谱系疾病预后分析[J].中华医学杂志,2017,97:827-832.
[26]杨丹,乔琳,赵丽丹.原发干燥综合征伴发脑梗塞1例报告附文献复习[J].北京大学学报:医学版,2016,48:1077-1080.
[27] Arinuma Y,Yanagida T,Hirohata S. Association of cerebrospinal fluid anti-NR2 glutamate receptor antibodies with diffuse neuropsychiatric systemic lupus erythematosus[J]. Arthritis Rheum,2008,58:1130-1135.
[28] Pars K,Pul R,Schwenkenbecher P,et al. Cerebrospinal fluid findings in neurological diseases associated with Sj9gren’s syndrome[J]. Eur Neurol,2017,77:91-102.
[29]杨娉婷,徐枫,肖卫国.干燥综合征神经系统损害临床表现及诊治[J].中国实用内科杂志,2017,37:503-505.
[30] Niu B,Zou Z,Shen Y,et al. A case report of Sj9gren syndrome manifesting bilateral basal ganglia lesions[J/OL]. Medicine(Baltimore),2017,96:e6715[2017-10-12]. http://insights. ovid.com/pubmed? pmid=28445282.
[31]刘静,王玉平.电生理检查在原发干燥综合征合并中枢及周围神经系统损害的应用[J].脑与神经疾病杂志,2016,24:199-201.
[32]屈继波.原发性干燥综合征并发中枢神经脱髓鞘与多发性硬化的鉴别研究进展[J].神经疾病与精神卫生,2012,12:210-213.
[33] Eckstein C,Saidha S,Levy M. A differential diagnosis of central nervous system demyelination:beyond multiple sclerosis[J]. J Neurol,2012,259:801-816.
[34] Ozgocmen S,Gur A. Treatment of central nervous system involvement associated with primary Sj9gren’s syndrome[J]. Curr Pharm Des,2008,14:1270-1273.
[35] Jayarangaiah A,Sehgal R,Epperla N. Sj9grens syndrome and neuromyelitis optica spectrum disorders(NMOSD)a case report and review of literature[J]. BMC Neurol,2014,14:200.
[36] Takahashi Y,Takata T,Hoshino M,et al. Benefit of IVIg for long-standing ataxic sensory neuronopathy with Sj9gren’s syndrome[J]. Neurology,2003,60:503-505.