Abstract：
BACKGROUND: Behcet\'s disease (BD) has a heterogeneous and unpredictable phenotype that differs in various geographical areas.
OBJECTIVE: To describe the clinical phenotype & outcome of Behcet\'s disease(BD) from Karnataka, India and compare them with large cohorts from endemic regions.
METHODS: Databases of practising rheumatologists from Karnataka were reviewed to retrieve clinical characteristics, course of illness, prescribing information and outcome at last follow-up of patients clinically diagnosed as BD. The classification criteria, namely revised International criteria for Behcet\'s disease (rICBD) and International study group (ISG) criteria were applied. Outcome was defined as complete or partial remission, persistent disease or relapse.
RESULTS: We included 72 patients, equal gender distribution and mean age 37.4 ± 12.8 years from 8 rheumatology centres. Commonest presentations were recurrent oral aphthosis 58(80.6%), genital ulcers 36(50%) and ocular manifestations 40(55.6%). Three-quarters [51/72(70.8%)] fulfilled rICBD criteria whereas only half [36/72(50%)] fulfilled ISG criteria. Apart from glucocorticoids [53/72(73.6%)], frequently prescribed therapies were colchicine 39(54.2%) and azathioprine 35(48.6%). Eleven-patients received biologics(anti-TNF-α) and JAK inhibitors to treat severe organ involvement. HLA-B*51 and pathergy tests were positive in 27/45(60%) and 12/34(35.3%) patients respectively. Outcomes were documented in 94.4%(68/72) patients at median follow-up of 24 (12;36) months. Majority [46/68(67.6%)] had complete remission, 17/68(25%) had partial remission, 4/68(5.9%) had persistent while 1/68(1.5%) had relapsing course.
CONCLUSIONS: Majority of BD patients had orogenital aphthosis and ocular manifestations and an excellent response to treatment. Key Points • In our region, Behçet\'s Disease primarily manifests with recurrent oral aphthae and ocular involvement, with comparatively lower incidence of severe genital ulcers and neurological involvement than in endemic regions. • Apart from glucocorticoids, colchicine and azathioprine are the most commonly used agents. Biologics and JAK inhibitors are prescribed infrequently, primarily in cases of severe organ involvement. • A significant proportion of patients achieved either complete or partial remission during follow-up, with no observed mortality suggesting a milder disease course and better outcome compared to endemic regions. • Gender, HLA-B*51 status, and pathergy response did not exert any significant influence on the clinical profile or outcome in BD patients in Karnataka.
OBJECTIVE: To describe the clinical phenotype & outcome of Behcet\'s disease(BD) from Karnataka, India and compare them with large cohorts from endemic regions.
METHODS: Databases of practising rheumatologists from Karnataka were reviewed to retrieve clinical characteristics, course of illness, prescribing information and outcome at last follow-up of patients clinically diagnosed as BD. The classification criteria, namely revised International criteria for Behcet\'s disease (rICBD) and International study group (ISG) criteria were applied. Outcome was defined as complete or partial remission, persistent disease or relapse.
RESULTS: We included 72 patients, equal gender distribution and mean age 37.4 ± 12.8 years from 8 rheumatology centres. Commonest presentations were recurrent oral aphthosis 58(80.6%), genital ulcers 36(50%) and ocular manifestations 40(55.6%). Three-quarters [51/72(70.8%)] fulfilled rICBD criteria whereas only half [36/72(50%)] fulfilled ISG criteria. Apart from glucocorticoids [53/72(73.6%)], frequently prescribed therapies were colchicine 39(54.2%) and azathioprine 35(48.6%). Eleven-patients received biologics(anti-TNF-α) and JAK inhibitors to treat severe organ involvement. HLA-B*51 and pathergy tests were positive in 27/45(60%) and 12/34(35.3%) patients respectively. Outcomes were documented in 94.4%(68/72) patients at median follow-up of 24 (12;36) months. Majority [46/68(67.6%)] had complete remission, 17/68(25%) had partial remission, 4/68(5.9%) had persistent while 1/68(1.5%) had relapsing course.
CONCLUSIONS: Majority of BD patients had orogenital aphthosis and ocular manifestations and an excellent response to treatment. Key Points • In our region, Behçet\'s Disease primarily manifests with recurrent oral aphthae and ocular involvement, with comparatively lower incidence of severe genital ulcers and neurological involvement than in endemic regions. • Apart from glucocorticoids, colchicine and azathioprine are the most commonly used agents. Biologics and JAK inhibitors are prescribed infrequently, primarily in cases of severe organ involvement. • A significant proportion of patients achieved either complete or partial remission during follow-up, with no observed mortality suggesting a milder disease course and better outcome compared to endemic regions. • Gender, HLA-B*51 status, and pathergy response did not exert any significant influence on the clinical profile or outcome in BD patients in Karnataka.
摘要：
背景：Behcet病（BD）具有异质性和不可预测的表型，在各个地理区域有所不同。
目的：描述卡纳塔克邦白塞病（BD）的临床表型和结局，印度，并将它们与来自流行地区的大型队列进行比较。
方法：回顾了来自卡纳塔克邦的风湿病专家数据库，以检索临床特征，病程,临床诊断为BD的患者最后一次随访时的处方信息和结果。分类标准，即采用修订的白塞病国际标准(rICBD)和国际研究组(ISG)标准。结果定义为完全缓解或部分缓解，持续性疾病或复发。
结果：我们纳入了72例患者，8个风湿病中心的性别分布相等，平均年龄37.4±12.8岁。最常见的表现是复发性口疮58（80.6%），生殖器溃疡36例(50%)和眼部表现40例(55.6%)。四分之三[51/72（70.8％）]符合rICBD标准，而只有一半[36/72（50％）]符合ISG标准。除了糖皮质激素[53/72（73.6%）]，常见的处方治疗是秋水仙碱39(54.2%)和硫唑嘌呤35(48.6%).11例患者接受生物制剂（抗TNF-α）和JAK抑制剂治疗严重器官受累。HLA-B*51和pathergy试验分别在27/45（60％）和12/34（35.3％）患者中阳性。在24（12;36）个月的中位随访中，94.4％（68/72）的患者记录了结果。大多数[46/68（67.6％）]完全缓解，17/68（25%）部分缓解，4/68（5.9％）持续，1/68（1.5％）复发。
结论：大多数BD患者有生殖口疮和眼部表现，对治疗反应良好。要点•在我们地区，Behçet病主要表现为复发性口疮和眼部受累，与流行地区相比，严重生殖器溃疡和神经系统受累的发生率相对较低。•除了糖皮质激素，秋水仙碱和硫唑嘌呤是最常用的药物。生物制剂和JAK抑制剂很少开处方，主要是在严重器官受累的情况下。•很大比例的患者在随访期间实现了完全或部分缓解，没有观察到的死亡率，表明与流行地区相比，病程较轻，结局更好。性别，HLA-B*51状态,在卡纳塔克邦的BD患者中,pathergy反应对临床特征或结局没有任何显著影响。
目的：描述卡纳塔克邦白塞病（BD）的临床表型和结局，印度，并将它们与来自流行地区的大型队列进行比较。
方法：回顾了来自卡纳塔克邦的风湿病专家数据库，以检索临床特征，病程,临床诊断为BD的患者最后一次随访时的处方信息和结果。分类标准，即采用修订的白塞病国际标准(rICBD)和国际研究组(ISG)标准。结果定义为完全缓解或部分缓解，持续性疾病或复发。
结果：我们纳入了72例患者，8个风湿病中心的性别分布相等，平均年龄37.4±12.8岁。最常见的表现是复发性口疮58（80.6%），生殖器溃疡36例(50%)和眼部表现40例(55.6%)。四分之三[51/72（70.8％）]符合rICBD标准，而只有一半[36/72（50％）]符合ISG标准。除了糖皮质激素[53/72（73.6%）]，常见的处方治疗是秋水仙碱39(54.2%)和硫唑嘌呤35(48.6%).11例患者接受生物制剂（抗TNF-α）和JAK抑制剂治疗严重器官受累。HLA-B*51和pathergy试验分别在27/45（60％）和12/34（35.3％）患者中阳性。在24（12;36）个月的中位随访中，94.4％（68/72）的患者记录了结果。大多数[46/68（67.6％）]完全缓解，17/68（25%）部分缓解，4/68（5.9％）持续，1/68（1.5％）复发。
结论：大多数BD患者有生殖口疮和眼部表现，对治疗反应良好。要点•在我们地区，Behçet病主要表现为复发性口疮和眼部受累，与流行地区相比，严重生殖器溃疡和神经系统受累的发生率相对较低。•除了糖皮质激素，秋水仙碱和硫唑嘌呤是最常用的药物。生物制剂和JAK抑制剂很少开处方，主要是在严重器官受累的情况下。•很大比例的患者在随访期间实现了完全或部分缓解，没有观察到的死亡率，表明与流行地区相比，病程较轻，结局更好。性别，HLA-B*51状态,在卡纳塔克邦的BD患者中,pathergy反应对临床特征或结局没有任何显著影响。
