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印度北部结节性多动脉炎:临床表现与预后

Polyarteritis nodosa in north India: clinical manifestations and outcomes.

作者信息

Sharma Aman, Pinto Benzeeta, Dhooria Aadhaar, Rathi Manish, Singhal Manphool, Dhir Varun, Sharma Kusum, Parkash Mahesh, Modi Manish, Vijayvergiya Rajesh, Sinha Saroj K, Nada Ritambhra, Minz Ranjana Walkar, Singh Surjit

机构信息

Department of Internal Medicine, Post Graduate Institute of Medical Education and Research, Chandigarh, India.

Department of Nephrology, Post Graduate Institute of Medical Education and Research, Chandigarh, India.

出版信息

Int J Rheum Dis. 2017 Mar;20(3):390-397. doi: 10.1111/1756-185X.12954. Epub 2016 Dec 19.

Abstract

OBJECTIVE

There has been a significant decrease in the number of published reports of classical polyarteritis nodosa (PAN) in the post-Chapel Hill consensus conference (CHCC) nomenclature era with only two series published from Asia. We report a case series of PAN from north India.

PATIENTS AND METHODS

A retrospective study of all patients diagnosed to have PAN according to American College of Rheumatology criteria/CHCC nomenclature. The details of clinical presentation, investigation findings, treatment details and outcomes were noted from the records. These findings between the hepatitis B positive and negative groups were compared.

RESULTS

Twenty-seven patients (20 male, seven female) were diagnosed as having PAN, out of which seven (25.9%) were hepatitis B surface antigen positive. Nervous system involvement was most common with 24 patients (88.9%) having mononeuritis multiplex. Weight loss was present in 20 (74%), fever in 14 (51.9%), renal involvement in 16 (59.3%), cutaneous in nine (33.3%), peripheral gangrene in eight (29.6%), gastrointestinal (GI) involvement in eight (29.6%), testicular pain in 6/20 (30%) and cardiac involvement in four (14.8%). Twenty-three (85.2%) patients recovered, three died (11.1%) and one was lost to follow-up. Median follow-up duration was 37 (interquartile range 22.00-69.75) months. The cumulative survival was 114.16 months (95% CI: 98.27-129.95). There was no significant difference in five factor score (FFS) or revised FFS between those patients who died and those who survived (P = 0.248, 0.894, respectively). Hepatitis B-related PAN had a lower FFS compared to non-hepatitis B-related PAN (P = 0.039). No other significant differences were noted between the two groups.

CONCLUSION

In comparison to classic PAN in other populations, classic PAN in north India is associated with higher neurological involvement and lower GI involvement.

摘要

目的

在查珀尔希尔共识会议(CHCC)命名法时代,经典结节性多动脉炎(PAN)的发表报告数量显著减少,亚洲仅发表了两个系列。我们报告了一组来自印度北部的PAN病例。

患者与方法

对所有根据美国风湿病学会标准/CHCC命名法诊断为PAN的患者进行回顾性研究。从记录中记录临床表现、检查结果、治疗细节和结局的详细信息。比较乙肝阳性和阴性组之间的这些发现。

结果

27例患者(20例男性,7例女性)被诊断为PAN,其中7例(25.9%)乙肝表面抗原阳性。神经系统受累最为常见,24例患者(88.9%)有多发性单神经炎。20例(74%)有体重减轻,14例(51.9%)有发热,16例(59.3%)有肾脏受累,9例(33.3%)有皮肤受累,8例(29.6%)有外周坏疽,8例(29.6%)有胃肠道(GI)受累,6/20例(30%)有睾丸疼痛,4例(14.8%)有心脏受累。23例(85.2%)患者康复,3例死亡(11.1%),1例失访。中位随访时间为37(四分位间距22.00 - 69.75)个月。累积生存率为114.16个月(95%CI:98.27 -  129.95)。死亡患者和存活患者之间的五因素评分(FFS)或修订FFS无显著差异(分别为P = 0.248,0.894)。与非乙肝相关的PAN相比,乙肝相关的PAN的FFS较低(P = 0.039)。两组之间未发现其他显著差异。

结论

与其他人群中的经典PAN相比,印度北部的经典PAN与更高的神经系统受累和更低的胃肠道受累相关。

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