Retrospective analysis of IgG4-related disease cases at a tertiary care centre in India

IF 1.4 Q3 RHEUMATOLOGY
M. Bhatt, Sanchit Kumar, M. Soneja, S. Vyas, M. Tripathi, R. Kumar, Prabhjot Singh, M. Sharma, N. Wig
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引用次数: 0

Abstract

Objectives IgG4-related disease (IgG4-RD) is a chronic inflammatory disorder with prominent fibrosis. This retrospective analysis was undertaken to study the clinical, laboratory, and radiological characteristics of patients with extra-pancreatic IgG4-RD and their response to treatment at a tertiary care centre located in northern India. Material and methods Patient data from our centre between January 2017 and January 2021 were reviewed. Probable/definite IgG4-RD cases were included in the analysis. Results A total of 14 cases were identified with a median age of 39 years (range 19–56 years). There were 10 males and 4 females. All patients presented with slowly progressive soft tissue swellings with pain/discomfort related to local mass effect. The median delay in diagnosis was 9.5 months (range 2–72 months). Cross-sectional imaging showed soft tissue masses in all cases. All contrast-enhanced studies (n = 7) showed enhancement on computed tomography and magnetic resonance imaging scan. F-18 fluorodeoxyglucose-avidity was observed in 8 of 9 (88.9%) cases. Biopsies performed in 12 of these were classified as definite in 8 and possible IgG4-RD in 4 cases. Patients were treated with a median dose of 1 mg/kg/day (range 0.5–1 mg/kg/day) prednisolone. Steroids were successfully tapered in all 12 cases with 41.6% (5 of 12) being off corticosteroids at a median follow-up of 10 months (range 0–18 months). Two patients were lost to follow-up. Conclusions IgG4-related disease is a chronic illness with a wide spectrum of manifestations, in which the diagnosis is often delayed, but it shows an excellent response to treatment. Efforts must be made to increase awareness among physicians about this disease to institute appropriate treatment as early as possible.
印度某三级保健中心igg4相关疾病病例的回顾性分析
igg4相关疾病(IgG4-RD)是一种以纤维化为主的慢性炎症性疾病。本回顾性分析研究了位于印度北部三级保健中心的胰腺外IgG4-RD患者的临床、实验室和放射学特征及其对治疗的反应。材料和方法回顾了本中心2017年1月至2021年1月的患者数据。可能/确诊的IgG4-RD病例被纳入分析。结果14例患者中位年龄39岁(19 ~ 56岁)。其中男性10人,女性4人。所有患者均表现为缓慢进行性软组织肿胀,伴有与局部肿块效应相关的疼痛/不适。中位诊断延迟为9.5个月(范围2-72个月)。横断成像均显示软组织肿块。所有对比增强研究(n = 7)显示计算机断层扫描和磁共振成像扫描增强。9例患者中有8例(88.9%)出现F-18氟脱氧葡萄糖贪食。其中12例活检确诊8例,4例可能为IgG4-RD。患者接受中位剂量1mg /kg/天(范围0.5-1 mg/kg/天)强的松龙治疗。12例患者中有41.6%(5 / 12)的患者在中位随访10个月(范围0-18个月)后停止使用皮质类固醇。2例患者未随访。结论igg4相关性疾病是一种表现广泛的慢性疾病,诊断常被延误,但治疗反应良好。必须努力提高医生对这种疾病的认识,以便尽早制定适当的治疗办法。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
Reumatologia
Reumatologia Medicine-Rheumatology
CiteScore
2.70
自引率
0.00%
发文量
44
审稿时长
10 weeks
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