Division of Rheumatology, Department of Internal Medicine, Marmara University, Istanbul, Turkey.
Division of Rheumatology, Ankara Numune Education and Research Hospital, Ankara, Turkey.
Clin Exp Rheumatol. 2020 Mar-Apr;38 Suppl 124(2):155-160. Epub 2020 Feb 11.
Immunoglobulin (Ig) A vasculitis affects children more commonly than adults and previous literature lacks any formal damage assessment. Our aim in this study is to investigate the disease course, relapse rates and prognostic factors in adult patients with IgA vasculitis and to evaluate the disease-related damage.
We assembled a retrospective cohort of adult IgA vasculitis from six tertiary Rheumatology Centres in Turkey. The demographics, clinical characteristics, treatment and outcomes of patients were abstracted from medical records.
The study included 130 (male/female: 85/45) patients and the mean age was 42.2±17 years. Cutaneous manifestations and arthritis/arthralgia were the most common clinical manifestations. One hundred thirteen patients (86.9%) were treated with oral glucocorticoids (GC). As additional immunosuppressive (IS) agents, azathioprine was given to 44 (34.9%) and pulse cyclophosphamide to 18 (12.6%) patients. Seventy-nine patients (60%) had follow-up of median 15 (IQR 7-40) months. Twelve (15%) patients relapsed during follow-up. The mean VDI score was 0.4 in the last visit. Nineteen (24.7%) patients had at least one damage item at the end of follow-up. Most frequent damage items were renal 11 (42%), ocular 4 (15%) and cardiovascular 4 (15%).
In this cohort the most frequent damage item was renal and was related to the disease itself. Damage score was higher in patients with more severe disease and treated more aggressively. Our results suggest that more effective treatment options are needed in a subgroup of patients with IgA vasculitis to prevent the damage related with the vasculitis, especially with more severe disease.
免疫球蛋白 A 血管炎在儿童中比在成人中更为常见，既往文献缺乏任何正式的损伤评估。我们在本研究中的目的是调查成人 IgA 血管炎患者的疾病过程、复发率和预后因素，并评估与疾病相关的损伤。
我们从土耳其的六个三级风湿病中心收集了一组回顾性的成人 IgA 血管炎队列。从病历中提取患者的人口统计学、临床特征、治疗和结局数据。
本研究纳入了 130 例（男/女：85/45）患者，平均年龄为 42.2±17 岁。皮肤表现和关节炎/关节痛是最常见的临床表现。113 例（86.9%）患者接受了口服糖皮质激素（GC）治疗。作为额外的免疫抑制（IS）药物，44 例（34.9%）患者给予了硫唑嘌呤，18 例（12.6%）患者给予了环磷酰胺冲击治疗。79 例（60%）患者的中位随访时间为 15（IQR 7-40）个月。在随访期间有 12 例（15%）患者复发。在最后一次就诊时，平均 VDI 评分为 0.4。19 例（24.7%）患者在随访结束时至少有一个损伤项目。最常见的损伤项目是肾脏 11 项（42%）、眼部 4 项（15%）和心血管 4 项（15%）。
在本队列中，最常见的损伤项目是肾脏，与疾病本身有关。损伤评分在疾病更严重和治疗更积极的患者中更高。我们的结果表明，IgA 血管炎患者中需要更有效的治疗方案来预防与血管炎相关的损伤，特别是在疾病更严重的患者中。
