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Juvenile idiopathic recurrent parotitis (JIRP) treated with short course steroids, a case series study and one decade follow up for potential autoimmune disorder
Pediatric Rheumatology ( IF 2.5 ) Pub Date : 2024-01-04 , DOI: 10.1186/s12969-023-00946-0
Farhad Salehzadeh , Rasol Molatefi , Ali Mardi , Negin Nahanmoghaddam

Juvenile idiopathic recurrent parotitis (JIRP) in children is a condition characterized with recurrent episodes of idiopathic parotid gland inflammation. Since there are no definitive guidelines for diagnosis and management of this condition, we present a consecutive case series of patients with more than one decade follow up and their dramatic response to short course treatment by prednisolone. We conducted this study by retrospectively reviewed medical charts of children who were diagnosed with JIRP, from 1 January 2002 to 29 February 2023. We performed usual serological tests to exclude some possible background. We administered short course prednisolone on first day of episode as divided dosage (0.5 mg /kg). In this case series of 10 patients (70%) were male, median age of onset was 5 years, duration of episodes 5 days, and the mean course of disease were 3.8 years. The average follows up of patients was near 10 years. In comparison with their natural course of disease all patients showed a dramatic response to treatment on the first day of administration of prednisolone (P Value 0.005). For ten years follow up there was not any additional accompanying autoimmune disorder. Short course prednisolone on first day of each episode and its dramatic and meaningful response in our patients, introduce a new, effective, fast, and inexpensive regimen of therapy in patients with JIRP.

中文翻译:

用短期类固醇治疗青少年特发性复发性腮腺炎 (JIRP)、病例系列研究和潜在自身免疫性疾病十年随访

儿童幼年特发性复发性腮腺炎(JIRP)是一种以特发性腮腺炎症反复发作为特征的疾病。由于没有明确的诊断和治疗这种情况的指南,我们提出了一系列经过十多年随访的患者的连续病例系列,以及他们对泼尼松龙短期治疗的显着反应。我们通过回顾性审查 2002 年 1 月 1 日至 2023 年 2 月 29 日期间被诊断为 JIRP 的儿童的病历来进行这项研究。我们进行了常规血清学测试以排除一些可能的背景。我们在发作的第一天以分次剂量(0.5 mg/kg)给予短程泼尼松龙。在该病例系列中,10 名患者 (70%) 为男性,中位发病年龄为 5 岁,发作持续时间为 5 天,平均病程为 3.8 年。患者的平均随访时间接近10年。与自然病程相比,所有患者在泼尼松龙给药第一天都表现出显着的治疗反应(P 值 0.005)。十年的随访中没有任何其他伴随的自身免疫性疾病。每次发作第一天使用短程泼尼松龙及其对我们患者的显着且有意义的反应,为 JIRP 患者引入了一种新的、有效的、快速的和廉价的治疗方案。
更新日期:2024-01-04
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