Natural history of Behçet's disease focusing on remission of oral ulcers.

OBJECTIVES: To describe the long-term clinical course of each manifestation of Behçet's disease (BD) and clarify factors involved in oral ulcer (OU) remission using clinical information of BD patients. METHODS: We retrospectively studied 155 BD patients visiting our hospital (1989-2020). We defined remission criteria for each manifestation and examined long-term clinical changes. Classification and regression trees and multivariable analyses were performed to investigate OU prognostic factors; hazard ratios were used to assign scores to prognostic factors deemed significant [OU prognosis score (OuP score)]. Risk stratification was examined by dividing the OuP scores into four stages. RESULTS: OUs appeared earliest, with the slowest decline in prevalence observed post-BD diagnosis. OU presence was the most common factor inhibiting complete remission. Young age at OU onset, never smoker, presence of genital ulcers, positive pathergy test, no usage of tumour necrosis factor inhibitors or of immunosuppressants, and long-term non-treatment or symptomatic treatment for OUs were poor OU prognostic factors. Based on multivariable analysis, the area under the curve of the OuP score to predict OU prognosis was 0.678. CONCLUSIONS: Remission criteria for each symptom clarified that OU had the greatest impact on complete BD remission. Faster OU remission was associated with earlier OU therapeutic intervention other than symptomatic treatment.

Is Kimura's disease associated with juvenile temporal arteritis? A case report and literature review of all juvenile temporal arteritis cases.

Both juvenile temporal arteritis (JTA) and Kimura's disease are eosinophilic inflammatory conditions but exhibit different clinical manifestations. Here, we describe a case involving a 40-year-old man who developed JTA secondary to Kimura's disease. Approximately 3 years before admission, masses appeared on both posterior auricles. A biopsy of the right posterior auricle mass led to a diagnosis of Kimura's disease. Approximately 4 months before admission, both masses increased in size, and almost simultaneously, the left temporal artery became distended. Histopathology of a biopsy of the left temporal artery revealed inflammatory findings with marked eosinophil infiltration and significant intimal hyperplasia with stenosis of the vascular lumen, indicating JTA. An analysis of the 48 reported cases of JTA, identified in a literature review, and the present case, revealed that Kimura's disease was detected in 6 cases, all of which involved Asians. In conclusion, this case and the literature review suggest that JTA can be accompanied by another eosinophilic inflammation-based disorder, Kimura's disease, particularly in Asians. This newly highlighted relationship between JTA and Kimura's disease could lead to a better understanding of JTA, which is an extremely rare disease.