Juvenile temporal arteritis with eosinophilia associated with systemic sclerosis

Abstract

Juvenile temporal arteritis with eosinophilia associated with systemic sclerosis Dear Editor, Juvenile temporal arteritis with eosinophilia (JTAE) is a rare disease, manifesting as an asymptomatic nodule in the temporal artery area in young adults. Despite an increasing number of reports, the etiology of JTAE is yet unknown. We report a case of JTAE in a patient with systemic sclerosis (SSc).A 39-year-old Japanese man had started treatment for bronchial asthma 10 years prior. Around the same time, he developed scleredema of the extremities and Raynaud's phenomenon. He was diagnosed with SSc based on clinical and histopathological findings and had been treated with daily 5 mg oral prednisolone until presentation. Two months before his initial examination, he noticed nodular thickening along the left temporal artery. The nodule became gradually larger, and he visited our clinic. Physical examination showed a non-tender cord-like induration measuring more than 5 cm in size on the superficial temporal left artery. Laboratory tests revealed a white blood cell count of 12 610/mm 3 with a total eosinophil count of 2520/mm 3 (20%), and antinuclear antibody (homogeneous and speckled pattern) test was positive. C-reactive protein test was negative, and the erythrocyte sedimentation rate was 10 mm/h. Total immunoglobulin E, interleukin (IL)-5 and eosinophil cationic protein (ECP) levels were normal, although the IL-4 level was elevated at 12.3 pg/mL (normal, <6.0). Ultrasonography of the left temporal artery showed hypoechoic vessel wall-thickening and vessel lumen occlusion, described as a halo sign. He underwent a 4-cm long left temporal artery biopsy. Histopathology revealed non-granulomatous panarteritis with lymphocytes and eosinophils, but giant cells were not found. Arteritis, occlusive fibrous intimal proliferation and disruption of the internal elastic lamina were present (Fig. 1). He was diagnosed with JTAE, but we decided to monitor the clinical course without any treatment. Shortly after biopsy, the nodule on the temporal artery became flattened, but hypereosinophilia did not resolve.

Concurrent bilateral juvenile temporal arteritis and hypereosinophilic syndrome: a case report and review of the literature

“…These patients complained of painless nodules on their temple, and the pathologic findings revealed nongiant cell inflammation of temporal arteries [4]. From then on until February 2019, according to our literature review through PubMed and Scopus, 18 more cases have been reported [23567891011121314151617181920]. Including the present case, a total of 23 cases of JTA were reported and their mean age was 27 years (range, 7 to 44 years) and there was the male predominance (18 men and 5 women).…”

“…JTA is a localized disease so that excision is curative and steroid treatment is usually not required, and after excision, recurrence is known to be rare [2]. Of the 22 cases identified through literature search, the excision of affected temporal artery was done in 19 cases [2345679101112141516171819], and only one case reported that the symptoms persisted after resection [14]. Two of the 18 cases that resolved after excision recurred later on the opposite side [1218].…”

“…In addition, one of the recurred cases reported that eosinophil count was elevated at recurrence [18]. Although only a small number of cases were available for the trend of eosinophil count after excision [8131819], eosinophilia may persist even if temporal arteritis improves without systemic steroids, and may lead to recurrence because documented recurrences occurred only in the patients with JTA with eosinophilia who treated locally. According to very recent case series (n = 12) from France, 41.7% (5 of 12) had peripheral eosinophilia, and this figure was similar to our estimate of 43.4% (10 of 23).…”

“…Since the first report of JTA in 1975 [4], a total of 22 cases have been reported so far [234567891011121314151617181920], but its etiology, pathogenesis, and long-term prognosis are still unknown. Although JTA is a localized disease, it is often accompanied by peripheral blood eosinophilia, so some authors have suggested the concept of JTA with eosinophilia [81213].…”

Juvenile temporal arteritis with eosinophilia associated with limited systemic sclerosis

“…Juvenile temporal arteritis with eosinophilia (JTAE) is a rare vascular disease involving children or young adults. To date, only one report of JTAE and systemic sclerosis (SSc) co‐occurrence has been reported 1 ; however, the detail of SSc, that is, limited or diffuse type, autoantibody, and internal organ involvement, was not described. In that case, serum IL‐4 level was elevated, and the authors speculate that IL‐4 may play an important role in SSc and JTAE via fibroblast proliferation and eosinophil attraction.…”