Temporal arteritis (TA), also known as giant cell arteritis (GCA), is typically a disease of middle-aged and older patients.[1-4] It is exceedingly rare in patients younger than 50 years with very few patients reported globally.[1-4] However, TA in young patients may differ in several aspects from the classical GCA seen in the elderly.[1-6]
Here, we report the case of TA in a 25-year-old male and also highlight the aspects in which it differs from its more popular counterpart of GCA in the elderly population.
A 25-year-old normotensive young male presented with a 3-year history of bilateral temporal headache and swelling in his left temporal region. Otherwise, the patient’s medical history was unremarkable.
The temporal artery swelling progressed in size for 4 months before presentation with an increase in symptoms.
There was an associated low-grade fever and malaise. However, there was no history of visual changes, jaw claudication, or other systemic symptoms. The patient was normotensive and the rest of his vascular and systemic examination was within normal limits. An ophthalmologic evaluation was also sought which showed a normal field of vision, visual acuity, and fundus and there were no features of retinopathy/arteritis.
The baseline laboratory investigations were as follows: hemoglobin – 13.8 g/dL (14–18); total leukocyte count – 7120 cells/cu mm (4400–11000); platelet count – 128000 cells/cu mm (150000–400000); creatinine – 1.22 mg/dL (0.8–1.6); aspartate aminotransferase – 23 IU/L (5–37); alanine aminotransferase – 36 IU/L (10–50); serum total protein – 7.6 mg/dL (6–8.2); and serum globulin – 2.9 mg/dL (1.8–3.4). C-reactive protein was 5 mg/L (<10 mg/L). Erythrocyte sedimentation rate (ESR) was 10 mm/h (0–12). Urine routine microscopy was normal. Viral markers by enzyme-linked immunoassay (ELISA) were negative (HIV 1 and 2; anti-hepatitis C virus antibody; hepatitis B surface antigen). Antineutrophil cytoplasmic antibodies by ELISA were negative.
Hence, on evaluation, our patient had normal renal and hepatic parameters with unremarkable blood counts and inflammatory markers. Ultrasonography (USG) with color Doppler showed focal areas of dilatation and narrowing with homogenous, hypoechoic wall thickening toward the luminal side of the frontal branch of the left temporal artery, both in longitudinal and transverse planes. The surrounding areas showed minimal inflammatory changes. Computed tomography imaging also revealed a dilated left superficial temporal artery, especially its frontal branch. However, there was no evidence of an inflammatory “halo” on USG. The right temporal artery was normal. Left temporal artery biopsy revealed thickening of the tunica media and adventitia with the fragmentation of internal elastic membrane and moderate lymphocytic, neutrophilic, and histiocytic infiltrations in intima and media. No necrosis or giant cells were seen [Figure 1].
These histological findings were consistent with those found in a noneosinophilic variant of juvenile TA (JTA) and hence supported our clinical diagnosis. His treatment was initiated with oral prednisolone at 30 mg/day. It resulted in rapid clinical improvement within 1 week with subsidence of headache and constitutional symptoms. Prednisolone was tapered every 3 weeks by 5 mg along with the addition of oral methotrexate (15 mg/week) with folate supplementation. Within a few weeks, symptoms of headache improved along with constitutional symptoms.
He was on regular follow-up, and after 6 months, in his last visit, he was doing well and symptoms and signs of TA were resolved. He is currently taking prednisolone at 5 mg/day and methotrexate orally at 15 mg/week. Figure 1 shows the appearance of the thickened left superficial temporal artery before and after the temporal artery biopsy (a, b) and histopathological findings(c, d).
Here, we report the case of a young 25-year-old male presenting with TA. In 1975, the term “juvenile temporal arteritis” was coined by Lie et al. Meyers and Lord, however, described similar presentation and histological features in 1948 and Bollinger et al. in 1986. Lie et al. described two children and two young adults with painless unilateral temporal nodules.
Subsequently, in 1994, Tomlinson et al. suggested diagnostic criteria for JTA with salient features, which mention patients to be (1) less than age 40; (2) having a nontender or painful palpable lump in the forehead area of the temporal artery; (3) with no associated signs such as fever, myalgia, visual symptoms, or anemia; (4) along with a normal ESR; (5) often showing eosinophilic panarteritis with intraluminal thrombus which may be associated with parietal microaneurysmal lesions and/or may have intimal proliferation with discreet damage to media and extensive cellular infiltrate made of lymphocytes, eosinophils, and plasma cells in the perivascular tissue; and (6) often with an absence of granulomatous lesions or giant cells.
Over the next few decades, only a few such cases have been reported in the literature. Vasculitic involvement of the temporal arteries in the young has often been divided into three groups.
- Eosinophilic arteritis, also called JTA, is the most common type and is a localized vasculitis confined to the temporal arteries
- The second group consists of systemic vasculitides, such as polyarteritis nodosa or Churg–Strauss syndrome, involving the temporal arteries
- Noneosinophilic vasculitis, with clinical and histological features resembling the classical GCA.
According to a literature review of 15 JTA patients by Nesher et al., as shown in Table 1.[4-16] there is often no evidence of systemic inflammation, ophthalmic symptoms, or other cranial ischemic manifestations. Peripheral eosinophilia may be present as well as histological examination often shows eosinophilic infiltrate which may extend to the perivascular area. Giant cells may be sparse or absent altogether. Thrombosis may be present. It has been also observed that in certain subsets, excision itself may be curative and no corticosteroid therapy was required. Although it remains unclear, whether a full excision biopsy of the affected artery is entirely necessary.
As reviewed by Nesher et al., the mean age of the JTA patients in the review was 23 years (range 7–39). There were 13 males and 2 females. Most of them (12 patients) had unilateral temporal nodule swelling as in our case and bilateral in three cases. In six patients, lesions were painful or tender and in nine cases, it was painless. In this subset of JTA patients, there were no associated symptoms. Although in most cases, lesions were noticed for several weeks or months. Strikingly, ESR, which was noted in 12 of them, was normal. Four patients had eosinophilia out of which one had a history of asthma and others had no history of allergic symptoms.
In this regard, features of JTA described by Lie et al. and others have apparent overlap with Kimura disease and the closely related angiolymphoid hyperplasia with eosinophilia (ALHE). Often, it is difficult to differentiate these even on biopsy. In JTA, inflammatory infiltrates are most often confined to the vessel wall while in Kimura disease and ALHE, they are mainly perivascular, and vascular proliferation is often conspicuous.
Vadlamudi and Schinella mentioned that owing to the proximity to the skin on one side and to the skull on the other side, susceptibility to traumatic injury may be more for temporal arteries and their branches. Hence, it has been hypothesized that trauma itself may result in pseudoaneurysm formation and reactive capillary proliferation. As a result, inflammatory reactions may develop in a subset of these cases. It is also possible that antigenic stimulus is provided by the wall of the temporal arteries. This in turn may trigger an immune reaction since artery excision alone heals JTA while the eosinophil count decrease.
On the other side of the spectrum, the noneosinophilic “elderly-type” TA in the young is even rare and only a few cases have been reported to date in the literature. Their age range was 17–45 years. Nesher et al. reviewed five such patients[4,17-20] out of which two had localized symptoms. Three of the five patients with “elderly-type” GCA had systemic features. No cranial ischemic manifestations were noted. ESR was normal in three patients and raised in two patients. Histology showed intimal hyperplasia and mononuclear cell infiltrates and giant cells in four out of five cases. No evidence of eosinophilic infiltrate was noted in any of them. Most of the patients were treated with short-course corticosteroids with the resolution of symptoms. No further relapse or ischemic complications were seen.
A recent review by Journeau et al. incorporated 44 patients (34 men and 10 women) with a median age of 30 and a maximum of 44. All of them presented with either a lump in the temporal region or prominent temporal arteries. Headache was present in 47.7% of patients. General symptoms were present in 11.4% of patients and 6.8% had a biological inflammatory syndrome. About 34.1% of patients had eosinophilia. About 83.7% of patients presented a single episode and 72.7% had complete excision without further relapses. Histopathology revealed inflammatory cells in the arterial wall in 97.6% of patients with sparse giant cells in 25% and 22.9% had granuloma. Sixty percent of the biopsies had the presence of lymphoid follicles or germinal centers, whereas 82.6% showed perivascular extension of inflammation. About 16.3% of cases showed clinical relapse. A clear distinction between JTA and GCA is important because high-dose corticosteroid therapy is a standard treatment for the latter but is often unnecessary in JTA.[3,4]
Our patient did not show much overlap with the classic/elderly GCA form. Clinically, it had its unique entity with predominantly localized symptoms and signs. Laboratory evaluation and imaging also showed no features of systemic inflammation. Histologically, it stands out uniquely having no giant cells, necrosis, or granuloma. It had thickening of the tunica media and adventitia with the fragmentation of the internal elastic membrane and moderate lymphocytic, neutrophilic, and histiocytic infiltration in intima and media. Thus, it rather conforms with a localized vasculitis restricted to the temporal artery without any systemic involvement. Our patient hence demonstrates and highlights these unique clinicopathologic spectra of JTA and paves way for further exploratory research into the domain of JTA.
Vasculitis of the temporal arteries is rare in the young.[1,4] Our patient emphasizes that TA can present even at a young age. It further demonstrates that this entity can differ significantly from elderly GCA both clinically and histopathologically. It, however, remains a rare and poorly understood entity. Data on the mid- and long-term evolution of this entity in young patients are still inadequate. Therapeutic management is yet not standardized and hence forms an unmet need in this domain of research.
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