GCA is a granulomatous vasculitis of unknown origin and targets large vessels with predominance for the aortic arch and the cranial branches [1, 2, 6]. Most patients in GCA present cranial manifestations described above; thus, GCA was previously called temporal arteritis. GCA usually occurs in elderly women over the age of 50 in western countries, while the incidence of GCA in Asian is relatively rare compared to that of Takayasu’s arteritis [2].
Some authors have recently reported a few subgroups of GCAs [3, 7,8,9,10]. Hayreh et al. investigated 85 GCA patients, of which occult GCA, defined as ocular involvement without any systemic symptoms, occupied 21% [7]. De Boysson et al. presented 143 GCA patients, and GCA without cranial manifestations occupied 22% [3]. According to studies in GCA without cranial symptoms, the characteristics were described as follows: lower inflammatory laboratory parameters, more frequent large arterial involvement, and less disease relapse [3, 7, 8]. Therefore, GCA should be increasingly recognized as a systemic vascular disease even if patients have no typical cranial manifestations in GCA.
Recent studies have suggested that GCA quite often manifests exclusively in large arteries, i.e., the aorta and proximal branches, with specific symptoms frequently not present. This has been referred to as “extracranial” GCA [5, 10]. Extracranial manifestations due to GCA are not commonly reported, and only 9% of patients with GCA exhibit involvement of extracranial sites such as the thorax, abdomen, and pelvis [4]. The diagnosis of extracranial GCA can be elusive because of the frequent paucity of symptoms [11, 12].
Mesenteric ischemia in GCA, which is one of the extracranial GCA entities, was first described by Hamrin et al. in 1965 [13], and since then, only 30 patients have been reported in English and French literatures [1, 11, 14,15,16]. Scola et al. reviewed 12 cases of mesenteric involvement in GCA and showed that survival was observed in only 50% [16]. The authors suggest that mesenteric vasculitis, only rarely described in GCA, represents serious complication resulting in small bowel infarction [14,15,16]. Grayson et al. analyzed massive cohort studies and showed that the frequency of arteriographic lesions of the mesenteric artery in GCA was 18% [17] and thus reports of mesenteric involvement in GCA may increase in future.
We reviewed our patient’s clinical course. She denied cranial symptoms such as temporal scalp pain, visual disturbance, masseter pain, or stroke. Extremity claudication was not determined. We examined additional studies containing laboratory tests and positron emission tomography (PET) imaging. Complements C3 and C4, hepatitis B and C serologies, antinuclear antibodies, and antineutrophil cytoplasmic antibodies were at normal range. PET imaging studies did not show cranial GCA nor extracranial GCA, namely no remarkable GCA findings of the aorta and other main branches were determined. According to our patient’s past medical history, she had already undergone abdominal contrast-enhanced CT 5 years prior to this clinical episode, at 71. The CT images did not show any typical GCA characteristics at the mesenteric arteries; thus, the patient’s clinical entity of mesenteric involvement in GCA might have gradually occurred during these 5 years in her 70s. Although neither perforation nor small bowel infarction was found in our case, and she could have suffered life-threatening critical episode prior to the surgical intervention.
Furthermore, we retrospectively studied our patient in histopathological finding. She presented an acute abdomen in extracranial GCA without any typical cranial symptoms. The thickening small bowel mesentery included a large mass, which was composed of small massive nodules and showed typical mesenteric vasculitis in GCA. The mesenteric tumor formation caused by GCA may have also induced massive ascites. Histological features also displayed intimal thickening and granulomatous inflammation, including giant cells, lymphocytes, and fragmented elastic lamina. In our case, these typical characteristics of GCA were determined in both the vein and the intestinal wall (Fig. 3). In histopathological, an excised specimen should be necessary to examine not only arteries but also veins and other organs for the patients with extracranial GCA.
