Juvenile temporal arteritis associated with Kimura's disease

A case of juvenile temporal arteritis, which is a rare vascular lesion in children and young adults, associated with Kimura's disease in a healthy 23-year-old asymptomatic man is described. The patient presented with a painless 2.5 cm nodule with eosinophilia and normal erythrocyte sedimentation rate. Histologically, the left superficial artery showed marked intimal thickening with moderate eosinophilic infiltrates, constriction of the vascular lumen, focal disruptions of the internal elastic lamina and media, moderate eosinophilic infiltrates in the adventia, and absence of giant cells. The subcutaneous tissue surrounding the artery was characterized by lymphofollicular hyperplasia, marked eosinophilic infiltrates in the intra- and extra-follicles with abscess, capillary proliferations, lymphocytic, plasma cell and mast cell infiltrates, and fibrosis in the interfollicular region. Immunohistochemically, reticular, positive IgE staining was observed in the germinal centers. Clinically and histologically, the lesion was consistent with juvenile temporal arteritis associated with Kimura's disease. The findings indicate that both entities are closely related and juvenile temporal arteritis may be secondary to Kimura's disease.     

Surgical anatomy of the superficial temporal artery to prevent facial nerve injury during arterial biopsy

To investigate the topographical relationship between the frontal branch of the superficial temporal artery (FSTA) and the temporal branch of the facial nerve (TFN) with the aim of preventing nerve injury during FSTA biopsy. Fifty‐seven hemifaces of 33 cadavers were dissected. Vertical lines drawn to the lateral orbital margin (LOM) and the superior root of the helix were used as the anterior and posterior reference positions, respectively. Horizontal lines drawn through the supraorbital margin and lateral canthus were used as the superior and inferior reference points, respectively. The depth and course relationships of the FSTA and TFN were examined. Midpoints between the FSTA and TFN are situated approximately 6.0 and 4.5 cm posterior to the lateral orbital margin at the levels of the lateral canthus and supraorbital margin, respectively. The TFN is generally situated 1–2 cm anteriorly and inferiorly to the FSTA in the temporal region. However, in two cases (3.6%), the TFN ran just underneath the FSTA with only a very small safe distance, making it highly vulnerable to iatrogenic injury. In conclusion, when performing an FSTA biopsy, the surgeon should not dissect below the superficial temporal fascia because there is an overlap between the course of the FSTA and the TFN in a minority of cases. Also, surgical incisions should be made outside the area delineated by an oblique line passing through the points 6.0 and 4.5 cm posterior to the lateral orbital margin at the levels of the lateral canthus and the supraorbital margin, respectively. Clin. Anat. 31:608–613, 2018. © 2017 Wiley Periodicals, Inc.     

A unique autopsy case of ascending aortic dissection caused by giant cell arteritis without drug therapy

Giant cell arteritis is a granulomatous inflammation of large and medium‐sized arteries, occurring predominantly in older women. In this case, a 76‐year‐old woman was hospitalized for examination because of a high C‐reactive protein (CRP) level, but nothing remarkable was found on thoracicoabdominal computed tomography (CT) or head magnetic resonanse imaging (MRI). On the 46th day from the first visit, she died suddenly due to cardiac tamponade. On pathological autopsy, we found the cause of death to be acute aortic dissection (Stanford type A) due to giant cell arteritis occurred in the ascending aorta. Histologically, granulomatous vasculitis with giant cells was recognized in the ascending aorta, thoracic descending aorta and abdominal aorta and their branches. Interestingly, similar granulomatous vasculitis was also found in the medium and small vessels of other plural organs, including the heart, liver, uterine corpus, and its appendages. To our knowledge, giant cell arteritis with multiple‐organ granulomatous changes has not been reported before. We herein reported a unique autopsy case of giant cell arteritis in a patient not treated with medication.     

Infection and temporal arteritis: a PCR-based study to detect pathogens in temporal artery biopsy specimens

The possibility of infectious triggers stimulating the development of inflammatory vascular diseases has generated much recent interest. This study uses PCR to detect the presence of Chlamydia pneumoniae, parvovirus B19 and all the human herpes viruses except HHV8 in temporal artery biopsy specimens. Samples from 37 temporal artery biopsies with histological evidence of arteritis and 66 samples from histologically negative temporal artery biopsies, all from different patients, were negative for C. pneumoniae, HSV, VZV, EBV, and HHV7 DNA. Two of the 37 histologically positive specimens were positive for HHV6, another two for CMV and a further two for parvovirus B19 DNA. Parvovirus B19 DNA was also detected in five histologically negative biopsies, one positive for HCMV DNA and a further one was positive for HHV6 DNA. There is no statistically significant difference to the presence of virus DNA in the two types of specimens (P = 0.538). This study does not support a role for C. pneumoniae, parvovirus B19 or human herpes viruses in the pathogenesis of temporal arteritis.     

Medin and medin-amyloid in ageing inflamed and non-inflamed temporal arteries.

Small amyloid deposits commonly occur along the internal elastic lamina of the temporal artery. In temporal artery biopsies from 22 patients with histological signs of giant cell arteritis and 25 without, amyloid deposits were found in 14 and 21 biopsies, respectively. Two specific peptide antisera show that this amyloid is identical to the recently identified medin-amyloid in the ageing aorta. On immunoelectron microscopy, the amyloid appeared topographically closely related to the elastic material. Furthermore, fragmented elastic material was often immunolabelled for medin and found to be engulfed by giant cells. Medin is an internal fragment of the larger precursor lactadherin and is presumably formed by specific enzymatic cleavage events. In situ hybridization showed that lactadherin is expressed locally by smooth muscle cells of the temporal artery. Given the potential role of lactadherin as a mediator for the adhesion of cells, including macrophages, to other cells or surfaces, lactadherin or its fragment medin may be important in the inflammatory process in giant cell arteritis.     

Association between giant cell (temporal) arteritis and HLA-Cw3

Thirty-five Caucasian patients with giant cell (temporal) arteritis were typed for HLA class I and II antigens. A significant increase was found for A31, B40, Cw3, and DR4. HLA-Cw3 was the most frequent antigen observed (57%) and had the highest relative risk (5.65), suggesting that Cw3 may be the primary HLA risk factor for this disease. The increased occurrence of A31, B40, and DR4 is probably secondary to their association with Cw3.     

Neutrophil and endothelial cell activation in the vasa vasorum in vasculo-Behçet disease

AIM: The aim of this study was to analyse the immunopathological mechanisms of vasculo-Beh?et disease, which were also compared to cases of Takayasu's arteritis and inflammatory aneurysm to evaluate differences in inflammatory mechanisms. METHOD AND RESULTS: We reviewed six cases of vasculo-Beh?et disease, four of Takayasu's arteritis and seven inflammatory aneurysms which underwent surgical repair. Immunohistochemical studies were performed on paraffin-embedded tissue using a labelled streptavidin-biotin method, as was in-situ hybridization for Epstein-Barr virus. Microscopically, neutrophils and lymphocytes accumulated around the vasa vasorum. Neutrophils were prominent as compared to Takayasu's arteritis and inflammatory aneurysm. Elastic fibres were not severely destroyed. Endothelial cells (ECs) of most vasa vasorum expressed HLA-DR. The number of vasa vasorum around which inflammatory infiltrating cells were observed in vasculo-Beh?et disease was significantly greater than in inflammatory aneurysms and Takayasu's arteritis (P < 0.001). The cytokines IL-1alpha, TNF-beta and IFN-gamma were expressed in neutrophils and lymphocytes which were distributed around vasa vasorum, as well as neutrophils adherent to HLA-DR positive ECs. CONCLUSION: Our results suggest that vasculo-Beh?et disease should be classified as a neutrophilic vasculitis targeting the vasa vasorum. Aneurysm formation may be related to degeneration of arterial wall caused by inflammation of the vasa vasorum.     

Infantile disseminated visceral giant cell arteritis presenting as sudden infant death

The rare clinicopathological entity 'disseminated visceral giant cell arteritis' (DVGCA) was first described in 1978. It is characterized by widespread small-vessel giant cell angitis and extravascular granulomas. A normal and healthy 7-month-old boy who presented unexpectedly with sudden infant death syndrome (SIDS) is reported. Histological examination at autopsy revealed giant cell angitis of the aorta, common carotid, coronary, pulmonary, celiac, mesenteric and common iliac arteries. There were also granulomas in the tracheal wall and liver. To our knowledge, this is the first documented case of DVGCA occurring in an infant younger than 12 months of age. A review of the literature on DVGCA is presented in this report, and the differential diagnosis is discussed.     

颞浅动脉额支的观测及其临床意义

目的为颞动脉活检提供颞浅动脉额支相关的解剖学资料。方法对16具防腐成人尸体标本进行解剖,对颞浅动脉额支进行相关的解剖学观测、测量。结果①颞浅动脉额支在起点和末梢分叉点之间的长度为(45.93±7.29)mm;②颞浅动脉额支在起点和末梢分叉点的坐标值分别为(22.95±3.81,17.32±4.54)mm和(59.93±8.94,37.48±5.37)mm;③颞浅动脉额支在起点和末梢分叉点之间直线相关,直线回归方程为y赞=7.06+0.50X,相关系数r=0.68,P<0.001。结论深入了解颞浅动脉额支的解剖学特点及其与周围结构的解剖学关系,有利于颞动脉活检中颞浅动脉额支的定位及防止周围结构的损伤。     

An autopsy case of aortic dissection due to giant cell arteritis

Giant cell arteritis (GCA) is a systemic vasculitis affecting mainly large and medium-sized arteries. GCA sometimes involves the aorta and its major branches and causes aortic dissection as a rare complication. We have experienced an autopsy case of aortic dissection due to GCA. The patient was an 87-year-old Japanese woman with Stanford type A aortic dissection who died 7 days after admission. Two years previously she had been diagnosed as having abdominal aortic aneurysm and undergone endovascular aneurysm repair (EVAR). Although she had no characteristic symptoms of GCA, autopsy revealed marked granulomatous inflammation in the dissected area and coronary arteries. Active arteritis was evident not only in the arteries of the upper extremity but also those in the lower extremity. Granulomatous inflammation was not evident in the aneurysm. The aortic dissection might have been an initial manifestation of GCA. We report the regions of GCA extension and its histology in detail.     

An approach to the diagnosis and management of systemic vasculitis

The systemic vasculitides are a complex and often serious group of disorders which, while uncommon, require careful management in order to ensure optimal outcome. In most cases there is no known cause. Multi‐system disease is likely to be fatal without judicious use of immunosuppression. A prompt diagnosis is necessary to preserve organ function. Comprehensive and repeated disease assessment is a necessary basis for planning therapy and modification of treatment protocols according to response. Therapies typically include glucocorticoids and, especially for small and medium vessel vasculitis, an effective immunosuppressive agent. Cyclophosphamide is currently the standard therapy for small vessel multi‐system vasculitis, but other agents are now being evaluated in large randomized trials. Comorbidity is common in patients with vasculitis, including the cumulative effects of potentially toxic therapy. Long‐term evaluation of patients is important in order to detect and manage relapses.     

An Uneven Expression of T Cell Receptor V Genes in the Arterial Wall and Peripheral Blood in Giant Cell Arteritis

The aim of the study was to investigate T cell receptor (TCR) usage at the time of diagnosis of giant cell arteritis (GCA) and to estimate the degree of clonality of T-cells infiltrating the lesion. Seven patients with biopsy-proven giant cell arteritis were included in the study. Immunocytochemistry in biopsies from the temporal arteries and flow cytometric analysis of peripheral blood lymphocytes (PBL) was performed using monoclonal antibodies specific for CD3, CD4 and CD8 and 13 TCR Vα and Vβ gene segment products. The CDR3 fragment length polymorphism was assessed by gel electrophoresis of PCR-amplified TCR segments. The T lymphocytes were found to be concentrated to the adventitia rather than the media or intima. Six of the seven patients with GCA had expansions of T lymphocytes, expressing selected TCR V genes in the arterial wall. None of these expansions was found in PBL. The infiltrating T-cells were poly- or oligoclonal. In conclusion, the dominating part of the inflammatory infiltrate in GCA emanates from the adventitial microvessels. There is an uneven expression of TCR V genes by T lymphocytes in the inflammatory infiltrates as compared to peripheral blood T lymphocytes at the time of diagnosis, consistent with an antigen-driven immunological reaction in the arterial wall.     

Giant cell arteritis localized to the colon associated with Crohn's disease

AIMS: Intestinal vasculitis is uncommon and usually accompanies systemic vasculitis. Although intestinal vascular changes including vasculitis have been studied intensively, and are found regularly in Crohn's disease, giant cell arteritis is distinctly unusual. We describe a case of giant cell arteritis localized to the colon of an 18-year-old girl suffering from Crohn's disease. METHODS and RESULTS: After three years of medical treatment, the patient underwent a proctocolectomy. The medium-sized arteries of the mesocolon demonstrated striking changes characterized by intimal fibrous thickening and an inflammatory infiltrate with giant cells, most predominant at the intima-media junction. Epithelioid cells and sarcoid-like granulomas were not observed. The internal elastic lamina was fragmented. Neither clinical symptoms nor laboratory findings showed evidence of systemic vasculitis. Neither the chest CT scan nor the echo-Doppler of the temporal arteries, supra-aortic and abdominal vessels revealed any abnormality. CONCLUSIONS: This case illustrates an extremely rare feature in the spectrum of vascular lesions in Crohn's disease which have to be differentiated from temporal and Takayasu's arteritis.     

Standard and biological treatment in large vessel vasculitis: guidelines and current approaches

Introduction: Giant cell arteritis and Takayasu arteritis are the two major forms of idiopathic large vessel vasculitis. High doses of glucocorticoids are effective in inducing remission in both conditions, but relapses and recurrences are common, requiring prolonged glucocorticoid treatment with the risk of the related adverse events.

Areas covered: In this article, we will review the standard and biological treatment strategies in large vessel vasculitis, and we will focus on the current approaches to these diseases.

Expert commentary: The results of treatment trials with conventional immunosuppressive agents such as methotrexate, azathioprine, mycophenolate mofetil, and cyclophosphamide have overall been disappointing. TNF-α blockers are ineffective in giant cell arteritis, while observational evidence and a phase 2 randomized trial support the use of tocilizumab in relapsing giant cell arteritis. Observational evidence strongly supports the use of anti-TNF-α agents and tocilizumab in Takayasu patients with relapsing disease. However biological agents are not curative, and relapses remain common.     

The arteritides

This article reviews the histopathological, clinical and immunological features of the arteritides. Based on these criteria, a classification scheme is proposed that includes infectious and non-infectious causes. Included in the non-infectious arteritides are: hypersensitivity vasculitis including serum sickness, Henoch–Schönlein purpura, mixed cryoglobulinaemia, hypocomplementaemia, drug and malignancy-associated vasculitis; arteritides of small and medium-sized arteries including polyarteritis nodosa, Kawasaki's disease, Wegener's granulomatosis, Churg–Strauss syndrome, necrotizing sarcoid granulomatosis, thromboangiitis obliterans (Buerger's disease) and localized forms of arteritis; arteritides involving large, medium and small-sized arteries which includes giant cell (temporal) arteritis, Takayasu's disease and arteritis of collagen-vascular disease (rheumatoid arthritis, rheumatic fever, Behçet's disease, Sjögren's syndrome, systemic lupus erythematosis and systemic sclerosis).     

Temporal (granulomatous) arteritis: a histopathological study of 32 cases

Thirty-two cases of temporal arteritis were reviewed. All patients were adults with a mean age of 69.6 years, and no sex predominance. The erythrocyte sedimentation rate was raised and there was a good response to steroid therapy in the 29 patients who were adequately documented and followed up. Detailed histopathological study of the temporal artery biopsies showed three main histopathological variants; 12 cases (37.5%) had predominantly intimal proliferative changes, four cases (12.5%) had granulomatous inflammation without giant cells and 16 (50%) had granulomatous inflammation with giant cells. The predominantly intimal change variant, consisting of a cellular proliferation of fibroblasts and myointimal cells with little or no changes in the media represent an active pathological process and not a healed disease as has been previously suggested. The internal elastic lamina showed abnormal features in all three morphological variants. The need to recognize this variant, the temporal relationship between these variants and the need to study multiple sections are discussed. Biopsy of clinically normal temporal arteries in patients suspected of having temporal arteritis is recommended as some of these vessels may show disease. No correlation was observed between the clinical picture and the histopathological findings.     

Parietal centripetal and centrifugal thickening neovascularization in the descending anterior coronary artery

Summary Left coronary arteries of 30 human hearts, obtained at autopsy, were injected with contrast medium. A control group was formed from anterior descending coronary arteries free of atherosclerosis and a study group from anterior descending coronary arteries with areas of atherosclerotic injury. The following differences in the two groups were noted. The control group did not show successfully injected vessels in intima and media, while cases with atherosclerotic injury have them; the number of injected vessels in presence of atherosclerotic injury was three times greater than in healthy coronary arteries; there was a decreasing gradient from outside to in, in the number of injected vessels in both groups; and finally in atherosclerotic vessels we noted a lack of balance between parietal thickening and the residual lumen (conspicuous thickening was accompanied by a small reduction in the lumen). We interpret centrifugal thickening as a possible compensatory mechanism in the major branches for an inadequate canalization of vessel, and suggest possible formation of coronary collateral circulation from vasa vasorum by a process of neovascularization.