Presence Of Aortitis Research Articles

Recent advances in the diagnosis and therapy of large vessel vasculitis.

Large vessel vasculitis (LVV), including Takayasu arteritis (TAK) and giant cell arteritis (GCA), causes granulomatous vascular inflammation mainly in large vessels, and is the most common primary vasculitis in adults. Vascular inflammation may evoke many clinical features including vision impairment, stroke, limb ischemia, and aortic aneurysms. The best way to diagnose LVV is to combine medical history, physical examination, various laboratory tests, and imaging modalities. Progress in imaging modalities facilitated early diagnosis and follow‑up of the disease activity. Conventional angiography is no longer the gold standard for the diagnosis of TAK. Similarly, temporal artery biopsy is no longer the only tool for diagnosing cranial GCA. In selected cases, color Doppler ultrasound may be used for this purpose. Despite some similarities, TAK and GCA differ in many aspects and they are different diseases. They also have different clinical subtypes. The presence of aortitis does not always implicate the diagnosis of TAK or GCA; infectious aortitis, as well as noninfectious aortitis associated with other autoimmune rheumatic diseases should be excluded. Treatment of LVV includes glucocorticoids (GCs), conventional immunosuppressive agents, and biological drugs. Tumor necrosis factor inhibitors are ineffective in GCA but effective in TAK. On the other hand, tocilizumab may be used to treat both diseases. Promising targeted therapies evaluated in ongoing clinical trials include, for example, anti‑IL‑12/23 (ustekinumab), anti‑IL‑17 (secukinumab), anti‑IL‑1 (anakinra), anti‑IL‑23 (guselkumab), anti‑cytotoxic T‑lymphocyte antigen 4 (abatacept), Janus kinase inhibitors (tofacitinib and upadacitinib), anti‑granulocyte / macrophage colony‑stimulating factor (mavrilimumab), and endothelin receptor (bosentan) therapies.

Are Temporal Artery Biopsy Findings Related to PET/CT Findings in Giant Cell Arteritis?

Two clinical subsets of giant cell arteritis have been identified with different histological and CT findings. However, PET/CT findings have not been compared with temporal artery biopsy (TAB). The aims of this study were to describe clinical and histological findings in patients with giant cell arteritis according to the presence or absence of aortitis in PET/CT at the disease diagnosis, and to identify independent factors related to aortic involvement. Patients were included and followed prospectively. Clinical symptoms and TAB findings were recorded. PET/CT was performed in the first 10 days of steroid therapy. Aortitis was defined if a grade 3 uptake on visual analysis was present on arterial wall. Clinical and histological variables were compared according to the presence or absence of aortitis on PET/CT. Multivariate analysis was performed to identify independent factors related to the presence of aortitis. Twenty-seven patients (median age, 77.6 years) were included. PET/CT was performed with a median delay of 5.0 days. Aortitis was observed in 8 patients. Patients with aortitis were younger (69.9 vs 83.7 years, P = 0.04) and had less frequently ischemic manifestations (25.0% vs 84.2%, P = 0.006) than patients without aortitis. Giant multinucleated cells were more frequent on TAB from patients with aortitis (71.4% vs 16.7%), and its presence was an independent risk factor for the occurrence of aortic involvement on PET/CT (odds ratio, 12.2; P = 0.046). Our study shows that giant cells on TAB are associated with the presence of aortitis on PET/CT. Patients with aortic involvement are younger and show less frequently ischemic manifestations.

Risk of ischaemic events at giant cell arteritis diagnosis according to PET/CT findings.

To analyse the risk of ischaemic events in patients with newly diagnosed giant cell arteritis (GCA) according to PET/CT findings. PET/CT was performed during the first 10days of steroid therapy. Clinical manifestations at diagnosis, and physical examination and PET/CT findings were recorded and compared according to the presence or absence of ischaemic symptoms at disease onset. Analysed territories included the ascending aorta, aortic arch, descending aorta, abdominal aorta, carotid arteries, brachiocephalic trunk, vertebral arteries, subclavian arteries and axillary arteries. The study group comprised 30 patients with a median age of 80.8years. Of these patients, 21 (70%) reported ischaemic symptoms at diagnosis, and 13 (43.3%) had permanent visual loss. Of the 30 patients, 77.8% showed large vessel vasculitis (including aortic and vertebral artery involvement) on PET/CT, and 60% had isolated involvement of the vertebral territory. Vertebral arteries were more frequently involved in patients with ischaemic symptoms (OR 5.0, 95% CI 0.99-24.86, p = 0.051). The presence of vertebral artery involvement in the absence of aortic involvement was associated with the presence of ischaemic manifestations (Fisher's exact test, p = 0.001). The presence of aortitis was found to protect against the development of permanent visual loss (OR 19.0, 95% CI 2.79-127.97, p = 0.001). Our findings suggest an association between the vascular pattern on PET/CT at the time of GCA diagnosis and the risk of ischaemic events.

Diagnostics différentiels des aortites inflammatoires

Aortitis are mainly described in inflammatory disorders such as Takayasu arteritis, giant cell arteritis or Behçet's disease. Aortitis is sometimes qualified as idiopathic. However, differential diagnoses must be searched since they need specific interventions. Infectious aortitis should be ruled out first as its rapid evolution and short-term poor prognosis makes it a therapeutic emergency. Furthermore, rarer differential diagnoses should be known as they require specific care that might sometimes differ from the treatment of inflammatory aortitis, such as retroperitoneal fibrosis mostly idiopathic but also secondary to neoplasia or malignant hemopathies. IgG4 related disease, Erdheim-Chester disease and inflammatory abdominal aortic aneurysm due to atherosclerosis are other differential diagnoses to mention in the presence of aortitis in order to adapt patients’ care consequently.

Lack of C4d deposition may reveal susceptibility for ascending aortic dissection

Objectives. Complement activation as evidenced by C4d deposition indicates immunological tissue reactivity. We sought to study the vascular reactivity of the aortic wall by characterizing C4d deposits. Design. Aortic wall histology and immunohistochemistry for C4d, leukocytes, T- and B-lymphocytes, plasma cells, macrophages, endothelial cells, smooth muscle cells, cell proliferation, elastase, and Van–Gieson-staining were performed to 91 consecutive patients that underwent surgery for ascending aorta, and the samples were grouped according to presence of C4d deposits. Results. Fifty-three out of 91 patients had C4d deposits mainly within the adventitia (C4d +), whereas 38 patients lacked C4d deposits (C4d−) including decreased staining of intra-aortic vessels (p < 0.005). Intimal thickness and cellularity, together with inflammation consisting of plasma cells were increased in C4d‐ as compared with C4d + (p < 0.05). Receiver operating characteristic curve (ROC) analysis showed that C4d was associated with stabile nondissecting ascending aorta (AUC 0.792; SE 0.053; p = 0.000; 95% CI 0.688–0.895), but not with presence of aortitis per se (AUC 0.523; SE 0.069; p = 0.752; 95 % CI 0.388–0.658). Conclusions. Lack of C4d may indicate active remodeling of the aortic wall leading to aortic dissection (AD). Immunologic complement factors may be amenable to diagnosis of instability after aortic surgery.

Role of imaging studies in the diagnosis and evaluation of giant cell arteritis in Japanese: report of eight cases

The objective of this study is to clarify the characteristics and imaging results of Japanese patients with giant cell arteritis (GCA). Eight patients with biopsy-proven GCA were enrolled. Their clinical data and imaging results were retrospectively examined from their medical records. All the patients met the criteria for the classification of GCA by the American College of Rheumatology. Although the clinical manifestations are similar to those previously reported, none of the eight patients presented ocular symptoms, and half of them presented jaw claudication. Ultrasonography (US) of temporal artery showed the halo sign in all the patients. Fluorodeoxyglucose positron emission tomography (FDG-PET) was performed in four patients and indicated the presence of aortitis of the patients. US is a quick and noninvasive test to detect inflammation of temporal artery, and FDG-PET is very helpful for early diagnosis of aortitis in GCA. Awareness of the disease and appropriate imaging tests will result in diagnosis of GCA.

Role of imaging studies in the diagnosis and evaluation of giant cell arteritis in Japanese: report of eight cases

The objective of this study is to clarify the characteristics and imaging results of Japanese patients with giant cell arteritis (GCA). Eight patients with biopsy-proven GCA were enrolled. Their clinical data and imaging results were retrospectively examined from their medical records. All the patients met the criteria for the classification of GCA by the American College of Rheumatology. Although the clinical manifestations are similar to those previously reported, none of the eight patients presented ocular symptoms, and half of them presented jaw claudication. Ultrasonography (US) of temporal artery showed the halo sign in all the patients. Fluorodeoxyglucose positron emission tomography (FDG-PET) was performed in four patients and indicated the presence of aortitis of the patients. US is a quick and noninvasive test to detect inflammation of temporal artery, and FDG-PET is very helpful for early diagnosis of aortitis in GCA. Awareness of the disease and appropriate imaging tests will result in diagnosis of GCA.

Infrarenal Aortic Infection and Rupture of a Pseudoaneurysm Secondary to Migration of Proximal Aortic Patch: Case Report and Literature Review

Aneurysm formation at the site of prior aortic coarctation patch repair is well documented. Migration of the patch though to the infrarenal aorta, as in this case, with subsequent aortitis and pseudoaneurysm formation has not been described. A 16 y old boy who was operated at the age of 10 for his aortic coarctation, presented with sepsis and abdominal pain. CT scan confirmed the presence of aortitis and an aortic pseudoaneurysm. This was treated with resection of the infrarenal aorta and replacement with superficial femoral vein. The PTFE patch from the previous coarctation repair was found and extracted from the infrarenal aorta.