[Large vessel vasculitis: update 2021]. / Update Großgefäßvaskulitis 2021.

In recent years, clinically significant advances have been made in the management of giant cell arteritis and Takayasu arteritis. This concise review article highlights important aspects of the diagnostic workup and imaging-based treatment surveillance of the large vessel vasculitides.

Ultrasonic Implantation and Imaging of Sound-Sensitive Theranostic Agents for the Treatment of Arterial Inflammation.

For site-specific diseases such as atherosclerosis, it is desirable to noninvasively and locally deliver therapeutics for extended periods of time. High-intensity focused ultrasound (HIFU) provides targeted drug delivery, yet remains unable to sustain delivery beyond the HIFU treatment time. Furthermore, methods to validate HIFU-enhanced drug delivery remain limited. In this study, we report on HIFU-targeted implantation of degradable drug-loaded sound-sensitive multicavity PLGA microparticles (mcPLGA MPs) as a theranostic agent for the treatment of arterial lesions. Once implanted into the targeted tissue, mcPLGA MPs eluted dexamethasone for several days, thereby reducing inflammatory markers linked to oxidized lipid uptake in a foam cell spheroid model. Furthermore, implanted mcPLGA MPs created hyperechoic regions on diagnostic ultrasound images, and thus noninvasively verified that the target region was treated with the theranostic agents. This novel and innovative multifunctional theranostic platform may serve as a promising candidate for noninvasive imaging and treatment for site-specific diseases such as atherosclerosis.

Adipose tissue-derived stem cells suppress coronary arteritis of Kawasaki disease in vivo.

BACKGROUND: Kawasaki disease (KD) is a systemic inflammatory disease resulting in an acute febrile syndrome commonly affecting children younger than 5 years. Coronary arteritis in KD is occasionally non-responsive to several treatments. Recently, adipose tissue-derived stem cells (ADSCs) have been shown to have anti-inflammatory, immunosuppressive, and tissue-repair characteristics and are considered a useful treatment for inflammatory disease. The present study aimed to elucidate whether the administration of ADSCs can suppress KD-associated vasculitis in vivo. METHODS: Candida albicans water-soluble fraction is often used to model KD via the induction of severe coronary arteritis. Kawasaki disease model mice were intravenously administered ADSCs and phosphate-buffered saline (PBS). On day 29, the mice were sacrificed and hearts from mice in each group were dissected. This was followed by serum collection. Cardiac tissue sections were subjected to histopathological examination to evaluate the inflammatory area. The levels of pro-inflammatory cytokines in the serum were analyzed at days 15 and 29. The survival rates of both groups were compared. RESULTS: The mean inflammatory area in coronary arteritis was significantly lower in the ADSC group compared to the PBS group (P < 0.01). Furthermore, the levels of pro-inflammatory cytokines, such as IL-1ß, IL-12, IL-17, RANTES, INF-γ, and TNF-α, in the ADSC group were significantly lower than those in the PBS group. Moreover, the ADSC group had a significantly higher survival rate than the PBS group. CONCLUSIONS: These findings highlight that ADSCs have anti-inflammatory and immune regulatory functions that could provide novel cell-based therapeutic strategies for severe KD.

Characteristics and prognosis of IgG4-related periaortitis/periarteritis: A systematic literature review.

OBJECTIVE: Immunoglobulin G4 (IgG4)-related disease is a systemic chronic fibroinflammatory disease that can affect almost every organ of the body. IgG4-related periaortitis/periarteritis is a newly recognized subset of IgG4-related disease, and its characteristics and prognosis remain unclear. We investigated the clinical characteristics and prognosis of IgG4-related periaortitis/periarteritis. METHODS: We performed a systematic literature review of IgG4-related periaortitis/periarteritis. Additionally, we have summarized the characteristics and prognosis of IgG4-related coronary arteritis. RESULTS: We investigated 248 patients with IgG4-related periaortitis/periarteritis. All studies reported the condition in elderly patients, and male predominance was observed. The infra-renal abdominal aorta and iliac arteries were the most commonly affected sites. Most reports showed the serum C-reactive protein elevation in this disease entity, in contrast to non-vascular IgG4-related disease. Based on radiological findings observed in 27 patients with IgG4-related coronary arteritis, vasculitic lesions were classified into 3 types: stenotic (67% of patients), aneurysmal (42%), and diffuse wall thickening type (92%). Serum IgG4 level, but not C-reactive protein level, was associated with the number of affected organs in IgG4-related coronary arteritis. Corticosteroid treatment with or without cardiac surgery or percutaneous coronary intervention was effective in most patients with IgG4-related coronary arteritis; however, 33% of patients showed an unfavorable clinical course including disease progression, relapse, or death. Pre-treatment stenosis and/or aneurysms were associated with progression of stenosis or aneurysm after corticosteroid treatment. CONCLUSION: Most clinical characteristics were similar between the IgG4-related periaortitis/periarteritis and the non-vascular IgG4-related disease groups; however, serum C-reactive protein level elevation was observed only in the former. Although corticosteroid treatment was effective, this disease can be life-threatening secondary to myocardial infarction, aortic dissection, and aneurysmal rupture. Pre-treatment evaluation of stenosis or aneurysms is important for predicting progression of stenosis or aneurysm after corticosteroid treatment.

[A Case of Subdural Empyema with Cerebral Arteritis and Brain Ischemia in the Middle Cerebral Artery Distribution, Secondary to Odontogenic Maxillary Sinusitis].

We present a rare case of subdural empyema with cerebral arteritis and brain ischemia in the middle cerebral artery distribution secondary to odontogenic maxillary sinusitis. A 32-year-old man was admitted to our hospital because of high fever and generalized convulsions. Computed tomography(CT)and magnetic resonance imaging(MRI)showed subdural empyema at the left convexity, with a small amount of air. An interruption of the right maxillary sinus floor corresponding to the alveolar process was evident on coronal CT. He was diagnosed as having subdural empyema caused by odontogenic maxillary sinusitis. MR angiography showed stenosis of the left middle cerebral artery(MCA). Despite antibiotic administration, he became drowsy and developed aphasia with right hemiparesis. Repeat MRI showed enlargement of the encapsulated subdural empyema with increased midline shift to the right. We performed prompt surgical evacuation with craniotomy, endoscopic drainage of the sinusitis, and tooth extraction. A hyperintense lesion was observed on subsequent diffusion-weighted imaging in the left MCA distribution. After repeat drainage of the re-enlarged subdural empyema, he was discharged without apparent neurological deficits. This case indicates that subdural empyema from odontogenic sinusitis requires a suitable imaging study of the brain, head, and neck region, and a multidisciplinary approach involving a neurosurgeon, otolaryngologist, and oral surgeon. Prompt initiation of appropriate antibiotic therapy with surgical intervention is recommended for treatment of subdural empyema from odontogenic sinusitis.

Lymphocytic Thrombophilic Arteritis: A Review.

Macular lymphocytic arteritis or lymphocytic thrombophilic arteritis (LTA) is a recently described cutaneous arteritis that is characterized by asymptomatic macules and patches of the extremities associated with lymphocytic arteritis at the deep dermal/subcuticular junction for which little information exists on demographics, evaluation, and management. There has been recent debate in the literature whether this disease is a new distinct entity, misdiagnosed cutaneous polyarteritis nodosa (cPAN), or a disease on a spectrum with cPAN. We systematically review the literature on demographic information, medical history, histopathology findings, and treatment management to analyze trends and clarify controversies in characterizations of LTA. Forty detailed cases of LTA have been published. We submit that, although literature is limited, a review of the data still suggests that LTA is distinct from cPAN and systemic PAN. In addition, to better reflect the pathophysiologic natural history of this condition and correct for the historical artifact of how the disease was identified, we encourage the disease to be referred to as LTA and discourage ongoing use of macular lymphocytic arteritis.

IgG4-related periaortitis/periarteritis: An under-recognized condition that is potentially life-threatening.

Periaortitis and periarteritis are one of the clinical features of IgG4-related disease. Such vascular manifestations are reported to be present in 10-30% of overall IgG4-related disease, and they may also appear as an isolated lesion of IgG4-related disease. As other types of large vessel vasculitis, IgG4-related vasculitis may cause significant clinical consequences. On the other hand, the diagnosis of IgG4-related periaortitis/periarteritis seems to have been made on the basis of different diagnostic criteria in reported studies; thus far, difficulty seems to be present in assessing the true frequency of the disease as well as determining the optimal therapeutic strategy. In this review, in addition presenting the clinical and pathological features of IgG4-related periaortitis/periarteirtis, we would like to discuss on what kind of points should be resolved for the better understanding of vascular involvement of IgG4-related disease in terms of diagnosis and treatment.

Fifty-one cases of fungal arteritis after kidney transplantation: A case report and review of the literature.

Fungal arteritis affecting graft arteries is a rare but life-threatening complication in kidney transplantation (KT). Here, we report the case of a patient with Aspergillus arteritis who experienced renal artery rupture 8 days after KT. We also reviewed 50 other reported cases of fungal arteritis after KT. We found that fungal contamination can occur during kidney graft harvest, preservation, and/or transplantation. Typically, early diagnosis, timely antifungal treatment, and emergency surgery seem crucial for avoiding life-threatening vascular complications.

Sclerosing Aortic and Coronary Arteritis Due to IgG4-Related Disease.

We present the case of a 55-year-old woman admitted for a coronary artery bypass operation because of three-vessel coronary artery disease based on angiographic findings and clinical symptoms. Unexpected intraoperative findings with diffuse tissue thickening of the ascending aorta and coronary arteries indicated an alternate pathogenesis rather than coronary artery atherosclerosis. Histopathologic findings and clinical evaluation could confirm IgG4-related disease (IgG4-RD). IgG4-RD is a newly recognized fibroinflammatory condition that can present in a variety of organs and is characterized by common histopathologic features. Low disease awareness among clinicians makes this condition underdiagnosed.

Cannabis arteritis.

Cannabis is the most consumed psychoactive substance by young people. Chronic use of cannabis can lead to cannabis arteritis, which is a very rare peripheral vascular disease similar to Buerger's disease. It is affecting young adults, especially men, consuming cannabis. A 27-year old woman, with no particular past medical history except for long-term use of cannabis and tobacco developed a digital necrosis in the left hand. She denied using other illicit drugs. Doppler ultrasound examination of the upper limbs was unremarkable. Toxicological analysis revealed the presence of cannabis in both biological fluid and hair strand. Despite medical treatment, cessation of the cannabis and tobacco consumption and hyperbaric oxygen therapy, an amputation of necrotic parts was then required. This case shows the prolonged use of cannabis could be a risk factor for young adult arteritis. Faced with a rapidly progressive arteritis occurring in young adult, the physician should consider the history of use of cannabis. Hair analysis can be useful for confirmation of the chronic consumption of drugs.

Cannabis arteritis: ever more important to consider.

Cannabis arteritis (CA) is a major and underdiagnosed cause of peripheral arterial disease in young patients. A 34-year-old man, daily smoker of 20 cigarettes and two cannabis cigarettes for 14 years, presented with a necrotic plaque of left hallux for 3 weeks. The Doppler ultrasound and angiography were compatible with severe Buerger's disease. Submitted to a revascularisation procedure and hypocoagulation with rivaroxaban. He had ceased smoking but maintained consumption of cannabis. Owing to the persistence of distal necrosis, amputation of the hallux was performed with good evolution. CA is a subtype of Buerger's disease. It is poorly known but increasingly prevalent and manifests in cannabis users regardless of tobacco use. The drug is considered at least a cofactor of the arteriopathy. The most effective treatment is cessation of consumption. Being cannabis one of the most consumed drugs, its mandatory to ask about its use in all young patients with arteriopathy.

Gastrointestinal aspects of vasculitides.

Systemic vasculitides are caused by inflammation of blood vessels and can affect any organ and any part of the gastrointestinal tract, hepatic and biliary system, as well as the pancreas. These disorders can cause a wide array of gastrointestinal manifestations, from asymptomatic elevated transaminase levels and mild abdominal pain to potentially life-threatening bowel perforations and peritonitis. A diagnosis based solely on gastrointestinal symptoms is challenging as these manifestations are not specific. Conversely, diagnostic and therapeutic delays can be rapidly detrimental. In this article, we review the epidemiology, characteristics and management of the main gastrointestinal manifestations of systemic vasculitides, including polyarteritis nodosa and antineutrophil cytoplasm antibody-associated vasculitides, as well as isolated vasculitides limited to the gastrointestinal tract.

iMAP™ imaging of tumorous lesions surrounding the coronary arteries in a patient with an elevated serum level of immunoglobulin G4.

A 76-year-old woman with multiple coronary risk factors was admitted to our hospital because of episodes of new-onset chest pain that had begun 3 days previously. She underwent percutaneous coronary intervention (PCI) for severe stenoses in the two high lateral (HL) branches. Intravascular ultrasound (IVUS) revealed massive stenotic lesions in the HL branches and tumorous nonstenotic lesions in the left anterior descending coronary artery (LAD) and the left circumflex coronary artery (LCx). iMAP™, optical coherence tomography (OCT), and coronary computed tomography angiography (CCTA) were performed. iMAP depicted fibrosis in the vessel (green areas) and nonfibrotic tissue change suggestive of inflammation outside the vessel (yellow/red areas). OCT revealed high-intensity homogenous intimal hyperplasia with superficial calcification, and CCTA showed massive periarterial soft lesions in the HL, LAD, and LCx. The serum IgG4 level was high at 252-427 mg/dL (8 measurements) (reference range, 4.8-105.0 mg/dL). We suspected IgG4-related coronary periarteritis on the basis of the comprehensive diagnostic criteria as a possible diagnosis. The clinical course was good after initial and subsequent PCIs for both the HL stenoses and the progressing LCx stenosis, and there was no recurrence of angina pectoris thereafter. Steroids were not administered because the massive lesions did not enlarge during the 16 months of follow-up. iMAP was able to evaluate the tissue characteristics of tumorous lesions in the stenosed HL branches and the nonstenotic LAD and LCx in a patient with an elevated level of IgG4.

Non-Arteritic Anterior Ischemic Optic Neuropathy (NAION): A Review and Update on Animal Models.

Non-arteritic anterior ischemic optic neuropathy (NAION) is a relatively common optic neuropathy in adults over the age of 50, typically causing sudden, painless, unilateral vision loss and always resulting in swelling of the optic nerve head. Though several anatomic and vascular risk factors have been identified, much remains unknown about its pathophysiology and there is no proven treatment. This article reviews the risk factors, clinical presentation, and therapies that have been investigated for NAION. In addition, we provide an update from recent rodent and primate models, which offer new insight into the pathophysiology of this disease and provide a platform for treatment trials.

Infectious arteritis of the internal carotid artery complicating retropharyngeal abscess.

INTRODUCTION: Retropharyngeal abscess is a well-known entity in children, but can also occur in adults. The two main vascular complications are vascular compression and pseudoaneurysm, while infectious arteritis of the internal carotid artery is exceptional. CASE REPORT: The authors describe a case of a retropharyngeal abscess in an adult woman complicated by infectious arteritis of the internal carotid artery. This rare complication was treated by endovascular occlusion of the internal carotid artery and incision and drainage of the abscess in combination with antibiotic and anticoagulant therapy. The patient did not present any neurological sequelae and follow-up MRI did not reveal any signs of vascular or neurological complications. DISCUSSION: This case highlights the importance of thorough examination of imaging performed in the context of deep neck space abscess to detect signs of vascular involvement. Treatment must be aggressive in view of the life-threatening risk of arterial rupture or septic embolism. This is the first reported case of infectious arteritis involving the internal carotid artery complicating retropharyngeal abscess.

Current concepts in the diagnosis, pathogenesis and management of nonarteritic anterior ischaemic optic neuropathy.

Nonarteritic anterior ischaemic optic neuropathy (NAION) is the most common acute optic neuropathy in patients over the age of 50 and is the second most common cause of permanent optic nerve-related visual loss in adults after glaucoma. Patients typically present with acute, painless, unilateral loss of vision associated with a variable visual field defect, a relative afferent pupillary defect, a swollen, hyperaemic optic disc, and one or more flame-shaped peripapillary retinal haemorrhages. The pathogenesis of this condition is unknown, but it occurs primarily in patients with structurally small optic discs that have little or no cup and a variety of underlying vascular disorders that may or may not be known at the time of visual loss. There is no consistently beneficial medical or surgical treatment for the condition, but there are now animal models that allow testing of various potential therapies. About 40% of patients experience spontaneous improvement in visual acuity. Patients in whom NAION occurs in one eye have a 15-19% risk of developing a similar event in the opposite eye over the subsequent 5 years.

Training improves walking capacity and cardiovascular function in arteritis.

Patients with arteritis have a high risk of mortality from cardiovascular disorders. However, whether these patients benefit from an intervention involving exercise remains unclear. In this study, we assessed the effects of an unsupervised exercise program on walking capacity, quality of life, and cardiovascular parameters of a patient with arteritis. A 33-year-old man reporting symptoms of claudication during walking was studied. Imaging tests revealed severe atherosclerosis and arteritis was diagnosed. Five weekly sessions of walking for 16 weeks increased claudication distance and total walking distance, produced improvements in six out of the eight health-related quality-of-life domains, decreased systolic blood pressure, and changed cardiac autonomic modulation toward parasympathetic modulation. This case report showed that unsupervised exercise training improved walking capacity, quality of life, and cardiovascular parameters in a patient with arteritis.

Femoral artery pseudoaneurysm due to Candida albicans in an injection drug user.

Candida arteritis is an uncommon condition but important to recognize due to the risk of significant morbidity and the difficulty in management of the enduring fungal infection. The authors report a rare case of a man with a femoral artery pseudoaneurysm with persistent Candida albicans infection, as a complication of infective endocarditis. The 23-year-old man, with a history of chronic intravenous drug use and Type I diabetes mellitus, presented with left groin pain, paresthesia of his left foot, and a pulsatile mass in the inguinal region. On imaging, he was found to have a pseudoaneurysm of the left common femoral artery, which later ruptured. Further investigation revealed vegetations on the mitral and aortic valves as well. Initial blood cultures were negative. He underwent multiple surgical interventions including replacement of the mitral and aortic valves and resection of the left common femoral artery with autogenous revascularization. In addition, he was commenced on intravenous antifungal therapy. Postoperatively, he continued to experience significant pain in the left groin and had two episodes of rerupture of the femoral artery that was consequently surgically repaired. Histological examination of the resected valves revealed vegetations with a mixture of fungal elements and bacterial cocci. The femoral artery resection specimens revealed evidence of infectious arteritis and the presence and persistence of C. albicans organisms in subsequent specimens. This case highlights the importance of an accurate diagnosis and aggressive management of fungal mycotic aneurysms in at-risk populations.