The cardiovascular system
C. Simon Herrington in Muir's Textbook of Pathology, 2020
The term ‘vasculitis’ refers to inflammation of blood vessels. Vasculitis can affect any of the blood vessels, including arteries, veins, and capillaries. Vessels of any type, in any organ, can be affected, resulting in a broad spectrum of symptoms and signs. The heterogeneous nature of vasculitides often presents a diagnostic challenge. The American College of Rheumatology classification criteria and the Chapel Hill Consensus Conference nomenclature are the most widely used to distinguish different forms of vasculitis. The latter defines 10 primary vasculitides based on vessel size (large, medium, and small). The diagnosis relies on the recognition of a compatible clinical presentation supported by specific laboratory or imaging investigations and confirmatory histology. Anti-neutrophil cytoplasmic antibody (ANCA) testing has been of particular benefit in defining a subgroup of small-vessel vasculitides. Arteritis affects arteries and arterioles, but, when veins and capillaries are affected, a broader term such as vasculitis or angiitis is preferred. Necrosis of the vessel wall may cause thrombosis and infarction, aneurysm formation, or rupture with haemorrhage. In the healing phase, luminal narrowing causes chronic ischaemia. There are four patterns of inflammation: granulomatous/giant cell, lymphoplasmacytic, acute neutrophilic, and eosinophilic. Mixed patterns, particularly acute/lymphoplasmacytic, also occur.
Connective Tissue Diseases: ENT Complications
John C Watkinson, Raymond W Clarke, Louise Jayne Clark, Adam J Donne, R James A England, Hisham M Mehanna, Gerald William McGarry, Sean Carrie in Basic Sciences Endocrine Surgery Rhinology, 2018
This is an autoimmune disorder of young people characterized by3: ocular symptoms with non-syphilitic interstitial keratitisaudio-vestibular symptoms similar to those of Meniere’s syndrome (sudden onset of tinnitus and vertigo accompanied by gradual hearing loss)an interval between the onset of the ocular and audio-vestibular features of less than 2 years. Atypical cases occur without any inflammatory ocular manifestations, with interstitial keratitis and with delays of more than 2 years between the eye and ear symptoms. There are often neurological or systemic features of vasculitis with possible infectious or autoimmune triggers. Anti-HSP 70 antibodies may be a marker for the autoimmune nature of the hearing loss. ANCA positivity may occur. Treatment is with corticosteroids followed by immunosuppressive therapy with cyclophosphamide, methotrexate, azathioprine, cyclosporine or anti-TNF blockers such as etanercept and infliximab. Rituximab is also used.
Cardiovascular cases
Lt Col Edward Sellon, David C Howlett, Nick Taylor in Radiology for Medical Finals, 2017
A AA may be associated with:Atherosclerosis (most common).Chronic aortic dissection.Vasculitis, e.g. Takayasu arteritis.Connective tissue disorders, e.g. Marfan’s or Ehlers–Danlos syndrome.Complications of AAA are:Bleeding due to leak or rupture.Fistula (aortoenteric would cause life-threatening bleeding into the bowel).What other imaging might show this abnormality in an emergency situation?
Transverse myelitis associated with primary biliary cirrhosis: clinical, laboratory, and neuroradiological features
Published in International Journal of Neuroscience, 2022
Mangsuo Zhao, Mingjie Zhang, Shimei Zhou, Bingxin Shi, Yan Wei, Fangjie Huang, Jing Wang, Jingfeng Huang, Liyan Qiao
Only seven patients with TM associated with PBC have been reported to date, and 71.4% (5/7) of these patients also had SS. Twenty percent of patients with SS have neurological diseases. TM has been suggested to occur in 1–5% of patients with SS [9], but the pathogenesis of TM in SS is unclear. Potential mechanisms include immunological injury of spinal vessels and/or the spinal cord driven by reactive T cells and/or the presence of anti-neuronal antibodies [4, 10–12]. Approximately one-third of patients with PBC were reported to have other coexisting autoimmune disorders, with SS being the most frequent autoimmune comorbidity [13]. In some series, SS was observed in 40% of PBC cases [14, 15]. PBC may be considered SS of the liver, whereas SS may be considered PBC of the salivary glands, and both are characterized by inflammation of target epithelial elements [16]. The mechanisms underlying the association of TM with PBC are unknown. As both PBC and SS belong to one disease spectrum, the same mechanisms may underlie the pathogenesis of TM in SS. Pathological examination in one patient reported by Rutan et al. [2] revealed angiitis and necrosis in the gray and white matter of the spinal cord; both arterioles and venules were thick and infiltrated by lymphocytes, macrophages, and a few plasma cells [2]. These observations suggest that vasculitis may be the main mechanism underlying this process.
Rationale for and clinical development of anti-fractalkine antibody in rheumatic diseases
Published in Expert Opinion on Biological Therapy, 2020
Sei Muraoka, Junko Nishio, Yoshikazu Kuboi, Toshio Imai, Toshihiro Nanki
Noninfectious vasculitis is categorized based on the predominant type of vessels involved [111]. Large vessel vasculitis includes Takayasu arteritis and giant cell arteritis; medium vessel vasculitis includes polyarteritis nodosa (PAN) and Kawasaki disease; and small vessel vasculitis is further divided into ANCA-associated vasculitis and immune complex (IC) small vessel vasculitis. ANCA-associated vasculitis includes microscopic polyangiitis (mPA), granulomatosis with polyangiitis (GPA), and eosinophilic granulomatosis with polyangiitis. IC small vessel vasculitis includes anti-glomerular basement membrane disease, cryoglobulinemic vasculitis, IgA vasculitis, and hypocomplementemic urticarial vasculitis. Other types of vasculitis have also been classified [111]. Systemic vasculitis affects small, medium, and large vessels, and causes various symptoms depending on the severity of inflammation and damage in vessels. In patients with ANCA-positive GN vasculitis, FKN mRNA is expressed in glomerular or tubulointerstitial lesions and strongly expressed in inflamed vascular sites. FKN is not expressed by infiltrating leukocytes [112]. The serum concentration of sFKN was found to be significantly higher in patients with mPA than in healthy subjects and correlated with disease activity [113]. CX3CR1 expression was also up-regulated on peripheral blood T cells in patients with mPA [113]. Moreover, the expression levels of FKN and CX3CR1 decreased with treatment-induced reductions in disease activity.
Prognosis and monitoring of giant cell arteritis and associated complications
Published in Expert Review of Clinical Immunology, 2018
Tanaz A. Kermani, Kenneth J. Warrington
Treatment with glucocorticoids is effective at controlling systemic inflammation, improving symptoms and preventing progressive vision loss, but has significant adverse effects [4–7]. Furthermore, GCA is a relapsing condition often requiring long-term and fluctuating doses of glucocorticoids to manage the disease. As a result, damage from the vasculitis and its treatment are not infrequent [8]. Prior studies evaluating adjunctive immunosuppressive therapy to reduce the burden of glucocorticoid-associated adverse effects and better maintain remission have yielded disappointing results [9]. There has been much interest recently about tocilizumab, an interleukin (IL)-6 antagonist, which is the first treatment with proven efficacy for GCA [10,11]. While tocilizumab appears effective at induction and maintenance of remission in most patients with GCA, its effects on long-term outcomes including protecting against vessel damage is unknown.