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Vasculitis
Published in Jason Liebowitz, Philip Seo, David Hellmann, Michael Zeide, Clinical Innovation in Rheumatology, 2023
Michelle L. Robinette, Eli Miloslavsky, Zachary S. Wallace
While the vast majority of vasculitis patients achieve disease remission with currently available treatments, many require ongoing immunosuppressive treatment with glucocorticoids and other medications. These ongoing treatment requirements and their associated toxicities make additional therapeutic options necessary. This has become even more apparent in the COVID-19 era, where immunosuppression may affect not only the body’s ability to combat the virus but also the efficacy of vaccination (122). Future therapeutic options must strive for a balance of efficacy, rapid onset of action similar to that observed with glucocorticoids, and safety. Therapeutic regimens that achieve all three parameters may ultimately enable glucocorticoid-free treatment of systemic vasculitis.
Rheumatology
Published in Kaji Sritharan, Jonathan Rohrer, Alexandra C Rankin, Sachi Sivananthan, Essential Notes for Medical and Surgical Finals, 2021
Kaji Sritharan, Jonathan Rohrer, Alexandra C Rankin, Sachi Sivananthan
A group of heterogeneous conditions that feature inflammation of the blood vessel walls. Can be primary or can occur as part of another condition (secondary). Clinical features: initial symptoms of a systemic vasculitis are often non-specific – general malaise, fever, weight loss. Management: varies slightly for each type but generally steroids +/- immunosuppresants (e.g. cyclophosphamide).
The cardiovascular system
Published in C. Simon Herrington, Muir's Textbook of Pathology, 2020
Mary N Sheppard, C. Simon Herrington
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.
A case of polyangiitis overlap syndrome of giant cell arteritis and granulomatosis with polyangiitis successfully treated with rituximab
Published in Modern Rheumatology Case Reports, 2021
Akiko Shibata, Tsuneo Kondo, Takahiko Kurasawa, Kentaro Chino, Yusuke Okada, Koichi Amano
Treatment of vasculitis has dramatically shifted from steroid-dependent therapy to steroid-sparing immunosuppressive therapy. Given the frequency of steroid-induced adverse events such as osteoporotic vertebral fracture and diabetes, particularly in the elderly, steroid sparing therapeutic strategies have been awaited [17]. Cyclophosphamide has been widely used for remission induction therapy for AAV [18], and in some patients with refractory GCA [19,20], but this therapy itself involves such problems as bone marrow suppression in aged patients. Recently, the anti-CD20 monoclonal antibody rituximab was proved to be effective for AAV [18,21,22], and was approved for MPA and GPA in June 2013 in Japan. Further, recent clinical studies revealed that tocilizumab, an anti-IL-6 receptor antibody, was effective for GCA [23]. Tocilizumab was also shown to be effective for Takayasu arteritis, albeit that the study failed to show statistical significance [24], and was approved for GCA and Takayasu arteritis in August 2017 in Japan. However, the efficacy of rituximab for these large vessel vasculitides is controversial [25], despite the efficacy shown in several case reports [26,27]. In our present case also, high-dose steroid plus rituximab therapy was effective for both GPA and GCA lesions. Rituximab might therefore be effective not only for GPA but also for GCA, which might be small vessel-type GCA, although one report showed that only a few B cells are seen in the vascular wall of GCA patients [28].
A Four-Year-Old with History of Kawasaki Disease Presenting in Acute Shock
Published in Prehospital Emergency Care, 2021
Katherine Staats, Adriana H. Tremoulet, Helen Harvey, Jane C. Burns, J. Joelle Donofrio-Odmann
KD was first described in Japan in 1967 and is the most frequently diagnosed acquired heart disease of children in the developed world (4,5). It is a medium vessel vasculitis that occurs in children where 85% of cases occur in patients less than five years of age. It can occur in any ethnic or racial group, however the highest incidence rate is among patients of Asian descent (4,5). This is a syndrome with an unknown etiology that leads to a vasculitis. The classic diagnosis is clinical. If a patient has ≥ 5 days of fever, and 4 of 5 clinical findings (Conjunctivitis that is limbic sparing, bilateral and without exudate, Rash [maculopapular, diffuse erythroderma, or erythema multiforme-like], Adenopathy [≥1.5 cm in diameter, often unilateral], Strawberry tongue, cracking of lips and/or erythema of oral and pharyngeal mucosa, and Hand swelling/redness/desquamation), they meet criteria for KD (Table 3) (7). Initial treatment is with aspirin and IV immunoglobulin (4,5). In recent years, previous treatment with high dose aspirin has been studied in greater depth, and dosing has been decreased from previous regiments, to continue to provide antiplatelet therapy, while also lessening side effects (Table 4) (13,14,19,20). Untreated KD leads to the development of coronary artery aneurysms in 25% of cases, which may progress to arterial stenosis in 20%, myocardial infarction in 7.5% and death in 3% (6). With proper recognition and treatment, the rate of coronary artery aneurysms decreases to 2-3% of patients (8).
Asymptomatic coronary aneurysms in a patient with eosinophilic granulomatosis with polyangiitis who developed a digital gangrene
Published in Modern Rheumatology Case Reports, 2021
Mayu Sato, Yusuke Yoshida, Tomohiro Sugimoto, Shinji Kishimoto, Takuji Omoto, Hirofumi Watanabe, Tadahiro Tokunaga, Kazutoshi Yukawa, Hiroki Kohno, Sho Mokuda, Takaki Nojima, Shintaro Hirata, Eiji Sugiyama
The manifestations of EGPA can be divided into three phases: prodromal phase, eosinophilic phase, and vasculitic phase. [4] In the prodromal phase, there are allergic symptoms such as bronchial asthma. In the eosinophilic phase, there is a serum elevation and organ infiltration by eosinophils. In the vasculitis phase, organ damage and symptoms associated with systemic vasculitis are seen. Cutaneous or cardiac involvements in EGPA are common in the vasculitic phase. Cutaneous involvements include itchy erythema or purpura, livedo, Raynaud’s phenomenon, or digital ulcer. [5] Rarely, digital gangrene is also reported in EGPA, [6–8] suggesting a severe ischaemic skin manifestation. Cardiac involvement is usually manifested as cardiomyopathy, but coronary aneurysms or stenoses due to coronary vasculitis are rarely reported. [9] Both digital gangrene and coronary aneurysms are observed in EGPA due to vasculitis of the medium-sized vessels. [1]