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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
Systemic vasculitis is a broad category of diseases that often have severe manifestations because of their predilection for vascular beds in the lungs, kidneys, and nerves, as well as the aorta and its main branches. Tissue damage occurs from ischemia, infarction, and/or hemorrhage and can be catastrophic. Vasculitides are most often classified according to their primary vessel involvement (small, medium, large, or variable), as defined in the Chapel Hill classification criteria (1) (Table 10.1).
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).
Diseases of the Peripheral Nerve and Mononeuropathies
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
Diana Mnatsakanova, Charles K. Abrams
Other blood tests that should be included in the work-up for a systemic vasculitis: Rheumatoid factor, anticyclic citrullinated peptide (CCP) antibody (rheumatoid arthritis). Rheumatoid factor can be positive in other autoimmune diseases, infections (hepatitis C), and following chemotherapy and radiation treatment for cancer. However, anti-CCP antibody has a high specificity for rheumatoid arthritis.35Antineutrophil cytoplasmic antibodies (ANCAs) are positive in 90% of granulomatosis with angiitis (anti-cANCA, usually against the PR3 antigen) and 50% of eosinophilic granulomatosis with polyangiitis (anti-pANCA usually against the myeloperoxidase [MPO] antigen).Serum complement levels, anti–double-stranded DNA antibodies (SLE).Anti-Ro/SSA, anti-La/SSB (Sjögren's syndrome).Hepatitis B and C panel.Cryoglobulins.
A case of intravascular large B cell lymphoma with brain involvement mimicking progressive multifocal leukoencephalopathy
Published in International Journal of Neuroscience, 2023
Elisabetta Belli, Chiara Milano, Ilaria Pesaresi, Ilaria Trivelli, Antonio Tavoni, Eugenio Ciancia, Greta Alì, Virna Zampa, Chiara Pizzanelli, Gabriele Siciliano, Giulia Ricci
Nevertheless, in our case, the rapidly progressive worsening of clinical conditions despite the interruption of Jak2 inhibitor therapy and the signs of systemic involvement, prompted us to take into account alternative differential diagnosis. Systemic vasculitis with CNS involvement should be considered in cases of nonspecific CNS clinical and radiological findings, together with a more systemic disease process. Due to similar features of systemic vasculitis and IVLBLC, biopsy of the affected organs is the gold standard for the differential diagnosis. In particular, multiple skin biopsies are considered very sensitive for the detection of IVLBLC [8]. Of note, tumour cells could be identified from random deep skin biopsies not only in European series in which the cutaneous variant is prevalent, but also in Asian series in which the cutaneous variant is rare [8]. This diagnostic procedure has allowed a better in vivo recognition of IVLBCL (up to 79%), with important implications in terms of early treatment start [9]. The neoplastic cells immunophenotype is generally typical of B cells (CD19+, CD20+, CD22+, CD79a+), with few cases expressing the T-cells marker CD5 [4].
Vasculitis issue – introduction
Published in Postgraduate Medicine, 2023
Sophia Panaccione, David A. Cohen
The terminology ‘systemic vasculitis’ encompasses an array of conditions characterized by inflammation of blood vessels of varying size that without recognition and treatment can lead to end organ damage and failure with subsequent mortality. Vasculitis disease states are categorized by the size of vessel they involve along with recognition of affiliated age group. Data over the last 40 years estimate varying but overall increasing incidence of vasculitis in the United States (U.S.) [1,2]. Since establishment of more standardized diagnostic tools and the evolution of immunomodulating therapy, survival has increased and mortality has decreased; however, estimated death count in the U.S. remains high at 13,048 individuals with vasculitis contributing to their death from 1999 to 2019. During this same period in the U.S., age-adjusted mortality rate of vasculitis as underlying cause of death has averaged 1.888 per million (CI 1.855–1.921) [2].
Chest discomfort leading to the diagnosis of pulmonary artery aneurysm due to isolated main pulmonary arteritis involving giant cells: a case report
Published in Acta Chirurgica Belgica, 2023
Sarah Sakalihasan, Vincent Tchana-Sato, Audrey Courtois, Jean Olivier Defraigne, Natzi Sakalihasan
To date, two cases of PAAs due to isolated PA vasculitis involving giant cells have been reported in the English literature [1,5]. In both cases, patients were asymptomatic and had no features of systemic vasculitis. In one of the cases, PAA was located in the main PA [5]. The two patients were managed by surgical resection alone, with one requiring an additional lobectomy due to the involvement of the peripheral PA [1]. The treatment of isolated PA vasculitis, and particularly PA vasculitis involving giant cells, is not well defined. In our case, surgery was performed because of an increasing PAA extending to the proximal portion of the right PA in a symptomatic patient. As in the two cases of PAAs due to isolated PA vasculitis involving giant cells [1,5], the vasculitis diagnosis was unanticipated before the surgery, and a positron emission tomography-computed tomography was not performed preoperatively.