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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
Primary systemic vasculitic neuropathy occurs in the setting of a disorder with mainly vasculitic manifestations: Eosinophilic granulomatosis with polyangiitis (Churg–Strauss syndrome): neuropathy is common, occurring in 65–80% of cases.Microscopic polyangiitis occurs in > 50% of cases.Polyarteritis nodosa: up to 75% of patients.Granulomatosis with angiitis (Wegener's granulomatosis): 14–40% of patients.Secondary systemic vasculitic neuropathy occurs in the setting of a disorder with nonvasculitic manifestations such as systemic lupus erythematosus (SLE), rheumatoid arthritis, and Sjögren's syndrome. Vasculitic neuropathy is uncommon in SLE and rheumatoid arthritis, but is a relatively common occurrence in Sjögren's syndrome.
Rheumatology
Published in Fazal-I-Akbar Danish, Essential Lists of Differential Diagnoses for MRCP with diagnostic hints, 2017
ANCA-associated (frequently also involves small/medium-sized blood vessels):1 Wegener’s granulomatosis.2 Churg–Strauss’ syndrome.3 Microscopic polyangiitis.
Diseases of Blood vessels
Published in P. Chopra, R. Ray, A. Saxena, Illustrated Textbook of Cardiovascular Pathology, 2013
Microscopic polyangiitis This is a pauci-immune necrotizing vasculitis that affects the capillaries, venules, arterioles and small arteries in skin (Fig. 13.32), lungs, gastrointestinal tract and kidneys. Microscopically, leukocytoclastic vasculitis in skin, necrotizing glomerulonephritis and pulmonary capillaritis in one or other form may be seen. About 90% patients of microscopic polyangiitis show the presence of p-ANCA in the serum. Immune-complex deposition in blood vessels as seen in other types of cutaneous leukocytoclastic vasculitis are not demonstrated in MPAN.
How best to manage relapse and remission in ANCA-associated vasculitis
Published in Expert Review of Clinical Immunology, 2022
Xavier Puéchal, Loïc Guillevin
It is now well recognized that a prior relapse, diagnosis of granulomatosis with polyangiitis (GPA), proteinase-3 (PR3)-ANCA positivity, and/or higher estimated glomerular filtration rate increase the probability of relapse [6]. Early drug withdrawal [7], mycophenolate mofetil (MMF) [8] or methotrexate (MTX) for remission induction [9], rather than CYC, maintenance therapy with MMF versus azathioprine (AZA) [10] or AZA versus rituximab (RTX) [11] also put the patient at higher risk of relapse. The abilities of CD19+ B-cell reconstitution and/or anti-PR3-ANCA-titer change to predict relapse have not been proven [12]. Continued regular clinical monitoring of AAV patients after remission induction enables the early detection of relapses with less advanced manifestations than initially [13,14]. Herein, maintenance therapies are reviewed, and we discuss new recommended strategies to prevent and treat relapses, focusing on GPA and microscopic polyangiitis (MPA).
Anti-neutrophil cytoplasmic antibody-positive vasculitis presenting with periaortitis and muscle vasculitis in a patient with chronic Chagas disease
Published in Scandinavian Journal of Rheumatology, 2020
V Garcia-Bustos, E Calabuig, J López-Aldeguer, P Moral Moral
In conclusion, large-vessel involvement and myalgia with muscular small-vessel vasculitis are infrequent predominant manifestations in microscopic polyangiitis and ANCA-positive vasculitides. We have described a case of microscopic polyangiitis presenting with both. Our review highlights that ANCA-associated vasculitis may be a cause of unknown-origin fever and may tend to involve large vessels and myalgia with perimysial muscle vasculitis. Other organ involvement must be screened, as several associations with the classical manifestations of the disease can coexist with this atypical presentation. Histological diagnosis is essential to establish prompt and appropriate treatment and prevent severe complications. Our findings show the potential effectiveness of corticoids and rituximab combination therapy for muscle and large-vessel manifestations of ANCA-associated vasculitis.
The effect and possible clinical efficacy of in vivo inhibition of neutrophil extracellular traps by blockade of PI3K-gamma on the pathogenesis of microscopic polyangiitis
Published in Modern Rheumatology, 2018
Hirotaka Kimura, Yasushi Matsuyama, Sachiko Araki, Atsushi Koizumi, Yumi Kariya, Shunsuke Takasuga, Satoshi Eguchi, Hiroki Nakanishi, Junko Sasaki, Takehiko Sasaki
Microscopic polyangiitis (MPA) is the most common among the clinicopathologic variants of anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) which is a necrotizing vasculitis predominantly affecting small vessels, including capillaries, venules and arterioles, with few or no immune deposits [1]. In patients with MPA, an autoantibody specific for myeloperoxidase (MPO-ANCA) is frequently detected, along with the presence of crescentic necrotizing glomerulonephritis and occasional alveolar hemorrhage due to pulmonary capillaritis with the absence of granulomatous inflammation. ANCAs recognized in AAV play crucial roles in the immunopathogenesis of small vessel vasculitis (SVV) through activating neutrophils, resulting in the release of ROS and endothelial injury with subsequent vascular damage [2–4].