Diseases of the Peripheral Nerve and Mononeuropathies
Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw in Hankey's Clinical Neurology, 2020
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.
Diseases of Blood vessels
P. Chopra, R. Ray, A. Saxena in 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.
Clinical Diagnosis of Pulmonary Hemorrhage
Philip T. Cagle, Timothy C. Allen, Mary Beth Beasley in Diagnostic Pulmonary Pathology, 2008
Microscopic polyangiitis often presents with constitutional symptoms, followed by renal diseases, characterized by a rapidly progressive glomerulonephritis. The lungs are affected in approximately 30% of cases (35), and diffuse alveolar hemorrhage is the most common pulmonary manifestation. The musculoskeletal and peripheral nervous systems are frequently involved, as is the skin and gastrointestinal mucosa. p-ANCA testing is positive in 50% to 75% and c-ANCA can be positive in 10% to 30% of patients (31,34).
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].
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.
Microscopic polyangiitis: an incidental finding in a patient with stroke
Published in Journal of Community Hospital Internal Medicine Perspectives, 2020
Abubakar Tauseef, Muhammad Sohaib Asghar, Mariam Amir, Maryam Zafar, Ayesha Anum, Haris Alvi, Mustafa Dawood, Uzma Rasheed, Mohammed Akram, Nisar Ahmed
Microscopic polyangiitis (MPA) associated with the presence of anti-neutrophilic cytoplasmic autoantibodies first defined in 1994 in Chapel Hill Consensus, is an autoimmune multisystem disease which includes renal symptoms, mononeuritis multiplex, arthritis, diffuse alveolar hemorrhages, hemoptysis, hematuria, weight loss, proteinuria, skin rash, gastrointestinal bleeding, seizures, and myalgias. ANCA associated with microscopic polyangiitis has a peri-nucleic staining pattern P-ANCA caused by autoantibodies against myeloperoxidase. The absence of circulating ANCA, however, does not exclude the diagnosis. ANCA antibodies can also be positive secondary to exposure to hydralazine or propylthiouracil, in some inflammatory conditions, various infections and cystic fibrosis.
Related Knowledge Centers
- Fatigue
- Fever
- Granuloma
- Inflammation
- Kidney Failure
- Purpura
- Vasculitis
- Autoimmune Disease
- Hemoptysis
- Pauci-Immune