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R. J. Falk - One of the best experts on this subject based on the ideXlab platform.
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Anca Vasculitis: Microscopic Polyangiitis, Wegener's Granulomatosis, and Churg-strauss Syndrome
Pathology Case Reviews, 2007Co-Authors: J. C. Jennette, R. J. FalkAbstract:Abstract: Antineutrophil cytoplasmic autoantibodies (ANCA) are associated with systemic and organ-limited vasculitis that most often is characterized immunopathologically by a paucity of vessel wall immunostaining for immunoglobulins. The systemic expressions of ANCA-associated vasculitis include Microscopic Polyangiitis, Wegener's granulomatosis, and Churg-Strauss syndrome. Microscopic Polyangiitis has systemic pauci-immune small vessel vasculitis without evidence for granulomatous inflammation or asthma. Wegener's granulomatosis has pauci-immune small vessel vasculitis accompanied by necrotizing granulomatous inflammation but no asthma. Churg-Strauss syndrome has pauci-immune small vessel vasculitis accompanied by blood eosinophilia and asthma. Although ANCA-associated vasculitis has a predilection for small vessels, such as glomerular capillaries, pulmonary alveolar capillaries, and dermal venules, arteries also can be involved with pathologic changes that are indistinguishable form those of polyarteritis nodosa. ANCA with specificity for proteinase 3 (PR3-ANCA) are predominant in patients with Wegener's granulomatosis, whereas ANCA with specificity for myeloperoxidase (MPO-ANCA) are predominant in patients with Microscopic Polyangiitis, Churg-Strauss syndrome or renal-limited disease; however, either type of ANCA can occur in any of the clinicopathologic expressions of ANCA-associated vasculitis. Clinical, in vitro, and experimental animal data indicate that ANCA IgG is directly involved in the pathogenesis of ANCA-associated vasculitis.
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Microscopic Polyangiitis (Microscopic polyarteritis).
Seminars in diagnostic pathology, 2001Co-Authors: J. C. Jennette, D. B. Thomas, R. J. FalkAbstract:Microscopic Polyangiitis ("Microscopic polyarteritis") is a form of necrotizing small vessel vasculitis that most often affects venules, capillaries, arterioles, and small arteries, although it occasionally involves medium-sized arteries. Microscopic Polyangiitis is a more appropriate name than Microscopic polyarteritis because some patients have no evidence for arterial involvement. The absence or paucity of immunoglobulin localization in vessel walls distinguishes Microscopic Polyangiitis from immune complex mediated small vessel vasculitis, such as Henoch-Schonlein purpura and cryoglobulinemic vasculitis. Clinical, epidemiological, and pathologic differences warrant the separation of Microscopic Polyangiitis from polyarteritis nodosa on the basis of involvement of capillaries and venules by the former but not the latter. Pauci-immune necrotizing and crescentic glomerulonephritis, and hemorrhagic pulmonary capillaritis are common in patients with Microscopic Polyangiitis. Microscopic Polyangiitis is the most common cause for pulmonary-renal vasculitic syndrome. The vasculitis in patients with Microscopic Polyangiitis is pathologically indistinguishable from the vasculitis of Wegener's granulomatosis and Churg-Strauss syndrome. Granulomatous inflammation distinguishes Wegener's granulomatosis from Microscopic Polyangiitis. Asthma and eosinophilia distinguish Churg-Strauss syndrome from Microscopic Polyangiitis. Microscopic Polyangiitis, Wegener's granulomatosis, and Churg-Strauss syndrome are all associated with circulating antineutrophil cytoplasmic autoantibodies.
J. C. Jennette - One of the best experts on this subject based on the ideXlab platform.
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Anca Vasculitis: Microscopic Polyangiitis, Wegener's Granulomatosis, and Churg-strauss Syndrome
Pathology Case Reviews, 2007Co-Authors: J. C. Jennette, R. J. FalkAbstract:Abstract: Antineutrophil cytoplasmic autoantibodies (ANCA) are associated with systemic and organ-limited vasculitis that most often is characterized immunopathologically by a paucity of vessel wall immunostaining for immunoglobulins. The systemic expressions of ANCA-associated vasculitis include Microscopic Polyangiitis, Wegener's granulomatosis, and Churg-Strauss syndrome. Microscopic Polyangiitis has systemic pauci-immune small vessel vasculitis without evidence for granulomatous inflammation or asthma. Wegener's granulomatosis has pauci-immune small vessel vasculitis accompanied by necrotizing granulomatous inflammation but no asthma. Churg-Strauss syndrome has pauci-immune small vessel vasculitis accompanied by blood eosinophilia and asthma. Although ANCA-associated vasculitis has a predilection for small vessels, such as glomerular capillaries, pulmonary alveolar capillaries, and dermal venules, arteries also can be involved with pathologic changes that are indistinguishable form those of polyarteritis nodosa. ANCA with specificity for proteinase 3 (PR3-ANCA) are predominant in patients with Wegener's granulomatosis, whereas ANCA with specificity for myeloperoxidase (MPO-ANCA) are predominant in patients with Microscopic Polyangiitis, Churg-Strauss syndrome or renal-limited disease; however, either type of ANCA can occur in any of the clinicopathologic expressions of ANCA-associated vasculitis. Clinical, in vitro, and experimental animal data indicate that ANCA IgG is directly involved in the pathogenesis of ANCA-associated vasculitis.
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Microscopic Polyangiitis (Microscopic polyarteritis).
Seminars in diagnostic pathology, 2001Co-Authors: J. C. Jennette, D. B. Thomas, R. J. FalkAbstract:Microscopic Polyangiitis ("Microscopic polyarteritis") is a form of necrotizing small vessel vasculitis that most often affects venules, capillaries, arterioles, and small arteries, although it occasionally involves medium-sized arteries. Microscopic Polyangiitis is a more appropriate name than Microscopic polyarteritis because some patients have no evidence for arterial involvement. The absence or paucity of immunoglobulin localization in vessel walls distinguishes Microscopic Polyangiitis from immune complex mediated small vessel vasculitis, such as Henoch-Schonlein purpura and cryoglobulinemic vasculitis. Clinical, epidemiological, and pathologic differences warrant the separation of Microscopic Polyangiitis from polyarteritis nodosa on the basis of involvement of capillaries and venules by the former but not the latter. Pauci-immune necrotizing and crescentic glomerulonephritis, and hemorrhagic pulmonary capillaritis are common in patients with Microscopic Polyangiitis. Microscopic Polyangiitis is the most common cause for pulmonary-renal vasculitic syndrome. The vasculitis in patients with Microscopic Polyangiitis is pathologically indistinguishable from the vasculitis of Wegener's granulomatosis and Churg-Strauss syndrome. Granulomatous inflammation distinguishes Wegener's granulomatosis from Microscopic Polyangiitis. Asthma and eosinophilia distinguish Churg-Strauss syndrome from Microscopic Polyangiitis. Microscopic Polyangiitis, Wegener's granulomatosis, and Churg-Strauss syndrome are all associated with circulating antineutrophil cytoplasmic autoantibodies.
Paraskeyi Katsaounou - One of the best experts on this subject based on the ideXlab platform.
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Pulmonary fibrosis predating Microscopic Polyangiitis by seven years
Respiratory Medicine CME, 2010Co-Authors: Angeliki M. Tsimogianni, Magda Stratiki, Grigoris Stratakos, Spyros Zakynthinos, Paraskeyi KatsaounouAbstract:Abstract A 63-year-old man, ex-smoker with renal failure of recent onset was admitted at the respiratory department with massive haemoptysis. Previous X-rays and CT scans showed pulmonary fibrosis of seven-year duration. Subsequently, he developed high fever, large haemoptysis, new infiltrates and respiratory failure despite broad-spectrum antibiotic treatment. Antineutrophilic antibodies of the perinuclear type with specificity against myeloperoxidase were detected and Microscopic Polyangiitis was diagnosed. Immunosuppressive treatment with methylprednisolone pulses and cyclophosphamide was started with initially favorable response, but later the patient developed a hospital-acquired pneumonia which was treated successfully with meropenem. As pulmonary haemorrhage recurred, he was transferred to intensive care for plasmapheresis which was considered the last treatment option. Unfortunately he died from septic shock. Conclusion Asymptomatic pulmonary fibrosis can predate Microscopic Polyangiitis by several years and is associated with unfavorable prognosis of the vasculitis. Appreciation of this finding would lead to faster diagnosis and better management of these patients.
Sobue - One of the best experts on this subject based on the ideXlab platform.
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Spatial distribution of nerve fiber pathology and vasculitis in Microscopic Polyangiitis-associated neuropathy.
Journal of neuropathology and experimental neurology, 2011Co-Authors: Saori Morozumi, Haruki Koike, Minoru Tomita, Yuichi Kawagashira, Masahiro Iijima, Masahisa Katsuno, Naoki Hattori, Fumiaki Tanaka, SobueAbstract:We analyzed the 3-dimensional distribution of pathologic findings in 8 autopsied cases of neuropathy associated with Microscopic Polyangiitis. Necrotizing vasculitis was commonly and diffusely present in the epineurium of the sciatic/tibial and median nerves. Although findings of vasculitis were distributed diffusely in proximal to distal segments of the nerve trunks, marked loss of myelinated fibers occurred only from the middle to distal segments of these nerves. Neurons of the sensory and sympathetic ganglia were well preserved, as were myelinated fibers of the anterior and posterior spinal roots. Central fascicular nerve fiber degeneration, suggesting direct ischemic damage, occurred in restricted segments of the proximal-middle portion of the sciatic/tibial and median nerve trunks. Vasculitis was also seen in various visceral organs in all patients, but its distribution differed among individual patients; the severity of vasculitis in the other organs did not correlate with that in the peripheral nerves. The distinct spatial distribution pattern of nerve fiber degeneration, in contrast to the ubiquitous presence of vasculitis, suggested that the ischemic zone that directly damages nerve fibers is present in the proximal-middle portion of peripheral nerve trunks in Microscopic Polyangiitis.
Guillevin L - One of the best experts on this subject based on the ideXlab platform.
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Polyarteritis nodosa, Microscopic Polyangiitis, and Churg-Strauss syndrome. Clinical aspects and treatment.
Rheumatic diseases clinics of North America, 1995Co-Authors: Lhote F, Guillevin LAbstract:In this article, three systemic vasculitides--polyarteritis nodosa, Microscopic Polyangiitis, and Churg-Strauss syndrome--are reviewed, focusing on advances in the understanding of the pathogenesis, new classification nomenclature, and major clinical features of these vasculitides. Distinguishing criteria between polyarteritis nodosa and Microscopic Polyangiitis are also presented in detail, and recent advances in the clinical management of these diseases according to appropriate therapeutic strategies are recommended.