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Microscopic Polyangiitis1 Microscopic polyangiitis1 Author: Professor J. Charles Jennette2 Creation Date: October 2002 Scientific Editor: Professor Loïc Guillevin 1This text was adapted from: Jennette JC, Thomas DB, Falk RJ. Microscopic polyangiitis (microscopic polyarteritis). Semin Diagn Pathol. 2001;18:3-13. 2Department of Pathology and Laboratory Medicine, University of North Carolina, 303 Brinkhous-Bullitt Building, NC 27599-7525 Chapel Hill, United States. [email protected] Abstract Keywords Disease name Definition Differential diagnosis Frequency Clinical manifestation Diagnostic methods Treatment Unresolved questions References Abstract Microscopic polyangiitis (MPA) refers to a necrotizing systemic vasculitis with few or no immune deposits that affects small vessels (ie, capillaries, venules and arterioles). Arteries, especially small arteries, are often but not always involved. Vessels of any type in any organ can be affected, resulting in a wide variety of signs and symptoms, and nonspecific clinical manifestations. Common signs and symptoms include nephritis, pulmonary hemorrhage, purpura, peripheral neuropathy, abdominal pain, myalgias and arthralgias. MPA is the most common antineutrophil cytoplasmic autoantibodies (ANCA)-associated small-vessel vasculitis. Most patients have positive myeloperoxidase MPOANCA (PANCA), although proteinase 3 PR3 ANCA (CANCA) may be also present. MPA has an incidence of approximately 1:100,000 with a slight predominance in men, and a mean age of onset of about 50 years. Treatment of patients with MPA consists of three phases: induction of remission, maintenance of remission, and treatment of relapse. Current induction therapy often consists of cyclophosphamide and corticosteroids. Keywords Micoscopic polyangiitis, small-vessel vasculitis, antineutrophil cytoplasmic autoantibodies (ANCA), corticosteroid, cyclophosphamide. Disease name few histological patterns of vascular Microscopic polyangiitis (MPA) inflammation. Small-vessel vasculitis is defined Microscopic polyarteritis as a vasculitis affecting vessels smaller than Microscopic polyangiitis is a more appropriate arteries, such as arterioles, venules and name than microscopic polyarteritis because capillaries. some patients show no evidence for arterial There is no widely accepted classification of involvement. primary vasculitis, although a working framework has been provided by a consensus reached by Definition the leading investigators at a meeting in Chapel Classification of systemic vasculitis Hill, North Carolina in 1995. Systemic Vasculitis is an inflammation of vessel walls. It vasculitides form a heterogeneous group of has many causes, although they result in only a Jennette J.C.; Microscopic polyangiitis. Orphanet encyclopedia, October 2002 http://www.orpha.net/data/patho/GB/uk-MPA.pdf 1 vascular inflammatory diseases that can be sub- the presence of ANCA and few or no immune divided on the basis of the underlying deposits in the involved vessels. The kidneys are histopathology, the type of vessels involved, the the most commonly affected organs and are target organs involved, and the resulting clinical involved in 90% of patients who have this type of picture (table 1). vasculitis. Patients often present with variable combinations of renal manifestations, palpable Table 1: The Chapel Hill Consensus purpura, abdominal pain, cough, and Conference on the Nomenclature of Systemic hemoptysis. Most patients have positive Vasculitis myeloperoxidase MPOANCA (PANCA), although Large Vessel Vasculitis proteinase 3 PR3 ANCA (CANCA) may be also Giant-cell arteritis – granulomatous arteritis of the aorta present. and its major branches, especially the extracranial branches of the carotid artery; usually in patients over 50 years old and associated with polymyalgia rheumatica. Differential diagnosis Takayasu arteritis – granulomatous inflammation of the In recent years there has been substantial aorta and its major branches usually in patients <50 years progress in identifying attributes of specific types old. of vasculitis, thus enabling accurate diagnosis. Medium Vessel Vasculitis MPA must be differentiated not only from other Polyarteritis Nodosa – necrotizing inflammation of medium or small arteries without glomerulonephritis or vasculitis in forms of small-vessel vasculitis, but also from arterioles, capillaries, or venules. medium-sized vessel vasculitis, such as Kawasaki’s Disease – arteritis of large, medium, and small polyarteritis nodosa and Kawasaki disease. arteries associated with the mucocutaneous lymph node MPA may cause a necrotizing arteritis that is syndrome, often involves the coronaries, usually occurs in children histologically identical to that caused by Small Vessel Vasculitis polyarteritis nodosa (PAN). Clinical, Wegener’s granulomatosis epidemiological and pathological differences Churg-Strauss syndrome enable differentiation between MPA and PAN, as Microscopic polyangiitis capillaries and venules are affected in MPA but Henoch-Schonlein purpura Cryoglobulinemic vasculitis not in PAN. Cutaneous Leukocytoclastic Vasculitis The absence or paucity of immunoglobulin localization in vessel walls helps distinguish Microscopic Polyangiitis MPA from immune complex-mediated small MPA is a necrotizing systemic vasculitis with few vessel vasculitis, such as Henoch-Schönlein or no immune deposits that affects small vessels purpura and cryoglobulinemic vasculitis. (ie, capillaries, venules and arterioles), although The vasculitis in patients with MPA is it occasionally involves medium-sized arteries. pathologically indistinguishable from the MPA belongs to a category of vasculitis that also vasculitis of WG and CSS. Granulomatous includes Wegener’granulomatosis (WG), Churg- inflammation distinguishes WG from MPA. Strauss syndrome (CSS), Henoch-Schönlein Asthma and eosinophilia distinguish CSS from purpura, cryoglobulinemic vasculitis and others MP. necrotizing polyangiitides. MP, WG and CSS are all associated with MPA is the most common antineutrophil circulating ANCA (table 2). cytoplasmic autoantibodies(ANCA)-associated small-vessel vasculitis, and is characterized by Jennette J.C.; Microscopic polyangiitis. Orphanet encyclopedia, October 2002 http://www.orpha.net/data/patho/GB/uk-MPA.pdf 2 Table 2: Features that allow differentiation of microscopic polyangiitis from several other forms of small-vessel vasculitis Henoch-Schonlein Cryoglobulinemic Microscopic Wegener’s Churg-Strauss Purpura Vasculitis Polyangiitis Granulomatosis Syndrome Small-vessel vasculitis Yes Yes Yes Yes Yes signs and symptoms IgA-dominant immune Yes No No No No deposits Cryoglobulins in blood No Yes No No No and vessels ANCA in blood No No Yes Yes Yes Necrotizing No No No Yes Yes granulomas Asthma and No No No No Yes eosinophilia If a patient has systemic pauci-immune small-vessel vasculitis with no evidence for granulomatous inflammation, or asthma, the appropriate diagnosis is MPA The diagnosis of MPA is essentially made by exclusion of other diseases, which is always a difficult task. pain as well as bloody stools, and possible Frequency intussusception, perforation, and pancreatitis. MPA has an incidence of approximately Respiratory tract: pulmonary manifestations 1:100,000, with a slight predominance in men, range from fleeting focal infiltrates to massive and a mean age at onset of about 50 years, pulmonary hemorrhage and hemoptysis although any age can be affected secondary to alveolar capillaritis. Histologic Findings Clinical manifestation The basic acute vascular lesion of MPA is Vessels of any type in any organ can be segmental vascular necrosis with infiltration of affected, resulting in a wide variety of signs and neutrophils and monocytes, often accompanied symptoms, and nonspecific clinical by leukocytoclasia and accumulation of fibrin. manifestations. Common signs and symptoms MPA may cause necrotizing arteritis that is include nephritis, pulmonary hemorrhage, histologically identical to that caused by PAN. purpura, peripheral neuropathy, abdominal pain, According to the Chapel Hill consensus myalgias and arthralgias. conference on the classification of vasculitis, Constitutional signs and symptoms such as PAN and MPA can be distinguished fever, arthralgias, myalgias, and "flu-like" pathologically by the absence of vasculitis in symptoms are often present early in the disease. vessels other than arteries in patients with PAN, Arthralgias tend to be migratory, affect large and and the presence of vasculitis in vessels smaller small joints with evidence of synovitis in 10-20%. than arteries, such as arterioles, venules, and Blood vessels in the skin, lungs, kidneys, gut, capillaries, in patients with MPA. nerves, and skeletal muscles are often involved The glomerulonephritis in MPA is characterized but vary between the different patients. MPA by focal necrosis, crescent formation, and the may cause pulmonary-renal syndrome. absence or paucity of immunoglobulin deposits. Skin: the most common cutaneous lesion is Pulmonary manifestations consist of pulmonary leukocytoclastic angiitis, which typically causes alveolar capillaritis. Biopsy of the muscle and purpura and ulcerations; other lesions include peripheral nerves may reveal necrotizing erythematous tender nodules, focal necrosis, vasculitis epineural arteries. and livedo reticularis. The most common cutaneous lesion is Peripheral neuropathy: mononeuritis multiplex leukocytocalstic angiitis affecting dermal is the most common neurological manifestation venules. Necrotizing dermal arteritis also occurs.
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