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Microscopic Polyangiitis

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Systemic Vasculitides: Current Status and Perspectives

Abstract

Microscopic polyangiitis (MPA) is a necrotizing, systemic vasculitis usually affecting capillaries, venules and arterioles. Midium arteries can also be involved. Necrotizing glomerulonephritis and pulmonary capillaritis are the major pathological findings, but granulomatosis is regularly absent. Its prevalence is lower than that of granulomatosis with polyangiitis and of eosinophilic granulomatosis with polyangiitis but, similarly to these two conditions, it belongs to the group of vasculitides associated with anti-neutrophil cytoplasmic antibodies (ANCA), directed (in most though not all MPA patients) against myeloperoxidase rather than against proteinase-3. In addition to general constitutional symptoms, clinical features may range from a renal-restricted vasculitis usually consisting of idiopathic, necrotizing and crescentic glomerulonephritis to pulmonary capillaritis whose severity may reach the level of pulmonary hemorrhage. Purpuric eruptions and mononeuritis multiplex can also be detected with variable prevalence. At diagnosis, prompt and intensive treatment with the combination of corticosteroids and cyclophosphamide is the usual first-line approach, that is able to achieve a complete or partial response in over two-thirds of the patients. Maintenance therapy includes lower doses of corticosteroids, azathioprine and methotrexate. Rituximab has been shown to be effective in resistant and relapsing patients. End-stage renal disease, infections and cardiovascular failure are the most frequent causes of mortality.

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Authors and Affiliations

  1. Department of Biomedical Sciences and Human Oncology, Section of Internal Medicine, University of Bari Medical School, 70124, Bari, Italy

    Franco Dammacco & Angelo Vacca

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  1. Franco Dammacco
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  2. Angelo Vacca
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Correspondence to Franco Dammacco .

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Editors and Affiliations

  1. University of Bari Medical School, Bari, Italy

    Franco Dammacco

  2. University of Bari Medical School, Bari, Italy

    Domenico Ribatti

  3. University of Bari Medical School, Bari, Italy

    Angelo Vacca

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Dammacco, F., Vacca, A. (2016). Microscopic Polyangiitis. In: Dammacco, F., Ribatti, D., Vacca, A. (eds) Systemic Vasculitides: Current Status and Perspectives. Springer, Cham. https://doi.org/10.1007/978-3-319-40136-2_10

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