Microscopic polyangiitis: Clinical presentation

Villiger, Peter M; Guillevin, Loïc (2010). Microscopic polyangiitis: Clinical presentation. Autoimmunity reviews, 9(12), pp. 812-9. Amsterdam: Elsevier 10.1016/j.autrev.2010.07.009

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Microscopic polyangiitis (MPA) is a member of the family of ANCA-associated vasculitides. Its characteristic histology shows a necrotizing small vessel vasculitis with little or absent immune deposits (pauci-immune vasculitis). In Western countries MPA shows a lower prevalence than Wegener's disease, it affects more men than women and commences at the age of > or = 50 years. The two organs most typically involved and often defining prognosis are the kidneys and the lungs. MPA may concomitantly or sequentially involve other organs such as the nervous system, the skin, the musculoskeletal system, but also the heart, the eye and the intestines. Treatment decisions should be based on severity and pattern of organ involvement and respect the five factor score (FFS). Life- or organ- threatening disease is treated with glucocorticoids and (pulse) cyclophosphamide. Plasmapheresis and i.v.immunoglobulins have been shown to be beneficial as additional measure in severe cases. If renal function is preserved, Methotrexate may be considered to induce remission, and if the FFS equals 0, remission may be induced with glucocorticoid monotherapy. Maintenance therapy is recommended with Azathioprin, mycophenolate mofetil may be used as a second line drug. Biologic agents such as monoclonal antibodies to tumor necrosis factor a and B cell depleting rituximab have been shown to bear remission-inducing quality.

Item Type:

Journal Article (Original Article)

Division/Institute:

04 Faculty of Medicine > Department of Dermatology, Urology, Rheumatology, Nephrology, Osteoporosis (DURN) > Clinic of Rheumatology, Clinical Immunology and Allergology

UniBE Contributor:

Villiger, Peter Matthias

ISSN:

1568-9972

Publisher:

Elsevier

Language:

English

Submitter:

Factscience Import

Date Deposited:

04 Oct 2013 14:10

Last Modified:

05 Dec 2022 14:01

Publisher DOI:

10.1016/j.autrev.2010.07.009

PubMed ID:

20656070

Web of Science ID:

000283211600005

URI:

https://boris.unibe.ch/id/eprint/1695 (FactScience: 203579)

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