Polyarteritis nodosa, microscopic polyangiitis and Churg–Strauss syndrome
- 1 May 1998
- journal article
- review article
- Published by SAGE Publications in Lupus
- Vol. 7 (4), 238-258
- https://doi.org/10.1191/096120398678920055
Abstract
Polyarteritis nodosa (PAN), first described by Küssmaul and Maier, is a well-known form of necrotizing angiitis whose manifestations are weight loss, fever, asthenia, peripheral neuropathy, renal involvement, musculoskeletal and cutaneous manifestations, hypertension, gastrointestinal tract involvement, and cardiac failure. Recently individualized from PAN, microscopic polyangiitis (MPA) is a systemic vasculitis of small-size vessels whose clinical manifestations are very similar to those of PAN, but it is characterized by the presence of rapidly progressive glomerulonephritis (RPGN), which is nearly constant, and pulmonary involvement usually absent in PAN. Churg–Strauss syndrome (CSS) is a disorder characterized by hypereosinophilia and systemic vasculitis similar to that of PAN and occurring in individuals with asthma and allergic rhinitis. Considering the etiologies of PAN, primary and secondary vasculitides can also be distinguished because PAN can be the consequence of hepatitis B virus (HBV) infection and sometimes of other etiologic agents. The prognosis of systemic vasculitides has been transformed by corticosteroids that are, except in HBV-related PAN, the basic treatment. Immunosuppressive drugs, especially cyclophophamide, have also contributed to improving the prognosis, but their precise role in the management of these vasculitides is still being elucidated.Keywords
This publication has 70 references indexed in Scilit:
- A prospective, multicenter, randomized trial comparing steroids and pulse cyclophosphamide versus steroids and oral cyclophosphamide in the treatment of generalized wegener's granulomatosisArthritis & Rheumatism, 1997
- Small-Vessel VasculitisNew England Journal of Medicine, 1997
- Polyarteritis Nodosa Related to Hepatitis B Virus A Prospective Study with Long-Term Observation of 41 PatientsMedicine, 1995
- Nomenclature of Systemic VasculitidesArthritis & Rheumatism, 1994
- The American college of rheumatology 1990 criteria for the classification of polyarteritis nodosaArthritis & Rheumatism, 1990
- Idiopathic Microscopic Polyarteritis Nodosa: Ultrastructural Observations on the Renal Vascular and Glomerular LesionsAmerican Journal of Kidney Diseases, 1986
- Systemic Vasculitis with Asthma and EosinophiliaMedicine, 1984
- The Spectrum of VasculitisAnnals of Internal Medicine, 1978
- ASSOCIATION BETWEEN POLYARTERITIS AND AUSTRALIA ANTIGENThe Lancet, 1970
- Periarteritis Nodosa: A Critical ReviewAmerican Journal of Clinical Pathology, 1952