Polyarteritis nodosa

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Teoría 
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Última revisión: 24 Sep 2025

Última actualización: 13 Feb 2024

Polyarteritis nodosa (PAN) is a rare form of systemic vasculitis that affects only medium-sized vessels (i.e., small and medium-sized arteries).

Hepatitis B virus (HBV)-related PAN has become very rare since the introduction of effective immunization programs against the virus.

Both non HBV-related PAN and HBV-related PAN are differentiated from the other small- and medium-vessel vasculitides by the absence of antineutrophil cytoplasmic antibodies, and by confirmation that small vessels (i.e., arterioles, capillaries, venules) are not involved.

Angiography typically demonstrates microaneurysms and focal narrowing in medium-sized blood vessels.

Pathology is characterized by focal and segmental transmural necrotizing inflammation with fibrinoid necrosis in medium-sized vessels.

Treatment for non HBV-related PAN is based on immunosuppression with corticosteroids and cyclophosphamide.

Treatment for HBV-related PAN utilizes a short course of high-dose corticosteroids, followed by a combination of antiviral therapy and plasma exchange.

Prognosis can be determined by use of the 5-factor score.

Definición

The term "periarteritis nodosa" was used in the 19th century to describe any form of systemic vasculitis without a known cause.[1] Since then, the name has changed to polyarteritis nodosa (PAN) and the definition has been refined to: "Necrotizing inflammation of medium-sized or small arteries without glomerulonephritis or vasculitis in arterioles, capillaries, or venules." This definition was proposed at the Chapel Hill Consensus Conference (CHCC) in 1992 and published in 1994.[2]

Anamnesis y examen

Principales factores de diagnóstico

age 40 to 60 years
fever
weight loss
myalgia or arthralgia
mononeuritis multiplex
paresthesia
muscle tenderness
abdominal pain
skin manifestations
diastolic blood pressure >90 mmHg
history of blood transfusion predating introduction of routine HBV screening
previous or current intravenous drug abuse
recent hepatitis B virus (HBV) infection
testicular pain

Todos los datos

Otros factores de diagnóstico

male sex
hairy cell leukemia
seizure
upper motor neuron weakness
gastrointestinal (GI) bleeding
peritonitis
monocular blindness
chest pain
congestive cardiac failure
tender breast lumps

Todos los datos

Factores de riesgo

hepatitis B virus (HBV) infection
age 40 to 60 years
hairy cell leukemia
history of blood transfusion predating introduction of routine HBV screening
hepatitis C virus (HCV) infection
male sex

Todos los datos

Pruebas diagnósticas

Primeras pruebas diagnósticas para solicitar

CRP
erythrocyte sedimentation rate (ESR)
CBC
complement
serum creatinine
midstream urine analysis
liver function tests
hepatitis B virus (HBV) serology
hepatitis C virus (HCV) serology
cryoglobulins
blood culture
creatine kinase
antineutrophil cytoplasmic antibodies (ANCA)
antinuclear antibodies (ANA)
anti-double-stranded DNA antibodies (anti-dsDNA)
rheumatoid factor
antibodies to cyclic citrullinated peptides (anti-CCP antibodies)
lupus anticoagulant
immunoglobulin G antiphospholipid antibodies
B2 glycoprotein
fibrinogen
conventional digital subtraction angiography
echocardiography

Todos los datos

Pruebas diagnósticas que deben considerarse

MR angiography (MRA)
CT angiography
biopsy of affected tissue
HIV serology
genetic test for adenosine deaminase 2 (ADA2) deficiency

Todos los datos

Algoritmo de tratamiento

Agudo

non HBV-related PAN

HBV-related PAN

En curso

relapse of disease

Colaboradores

Autores

Ravi Suppiah, MBChB, PGDipSportMed, MD, FRACP

Consultant Rheumatologist

Auckland and Counties Manukau District Health Boards

Auckland

New Zealand

Divulgaciones

RS declares that he has no competing interests.

Joanna Robson, MBBS, PhD, MRCP

Rheumatology Registrar

Nuffield Orthopaedic Centre

Oxford

Declarações

JR declares that she has no competing interests.

Raashid Luqmani, DM, FRCP, FRCP(E)

Professor of Rheumatology

NIHR Musculoskeletal Biomedical Research Unit

Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Science

University of Oxford

Oxford

Declarações

RL is an author of a number of references cited in this topic.

Agradecimentos

Dr Ravi Suppiah, Dr Joanna Robson and Dr Raashid Luqmani would like to gratefully acknowledge Dr Loic Guillevin, the previous contributor to this topic. LG is an author of a number of references cited in this topic.

Revisores

Alan Bridges, MD

Professor and Vice Chair

Department of Medicine

University of Wisconsin Hospital

Madison

Declarações

AB declares that he has no competing interests.

Richard Watts, MA, DM, FRCP

Consultant Rheumatologist

Ipswich Hospital

Ipswich

Declarações

RW has received fees for consulting from Roche Pharmaceuticals, manufacturer of rituximab. His department has received financial support from Wyeth Pharmaceuticals, manufacturer of etanercept, and from Schering-Plough, manufacturer of infliximab. RW is an author of a number of references cited in this topic.

Ellen C. Ebert, MD

Professor of Medicine

Department of Medicine

UMDNJ-Robert Wood Johnson Medical School

New Brunswick

Declarações

ECE declares that she has no competing interests.

Créditos aos pareceristas

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As afiliações e declarações dos pareceristas referem--se ao momento da revisão.

Referências

Nossas equipes internas de editoria e de evidências trabalham em conjunto com colaboradores internacionais especializados e pares revisores para garantir que forneçamos acesso às informações o mais clinicamente relevantes possível.

Principais artigos

Masi AT, Hunder GG, Lie JT, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum. 1990;33:1094-1100. Resumo

Mahr A, Guillevin L, Poissonnet M, et al. Prevalences of polyarteritis nodosa, microscopic polyangiitis, Wegener's granulomatosis, and Churg-Strauss syndrome in a French urban multiethnic population in 2000: a capture-recapture estimate. Arthritis Rheum. 2004;51:92-99. Resumo

Colmegna I, Maldonado-Cocco JA. Polyarteritis nodosa revisited. Curr Rheumatol Rep. 2005;7:288-296. Resumo

Mukhtyar C, Guillevin L, Cid MC, et al. EULAR recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis. 2009 Mar;68(3):310-7Texto completo Resumo

Ntatsaki E, Carruthers D, Chakravarty K, et al; BSR and BHPR Standards, Guidelines and Audit Working Group. BSR and BHPR guideline for the management of adults with ANCA-associated vasculitis. Rheumatology (Oxford). 2014 Dec;53(12):2306-9.Texto completo Resumo

Jayne D, Rasmussen N, Andrassy K, et al. A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies. N Engl J Med. 2003 Jul 3;349(1):36-44.Texto completo Resumo

De Groot K, Rasmussen N, Bacon PA, et al. Randomized trial of cyclophosphamide versus methotrexate for induction of remission in early systemic antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum. 2005 Aug;52(8):2461-9.Texto completo Resumo

Artigos de referência

Uma lista completa das fontes referenciadas neste tópico está disponível para os usuários com acesso total ao BMJ Best Practice.

Diagnósticos diferenciais
- Granulomatosis with polyangiitis (formerly known as Wegener granulomatosis)
- Microscopic polyangiitis
- Churg-Strauss syndrome
Mais Diagnósticos diferenciais
Diretrizes
- Guideline for vaccinations in patients with rheumatic and musculoskeletal diseases
- BSR and BHPR guidelines for the management of adults with ANCA-associated vasculitis
Mais Diretrizes
Folhetos informativos para os pacientes
Pneumonia: do I need a vaccine?
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Polyarteritis nodosa

Resumen

Definición

Anamnesis y examen

Principales factores de diagnóstico

Otros factores de diagnóstico

Factores de riesgo

Pruebas diagnósticas

Primeras pruebas diagnósticas para solicitar

Pruebas diagnósticas que deben considerarse

Algoritmo de tratamiento

non HBV-related PAN

HBV-related PAN

relapse of disease

Colaboradores

Autores

Ravi Suppiah, MBChB, PGDipSportMed, MD, FRACP

Divulgaciones

Joanna Robson, MBBS, PhD, MRCP

Declarações

Raashid Luqmani, DM, FRCP, FRCP(E)

Declarações

Agradecimentos

Revisores

Alan Bridges, MD

Declarações

Richard Watts, MA, DM, FRCP

Declarações

Ellen C. Ebert, MD

Declarações

Créditos aos pareceristas

Declarações

Referências

Principais artigos

Artigos de referência

Uma lista completa das fontes referenciadas neste tópico está disponível para os usuários com acesso total ao BMJ Best Practice.

Diagnósticos diferenciais

Diretrizes

Folhetos informativos para os pacientes

Inicie sesión o suscríbase para acceder a todo el BMJ Best Practice

Inicie sesión o suscríbase para acceder a todo el BMJ Best Practice

Inicie sesión para acceder a todo el BMJ Best Practice