Last reviewed: 20 Sep 2020
Last updated: 05 Jan 2018



History and exam

Key diagnostic factors

  • age 40 to 60 years
  • fever
  • weight loss
  • myalgia or arthralgia
  • mononeuritis multiplex
  • paraesthesia
  • muscle tenderness
  • abdominal pain
  • skin manifestations
  • diastolic blood pressure >90 mmHg
  • blood transfusion at a time before routine screening for hepatitis B virus (HBV)
  • previous or current intravenous drug abuse
  • recent hepatitis B virus (HBV) infection
  • testicular pain

Other diagnostic factors

  • male sex
  • hairy cell leukaemia
  • seizure
  • upper motor neuron weakness
  • GI bleeding
  • peritonitis
  • monocular blindness
  • chest pain
  • congestive cardiac failure
  • tender breast lumps

Risk factors

  • hepatitis B virus (HBV) infection
  • age 40 to 60 years
  • hairy cell leukaemia
  • blood transfusion at a time before routine screening for HBV
  • hepatitis C virus (HCV) infection
  • male sex

Diagnostic investigations

1st investigations to order

  • CRP
  • ESR
  • FBC
  • complement
  • serum creatinine
  • midstream urine analysis
  • liver function tests
  • HBV serology
  • hepatitis C virus (HCV) serology
  • cryoglobulins
  • blood culture
  • creatine kinase
  • anti-neutrophil cytoplasmic antibodies (ANCA)
  • ANA
  • anti-double-stranded DNA antibodies (anti-dsDNA)
  • rheumatoid factor
  • antibodies to cyclic citrullinated peptides (anti-CCP antibodies)
  • lupus anticoagulant
  • IgG antiphospholipid antibodies
  • B2 glycoprotein
  • fibrinogen
  • conventional digital subtraction angiography
  • echocardiography
More 1st investigations to order

Investigations to consider

  • magnetic resonance angiography (MRA)
  • CT angiography
  • biopsy of affected tissue
  • HIV serology
More investigations to consider

Treatment algorithm


Consultant Rheumatologist

Auckland and Counties Manukau District Health Boards


New Zealand


RS declares that he has no competing interests.

Rheumatology Registrar

Nuffield Orthopaedic Centre




JR has attended symposia sponsored by Roche Ltd.

Professor of Medicine

Rheumatology Department

Hopital Cochin




LG is an author of a number of references cited in this monograph.

Professor of Rheumatology

NIHR Musculoskeletal Biomedical Research Unit

Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Science

University of Oxford




RL has attended symposia sponsored by GlaxoSmithKline, the manufacturer of Epivir (lamivudine), and has received sponsorship from Roche, Abbot Ltd, Schering Plough (the manufacturer of Remicade [infliximab]), and Wyeth (the manufacturer of Enbrel [etanercept]) to attend conferences. RL has received sponsorship from Sanofi Aventis (the manufacturer of Arava [leflunomide]) to support the costs of a meeting, and is on the Roche advisory board looking at treatment of vasculitis with B cell therapy. RL is an author of a number of references cited in this monograph.

Peer reviewersVIEW ALL

Professor and Vice Chair

Department of Medicine

University of Wisconsin Hospital




AB declares that he has no competing interests.

Consultant Rheumatologist

Ipswich Hospital




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 monograph.

Professor of Medicine

Department of Medicine

UMDNJ-Robert Wood Johnson Medical School

New Brunswick



ECE declares that she has no competing interests.

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