Last reviewed: 27 Jun 2021
Last updated: 13 Jan 2021

Summary

Definition

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
  • history of blood transfusion predating introduction of routine HBV screening
  • 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
  • gastrointestinal (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
  • history of blood transfusion predating introduction of routine HBV screening
  • hepatitis C virus (HCV) infection
  • male sex

Diagnostic investigations

1st investigations to order

  • CRP
  • erythrocyte sedimentation rate (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)
  • 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

Investigations to consider

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

Treatment algorithm

Contributors

Authors

Ravi Suppiah, MBChB, PGDipSportMed, MD, FRACP

Consultant Rheumatologist

Auckland and Counties Manukau District Health Boards

Auckland

New Zealand

Disclosures

RS declares that he has no competing interests.

Joanna Robson, MBBS, PhD, MRCP

Rheumatology Registrar

Nuffield Orthopaedic Centre

Oxford

UK

Disclosures

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

UK

Disclosures

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

Acknowledgements

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.

Peer reviewers

Alan Bridges, MD

Professor and Vice Chair

Department of Medicine

University of Wisconsin Hospital

Madison

WI

Disclosures

AB declares that he has no competing interests.

Richard Watts, MA, DM, FRCP

Consultant Rheumatologist

Ipswich Hospital

Ipswich

UK

Disclosures

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

NJ

Disclosures

ECE declares that she has no competing interests.

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