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Last reviewed: 14 Mar 2025
Last updated: 26 Sep 2023

Summary

Definition

History and exam

Key diagnostic factors

  • presence of risk factors
  • numbness
  • weakness
  • pain
  • sicca symptoms
  • parotid gland enlargement
  • rash, ulcerations, or pigment changes
  • wheeze, cough, other pulmonary signs
  • fever, night sweats, weight loss, and malaise

Other diagnostic factors

  • predisposing conditions causing vasculitis, inflammation, or other nerve damage

Risk factors

  • age over 50 years
  • sarcoidosis
  • hepatitis C
  • cryoglobulinaemia
  • hepatitis B
  • connective tissue disease
  • livedo reticularis
  • primary vasculitis
  • medications
  • HIV infection
  • non-HIV, non-hepatitis infections
  • severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection
  • recreational drug use
  • genetic predisposition

Diagnostic investigations

1st investigations to order

  • electromyogram (EMG)
  • FBC with differential
  • erythrocyte sedimentation rate (ESR)
  • C-reactive protein (CRP)
  • serum creatinine
  • serum glucose
  • cryoglobulins
  • serum complement
  • hepatitis B surface antigen
  • hepatitis C antibodies or RNA
  • anti-HIV antigens or HIV RNA
  • Lyme disease antibodies
  • cytoplasmic and perinuclear antineutrophil cytoplasmic antibodies (c-ANCA and p-ANCA)
  • rheumatoid factor
  • antinuclear antibodies (ANA)
  • antidouble-stranded (ds) DNA
  • anti-Sjogren syndrome-related antigen A (SSA) or -SSB antibodies
  • serum angiotensin-converting enzyme
  • protein electrophoresis and immunofixation
  • chest x-ray
  • urinalysis
  • muscle and nerve biopsy

Investigations to consider

  • anti-Smith (anti-Sm) antibodies
  • anti-topoisomerase I (anti-Scl 70) and anticentromere (ACA) antibodies
  • skin biopsy
  • lip biopsy
  • anti-Hu antibodies
  • cerebrospinal fluid (CSF) analyses
  • CT of chest, abdomen/pelvis
  • positron emission tomography (PET) scan of chest, abdomen, or pelvis
  • conventional angiography
  • magnetic resonance angiography

Treatment algorithm

Contributors

Authors

​Ashok Verma, MD, DM, MBA, FAAN, FANA

Professor of Neurology

Director, Neuromuscular Medicine Fellowship Training Program

University of Miami Miller School of Medicine

Don Soffer Clinical Research Center

Miami

FL

Disclosures

AV declares that he has no competing interests.

Acknowledgements

Professor Ashok Verma would like to gratefully acknowledge Dr Kevin Scott, Dr Milind Kothari, and Dr Jenice Robinson, the previous contributors to this topic.

Disclosures

JR, KS, and MK declare that they have no competing interests.

Peer reviewers

John J. Kelly, MD

Professor and Chairman

Department of Neurology

The George Washington University Medical Center

Washington

DC

Disclosures

JJK declares that he has no competing interests.

Cory Toth, BSc, MD, FRCP(C)

Assistant Professor of Neurosciences

Hotchkiss Brain Institute

University of Calgary

Alberta

Canada

Disclosures

CT declares that he has no competing interests.

Jeremy Bland, FRCP

Consultant Neurophysiologist

East Kent Hospitals NHS Trust

Canterbury

Kings College Hospital NHS Trust

London

UK

Disclosures

JB declares that he has no competing interests.

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