Last reviewed: November 2017
Last updated: August  2017

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, 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
  • primary vasculitis
  • drug use
  • HIV infection
  • non-HIV, non-hepatitis infections
  • recreational intravenous drug use
  • genetic predisposition

Diagnostic investigations

1st investigations to order

  • EMG
  • FBC with differential
  • ESR
  • 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
  • ANA
  • antidouble-stranded (ds) DNA
  • anti-SSA or SSB antibodies
  • serum angiotensin-converting enzyme
  • protein electrophoresis and immunofixation
  • CXR
  • urinalysis
  • muscle and nerve biopsy
Full details

Investigations to consider

  • anti-Sm antibodies
  • antitopoisomerase I (anti-Scl 70) and anticentromere (ACA) antibodies
  • skin biopsy
  • lip biopsy
  • anti-Hu antibodies
  • CSF analyses
  • CT of chest, abdomen/pelvis
  • PET scan of chest, abdomen, or pelvis
  • conventional angiography
  • MR angiography
Full details

Treatment algorithm

Contributors

Authors VIEW ALL

Associate Professor of Neurology

Colorado Springs Neurological Associates

Colorado Springs

CO

Disclosures

KRS declares that he has no competing interests.

Professor of Neurology

Penn State College of Medicine

Hershey

PA

Disclosures

MJK declares that he has no competing interests.

Dr Kevin Scott and Dr Milind Kothari would like to gratefully acknowledge Dr Jenice Robinson, the previous contributor to this monograph. JR declares that she has no competing interests.

Peer reviewers VIEW ALL

Professor and Chairman

Department of Neurology

The George Washington University Medical Center

Washington

DC

Disclosures

JJK declares that he has no competing interests.

Assistant Professor of Neurosciences

Hotchkiss Brain Institute

University of Calgary

Alberta

Canada

Disclosures

CT declares that he has no competing interests.

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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