Last reviewed: November 2017
Last updated: November  2017

Summary

Definition

History and exam

Key diagnostic factors

  • presence of risk factors
  • Gottron's papules
  • heliotrope rash with or without peri-orbital oedema
  • macular violaceous erythema
  • peri-ungual erythema, nail-fold capillary dilation, cuticular over-growth
  • 'mechanic's' hands
  • proximal muscle weakness

Other diagnostic factors

  • photosensitivity
  • poikiloderma vasculare atrophicans
  • pruritus
  • fatigue and malaise
  • dyspnoea
  • weight loss
  • fever
  • myalgia
  • arthralgia
  • dysphagia
  • palpitations and syncope
  • Raynaud's phenomenon
  • cutaneous calcinosis
  • erythroderma
  • vesicobullous lesions
  • leukocytoclastic vasculitis
  • cutaneous necrosis
  • non-scarring alopecia

Risk factors

  • genetic predisposition
  • bimodal age distribution: children and age >40 years
  • female gender
  • black race
  • UV radiation
  • infectious agents
  • drugs
  • immunisation

Diagnostic investigations

1st investigations to order

  • serum CK
  • serum aldolase
  • muscle biopsy
  • EMG
  • skin biopsy
  • ANA
Full details

Investigations to consider

  • myositis-specific antibodies (MSAs)
  • muscle MRI
  • ECG
  • echocardiography
  • cardiac troponin I
  • CXR
  • PFTs
  • high-resolution CT of chest
  • barium swallow or video-fluoroscopic assessment of swallow
Full details

Treatment algorithm

Contributors

Authors VIEW ALL

Consultant Physician and Rheumatologist

Department of Rheumatology

Centre for Rheumatic Diseases

Glasgow Royal Infirmary

Glasgow

UK

Disclosures

SAS declares that she has no competing interests.

Consultant Physician and Rheumatologist

Department of Rheumatology

Glasgow Royal Infirmary

Glasgow

UK

Disclosures

RM holds shares in GSK to the value less than £12,000.

Dr Sarah A. Saunders and Dr Rajan Madhok would like to gratefully acknowledge Dr Nicola Alcorn, a previous contributor to this monograph. NA declares she has no competing interests.

Peer reviewers VIEW ALL

Assistant Professor of Neurology

Director of EMG Laboratory & Peripheral Neuropathy Center

Department of Neurology & Neurosciences

New Jersey Medical School

Newark

NJ

Disclosures

NS declares that he has no competing interests.

Physician

Department of Neurology

Penn Presbyterian Medical Center

University of Pennsylvania Health System

Philadelphia

PA

Disclosures

SK declares that he has no competing interests.

Assistant Professor

Associate Director

Arthritis Program

Director

Behcet's Syndrome Center

Seligman Center for Advanced Therapeutics

New York University Hospital for Joint Diseases

New York

NY

Disclosures

YY has served as a consultant for Bristol-Myers Squibb, Roche, Celgene, Centocor, and UCB, and has been a speaker for Pfizer and Bristol-Myers Squibb.

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