Summary
Definition
History and exam
Key diagnostic factors
- Gottron papules
- heliotrope rash with or without periorbital edema
- macular violaceous erythema
- periungual erythema, nail-fold capillary dilation, cuticular overgrowth
- "mechanic's" hands
- proximal muscle weakness
Other diagnostic factors
- photosensitivity
- poikiloderma vasculare atrophicans
- pruritus
- fatigue and malaise
- dyspnea
- weight loss
- fever
- myalgia
- arthralgia
- dysphagia
- palpitations and syncope
- Raynaud phenomenon
- cutaneous calcinosis
- erythroderma
- vesicobullous lesions
- leukocytoclastic vasculitis
- cutaneous necrosis
- nonscarring alopecia
Risk factors
- genetic predisposition
- bimodal age distribution: children and age >40 years
- female gender
- black race
- ultraviolet radiation
- infectious agents
- drugs
- immunization
Diagnostic investigations
1st investigations to order
- serum creatine kinase (CK)
- serum aldolase
- muscle biopsy
- electromyogram (EMG)
- skin biopsy
- antinuclear antibody (ANA)
Investigations to consider
- myositis-specific antibodies (MSAs)
- muscle MRI
- ECG
- echocardiography
- cardiac troponin I
- chest x-ray (CXR)
- pulmonary function tests (PFTs)
- high-resolution CT of chest
- barium swallow or videofluoroscopic assessment of swallow
Treatment algorithm
Contributors
Authors
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
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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