Summary
Definition
History and exam
Key diagnostic factors
- digital pallor/pain
- arthritis/arthralgia
- swollen hands
- sclerodactyly
- nail fold vascular changes
- dyspnea or cough
- GERD and heartburn
- myalgias or myositis
Other diagnostic factors
- hematuria
- lymphadenopathy
- alopecia
- skin rashes
- proximal muscle weakness
- trigeminal neuralgia
- headaches
- neuropsychiatric disease including psychosis and seizures
- peripheral neuropathy
- fever
Risk factors
- female sex
- age 20 to 40 years
- presence of specific HLA genotypes
Diagnostic investigations
1st investigations to order
- CBC
- erythrocyte sedimentation rate
- CRP
- serum BUN and creatinine
- rheumatoid factor
- antinuclear antibodies
- anti-cyclic citrullinated peptide (anti-CCP) antibody
- urinalysis
- anti-U1 ribonucleoprotein
- anti-Jo-1
Investigations to consider
- creatine kinase
- anti-double-stranded DNA
- Smith antigen
- anti-SS-A and anti-SS-B
- pulmonary function tests (spirometry, lung volumes and diffusion capacity measurement)
- CXR
- high-resolution CT scan of chest
- echocardiogram
- right-heart catheterization
- barium swallow
- upper gastrointestinal endoscopy ± biopsy
- plain x-ray of affected joint(s)
- electromyography
- nerve conduction studies
- muscle biopsy
- lung biopsy
- renal biopsy
Emerging tests
- anti-Ku antibodies
- antipolymyositis/scleroderma antibodies
- other antisynthetase antibodies (PL7, PL12, OJ, EJ, KS, Ha and others)
Treatment algorithm
all patients
Contributors
Authors
Larry Young, MD
Associate Professor of Medicine
Division of Rheumatology
Miller School of Medicine
University of Miami
Miami
FL
Disclosures
LY has developed educational materials for the Duke University workshop on evidence-based practice and received sponsored travel and hotel accommodations as one of the workshop co-directors.
Carlos Lozada, MD
Associate Professor of Medicine
Division of Rheumatology and Immunology
Miller School of Medicine
University of Miami
Miami
FL
Disclosures
CL declares that he has no competing interests.
Acknowledgements
Dr Larry Young and Dr Carlos Lozada would like to gratefully acknowledge Dr Robert Hoffman, a previous contributor to this topic. RH has received NIH grants greater than 6 figures USD. RH is the author of some studies referenced in this topic. RH declares that he has no competing interests.
Peer reviewers
Robert Ortmann, MD
Associate Professor and Director
Division of Rheumatology and Immunology
University of Arkansas for Medical Sciences
Little Rock
AR
Disclosures
RO declares that he has no competing interests.
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.
Simon Bowman, PhD, FRCP
Consultant Rheumatologist and Honorary Senior Clinical Lecturer in Rheumatology
Selly Oak Hospital
Birmingham
UK
Disclosures
SB declares that he has no competing interests.
Differentials
- Systemic lupus erythematosus (SLE)
- Polymyositis
- Scleroderma
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