Last reviewed: June 2020
Last updated: May  2020

Summary

Definition

History and exam

Key diagnostic factors

  • presence of risk factors
  • digital pallor/pain
  • arthritis/arthralgia
  • swollen hands
  • sclerodactyly
  • nail fold vascular changes
  • dyspnoea or cough
  • GORD and heartburn
  • myalgias or myositis

Other diagnostic factors

  • haematuria
  • 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

  • FBC
  • erythrocyte sedimentation rate
  • CRP
  • serum urea and creatinine
  • rheumatoid factor
  • antinuclear antibodies
  • anti-cyclic citrullinated peptide (anti-CCP) antibody
  • urinalysis
  • anti-U1 ribonucleoprotein
  • anti-Jo-1
More 1st investigations to order

Investigations to consider

  • creatine kinase
  • anti-double-stranded DNA
  • Smith's 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 catheterisation
  • barium swallow
  • upper gastrointestinal endoscopy ± biopsy
  • plain x-ray of affected joint(s)
  • electromyography
  • nerve conduction studies
  • muscle biopsy
  • lung biopsy
  • renal biopsy
More investigations to consider

Emerging tests

  • anti-Ku antibodies
  • antipolymyositis/scleroderma antibodies
  • other antisynthetase antibodies (PL7, PL12, OJ, EJ, KS, Ha and others)
More emerging tests

Treatment algorithm

Contributors

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.

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.

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 reviewersVIEW ALL

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.

Professor and Vice Chair

Department of Medicine

University of Wisconsin Hospital

Madison

WI

Disclosures

AB declares that he has no competing interests.

Consultant Rheumatologist and Honorary Senior Clinical Lecturer in Rheumatology

Selly Oak Hospital

Birmingham

UK

Disclosures

SB declares that he has no competing interests.

Use of this content is subject to our disclaimer