Last reviewed: November 2017
Last updated: December  2017

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
  • ESR
  • CRP
  • serum urea and creatinine
  • rheumatoid factor
  • ANA
  • anti-cyclic citrullinated peptide (anti-CCP) antibody
  • urinalysis
  • U1-ribonucleoprotein
  • anti-Jo-1
Full details

Investigations to consider

  • CK
  • anti-double-stranded DNA
  • Smith's antigen
  • anti-SS-A and anti-SS-B
  • PFTs (spirometry, lung volumes and diffusion capacity measurement)
  • CXR
  • high-resolution CT scan of chest
  • echocardiogram
  • right-heart catheterisation
  • barium swallow
  • upper GI endoscopy ± biopsy
  • plain x-ray of affected joint(s)
  • EMG
  • nerve conduction studies
  • muscle biopsy
  • lung biopsy
  • renal biopsy
Full details

Emerging tests

  • anti-Ku antibodies
  • antipolymyositis/scleroderma antibodies
Full details

Treatment algorithm

ONGOING

Contributors

Authors VIEW ALL

Associate Professor of Medicine

Division of Rheumatology

Miller School of Medicine

University of Miami

Miami

FL

Disclosures

LY declares that he has no competing interests.

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 monograph. RH has received NIH grants greater than 6 figures USD. RH is the author of some studies referenced in this monograph. RH declares that he has no competing interests.

Peer reviewers VIEW 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.

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