What confirms the diagnosis of scleroderma?

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Last updated: February 7, 2025View editorial policy

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From the Guidelines

The diagnosis of scleroderma is confirmed by a combination of clinical features, including skin thickening, Raynaud phenomenon, and specific antibodies, as well as imaging and laboratory tests.

Key Diagnostic Features

  • Skin involvement: Characterized by thickening and fibrosis, which can be limited (lcSSc) or diffuse (dcSSc) 1
  • Raynaud phenomenon: Nearly all patients with SSc have Raynaud phenomenon, and half have digital ulcers 1
  • Specific antibodies: Such as anti-centromere, anti-topoisomerase (Scl-70), and anti-RNA polymerase antibodies, which are associated with SSc 1

Diagnostic Tests

  • Imaging tests: High-resolution computed tomography (HRCT) of the lungs to assess for interstitial lung disease (ILD) 1
  • Laboratory tests: Pulmonary function testing, electrocardiography, and blood tests to assess for organ involvement and specific antibodies 1

Diagnostic Criteria

  • American College of Rheumatology (ACR) criteria: Include a combination of clinical features, such as skin thickening, Raynaud phenomenon, and specific antibodies, as well as imaging and laboratory tests 1
  • European League against Rheumatism (EULAR) recommendations: Emphasize the importance of early diagnosis and screening for organ involvement, including ILD and pulmonary arterial hypertension (PAH) 1

From the Research

Diagnosis of Scleroderma

The diagnosis of scleroderma can be confirmed by the presence of several key factors, including:

  • Raynaud's phenomenon 2, 3
  • Antinuclear antibodies (ANA) positivity 2, 3
  • Distribution of sclerosis 3
  • Scleroderma-specific autoantibodies 4
  • Nailfold video capillaroscopy and evaluation of specific disease antibodies (anticentromere and anti-topoisomerase I) 2

Diagnostic Clues

The lack of certain characteristics can also be important diagnostic clues, including:

  • Absence of Raynaud's phenomenon 4
  • Absence of capillaroscopic abnormalities 4
  • Absence of scleroderma-specific autoantibodies 4

Laboratory Diagnostics

Laboratory diagnostics play a crucial role in the diagnosis and monitoring of systemic scleroderma, including:

  • Laboratory tests to detect autoantibodies and other biomarkers 5
  • Imaging studies to assess internal organ involvement 5

Early Diagnosis

Early diagnosis of systemic scleroderma is essential to prevent disease progression and improve patient outcomes, with a focus on:

  • Early identification of Raynaud's phenomenon 2
  • Prompt initiation of therapy 2, 6

References

Guideline

Guideline Directed Topic Overview

Dr.Oracle Medical Advisory Board & Editors, 2025

Research

Differential diagnosis of scleroderma and pseudoscleroderma.

Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG, 2007

Research

[Early diagnosis of systemic scleroderma].

Vnitrni lekarstvi, 2022

Professional Medical Disclaimer

This information is intended for healthcare professionals. Any medical decision-making should rely on clinical judgment and independently verified information. The content provided herein does not replace professional discretion and should be considered supplementary to established clinical guidelines. Healthcare providers should verify all information against primary literature and current practice standards before application in patient care. Dr.Oracle assumes no liability for clinical decisions based on this content.

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