Home

Search

Pathway AI

Account ⋅ Sign Out

Table of contents

Systemic sclerosis

Definition
SSc is a systemic autoimmune disease characterized by diffuse fibrosis and vasculopathy of the skin and internal organs.
1
Pathophysiology
SSc is caused by a complex interplay of environmental factors that trigger an abnormal immune response in genetically predisposed individuals.
3
Epidemiology
The overall incidence and prevalence of SSc in the US are 5.6 cases per 100,000 person-years and 13.5-18.4 cases per 100,000 persons, respectively.
2
Disease course
The resultant vascular injury and endothelial dysfunction lead to vasoconstriction, excessive deposition of extracellular matrix and collagen, hypertrophy of intimal and medial layers of small blood vessels, and endothelial cell apoptosis, resulting in clinical manifestations of Raynaud's phenomenon, digital ulcers, pulmonary arterial hypertension, renal failure, obstructive vasculopathy, and fibrosis of the skin and internal organs.
3
Prognosis and risk of recurrence
The estimated 5-year and 10-year cumulative survival from the time of diagnosis is 74.9% and 62.5%, respectively.
4
Key sources
The following summarized guidelines for the evaluation and management of systemic sclerosis are prepared by our editorial team based on guidelines from the American Thoracic Society (ATS 2024), the American College of Rheumatology (ACR 2023), the European Society of Cardiology (ESC/ERS 2022), the European League Against Rheumatism (EULAR 2022; 2017), the Canadian Cardiovascular Society (CCS/CTS 2020), the French Society of Pulmonology (SPLF 2017), the European Society for Vascular Medicine (ESVM 2017), and the British Thoracic Society (BTS/TSANZ/ITS 2008).
1
2
3
4
5
6
7
8
9
10
11
12
13
14
15

Guidelines

1.Diagnostic investigations

Screening for pulmonary hypertension: as per ESC 2022 guidelines, obtain an annual assessment of the risk of having pulmonary arterial hypertension in patients with SSc.
B
consider obtaining an annual assessment of the risk of pulmonary arterial hypertension in patients with connective tissue disease with overlap features of SSc.
B
Show 5 more
Create free account

More topics in this section

  • Autoantibody titers

  • Screening for chronic infections

2.Medical management

Management of skin disease: consider methotrexate for the treatment of skin manifestations in early diffuse SSc.
B

More topics in this section

  • Management of Raynaud's phenomenon

  • Management of digital ulcerations

  • Management of pulmonary hypertension

  • Management of ILD (immunosuppressants)

  • Management of ILD (corticosteroids)

  • Management of ILD (antifibrotic agents)

  • Management of ILD (IVIG/plasma exchange)

  • Management of ILD (stem cell transplantation and/or lung transplantation)

  • Management of gastrointestinal disease

  • Management of scleroderma renal crisis

3.Therapeutic procedures

Hematopoietic stem cell transplantation: consider HSCT for the treatment of selected patients with rapidly progressive SSc who are at risk of organ failure.
B

4.Preventative measures

Routine immunizations: consider offering high-dose or adjuvanted influenza vaccination, rather than regular-dose influenza vaccination, in ≥ 65 years old patients with rheumatic or musculoskeletal diseases and in 18-65 years old patients with rheumatic or musculoskeletal diseases on immunosuppressive medications.
C
Show 17 more

More topics in this section

  • Prophylaxis for P. jirovecii pneumonia

5.Follow-up and surveillance

Serial respiratory assessment: as per ACR 2023 guidelines, consider obtaining pulmonary function testing, HRCT of the chest, or both, and ambulatory desaturation testing for monitoring patients with SSc-related ILD.
C
Show 3 more