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Systemic sclerosis

What's new

Updated 2023 ATS and 2023 ACR guidelines for the management of systemic sclerosis-related interstitial lung disease.

Background

Overview

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.
2
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.
3
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.
2
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

Guidelines

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 League Against Rheumatism (EULAR 2022,2017), the European Society of Cardiology (ESC/ERS 2022), the Canadian Cardiovascular Society (CCS/CTS 2020), the European Society for Vascular ...
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Diagnostic investigations

Screening for pulmonary hypertension: as per ERS/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
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  • Autoantibody titers

  • Screening for chronic infections

Medical management

Management of skin disease: as per EULAR 2017 guidelines, consider methotrexate for the treatment of skin manifestations in early diffuse SSc.
B

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  • 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

Therapeutic procedures

HSCT: as per EULAR 2017 guidelines, consider HSCT for the treatment of selected patients with rapidly progressive SSc who are at risk of organ failure.
B

Preventative measures

Routine immunizations: as per ACR 2023 guidelines, 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
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  • Prophylaxis for P. jirovecii pneumonia

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
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