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Updated: Jan 7, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Systemic Sclerosis-Recent Advances in Diagnosis and Management
William J Kerrigan1, Alistair C Church1,2, Ariane L Herrick3,4
1Scottish Pulmonary Vascular Unit, Golden Jubilee National Hospital, NHS Golden Jubilee, Glasgow, UK.
Systemic sclerosis treatment has advanced significantly, with new options for early diffuse disease, digital ulcers, lung disease, and pulmonary arterial hypertension. Ongoing research promises further progress in managing this complex connective tissue disease.
Area of Science:
- Rheumatology
- Immunology
- Pulmonology
Background:
- Systemic sclerosis is a severe multisystem connective tissue disease.
- It presents significant morbidity and mortality.
- Recent therapeutic progress has been substantial.
Purpose of the Study:
- To provide an overview of systemic sclerosis.
- To highlight recent advances in managing specific complications.
- To focus on early diffuse cutaneous disease, digital vasculopathy, interstitial lung disease, and pulmonary arterial hypertension.
Main Methods:
- Review of recent scientific literature and clinical trial data.
- Synthesis of information on emerging and established therapies.
- Focus on evidence-based treatment strategies.
Main Results:
- Autologous hematopoietic stem cell transplantation is an option for select early diffuse systemic sclerosis patients.
- Phosphodiesterase type 5 inhibitors and bosentan improve digital ulceration.
- Understanding of immunosuppressants and antifibrotics for interstitial lung disease has improved.
- Early combination therapies are increasingly used for pulmonary arterial hypertension.
Conclusions:
- Substantial advances have been made in managing systemic sclerosis.
- Current therapeutic strategies offer improved outcomes for key complications.
- Ongoing clinical trials are expected to yield further therapeutic innovations.
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