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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
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Systemic sclerosis
1Service de médecine interne, hôpital Cochin, Centre de référence national pour les maladies systémiques auto-immunes rares, DHU Authors, Assistance publique-Hôpitaux de Paris, Paris, France.
La Revue Du Praticien
|December 5, 2018
Summary
Systemic sclerosis (SSc) is a connective tissue disease more common in women, characterized by fibrosis and vascular issues. Early diagnosis and treatment of complications like pulmonary arterial hypertension are crucial for managing this condition.
Area of Science:
- Rheumatology
- Immunology
- Pulmonology
Background:
- Systemic sclerosis (SSc) is a rare autoimmune disease affecting women disproportionately.
- Prevalence ranges from 30-240 cases per million people.
- SSc presents in limited and diffuse forms based on skin involvement.
Purpose of the Study:
- To outline the key characteristics, manifestations, and treatment approaches for Systemic Sclerosis.
- To highlight the prognostic implications of SSc subtypes and complications.
Main Methods:
- Review of clinical manifestations, including fibrosing lesions and vascular abnormalities.
- Identification of auto-antibodies associated with SSc.
- Summary of current treatment strategies for SSc and its visceral complications.
Main Results:
- SSc involves skin and pulmonary fibrosis, Raynaud's phenomenon, and pulmonary arterial hypertension (PAH).
- Key auto-antibodies include anti-centromere, anti-topoisomerase 1, and anti-RNA polymerase III.
- Diffuse SSc with pulmonary involvement indicates a poor prognosis.
Conclusions:
- Systemic sclerosis requires comprehensive management addressing fibrotic and vascular complications.
- Immunosuppressive therapy is indicated for early-onset or rapidly progressing diffuse SSc with lung disease.
- Treatment focuses on symptom management, visceral complication treatment (PAH, renal crisis), and immunosuppression.
Keywords:
systemic sclerosis
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