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Related Experiment Video

Updated: Apr 4, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

Published on: June 16, 2020

Interstitial lung disease in systemic sclerosis: where do we stand?

Susanna Cappelli¹, Silvia Bellando Randone², Gianna Camiciottoli³

¹Dept of Experimental and Clinical Medicine, Division of Rheumatology AOUC, School of Medicine, University of Florence, Florence, Italy susicappelli82@yahoo.it.

European Respiratory Review : an Official Journal of the European Respiratory Society

|September 2, 2015

Summary

This summary is machine-generated.

Systemic sclerosis-associated interstitial lung disease (SSc-ILD) is a major cause of death. Early diagnosis and tailored treatment intensity, ranging from low to high intensity therapies, are crucial for improving patient prognosis and managing SSc-ILD progression.

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Refined Murine Model of Idiopathic Pulmonary Fibrosis

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Refined Murine Model of Idiopathic Pulmonary Fibrosis

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Published on: June 17, 2025

Area of Science:

Rheumatology
Pulmonology
Immunology

Background:

Interstitial lung disease (ILD) is a frequent and fatal complication of systemic sclerosis (SSc).
Despite advances, ILD remains the primary cause of mortality in SSc patients.
Early diagnosis is critical for improving prognosis.

Purpose of the Study:

To review recent advances in the pathogenesis, diagnosis, and treatment of SSc-ILD.
To highlight the importance of early diagnosis and risk stratification for treatment decisions.
To categorize current and emerging treatment strategies for SSc-ILD.

Main Methods:

Review of current literature on SSc-ILD pathogenesis, diagnosis, and treatment.
Discussion of diagnostic tools including pulmonary function tests, high-resolution computed tomography, and lung ultrasound.
Analysis of treatment strategies, including immunosuppressors and novel targeted therapies.

Main Results:

Pulmonary function tests and HRCT are standard diagnostic tools; lung ultrasound shows promise.
Peripheral blood biomarkers correlate with fibrosis severity.
Treatment is stratified into high (stem cell transplantation), medium (cyclophosphamide, rituximab), and low intensity (azathioprine, mycophenolate mofetil) approaches.

Conclusions:

Effective management of SSc-ILD requires early diagnosis and risk assessment.
Nonselective immunosuppressors like cyclophosphamide are mainstays, while rituximab shows potential.
Treatment strategies should be tailored to disease severity, with maintenance therapy following remission.