Introduction:

Systemic sclerosis can involve the lung parenchyma leading to serious complications and even death.

Objectives:

To describe the most relevant aspects of interstitial lung disease related to systemic sclerosis emphasizing diagnosis and treatment.

Materials and methods:

A literature review was performed searching in the databases Medline and EMBASE using the MeSH terms «Scleroderma, Systemic", «Lung Diseases, Interstitial» and «Pulmonary Fibrosis»

Results and conclusions:

Interstitial lung disease is a common clinical manifestation of systemic sclerosis and one of the main causes of death. Treatment options are limited and have a modest effect in most of the cases.

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