Efficacy of mycophenolate on lung disease and autoimmunity in children with immunodeficiency.

Abstract:

:The autoimmune manifestations of primary immunodeficiencies, such as autoimmune lymphoproliferative syndrome (ALPS) and common variable immunodeficiency (CVID), often constitute a great therapeutic challenge and have a significant impact on patients' morbidity and mortality. The most common autoimmune presentations are autoimmune cytopenias, but organ-related autoimmunity is also frequently observed. From a pulmonology perspective, granulomatous/lymphocytic interstitial lung disease (GLILD) is a severe immunological complication which significantly worsens the clinical outcome of these patients and for which there are currently few guidelines or protocols for treatment. We present three cases where the use of Mycophenolate in the context of autoimmune cytopenias proved beneficial also on the lung disease.

journal_name

Pediatr Pulmonol

journal_title

Pediatric pulmonology

authors

Bucciol G,Petrone A,Putti MC

doi

10.1002/ppul.23757

subject

Has Abstract

pub_date

2017-10-01 00:00:00

pages

E73-E76

issue

10

eissn

8755-6863

issn

1099-0496

journal_volume

52

pub_type

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