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Presse Med. 2021 Nov;50(3):104073. doi: 10.1016/j.lpm.2021.104073. Epub 2021 Sep 20.

Autoimmunity in lipodystrophy syndromes.

Presse medicale (Paris, France : 1983)

Giovanni Ceccarini, Silvia Magno, Donatella Gilio, Caterina Pelosini, Ferruccio Santini

Affiliations

  1. Obesity and Lipodystrophy Center, Endocrinology Unit, University Hospital of Pisa, Via Paradisa 2, 56124 Pisa, Italy. Electronic address: [email protected].
  2. Obesity and Lipodystrophy Center, Endocrinology Unit, University Hospital of Pisa, Via Paradisa 2, 56124 Pisa, Italy.
  3. Chemistry and Endocrinology Laboratory at University Hospital of Pisa, Pisa, Italy.

PMID: 34547374 DOI: 10.1016/j.lpm.2021.104073

Abstract

Lipodystrophy syndromes are rare, heterogeneous disorders characterized by the complete or partial deficiency of adipose tissue and are classified according to the extent of fat loss in generalized or partial subtypes, or based on the pathogenic mechanisms in genetic or acquired. While in most cases of congenital forms of lipodystrophy a genetic alteration can be identified, the pathogenic mechanisms responsible for the acquired diseases are not fully clarified. Based on the evidence of a positive association between most acquired lipodystrophies and autoimmune disorders including immune mediated alterations in the adipose tissue of patients affected by acquired lipodystrophy, a reaction against white adipose tissue antigens is postulated. Recent acquisitions have shed new light on the possible pathogenic mechanisms and identified novel forms of acquired lipodystrophy which are possibly immune-mediated. The aim of this review is to give an update on acquired lipodystrophies describing pathogenic mechanisms involved and the relationships between acquired lipodystrophies and other autoimmune disorders. Larger studies based on international disease registries are needed to collect accurate information on the prevalence, risk factors, genetic predisposition, natural history, disease markers and treatment efficacy of these ultrarare disorders.

Copyright © 2021 The Authors. Published by Elsevier Masson SAS.. All rights reserved.

Conflict of interest statement

Declaration of Competing Interest G.C. has received fees for consulting and/or received travel funds from the following companies, which are involved with lipodystrophy and/or diabetes: AstraZeneca, A

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