Disease heterogeneity in IgG4-related hypophysitis: report of two histopathologically proven cases and review of the literature.

Détails

ID Serval
serval:BIB_790A4F9C47AE
Type
Article: article d'un périodique ou d'un magazine.
Collection
Publications
Titre
Disease heterogeneity in IgG4-related hypophysitis: report of two histopathologically proven cases and review of the literature.
Périodique
Virchows Archiv
Auteur(s)
Uccella S., Amaglio C., Brouland J.P., Bianconi E., Ippolito S., Messerer M., Rouiller N., Tanda M.L., Sessa F., La Rosa S.
ISSN
1432-2307 (Electronic)
ISSN-L
0945-6317
Statut éditorial
Publié
Date de publication
09/2019
Peer-reviewed
Oui
Volume
475
Numéro
3
Pages
373-381
Langue
anglais
Notes
Publication types: Journal Article
Publication Status: ppublish
Résumé
IgG4-related hypophysitis (IgG4-RH) is a rare disease, which can occur singularly or as manifestation of a systemic IgG4-related disease (IgG4-RD). Less than one hundred cases have been reported in the literature, very few of which were histopathologically documented. We analyzed the clinical, radiological, and histopathological features of two cases of IgG4-RH, the former observed in a 66-year-old man in the context of an IgG4-RD, and the latter affecting a 21-year-old woman, as an isolated lesion. In addition, we performed a comprehensive review of the previously published histopathologically documented cases of IgG4-RH. Pituitary samples from both patients showed dense lymphoplasmacytic infiltration, interstitial and storiform fibrosis, and high numbers of IgG4-positive plasma cells, consistent with IgG4-RH. From the literature review, we retrieved 18 papers reporting a total of 22 cases of histopathologically documented IgG4-RH. The revision of these cases, also including the two reported herein, showed an equal distribution of IgG4-RH in the two sexes, albeit significant clinico-pathological variation was found between cases arisen in female and male patients, respectively. In detail, IgG4-RH females were affected in their second-third decade of life, with a solitary pituitary lesion, low IgG4 serum level, and frequent association with autoimmune disorders. By contrast, IgG4-RH in men was a disease of the elderly, often in the context of a systemic IgG4-RD, with high IgG4 serum levels. Our study shows that IgG4-RH, as currently defined, is a clinically heterogenous disease, with different features in the two sexes. Indeed, cases diagnosed in young women, as our case 2, mostly do not present other evidence of IgG4-RD and might be better classified as lymphocytic hypophysitis with abundant IgG4+ plasma cells. For this reason, the histopathological examination of the pituitary lesion, particularly in female patients, may still be useful for a correct differential diagnosis with other variants of primary hypophysitis.
Mots-clé
Hypophysitis, IgG4-related disease, IgG4-related hypophysitis, Pituitary gland
Pubmed
Création de la notice
29/03/2019 8:26
Dernière modification de la notice
04/09/2019 5:10
Données d'usage