Maladie systémique associée aux IgG4 : une pathologie émergente ? Mise au point et revue de la littérature
La Revue de Médecine Interne, ISSN: 0248-8663, Vol: 33, Issue: 1, Page: 23-34
2012
- 42Citations
- 35Captures
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Metrics Details
- Citations42
- Citation Indexes42
- 42
- CrossRef35
- Captures35
- Readers35
- 18
- 17
Article Description
Le syndrome d’hyper-IgG4, ou maladie systémique associée aux IgG4, est une entité de description récente caractérisée par la présence d’une ou plusieurs atteintes fibro-inflammatoires d’organe, associées le plus souvent à une élévation des IgG4 sériques. Les lésions tissulaires sont caractérisées par une fibrose et un infiltrat inflammatoire lymphocytaire et plasmocytaire, avec prédominance de plasmocytes IgG4+. Outre les tableaux de pancréatite sclérosante (dite « auto-immune ») les premiers décrits, les atteintes d’organe rapportées sont nombreuses et peuvent être associées chez un même patient. Parmi ces atteintes, on rapporte des tableaux de sialadénite, de dacryoadénite, de polyadénopathies, d’aortite, de cholangite sclérosante, de néphrite interstitielle, de fibrose rétropéritonéale ou encore de pseudotumeurs inflammatoires. L’ensemble de ces atteintes d’organes, survenant le plus souvent après 50 ans, partagent des lésions histologiques communes. L’évolution est habituellement marquée par une grande corticosensibilité. Cette revue de la littérature souligne les difficultés diagnostiques, en particulier biologiques et histologiques, essentiellement en rapport avec l’absence de critères validés pour le diagnostic de maladie systémique associée aux IgG4. Il reste aussi à caractériser à l’avenir l’épidémiologie et la physiopathologie de ce nouveau syndrome, mais aussi à en préciser la prise en charge thérapeutique. Hyper-IgG4 syndrome, or IgG4-related systemic disease (IgG4-RSD), has been recently characterized by the association of a focal or diffuse enlargement in one or more organs, elevated levels of serum IgG4 and histopathological findings including “storiform” fibrosis and prominent infiltration of lymphocytes and IgG4-positive plasma cells. Pancreas was the first organ involved with sclerosing pancreatitis (or autoimmune pancreatitis). Since this first description, many extrapancreatic lesions have been described, even in the absence of pancreatitis and include sialadenitis, lacrimal gland inflammation, lymphadenopathy, aortitis, sclerosing cholangitis, tubulointerstitial nephritis, retroperitoneal fibrosis or inflammatory pseudotumors. Multiorgan lesions can occur synchronously or metachronously in a same patient, usually after 50 years of age. They all share common histopathological findings. The disease often responds well to corticosteroid therapy. In this literature review on IgG4-RSD, we present historical, epidemiological and clinical characteristics, and we review the biological and histological diagnostic criteria. To date there is no international validated diagnostic criteria. Pathophysiological hypothesis and therapeutic approaches are also discussed.
Bibliographic Details
http://www.sciencedirect.com/science/article/pii/S0248866311006916; http://dx.doi.org/10.1016/j.revmed.2011.08.012; http://www.scopus.com/inward/record.url?partnerID=HzOxMe3b&scp=84855194936&origin=inward; http://www.ncbi.nlm.nih.gov/pubmed/21955722; https://linkinghub.elsevier.com/retrieve/pii/S0248866311006916; https://dx.doi.org/10.1016/j.revmed.2011.08.012
Elsevier BV
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