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IgG4 multiorgan lymphoproliferative syndrome as a differential diagnosis of primary Sjögren's syndrome in men?
  1. G Nocturne1,
  2. S Pavy1,
  3. T Lazure2,
  4. Y Taoufik3,
  5. C Miceli1,
  6. X Mariette1
  1. 1Department of Rheumatology, Université Paris-Sud 11, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
  2. 2Department of Pathology, Université Paris-Sud 11, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
  3. 3Department of Immunology, Université Paris-Sud 11, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
  1. Correspondence to X Mariette, Service de Rhumatologie, Hôpital Bicêtre, 78 rue du Général Leclerc, 94275 Le Kremlin Bicêtre, France; xavier.mariette{at}

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In 1888, Mikulicz-Radecki reported a case exhibiting bilateral, painless and symmetrical swellings of the lacrimal, parotid and submandibular glands. In 1953, Morgan and Castleman suggested that Mikulicz disease (MD) should be reconsidered as one of the manifestations of primary Sjögren's syndrome (pSS) as they shared many histopathological characteristics. But recently, several groups demonstrated that MD may differ from pSS. Actually, MD may partly be an IgG4-related systemic disease, the other components of which are still undelineated and include autoimmune pancreatitis, chronic sclerosing sialoadenitis, Riedel's thyroiditis, some cases of cholangitis, retroperitoneal fibrosis.1 Masaki defined a new clinical entity, the IgG4 multiorgan lymphoproliferative syndrome (MOLPS), which includes these manifestations.2 It is characterised by hyper-IgG4 γ-globulinaemia and IgG4 plasma cell infiltration. There are a number of differences with …

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  • Ethics approval This study was conducted with the approval of the CPP Bicêtre.

  • Provenance and peer review Not commissioned; externally peer reviewed.