Follicular T cells and tissue IgG3 or IgG4 expressing plasma cells in a case of IgG4-related systemic disease with interstitial nephritis - Sorbonne Université Access content directly
Preprints, Working Papers, ... Year : 2010

Follicular T cells and tissue IgG3 or IgG4 expressing plasma cells in a case of IgG4-related systemic disease with interstitial nephritis

Pascale Cervera-Pierot
  • Function : Author
  • PersonId : 890507
Sophie de Seigneux
  • Function : Author
  • PersonId : 890508
Karine Dahan
  • Function : Author
  • PersonId : 890509
Bettina Fabiani
  • Function : Author
  • PersonId : 890510
Patrice Callard
  • Function : Author
  • PersonId : 890511

Abstract

An 83-year-old man presented with weight loss, lymphadenopathies, anicteric cholestasis, and a striking hypergammaglobulinemia. He developed renal failure with tubular proteinuria. Investigations revealed pronounced increase of serum polyclonal IgG1, IgG3 and IgG4, and salivary gland and kidney interstitium infiltrates that included B-lymphocytes, T-lymphocytes and IgG3+ and IgG4+ plasma cells. Remarkably, lymph node B-cell follicles germinal centers were invaded by numerous T-cells, mostly CD4+. The diagnosis was IgG4-related systemic disease. Rituximab (375 mg/m²/week for 4 weeks) induced a dramatic and sustained clinical and biological improvement. To date, very little is known on the mechanisms underlying IgG4-related systemic disease. In addition to perfectly illustrate the broad spectrum of this protean condition, the present case provides further insights on its pathophysiology. The efficacy of rituximab suggests a critical role of CD20+ B-cells in the disease clinical expression and progression. The overexpression of IgG1 and IgG3, in addition to IgG4, and the unusual abundance of CD4+ T-cells in germinal centers of the B-cell follicles points to a possible role of B follicular helper T-cells in enhancing a skewed B-cell terminal maturation.
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Dates and versions

hal-00559440 , version 1 (25-01-2011)

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  • HAL Id : hal-00559440 , version 1

Cite

Mohamad Zaidan, Pascale Cervera-Pierot, Sophie de Seigneux, Karine Dahan, Bettina Fabiani, et al.. Follicular T cells and tissue IgG3 or IgG4 expressing plasma cells in a case of IgG4-related systemic disease with interstitial nephritis. 2010. ⟨hal-00559440⟩
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