Abstract
IgG4-related systemic disease is a protean disorder that covers a wide variety of lesions. We report on a patient with tubulointerstitial nephritis, lymphadenopathies, sialadenitis and retroperitoneal fibrosis. The salivary gland and kidney interstitium were infiltrated with B lymphocytes and T lymphocytes and IgG3(+) and IgG4(+) plasma cells. The overexpression of IgG1 and IgG3, in addition to IgG4, the unusual abundance of interfollicular plasma cells and CD4(+) T cells in germinal centres of lymph nodes, and the dramatic response to rituximab point to possible roles of follicular helper T cells in enhancing a skewed B-cell terminal maturation and of CD20(+) B cells in disease progression.
Publication types
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Case Reports
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Research Support, Non-U.S. Gov't
MeSH terms
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Aged, 80 and over
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Disease Progression
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Humans
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Immunoglobulin G / immunology*
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Lymph Nodes / immunology
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Lymph Nodes / pathology
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Lymphatic Diseases / complications
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Lymphatic Diseases / immunology
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Lymphatic Diseases / pathology*
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Male
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Nephritis, Interstitial / complications
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Nephritis, Interstitial / immunology
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Nephritis, Interstitial / pathology*
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Plasma Cells / immunology
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Plasma Cells / pathology
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Retroperitoneal Fibrosis / complications
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Retroperitoneal Fibrosis / immunology
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Retroperitoneal Fibrosis / pathology*
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Sialadenitis / complications
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Sialadenitis / immunology
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Sialadenitis / pathology*
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T-Lymphocytes / immunology
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T-Lymphocytes / pathology*
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T-Lymphocytes, Helper-Inducer / immunology
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T-Lymphocytes, Helper-Inducer / pathology*