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ABSTRACT: Background
IgG4 is associated with two emerging groups of rare diseases: 1) IgG4 autoimmune diseases (IgG4-AID) and 2) IgG4-related diseases (IgG4-RLD). Anti-neuronal IgG4-AID include MuSK myasthenia gravis, LGI1- and Caspr2-encephalitis and autoimmune nodo-/paranodopathies (CNTN1/Caspr1 or NF155 antibodies). IgG4-RLD is a multiorgan disease hallmarked by tissue-destructive fibrotic lesions with lymphocyte and IgG4 plasma cell infiltrates and increased serum IgG4 concentrations. It is unclear whether IgG4-AID and IgG4-RLD share relevant clinical and immunopathological features.Methods
We collected and analyzed clinical, serological, and histopathological data in 50 patients with anti-neuronal IgG4-AID and 19 patients with IgG4-RLD.Results
A significantly higher proportion of IgG4-RLD patients had serum IgG4 elevation when compared to IgG4-AID patients (52.63% vs. 16%, p = .004). Moreover, those IgG4-AID patients with elevated IgG4 did not meet the diagnostic criteria of IgG4-RLD, and their autoantibody titers did not correlate with their serum IgG4 concentrations. In addition, patients with IgG4-RLD were negative for anti-neuronal/neuromuscular autoantibodies and among these patients, men showed a significantly higher propensity for IgG4 elevation, when compared to women (p = .005). Last, a kidney biopsy from a patient with autoimmune paranodopathy due to CNTN1/Caspr1-complex IgG4 autoantibodies and concomitant nephrotic syndrome did not show fibrosis or IgG4+ plasma cells, which are diagnostic hallmarks of IgG4-RLD.Conclusion
Our observations suggest that anti-neuronal IgG4-AID and IgG4-RLD are most likely distinct disease entities.
SUBMITTER: Endmayr V
PROVIDER: S-EPMC8795769 | biostudies-literature | 2021
REPOSITORIES: biostudies-literature
Endmayr Verena V Tunc Cansu C Ergin Lara L De Rosa Anna A Weng Rosa R Wagner Lukas L Yu Thin-Yau TY Fichtenbaum Andreas A Perkmann Thomas T Haslacher Helmuth H Kozakowski Nicolas N Schwaiger Carmen C Ricken Gerda G Hametner Simon S Klotz Sigrid S Dutra Lívia Almeida LA Lechner Christian C de Simoni Désirée D Poppert Kai-Nicolas KN Müller Georg Johannes GJ Pirker Susanne S Pirker Walter W Angelovski Aleksandra A Valach Matus M Maestri Michelangelo M Guida Melania M Ricciardi Roberta R Frommlet Florian F Sieghart Daniela D Pinter Miklos M Kircher Karl K Artacker Gottfried G Höftberger Romana R Koneczny Inga I
Frontiers in immunology 20220114
<h4>Background</h4>IgG4 is associated with two emerging groups of rare diseases: 1) IgG4 autoimmune diseases (IgG4-AID) and 2) IgG4-related diseases (IgG4-RLD). Anti-neuronal IgG4-AID include MuSK myasthenia gravis, LGI1- and Caspr2-encephalitis and autoimmune nodo-/paranodopathies (CNTN1/Caspr1 or NF155 antibodies). IgG4-RLD is a multiorgan disease hallmarked by tissue-destructive fibrotic lesions with lymphocyte and IgG4 plasma cell infiltrates and increased serum IgG4 concentrations. It is un ...[more]