Author:
Endmayr Verena,Tunc Cansu,Ergin Lara,De Rosa Anna,Weng Rosa,Wagner Lukas,Yu Thin-Yau,Fichtenbaum Andreas,Perkmann Thomas,Haslacher Helmuth,Kozakowski Nicolas,Schwaiger Carmen,Ricken Gerda,Hametner Simon,Klotz Sigrid,Dutra Lívia Almeida,Lechner Christian,de Simoni Désirée,Poppert Kai-Nicolas,Müller Georg Johannes,Pirker Susanne,Pirker Walter,Angelovski Aleksandra,Valach Matus,Maestri Michelangelo,Guida Melania,Ricciardi Roberta,Frommlet Florian,Sieghart Daniela,Pinter Miklos,Kircher Karl,Artacker Gottfried,Höftberger Romana,Koneczny Inga
Abstract
BackgroundIgG4 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.MethodsWe collected and analyzed clinical, serological, and histopathological data in 50 patients with anti-neuronal IgG4-AID and 19 patients with IgG4-RLD.ResultsA 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.ConclusionOur observations suggest that anti-neuronal IgG4-AID and IgG4-RLD are most likely distinct disease entities.
Subject
Immunology,Immunology and Allergy
Cited by
11 articles.
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