Details

Autor(en) / Beteiligte

• 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

Titel

Anti-Neuronal IgG4 Autoimmune Diseases and IgG4-Related Diseases May Not Be Part of the Same Spectrum: A Comparative Study

Ist Teil von

• Frontiers in immunology, 2022-01, Vol.12, p.785247-785247

Ort / Verlag

Switzerland: Frontiers Media S.A

Erscheinungsjahr

2022

Link zum Volltext

Quelle

MEDLINE

Beschreibungen/Notizen

• 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. We collected and analyzed clinical, serological, and histopathological data in 50 patients with anti-neuronal IgG4-AID and 19 patients with IgG4-RLD. A significantly higher proportion of IgG4-RLD patients had serum IgG4 elevation when compared to IgG4-AID patients (52.63% vs. 16%, = .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 ( = .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. Our observations suggest that anti-neuronal IgG4-AID and IgG4-RLD are most likely distinct disease entities.