| Autor: |
Inga Koneczny, Poppert K, Lechner C, Tunc C, Yu T, Valach M, Pinter M, Michelangelo Maestri, de Rosa A, Pirker W, Roberta Ricciardi, Florian Frommlet, Angelovski A, Daniela Sieghart, Endmayr, Nicolas Kozakowski, Pirker S, Ludwig Wagner, Carmen Schwaiger, de Simoni D, Almeida Dutra L, Helmuth Haslacher, H oumlftberger R, Thomas Perkmann, Ergin L, Melania Guida, Gerda Ricken, Hametner S, M uumlller Gj, Fichtenbaum A, Weng R |
| Rok vydání: |
2021 |
| Předmět: |
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| Popis: |
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 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 analysed serological, clinical, and histopathological data in 50 patients with anti-neuronal IgG4-AID and 16 patients with IgG4-RLD.ResultsA significantly higher proportion of IgG4-RLD patients had serum IgG4 elevation when compared to IgG4-AID patients (50% vs. 16%, p = .015). Moreover, those IgG4-AID patients with elevated IgG4 did not meet the diagnostic criteria of IgG4-RLD, and their autoantibody titres 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 = .041). 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. |
| Databáze: |
OpenAIRE |
| Externí odkaz: |
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