Highlights
- •IgG4-RD can rarely occur together with pre-existing or concomitant systemic immune-mediated disorders.
- •IgG4-RD can occur in association with systemic autoimmune diseases and chronic granulomatous conditions.
- •Clinicians should consider the distinction between primary and secondary IgG4-RD.
Abstract
Purpose
Autoimmune disorders can occur together especially in genetically predisposed individuals.
We here aimed to assess the occurrence of IgG4-related disease (IgG4-RD) in association
with other systemic immune-mediated conditions.
Methods
We retrospectively analyzed the clinical records of patients with IgG4-RD followed
at the IgG4-RD Clinic of San Raffaele Hospital (Milan, Italy) for pre-existing or
concomitant immune-mediated disorders. IgG4-RD was diagnosed based on histological
findings and on the 2011 Comprehensive Diagnostic criteria. Associated immune-mediated
disorders were diagnosed based on available classification and/or diagnostic criteria.
Results
Two-hundred and thirty-four patients with a definitive diagnosis of IgG4-RD were included
in this study. A pre-existing immune-mediated connective tissue disease was reported
in 6/234 patients (3%): one case each of sarcoidosis, Takayasu arteritis (TA), eosinophilic
granulomatosis with polyangitis (EGPA), and rheumatoid arthritis; and two cases of
granulomatosis with polyangitis (GPA). Organs involved by IgG4-RD included the lungs,
the pancreas, the peritoneum, lacrimal glands, meninges and orbits. Sarcoidosis, EGPA,
and TA preceded the onset of IgG4-RD. GPA preceded IgG4-RD onset in one case and occurred
simultaneously in the other case. Rheumatoid arthritis occurred together with IgG4-RD
in one case.
Conclusion
Our observation suggests that “secondary” IgG4-RD can present in the context of pre-existing
systemic immune-mediated disorders and complicate systemic autoimmune diseases as
well as chronic granulomatous conditions. Further studies are needed to define whether
this peculiar clinical scenario is associated with different genetic backgrounds,
pathological bases, and long-term outcomes.
Keywords
To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to European Journal of Internal MedicineAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Polyautoimmunity in rheumatological conditions.Int J Rheum Dis. 2019; 22: 386-391https://doi.org/10.1111/1756-185X.13454
- Consensus statement on the pathology of IgG4-related disease.Mod Pathol. 2012; 25: 1181-1192https://doi.org/10.1038/modpathol.2012.72
- Antineutrophil cytoplasmic antibody positivity in IgG4-related disease.Medicine. 2016; 95: e4633https://doi.org/10.1097/MD.0000000000004633
- Do ANCA-associated vasculitides and IgG4-related disease really overlap or not?.Int J Rheum Dis. 2019; 22: 1926-1932https://doi.org/10.1111/1756-185X.13693
- IgG4-Related Pachymeningitis: Evidence of Intrathecal IgG4 on Cerebrospinal Fluid Analysis.Ann Intern Med. 2012; 156: 401https://doi.org/10.7326/0003-4819-156-5-201203060-00025
- Antineutrophil cytoplasmic antibody-associated vasculitides and IgG4-related disease: A new overlap syndrome.Autoimmun Rev. 2017; 16: 1036-1043https://doi.org/10.1016/j.autrev.2017.07.020
- Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD).Mod Rheumatol. 2011; 22 (2012): 21-30https://doi.org/10.1007/s10165-011-0571-z
- Diagnostic criteria for Takayasu arteritis.Int J Cardiol. 1996; 54: S141-S147https://doi.org/10.1016/S0167-5273(96)88783-3
- Antineutrophil Cytoplasmic Antibody-Associated Vasculitis in Korea: A Narrative Review.Yonsei Med J. 2019; 60: 10https://doi.org/10.3349/ymj.2019.60.1.10
- The American College of Rheumatology 1990 criteria for the classification of churg-strauss syndrome (allergic granulomatosis and angiitis).Arthritis Rheum. 2010; 33: 1094-1100https://doi.org/10.1002/art.1780330806
- 2010 rheumatoid arthritis classification criteria: an American college of rheumatology/European league against rheumatism collaborative initiative.Arthritis Rheum. 2010; 62: 2569-2581https://doi.org/10.1002/art.27584
- The 2019 American College of Rheumatology/European League against Rheumatism classification criteria for IgG4-related disease.Ann Rheum Dis. 2020; 79: 77-87https://doi.org/10.1136/annrheumdis-2019-216561
- Characteristics of Sjögren's syndrome associated with rheumatoid arthritis.RMD Open. 2022; 8https://doi.org/10.1136/rmdopen-2022-002234
- Inflammatory myositis in systemic lupus erythematosus.Lupus. 2020; 29: 776-781https://doi.org/10.1177/0961203320918021
- Coexistent Takayasu arteritis and sarcoidosis: a case report and review of the literature.Sarcoidosis Vasc Diffuse Lung Dis. 2019; 36: 311-317https://doi.org/10.36141/svdld.v36i4.8667
- IgG4-Related Disease: Results From a Multicenter Spanish Registry.Medicine. 2015; 94: e1275https://doi.org/10.1097/MD.0000000000001275
- IgG4-related systemic disease: features and treatment response in a French cohort: results of a multicenter registry.Medicine. 2012; 91: 49-56https://doi.org/10.1097/MD.0b013e3182433d77
- Nat Rev Dis Primers. 2019; 5https://doi.org/10.1038/s41572-019-0096-x
- New Insights on the Pathogenesis of Takayasu Arteritis: Revisiting the Microbial Theory.Pathogens. 2018; 7https://doi.org/10.3390/pathogens7030073
- Immunopathogenesis of ANCA-associated vasculitis.Int J Mol Sci. 2020; 21: 1-27https://doi.org/10.3390/ijms21197319
- CD8+ T lymphocyte is a main source of interferon-gamma production in Takayasu's arteritis.Sci Rep. 2021; 11: 17111https://doi.org/10.1038/s41598-021-96632-w
- An imbalance between blood CD4+CXCR5+Foxp3+ Tfr cells and CD4+CXCR5+Tfh cells may contribute to the immunopathogenesis of rheumatoid arthritis.Mol Immunol. 2020; 125: 1-8https://doi.org/10.1016/j.molimm.2020.06.003
Article info
Publication history
Published online: March 17, 2023
Accepted:
March 10,
2023
Received in revised form:
February 27,
2023
Received:
January 23,
2023
Publication stage
In Press Corrected ProofIdentification
Copyright
© 2023 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.
