IgG4-related disease-rare but you should not forget it

Frederico Augusto Gurgel Pinheiro; Ivanio Alves Pereira; Alexandre Wagner Silva de Souza; Henrique Ayres Mayrink Giardini; Rafael Alves Cordeiro

doi:10.1186/s42358-024-00374-y

IgG4-related disease-rare but you should not forget it

Adv Rheumatol. 2024 May 3;64(1):35. doi: 10.1186/s42358-024-00374-y.

Authors

Frederico Augusto Gurgel Pinheiro^{1

2}, Ivanio Alves Pereira³, Alexandre Wagner Silva de Souza⁴, Henrique Ayres Mayrink Giardini⁵, Rafael Alves Cordeiro⁵

Affiliations

¹ Rheumatology Division, Universidade Federal de São Paulo (UNIFESP), São Paulo, SP, Brazil. [email protected].
² Universidade Federal de São Paulo - Disciplina de Reumatologia, Rua Botucatu, 740, 3o andar, São Paulo, SP, 04023-062, Brazil. [email protected].
³ Universidade do Sul de Santa Catarina (UNISUL), Florianopolis, SC, Brazil.
⁴ Rheumatology Division, Universidade Federal de São Paulo (UNIFESP), São Paulo, SP, Brazil.
⁵ Rheumatology Division, Faculdade de Medicina FMUSP, Universidade de Sao Paulo, Sao Paulo, SP, Brazil.

PMID: 38702764
DOI: 10.1186/s42358-024-00374-y

Abstract

Immunoglobulin G4-related disease is a systemic immune-mediated disease with insidious evolution characterized by fibroinflammatory lesions over virtually any organ system. Despite the remarkable progression of knowledge, its etiology remains undefined. Due to its relapse-remitting pattern, it could accumulate irreversible damage, increasing comorbidities and mortality. This paper emphasizes key concepts for diagnosing and treating patients with this condition.

Keywords: Chronic relapsing-remitting inflammatory condition; Fibroinflammatory disease; IgG4-RD; Immunoglobulin G4-related disease; Rare diseases; Review; Rheumatic diseases.

Publication types

Review

MeSH terms

Humans
Immunoglobulin G / blood
Immunoglobulin G4-Related Disease* / complications
Immunoglobulin G4-Related Disease* / diagnosis
Rare Diseases

Substances

Immunoglobulin G