TY - JOUR
T1 - Update in ethiopathogeny, diagnosis and treatment of the IgG4 related disease
AU - Martínez-Valle, Fernando
AU - Orozco-Gálvez, Olimpia
AU - Fernández-Codina, Andreu
PY - 2018/7/13
Y1 - 2018/7/13
N2 - © 2017 Elsevier España, S.L.U. IgG4 related disease (IgG4-RD) is probably an autoimmune pathology of unknown etiology. Diverse interactions participate in its pathogen between the adaptive and innate immune systems, activating lymphocytes B and T which trigger the inflammatory cascade, which culminates in fibrosis of the organs and their malfunction. It can affect a multitude of organs simultaneously. The diagnosis is based on the correlation of clinical findings with anatomopathological results (lymphoplasmocitary infiltrate, storiform fibrosis, obliterative phlebitis and IgG4 + plasmatic cell count) and with the presence of elevated IgG4 in serum, depending on the criteria used. Corticoids and rituximab are among the few validated treatments available. There are multiple biomarkers and treatments in development. In this review, we aim to go over the principal pathogenic and clinical characteristics of IgG4-RD, as well as its handling, in accordance with the available scientific evidence.
AB - © 2017 Elsevier España, S.L.U. IgG4 related disease (IgG4-RD) is probably an autoimmune pathology of unknown etiology. Diverse interactions participate in its pathogen between the adaptive and innate immune systems, activating lymphocytes B and T which trigger the inflammatory cascade, which culminates in fibrosis of the organs and their malfunction. It can affect a multitude of organs simultaneously. The diagnosis is based on the correlation of clinical findings with anatomopathological results (lymphoplasmocitary infiltrate, storiform fibrosis, obliterative phlebitis and IgG4 + plasmatic cell count) and with the presence of elevated IgG4 in serum, depending on the criteria used. Corticoids and rituximab are among the few validated treatments available. There are multiple biomarkers and treatments in development. In this review, we aim to go over the principal pathogenic and clinical characteristics of IgG4-RD, as well as its handling, in accordance with the available scientific evidence.
KW - Diagnosis
KW - IgG4
KW - Treatment
U2 - 10.1016/j.medcli.2017.10.034
DO - 10.1016/j.medcli.2017.10.034
M3 - Review article
C2 - 29241876
VL - 151
SP - 18
EP - 25
JO - Medicina Clinica
JF - Medicina Clinica
SN - 0025-7753
IS - 1
ER -