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2018 ; 35
(4
): 292-300
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The Clinicopathologic Spectrum of IgG4-Related Disease
#MMPMID29866638
Obiorah IE
; Henao Velasquez A
; Özdemirli M
Balkan Med J
2018[Jul]; 35
(4
): 292-300
PMID29866638
show ga
Immunoglobulin G4-related disease is a fibroinflammatory systemic disease that is
characterized by focal or diffuse organ infiltration by immunoglobulin G4-bearing
plasma cells. Immunoglobulin G4-related disease may affect any organ, and a high
index of suspicion is necessary for early detection to avoid irreversible
fibrosis, organ dysfunction, and death. Tumor-forming lesions are common
radiological features of immunoglobulin G4-related disease that need to be
differentiated from malignancies. The diagnostic approach requires the
integration of clinical, biochemical, and radiographic manifestations with
classic histopathologic features, which remain crucial to diagnosis. The
histology of immunoglobulin G4-related disease is determined by a dense
lymphoplasmacytic infiltrate, storiform fibrosis, and obliterative phlebitis in
the presence of increased immunoglobulin G4-positve plasma cells. Although
immunoglobulin G4-related disease forms a distinct, clinically independent
disease category, many questions and problems remain unanswered, especially on
its pathogenesis and the role of immunoglobulin G4. Advances in the understanding
of immunoglobulin G4-related disease are likely to change the diagnostic approach
in the future and create potential targets for therapeutic purposes. Here we
describe the concept of immunoglobulin G4-related disease and the most recent
knowledge in the clinico-pathological characteristics on this emerging disease.
This study can guide clinicians in early diagnosis and prevent unnecessary
surgical resections.