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Vol. 145. Issue 12.
Pages 539-544 (December 2015)
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Vol. 145. Issue 12.
Pages 539-544 (December 2015)
Review
DOI: 10.1016/j.medcle.2016.04.023
IgG4-related disease
Enfermedad relacionada con IgG4
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Juan González-Morenoa,
Corresponding author
juan.glzmr@gmail.com

Corresponding author.
, Inés Losada Lópeza, Norberto Ortego Centenob
a Servicio de Medicina Interna, Hospital Son Llàtzer, Palma de Mallorca, Spain
b Unidad de Enfermedades Autoinmunes Sistémicas, Hospital Clínico San Cecilio, Granada, Spain
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Tables (5)
Table 1. Different nomenclature used for IgG4 related disease.
Table 2. Other pathologies with presence of IgG4-positive plasma cells.
Table 3. Terminology recommended to describe the histopathological patterns of IgG4-related disease (IgG4-RD).
Table 4. Organs and tissues involved in IgG4-related disease and nomenclature accepted.
Table 5. Differential diagnosis of IgG4-related disease.
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Abstract

IgG4-related disease is a recently described clinicopathological entity showing a wide spectrum of clinical manifestations that share a common pathology. Its most characteristic feature is the formation of inflammatory tumors in different organs, which makes differentiation mainly with neoplastic diseases fundamental. The inflammatory process is typically comprised of IgG4 lymphoplasmacytic cells. The pathophysiological role of the immunoglobulin is not clear. The treatment of choice is corticosteroids. This article aims to summarize the main features of the disease.

Keywords:
IgG4-related disease
Autoimmune pancreatitis
Resumen

La enfermedad relacionada con IgG4 es una entidad clínico-patológica descrita recientemente con un amplio espectro de manifestaciones clínicas que comparten una histopatología en común. Su manifestación más característica es la formación de tumores inflamatorios en diferentes órganos, lo que hace fundamental la diferenciación principalmente de enfermedades neoplásicas. Este fenómeno inflamatorio está compuesto característicamente por linfoplasmocitos productores de IgG4. El papel fisiopatogénico de la inmunoglobulina no está aclarado. El tratamiento de elección son los glucocorticoides. Este trabajo pretende resumir las principales características de la enfermedad.

Palabras clave:
Enfermedad relacionada con IgG4
Pancreatitis autoinmune

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