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Case report
Clinical spectrum of IgG4-related disease in Colombia
Espectro clínico de la enfermedad relacionada con IgG4 en Colombia
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Erika-Paola Navarroa,b, Juan-Pablo Susoc, Magali Chamorrod, Andrés Hormazac, Alex Echeverric, Iván Posso-Osorioc, Fabio Bonilla-Abadíac, Juliana Escobar-Steine, Carlos A. Cañasc, Juan Carlos Bravoe, Gabriel J. Tobónc,f,
Corresponding author
gtobon1@yahoo.com

Corresponding author.
a Facultad de Medicina, Universidad CES, Medellín, Colombia
b Departamento de Medicina Interna, Fundación Valle del Lili, Cali, Colombia
c Unidad de Reumatología, Fundación Valle del Lili, Universidad Icesi, Cali, Colombia
d Departamento de Medicina Interna, Universidad del Cauca, Popayán, Colombia
e Unidad de Patología, Fundación Valle del Lili, Cali, Colombia
f Laboratorio de Inmunología, Fundación Valle del Lili, Cali, Colombia
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Received 19 September 2016. Accepted 09 December 2016
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Table 1. Diagnostic criteria for IgG4-related disease.
Abstract

IgG4-related disease is an inflammatory systemic condition noted by the infiltration of different organs by IgG4-bearing plasma cells, as well as elevated serum IgG4 levels. Diagnosis of this condition is complex, and clinical findings are not particularly indicative. In this case series, a description is presented on 4 patients with a wide spectrum of clinical manifestations, in whom, after ruling out different options, a diagnosis of IgG4 related disease was confirmed. Despite this disease not being common, it should be considered among the options whenever multiple organs are affected. This report of patients with IgG4-related disease in Colombia highlights a wide spectrum of clinical presentations, including subglottic stenosis, autoimmune pancreatitis, retroperitoneal fibrosis, and systemic compromise.

Keywords:
IgG4 related disease
Ormond's disease
Mikulicz syndrome
Autoimmune pancreatitis
Resumen

La enfermedad relacionada con IgG4 es una condición inflamatoria sistémica, caracterizada por la infiltración de diversos órganos por complejos formados por células plasmáticas IgG4 positivas, asociadas con niveles elevados de IgG4 en el suero. El diagnóstico de esta enfermedad es complejo y los hallazgos clínicos no son patognomónicos. En esta serie de casos, describimos un amplio espectro clínico en 4 pacientes, en quienes, después de descartar otros diagnósticos, se confirmó la enfermedad relacionada con IgG4. A pesar de que esta enfermedad no es común, se debe considerar entre los diagnósticos diferenciales de enfermedades con afectación de múltiples órganos. Este reporte de pacientes con enfermedad relacionada con IgG4, en Colombia, resalta un amplio espectro de presentaciones clínicas, incluyendo estenosis subglótica, pancreatitis autoinmune, fibrosis retroperitoneal y compromiso sistémico.

Palabras clave:
Enfermedad relacionada con IgG4
Enfermedad de Ormond
Síndrome de Mikulicz
Pancreatitis autoinmune

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