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Revista de la Asociación Médica del Centro Médico ABC

2015, Number 2

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An Med Asoc Med Hosp ABC 2015; 60 (2)

Immunoglobulin G4-related disease

Arana RJC, Villalba OP, Baquera HJJ, Amigo CMC

Language: Spanish
References: 22
Page: 129-136
PDF size: 318.05 Kb.


ABSTRACT

IgG4-related disease is an entity recognized a few years ago characterized by lymphocytic infiltration of IgG4-positive plasma cells associated with Th2-type response, with involvement of one or several organs, the pancreas being the most frequent. Most respond to glucocorticoids, but refractory cases usually respond to rituximab. We present a 63-year-old man who started with sicca syndrome associated with periorbital edema, respiratory symptoms, parotid hypertrophy and lymphadenopathy; he received glucocorticoids, azathioprine and, because of unresponsiveness, eight doses of rituximab in two cycles, showing thereafter a considerable improvement. A review of the literature regarding this condition is made.


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