Buscar en
Revista Colombiana de Reumatología (English Edition)
Toda la web
Inicio Revista Colombiana de Reumatología (English Edition) Intranasal eosinophilic angiocentric fibrosis associated with an IgG4-related di...
Journal Information
Download PDF
More article options
Case report
DOI: 10.1016/j.rcreue.2017.02.004
Intranasal eosinophilic angiocentric fibrosis associated with an IgG4-related disease
Fibrosis angiocéntrica eosinofílica intranasal asociada a enfermedad relacionada con IgG4
Jesica Galloa, Cecilia Pisonib, Daniel González Cuetoc, John H. Stoned, Sergio Pairaa,
Corresponding author

Corresponding author.
a Servicio de Reumatología, Hospital J.M. Cullen, Santa Fe, Argentina
b Servicio de Reumatología, CEMIC, Buenos Aires, Argentina
c Servicio de Anatomía Patológica, Buenos Aires, Argentina
d Rheumatology Unit, Massachusetts General Hospital, Boston, MA, United States
Received 05 October 2016. Accepted 20 February 2017
Article information
Full Text
Download PDF
Figures (4)
Show moreShow less
Tables (1)
Table 1. Patient's laboratory data.

Eosinophilic angiocentric fibrosis is an uncommon tumefactive lesion of the ocular orbit and upper respiratory tract. We present a 44-year old man with a history of severe nasal congestion and nasal obstruction. The patient also reported symptoms of sinusitis. Submucous thickening tissue had been locally resected and the involved anterior nasal septum cartilage partially removed. Histopathological examination of the biopsy indicated eosinophilic angiocentric fibrosis. A CT scan of the sinuses showed a high-density focal mass of soft tissue arising from the anterior aspect of the nasal septum. Examination under low power view showed dense concentric fibrosis and mixed inflammatory cells. The fibrosis was in an angiocentric pattern, resembling an onionskin. High power view showed a mixture of lymphocytes, plasma cells, numerous eosinophils, and proliferating fibroblasts. A test for serum IgG performed ten years after the patient's initial presentation was 1421mg/dl (normal range 540–1822mg/dl), and the serum concentration of IgG4 was 168.70mg/dl (normal range 6.1–121mg/dl). A review of the original hematoxylin and eosin-stained slides revealed that this case was stained immunohistochemically with an IgG4 stain showing 50 IgG4-positive plasma cells/HPF.

Eosinophilic angiocentric fibrosis
Sinonasal tract
IgG4-related disease

La fibrosis angiocéntrica eosinofílica es una lesión tumoral infrecuente de la órbita ocular y del tracto respiratorio superior. Presentamos el caso de un hombre de 44 años, con antecedentes de severa congestión y obstrucción nasal, y síntomas de sinusitis. Una TAC de senos paranasales mostró una masa focal de tejidos blandos de alta densidad que crecía desde la cara anterior del septum nasal. Se resecó parte del tabique. Los cortes histológicos mostraron una fibrosis concéntrica densa con patrón angiocéntrico y células inflamatorias mixtas. Se demuestra la presencia de fibrosis angiocéntrica eosinofílica. El dosaje sérico de IgG: 1.421mg/dl (valor normal: 540–1.822) y el de IgG4: 168,70mg/dl (valor normal: 6.1–121). Los preparados histológicos fueron inmunomarcados para IgG4 mostrando 50 plasmocitos IgG4(+)/HPF.

Palabras clave:
Fibrosis angiocéntrica eosinofílica
Tracto sinuso-nasal
Enfermedad relacionada con IgG4


These are the options to access the full texts of the publication Revista Colombiana de Reumatología (English Edition)

If you already have your login data, please click here .

If you have forgotten your password you can you can recover it by clicking here and selecting the option “I have forgotten my password”
Purchase article

Purchasing article the PDF version will be downloaded

Price 19.34 €

Purchase now
Phone for subscriptions and reporting of errors
From Monday to Friday from 9 a.m. to 6 p.m. (GMT + 1) except for the months of July and August which will be from 9 a.m. to 3 p.m.
Calls from Spain
932 415 960
Calls from outside Spain
+34 932 415 960
Article options