Mikulicz´s disease with unilateral exophtalmia – IgG4 related disease


Authors: Hrnčíř Zb 1;  J. Laco 2;  R. Slezák 3;  E. Rencová 4;  M. Drahošová 5;  J. Brtková 6
Authors‘ workplace: II. interní klinika, oddělení revmatologie a klinické farmakologie, 2Fingerlandův ústav patologie, 3Stomatologická klinika, 4Oční klinika, 5Ústav klinické imunologie a alergologie, 6Radiodiagnostická klinika, Lékařská fakulta UK a Fakultní nemocnice, Hrad 1
Published in: Čes. Revmatol., 19, 2011, No. 3, p. 125-130.
Category: Case Report

Overview

A 63 y/o woman was examined for painless enlargement of both submandibular sialic glands, lasting about four past years. Within two past years it was associated with insidious exophtalmia of the right eye and with swelling of face below this eye. Deficiency of lacrimal capacity, but normal salivation were found by functional tests. Anti SSA/Ro and anti SSB/La antibodies were negative. MR scan demonstrated bilateral enlargement of lacrimal glands and orbital infiltration, both with asymetric predilection on the right site, and exophtalmia of the right eye. Excision of the right submandibular sialic gland and of the face swelling were made. Microscopic analysis demonstrated sclerosing sialadenitis and orbital infiltration, penetrating by infraorbital canaliculus into subcutaneous tissue of face. Using immunohistochemistry analysis IgG4+ plasma cells were found in excessive amount 400/l high-power field. IgG4 serum level was 44.68 g/l (82% of total IgG); paraproteinaemia was excluded. The Mikuliczęs disease with unilateral exophtalmia was diagnosed, and glucocorticoid therapy started. Maximal initial daily dose of methylprednisolone was 32 mg with gradual decrease up to 8 mg/day. The face swelling disappeared within 3 days, exophtalmia of the right eye and enlargement of the left submandibular sialic gland within 3 months. At this time the orbital MR scan was repeated: enlargement of lacrimal gland disappeared, and orbital infiltration was markedly reduced. After 6 month therapy the patient is symptomless, without exophtalmia and swelling of face, and with only moderate elevation of IgG4 in serum.

Key words:
Mikuliczęs disease, sclerosing sialadenitis, unilteral exophtalmia, IgG4-related disease, IgG4-positive plasma cells, IgG4 serum level, response to glucocorticoid therapy


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Labels
Dermatology & STDs Paediatric rheumatology Rheumatology
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