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Nathalie Butel, Mathieu ZMUDA, Olivier Galatoire; OPHTHALMIC AND SYSTEMIC MANIFESTATIONS OF IgG4 RELATED DISEASE : ABOUT 6 PATIENTS IN A SINGLE CENTER. Invest. Ophthalmol. Vis. Sci. 2015;56(7 ):5791.
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© ARVO (1962-2015); The Authors (2016-present)
We presented a french descriptive dataset of orbital, eyelid and systemic manifestations in lymphoproliferative disorder of IgG4 related disease (IgG4-RD) in 6 patients. The main manifestation is a non-specific inflammatory orbitopathy which is cortico-sensitive. This recent syndrome, underknown, often makes a Masquerade syndrome, responsible of misdiagnosis.
Retrospective and monocentric descriptive evaluation conducted between 2012 and 2013 in a tertiary center in Paris, including patients with orbital or eyelid impairment in IgG4-RD according to the Kawa et al criteria.
6 patients (4 men, 2 women) were included. 2 patients were childrens. Median age was 45 years (9-62 years). The average diagnostic delay between the onset ocular symptoms and immuno histo chemical confirmation was 18 months (2-72 months). Mean follow-up was 32 months (2-72 months). 1 patient (age 9) had a single eye-lid reached, while 5/6 patients also had systemic involvement like parotidis,affected lymph node and tonsil, thyroiditis, aortitis, skin involvement. In 2 cases we found an infraorbital nerve enlargement. All patients had a permanent or transient clinical improvement with corticotherapy.
IgG4-RD is rarely described in children, this study shows that it is important to think about it in an orbital or eyelid unexplained inflammatory disease, even in children. Involvement of cranial nerves in IgG4-RD was once described in the literature. We found 2 patients with infra orbital nerve enlargement which seems to be a very typical manifestations in IgG4-RD.
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