Endocrine Abstracts

Introduction: Immunoglobulin-G4-related disease (IgG4RD) is a multidisciplinary problem due to plasmatic cells infiltration and areas of fibrosis in the affected tissues. In IgG4RD, elevation of serum IgG4 immunoglobulins is present. Clinically, the disease is mainly manifested as: autoimmune pancreatitis, salivary gland involvement, sclerosing cholangitis, lymphadenopathy, lachrymal gland enlargement, retroperitoneal fibrosis or orbital pseudotumor. The simultaneous involvement of multiple organs is common. Treatment is based on glucocorticoids in case of recurrence or resistance to steroids, immunosuppresive therapy, involving disease-modifying antirheumatic drugs or rituximab are used.

Material and methods: The aim of the study was to present a case of a patient with orbitopathy in whom in the course of diagnostic procedure, IgG4-related ophthalmic disease was detected.

Results: A 68-year old man, without past history of any chronic diseases, was admitted to the outpatient endocrinology clinic because of increased protrusion of both eyeballs with swallowing conjunctivitis, eyelids redness and retro orbital pain. The MRI scans revealed enlargement and swallowing of all periorbital muscles. The thyroid function was normal with negative all anthythyroid immunoglobulins thus excluding Graves orbitopathy. Since elevated serum total IgG and IgG4 was stated, systemic glicocorticosteroid therapy followed by oral prednisolone treatment was introduced. The evident regression of symptoms and protrusion of eyeballs was achieved. The above therapy was repeated one year later because of recurrence of symptoms. Yet, at that moment it was followed by methotrexate treatment (20 mg/week). Until now, 5 months after second glucocorticoids pulse, the patient does not present any symptoms of orbitopathy; well tolerated metotrexate therapy is continued.

Conclusion: Presented case highlights the importance of considering IgG4RD in the differential diagnosis of orbitopathy.