Abstract
IgG4-related diseases are associated with systemic effects in which CD4- or CD8-positive T-lymphocytes and IgG4-positive plasma cells infiltrate internal organs. The disease groups in which infiltration of IgG4-positive plasma cells has been proved in internal organs have been proposed since Hamano reported elevated levels of serum IgG4 in autoimmune pancreatitis in 2001. It is thought that Mikulicz's Disease, Küttner's Tumor (Chronic Sclerotic Sialadenitis), and Riedel's thyroiditis are IgG4-related diseases in the field of otorhinolaryngology.
We report on a case of orbital and paranasal sinus lesions associated with an IgG4-related disease. A 62-year-old man was admitted for right periorbital swelling and nasal discharge from June 2009. His local physician found no abnormalities. Radiography revealed shadows in his right maxillary sinus in January 2010. He was suspected of having periorbital swelling caused by sinusitis and was referred to our hospital two days later. Neither visual impairment nor ocular motility were seen but mild edema of right middle nasal meatus was noted. Computed tomography (CT) and magnetic resonance imaging (MRI) showed shadows in the bilateral ethmoidal sinuses, the right maxillary sinus, the right sphenoidal sinus, and in the right orbit. We conducted endoscopic sinus surgery due to orbital complications of sinusitis. Edema was induced in the right ethmoid sinus and the mucosa demonstrated an inflammatory response. We removed the right orbital plate and when we made an incision in the orbital synovial membrane, the orbital tissues looked like low-fat connective tissues. We submitted specimens from the orbital synovia, intraorbital tissues, and mucous membrane of the ethmoid sinus for pathological analysis. Hematoxylin and eosin staining revealed a plasmocytic infiltrate. Based on the immunostaining findings which showed IgG4 positive phlogocytes, we reached a diagnosis of an IgG4-related disease.
