A 76-year old man with a history of bilateral lacrimal gland masses and an otherwise unremarkable ocular examination underwent left orbital biopsy at another institution. Clinical suspicion was high for possible lymphoma due to the additional presence of cervical and axillary lymphadenopathy. The histologic diagnosis at that time was reactive lymphoid hyperplasia with fibrosis. Immunohistochemistry, kappa/lambda in situ hybridization, and flow cytometry were negative for lymphoma. The patient was lost to follow-up without any further work-up. He presented a year later due to recurrence of orbital inflammation, at which point the surgeon requested histologic work-up for IgG4-related disease. The slides from the prior orbital biopsy were requested and reviewed, showing lymphoid follicles with intervening bands of fibrosis and mixed chronic inflammatory cells. The tissue block was also received, and additional sections were cut for CD138 and IgG4 immunostaining. There were at least 150–180 IgG4-positive plasma cells per high-power field, comprising over 40 % of the total plasma cell population (as assessed by CD138). (Serum IgG4 was not obtained.) This was considered consistent with IgG4-associated orbital inflammatory pseudotumor with sclerosing features. The patient was initially treated with prednisone and then transitioned to intravenous rituximab as first-line therapy and had an excellent response with no recurrence at 2 years following therapy.
A 43-year-old woman with a history of recurrent headaches and associated vision disturbances for the past 4 years presented to our emergency department with a 4-week history of progressive left-eye pain that radiated to the back of her head, swelling around the left eye, double vision, and a unilateral left-sided headache. There were no factors that worsened or improved her symptoms. She first developed this 4 years ago while living in another state. At that time, she was admitted to a hospital, given intravenous (IV) then oral steroids which helped suppress the pain and swelling, but she did not recall a specific diagnosis. Since that time, she has had three separate flare-ups, each resolving after steroid treatment, with the current episode being the most recent and severe. On physical exam she had left periorbital swelling and edema, 20/25 vision in her right eye and 20/30 in her left eye, and extra ocular muscles were intact, but she reported left-eye pain when at extreme medial and lateral gaze. She did not have temporal lobe tenderness on examination. There was normal muscle tone, range of motion, and sensation in all four extremities bilaterally. Laboratory results at admission were as follows: erythrocyte sedimentation rate (ESR) of 30 mm/h and normal complete blood count, renal function, hepatic function, and transaminases. Computed tomography (CT) of the head without contrast, CT of the orbits with contrast (), CT angiogram of the head, and an electrocardiogram (EKG) were performed. All were normal aside from the CT angiogram of the head which suggested a varix versus aneurysm involving the anterior cerebral arteries in the region of the inferior sagittal sinus (). Ophthalmology was consulted, excluded acute angle closure glaucoma, retinal disorders, diagnosed her with recurrent idiopathic orbital inflammation syndrome and possibly Tolosa-Hunt syndrome, and recommended treatment with methylprednisolone 80 mg IV daily for 3 days. Within 24 h she noted a 50% reduction in pain and had complete resolution of her symptoms within 48 h. Magnetic resonance imaging (MRI) of the brain with and without contrast conducted the next day was normal and did not confirm the presence of a vascular anomaly suggested by the CT scan. At this point she was discharged on 60 mg oral prednisone daily with a taper over the course of 2 weeks. One week after discharge she was seen at her ophthalmologist’s office and remained asymptomatic.