Case Report: Orbital and intracranial inflammatory syndrome in rheumatoid arthritis

To describe a case of rheumatoid arthritis (RA)-associated orbital and intracranial inflammatory disorder (OIID).

RA is a systemic autoimmune disease associated with a multitude of extra-articular manifestations. Cigarette smoking increases the risk of RA vasculitis and adversely influences the expression of the proto-oncogene p53 in RA. Although ocular manifestations of RA are well known, orbital and intracranial inflammatory presentations are much less common.

A 63-year-old woman with seropositive RA and a significant tobacco smoking history presented with intermittent jaw pain, headache, left temporal tenderness, blurred vision of the left eye, and intermittent diplopia. Serum laboratory studies demonstrated elevated RF and erythrocyte sedimentation rate (ESR). Cranial and orbital magnetic resonance imaging (MRI) showed left orbital apex enhancement with extension into the left cavernous sinus and Meckel cave. The patient improved with intravenous methylprednisolone and high-dose prednisone, but symptoms recurred with each steroid taper. 13 months later, the patient developed new contralateral right-sided orbital inflammation with cavernous sinus and superior orbital fissure involvement.

Clinicians should be aware that orbital (e.g., orbital inflammatory pseudotumor) and intracranial presentations may occur in RA in addition to the more common ocular (e.g., scleritis) presentations.