THE OFFICIAL NEWS SOURCE OF ACR CONVERGENCE 2022 • NOVEMBER 10-14



Rheumatologists can take the lead in orbital inflammatory disease

Inflammation of the orbit may be an old problem, but it’s one that rheumatologists are starting to notice. The eye socket is a limited space encompassing inflammatory diseases that can be best treated by a rheumatologist.

“Rheumatologists have a degree of comfort and familiarity with disease modifying medications; that is the most important reason we are best equipped to manage these patients,” said James T. Rosenbaum, MD, Professor of Ophthalmology, Medicine, and Cell Biology; Chair of Rheumatology; and Edward E Rosenbaum Professor of Inflammation Research at Oregon Health and Sciences University, Portland, OR.

“The orbit is an old problem area that rheumatologists don’t think much about or know much about,” he continued. “Patients with orbital inflammation frequently have a disease that may be a clue to a rheumatic disease elsewhere in the body. And that rheumatic disease frequently requires immunosuppression that is best managed by a rheumatologist.”

Dr. Rosenbaum will explore the field of orbital inflammation in the presentation Orbital Invaders: Differential Diagnosis & Management of Orbital Mass Lesions on Sunday from 9:00 – 10:00 am in Room W183a.

Orbital adipose tissue is the most common site of orbital inflammatory disease. The extraocular muscles that control eye movement are subject to orbital myositis. And the lacrimal gland, which produces the aqueous portion of tears, is subject to inflammation, called dacryoadenitis.

Graves eye disease, often referred to as thyroid eye disease (TED), is the most common cause of orbital inflammation. Other systemic conditions with orbital manifestations include sarcoidosis, granulomatosis with polyangiitis (GPA), Crohn’s disease, IgG4-related disease, and histiocytosis. A significant number of patients have nonspecific orbital inflammation, often diagnosed as orbital pseudotumor or idiopathic orbital inflammation.

Ophthalmologists are often the first clinicians to examine inflammatory conditions that affect the eye and the orbit. And while ophthalmologists routinely treat uveitis, scleritis, mucus membrane pemphigoid, and other inflammatory conditions of the eye, they are less familiar with rheumatologic conditions that can affect the orbit.

Dr. Rosenbaum is the head of the international Orbital Disease Consortium that is investigating gene expression as a diagnostic and prognostic tool to distinguish between the forms of orbital inflammatory disease. The group has identified gene expression patterns associated with different orbital inflammatory conditions, but molecular diagnosis remains in the early stages of investigation.

Imaging, most often CT or MRI, plus a biopsy can generally rule out metastatic malignancies, lymphoma, or infection. Ophthalmic signs and symptoms alone can make a definitive diagnosis difficult.

The inflammatory infiltrate from TED, for example, can be so minor that it is difficult to distinguish TED from normal orbital adipose tissue. A definitive GPA diagnosis requires vasculitis in a medium-sized vessel, which is seldom obtained during an orbital biopsy. Ophthalmologists receive limited training to look for evidence of vasculitis or other conditions outside the eye and its immediate surroundings.

“Ophthalmologists can have tunnel vision focused on the ophthalmic area and not on the relationship between different diseases and different systems,” Dr. Rosenbaum explained. “Oftentimes the ophthalmologist hasn’t stopped to think that the symptom that affects hearing and another problem with the sinuses are both related to this inflammation of the orbit. The rheumatologist is prepared to tie it all together, recognize that it is one illness, and treat them all at once.”

Rheumatologists are also more experienced in treating inflammatory conditions. The typical ophthalmologist is comfortable prescribing prednisone, dexamethasone, or some other steroid for inflammation. They are less familiar with immunosuppressive agents such as methotrexate. And few have experience with the broad range of biologics that rheumatologists use routinely.

“Patients and ophthalmologists both need the kind of expertise and experience that the rheumatologist can bring,” Dr. Rosenbaum said. “Orbital inflammation and inflammatory masses usually cause pain or double vision by limiting the mobility of the eye. People just don’t feel comfortable driving with double vision, or reading, using a computer, or phone. All kinds of daily activities are impaired. Managing the problem is incredibly important.”