Rheumatologic and inflammatory systemic diseases often cause similar neurologic and neuro-ophthalmologic manifestations. A wide variety of conditions can be included in these categories. This article summarizes the most common of these conditions, including sarcoidosis, idiopathic orbital inflammation, inflammatory bowel disease, Wegener's granulomatosis, polyarteritis nodosa, Churg-Strauss syndrome, Behcet's disease, systemic lupus erythematosis, scleroderma, Sjogren's syndrome, and rheumatoid arthritis.