Scleritis Associated with Relapsing Polychondritis

Abstract

Purpose: To evaluate clinical features, ocular complications, and successful therapeutic regimens in patients with scleritis associated with relapsing polychondritis (RP).

Methods: Records of 13 scleritis patients with RP were analyzed and compared with those of 111 scleritis patients with other connective tissue diseases.

Results: Scleritis patients with RP were 8 women and 5 men with a mean age of 50 years (range, 22 to 76 years). Ten patients had diffuse scleritis, 1 had necrotizing scleritis, and 2 had necrotizing scleritis and posterior scleritis. Ocular complications included anterior uveitis (5), peripheral keratitis (2), glaucoma (1), optic neuritis (3) and decrease of vision (5, 3 of them with legal blindness, p<0.001). Scleritis was the initial manifestation whose study led to the diagnosis of RP in 8 of the 13 patients (62%). Five patients (38%) had one or more systemic disorders, including systemic vasculitis (3), rheumatoid arthritis (1), systemic lupus erythematosus (1), and ankylosing spondylitis (AS) (2) (p<0.001); these disorders antedated the RP by a mean of 9 years (2-21). Two patients developed scleritis associated with RP while on infliximab (INFLI) due to their AS with uveitis; changing to adalimumab did not solve the problem. All 13 patients had recurrent scleritis. Successful therapeutic regimens included cyclosphosphamide (6), methotrexate (3), azathioprine (2), mycophenolate mofetil (1), and INFLI (1).

Conclusions: Scleritis associated with RP may be a severe disease often associated with ocular complications which may lead to decrease of vision and even blindness. Scleritis may be the initial manifestation whose study leads to the diagnosis of RP. About 38% of patients will have another systemic disorder, the most frequent being systemic vasculitis. Scleritis associated with RP most often will require immunomodulatory therapy, mainly alkylating or antimetabolite agents, or biologic response modifier drugs, mainly anti-TNFα. However, occasionally, scleritis associated with RP may be an adverse event secondary to the use of anti-TNFα.

Keywords: 708 sclera • 462 clinical (human) or epidemiologic studies: outcomes/complications • 557 inflammation