title: Scleritis associated with relapsing polychondritis.
abstract: To evaluate ocular disease characteristics and successful therapeutic regimens in patients with scleritis associated with relapsing polychondritis (RP). To compare these features with those seen in patients with scleritis associated with other systemic immune-mediated diseases (SIMD).
  
  
  
  Electronic health records of 13 scleritis patients associated with RP were analysed and compared with those of 113 scleritis patients associated with other SIMD seen at two tertiary referral centres.
  
  
  
  Scleritis in patients with RP was often bilateral (92.3%), diffuse (76.9%), recurrent (84.6%), sometimes with decreased vision (46.2%), anterior uveitis (38.5%), peripheral keratitis (15.4%) and ocular hypertension (30.8%). Patients with scleritis associated with RP more often had bilateral scleritis (p=0.001), necrotising scleritis (23.1%; p=0.02), recurrences (p=0.001) and decreased vision (three of the six with legal blindness; p=0.012), as compared with patients who had scleritis associated with other SIMD. Nine patients (69.2%) had one or more SIMD other than RP, including systemic vasculitis (4) or other autoimmune disease (8); they antedated RP by 9 years (range 2-21 years). Successful therapy included cyclophosphamide (5), methotrexate (3), azathioprine (3), mycophenolate mofetil (2), infliximab (2) and adalimumab (1).
  
  
  
  Scleritis may be the first manifestation whose study leads to the diagnosis of RP. Scleritis associated with RP is more often bilateral, necrotising, recurrent and associated with decrease of vision than scleritis associated with other SIMD. About 69.2% of patients will have an additional SIMD disorder. Scleritis associated with RP most often will require immunomodulatory therapy. Occasionally, scleritis with RP may appear while using antitumor necrosis factor α agents.
authors.affiliation: Institute Clinic of Ophthalmology, Hospital Clinic of Barcelona, Barcelona, Spain.
authors.forename: Maite
authors.initials: M
authors.lastname: Sainz-de-la-Maza
authors.affiliation: Institute Clinic of Ophthalmology, Hospital Clinic of Barcelona, Barcelona, Spain.
authors.forename: Nicolas
authors.initials: N
authors.lastname: Molina
authors.affiliation: Massachusetts Eye Research and Surgery Institution (MERSI), Cambridge, MA, USA Ocular Immunology and Uveitis Foundation, Cambridge, Massachusetts, USA.
authors.forename: Luis Alonso
authors.initials: LA
authors.lastname: Gonzalez-Gonzalez
authors.affiliation: BayView Clinic, Mumbai, India.
authors.forename: Priyanka P
authors.initials: PP
authors.lastname: Doctor
authors.affiliation: Tauber Eye Center, Kansas City, Missouri, USA.
authors.forename: Joseph
authors.initials: J
authors.lastname: Tauber
authors.affiliation: Massachusetts Eye Research and Surgery Institution (MERSI), Cambridge, MA, USA Ocular Immunology and Uveitis Foundation, Cambridge, Massachusetts, USA Harvard Medical School, Boston, Massachusetts, USA.
authors.forename: C Stephen
authors.initials: CS
authors.lastname: Foster
mesh_terms: D000328:Adult; D000368:Aged; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D011081:Polychondritis, Relapsing / Q000150:complications* / Q000175:diagnosis; D012008:Recurrence; D012189:Retrospective Studies; D015423:Scleritis / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D055815:Young Adult
chemical_list: D007155:Immunologic Factors
keywords: Anterior chamber; Diagnostic tests/Investigation; Immunology; Inflammation; Sclera and Episclera