title: Successful Treatment Strategies in Granulomatosis With Polyangiitis-Associated Peripheral Ulcerative Keratitis.
abstract: OBJECTIVE
  Management of granulomatosis with polyangiitis (GPA)-associated peripheral ulcerative keratitis (PUK) is challenging and lacks definite guidelines. We aimed to summarize our treatment and outcome experience with patients with GPA-PUK.
  
  
  METHODS
  The Massachusetts Eye Research and Surgery Institution patient database was searched from 2005 to 2015 to identify patients with diagnosis of PUK who suffered from GPA. Individual patient histories were examined, and treatment strategies and outcomes were summarized.
  
  
  RESULTS
  There were 16 patients who started treatment with a mean duration follow-up of 64 months (range: 12-110 mo). Rituximab and cyclophosphamide, either alone or in combination with other agents, were the most successful agents in controlling inflammation. Rituximab was administered in 11 patients with remission being achieved in all. Cyclophosphamide successfully controlled inflammation in 50% (5/10). Two of the patients (2/5, 40%) who had achieved initial control on cyclophosphamide had flares of their PUK. Two of 11 (18%) patients on rituximab had flares of scleritis and orbital inflammation but not PUK. Two patients, one in each treatment group, stopped treatment after achieving remission after 6 months of therapy but suffered disease recurrence within 2 months of treatment cessation.
  
  
  CONCLUSIONS
  Rituximab achieved a high rate of disease control in PUK patients with GPA and is the preferred agent in halting disease progression.
authors.affiliation: *Massachusetts Eye Research and Surgery Institution, Waltham, MA; †Ocular Immunology and Uveitis Foundation, Waltham, MA; ‡Massachusetts Eye and Ear Infirmary, Boston, MA; §Eye Research Center, Farabi Eye Hospital, Tehran University of Medical Sciences, Tehran, Iran; and ¶Department of Ophthalmology, Harvard Medical School, Boston, MA.
authors.forename: Nazanin
authors.initials: N
authors.lastname: Ebrahimiadib
authors.forename: Bobeck S
authors.initials: BS
authors.lastname: Modjtahedi
authors.forename: Ramak
authors.initials: R
authors.lastname: Roohipoor
authors.forename: Stephen D
authors.initials: SD
authors.lastname: Anesi
authors.forename: C Stephen
authors.initials: CS
authors.lastname: Foster
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D003320:Corneal Ulcer / Q000175:diagnosis / Q000188:drug therapy*; D003520:Cyclophosphamide / Q000627:therapeutic use*; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis / Q000188:drug therapy*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D000069283:Rituximab / Q000627:therapeutic use*; D016896:Treatment Outcome
chemical_list: D007166:Immunosuppressive Agents; D000069283:Rituximab; D003520:Cyclophosphamide