Objectives. (1) To review the visual and systemic outcomes of patients who
developed rheumatoid arthritis (RA)-associated peripheral ulcerative kerati
tis (PUK). (2) To describe the clinical and serological characteristics of
the patients' arthropathy at the time of presentation of this rare conditio
n. (3) To review the aetiology and management of RA-associated PUK.
Patients and methods. A case series is given of all nine patients within ou
r unit who have developed RA-associated PUK since 1996. Details of the pati
ents' arthropathy and the serological characteristics of the RA at presenta
tion of PUK were noted. The patients' visual outcomes and the development o
f any significant systemic complications were recorded.
Results. All patients had long-standing seropositive, erosive RA. PUK was a
ssociated with a poor visual outcome in most patients, five requiring emerg
ency corneal surgery to prevent perforation of the globe. Two patients deve
loped systemic vasculitis within 1 month of PUK onset, one of whom died.
Conclusion. RA-associated PUK often has a poor visual outcome and its appea
rance may herald the transformation of a patient's RA into the systemic vas
culitic phase. RA-associated PUK should be managed with aggressive immunosu
ppression if the associated morbidity and mortality are to be avoided. Cell
-mediated mechanisms appear to be important in the aetiopathogenesis of PUK
and a combination of corticosteroids and cyclosporin is therefore probably
the regimen of choice.