*6.2.1 Systemic treatment for the management of ocular morbidity*


## *6.2.2 Systemic treatment for the management of the underlying systemic condition*

The current treatment regimen at the active phase includes application of systemic steroids with their rapid therapeutic effect along with immunomodulatory agents, which are often necessary to induce remission of autoimmune disease. This is followed by gradual tapering of steroids and maintaining the immunomodulatory agent to avoid disease recurrence. Foster et al. found that the mean survival rate in patients having PUK and scleritis in the course of RA, GPA, and SLE is 24.7 years if systemic immunomodulatory therapy is administered versus 10.7 years without this treatment [61].

First-line management of RA-associated PUK involves systemic steroids and a cytotoxic agent (e.g., methotrexate (MTX)) [62]. Second-line agents such as azathioprine and cyclophosphamide are used for severe, refractory PUK cases unresponsive to MTX [63]. Immunosuppressive treatment in the acute phase of GPA is usually initiated with systemic corticosteroids along with cyclophosphamide, and if no improvement is observed, treatment may be changed to rituximab [64].

In pediatric patients, MTX is considered a first-line immunosuppressant in the treatment of underlying systemic treatment, but if it is ineffective second-line cyclosporine is considered [65]. In pregnant women, immunomodulatory therapy should be avoided due to its teratogenic effects, and oral steroids should be used with greater caution [5].
