a treatment option for refractory vasculitis, and may be needed intermittently for long-term maintenance treatment. As soon as steroid treatment was discontinued in our patient, her neurological symptoms recurred. HCQ has immunosuppressive and anti-inflammatory effects. In patients with SS, it may be useful for rheumatological symptoms. Before treatment, an eye examination should be performed and must be repeated every 6 months. Corneal deposits occur in most patients on long-term HCQ treatment, but are often undetected, as 95% of these patients are asymptomatic. Duration of the treatment increases the risk of eye toxicity. In around 0.5– 2% of cases, a potentially irreversible retinopathy may develop. Our patient did not have any ophthalmological abnormality during the initial follow-up and did not have coexisting liver or renal disease or concomitant retinal disease. We suggest that eye toxicity may be related to patient age and treatment duration because our patient s retinal toxicity disappeared 6 months after HCQ was stopped. She had taken HCQ for 9 years. Clearance of HCQ from the body takes a long time because of its long elimination half-life. Our patient developed systemic vasculitis after HCQ was discontinued, and we believe this was related to interruption of the drug. In patients with systemic lupus erythematosus (SLE), low blood HCQ concentration is a strong predictor of disease exacerbation. Vallat et al. reported a patient with SS who had vasculitic neuropathy. Six years after HCQ treatment, the neuropathy did not recur. B cells are involved in the pathophysiology of SS. Rituximab is an option for some cases of lymphomaassociated SS. Saigal et al. successfully treated a patient with SLE who had urticarial vasculitis with rituximab. However, we were not able to use rituximab for our patient because of its cost. B-cell targeted therapies may be offered to both severe cases with SS and in cases of contraindication to HCQ. Our case is interesting because systemic vasculitis occurred after withdrawal of HCQ. Therefore, developing new treatments and new ways of using already available therapies are essential for treatment of SS.