Abstract
BackgroundGranulomatosis with polyangiitis (GPA) is a systemic autoimmune disease characterized by small and medium vessel vasculitis. The use of biological therapies such as rituximab and infliximab has improved the treatment of ocular manifestations in GPA.Case reportWe report a case of a 45-year-old Caucasian male suffering with rhinitis, sinubronchitis and exophthalmos. These clinical findings, subsequent biopsy and MRI were consistent with positive anti-neutrophil cytoplasm antibody (ANCA)/proteinase-3 and he was diagnosed with GPA with orbital involvement. He was refractory to cyclophosphamide at stable doses of methotrexate and a therapy with rituximab was started. Eventually and because of family planning methotrexate was replaced by azathioprine. Symptoms worsened and MRI revealed an increase in the granulomatous lesion in the orbit. Therefore, we decided to add infliximab to the combination of azathioprine and rituximab, our patient achieved then a long-term response. During the 10 years of the combined treatment, no adverse effects or systemic involvement occurred.ConclusionsThis case suggests that the individual use of a combination of rituximab and infliximab may be a promising strategy for the treatment in the long term of refractory orbital GPA.
Highlights
Granulomatosis with polyangiitis (GPA) is a systemic autoimmune disease characterized by small and medium vessel vasculitis
This case suggests that the individual use of a combination of rituximab and infliximab may be a prom‐ ising strategy for the treatment in the long term of refractory orbital GPA
Rituximab has strongly improved the treatment of GPA including eye manifestations [4,5,6]
Summary
Rituximab has strongly improved the treatment of GPA including eye manifestations [4,5,6]. In two previous publications it was reported, that a patient diagnosed with GPA received a sequential treatment with cyclophosphamide, with infliximab and, due to recurrence of symptoms with rituximab [7] In another patient with a delayed diagnosis of GPA due to an unusual presentation, sequential treatment with steroids and infliximab was started, and due to relapse the patient was treated with three courses of cyclophosphamide and a posteriori with rituximab [8]. This case report suggests that the use of a combination of rituximab and infliximab might be a promising strategy for the treatment of refractory GPA, the combination treatment has been well tolerated for a long time under tight monitoring and did not trigger an immune state that precipitates adverse events. It has been reported in 2012 that increased risk of both solid and hematologic malignancies in ANCA associated vasculitis, this publication states that the risk might be both, inherent to the disease or to the treatment, which is difficult to determine [9]
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