Infliximab for IgG4-Related Orbital Disease

IgG4-related disease (IgG4-RD) is a systemic fibroinflammatory condition with unclear pathophysiology. It may occur as a single organ disorder, but multiorgan presentation is common and can mimic several conditions. The preferred therapy consists of steroids, but definite maintenance strategy remains unclear. The authors describe a case of a 61-year-old woman, initially diagnosed with idiopathic orbital inflammation refractory to multiple immunosuppressive agents. The disease was complicated with epilepsy, vision loss, and trismus. Treatment with various immunosuppressive agents was unsuccessful. Eventually the patient was effectively treated with infliximab. This is the second case of IgG4-RD treated with a TNF-blocker documented in literature and the first description to demonstrate its superiority over steroid sparing agents. Although speculative, TNF-blockers might exert their effect in IgG4-RD by interfering with the possible overexpressed TNF alpha due to fibrosis in this disease. Treatment with infliximab appears a good alternative for refractory IgG4RD. However, further studies are required to define the value of infliximab in IgG4-RD.