Rheumatology: Current Research
Open Access

Abstract

An Unexpected Cause of Amaurosis Fugax

Aaron Heath, Robert Digiovanni, Ila Sehgal and Jian Ma

IgG4-RD is an increasingly recognized group of immune-mediated conditions that share specific pathologic, serologic, and clinical features. It is a systemic inflammatory disorder that typically causes tumor-like swelling of involved organs, a lymphoplasmacytic infiltrate with predominance of IgG4-positive plasma cells with a variable degree of fibrosis, and elevated levels of serum IgG4. Lacrimal and orbital involvement is rare and when present is referred to as IgG4-related ophthalmic disease. Biopsy is the gold standard for diagnosis. The optimal treatment strategy has yet to be determined but glucocorticoids and rituximab have shown success. We report a case of amaurosis fugax and orbital inflammatory pseudotumor as an unusual presentation of IgG4-RD. IgG4-RD though rare, is likely underdiagnosed and often misdiagnosed. It is important that physicians are aware of the clinical spectrum of IgG4-RD as recognition and accurate diagnosis is crucial and early treatment may induce remission and prevent progression.