Methods: Two cases of Schnitzler syndrome recently treated at Brigham and Women’s Hospital were identified.
Results: Patient 1, a 45 year-old woman, presented with two months of urticaria, fever, arthritis and fatigue and was found to have elevated CRP and ferritin. She was seen by a rheumatologist and received anakinra for presumed adult Still’s disease with excellent response. When urticaria and fevers recurred with anakinra discontinuation, she was seen in allergy clinic where SPEP revealed IgM lambda M component and Schnitzler syndrome was diagnosed. Anakinra was restarted with good result. Patient 2, a 63 year-old man, presented to rheumatology clinic with three years of recurrent urticaria and recent fever and myalgias. CRP was elevated and SPEP revealed an IgM Kappa M component. He was referred to oncology, and was diagnosed with and treated for Waldenstrom macroglobulinemia. He was seen in allergy clinic where the diagnosis of Schnitzler syndrome was reaffirmed and anakinra treatment was planned.
Conclusions: Schnitzler syndrome is rare, with approximately 200 reported cases as of 2013. It can be effectively treated with anakinra and other agents. Expedient diagnosis and management requires cooperation between and familiarity with the disease among allergists, immunologists, oncologists, and rheumatologists.