Successful Anakinra Therapy in 2 Patients with Schnitzler Syndrome

Abstract

Background: Schnitzler syndrome is a rare idiopathic disease characterized by chronic urticaria, presence of monoclonal IgM immunoglobulin, signs of inflammation and further symptoms. Often unrecognized, it may evolve into a lymphoproliferative disorder. Case Report: Two male patients presented pruritic and nonpruritic urticarias at the age of 45 and 43 years, respectively. Based on further symptoms (fever or subfebrile temperatures, bone and joint pains), laboratory tests (monoclonal IgM kappa, leukocytosis, elevated ESR and CRP) and radiological findings (osteolytic-osteosclerotic and hyperostotic skeletal changes), they were subsequently diagnosed with Schnitzler syndrome. Follow-up times were 15 years and 1 year, respectively. We monitored disease activity in the second patient, finding a correlation with CRP, ESR and interleukin IL-6 and IL-18 serum levels. As regards therapy, we used several medications (antihistamines, bisphosphonates, corticoids, 2-chlorodeoxyadenosine, interferon-alpha, cyclosporine, thalidomide, bortezomib) and PUVA treatment without achieving complete remission. Only anakinra (interleukin-1 receptor antagonist) minimized Schnitzler symptoms in both patients with very good drug tolerance. The first patient has been on anakinra monotherapy for 3 years (10/2007-10/2010) at a dosing interval of 24-48 hours without any signs of Schnitzler syndrome. Conclusions: Biological therapy using anakinra proved effective in Schnitzler syndrome, a rare entity with potentially life-threatening complications (malignancy, systemic amyloidosis).