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KineretCareFirst (Caremark)

Schnitzler syndrome

Initial criteria

  • Member has an urticarial rash, monoclonal IgM (or IgG) gammopathy, and at least two of the following: fever, joint pain or inflammation, bone pain, lymphadenopathy, hepatomegaly, splenomegaly, leukocytosis, elevated erythrocyte sedimentation rate (ESR), or abnormalities on bone morphological study (e.g., increased bone density).
  • Other possible causes of the signs and symptoms have been ruled out, including but not limited to hyperimmunoglobulin D syndrome, adult-onset Still’s disease, hypocomplementemic urticarial vasculitis, acquired C1 inhibitor deficiency, and cryoglobulinemia.

Approval duration

12 months