Schnitzler Syndrome Treatment & Management

Updated: Mar 12, 2019
  • Author: Brian J Thomas, MD; Chief Editor: Dirk M Elston, MD  more...
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Treatment

Approach Considerations

The patient should be evaluated for alterations in quality of life as well as serial evaluation of inflammatory markers. In patients without significant elevations in these markers and without significant quality-of-life impairment, a less aggressive treatment course may be acceptable. This includes observation, colchicine, a short course of NSAIDs, or hydroxychloroquine. However, in patients with significantly impaired quality of life or regularly elevated inflammatory markers, a more aggressive course is recommended. This includes treatment with IL-1 inhibitors, such as anakinra. [14, 19]

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Medical Care

Up until about 2005, the urticarial eruption of Schnitzler syndrome was typically resistant to treatment. No treatment was consistently effective.

NSAIDs, corticosteroids, and immunosuppressive agents have been reported to provide variable relief from the symptoms of bone pain and arthralgias associated with Schnitzler syndrome.

Skin and extracutaneous manifestations respond poorly to H1 and H2 antihistamines. Colchicine and dapsone have been tried with variable success in different patients. A few patients were responsive to treatment with thalidomide but the occurrence of peripheral neuropathy limits its use. [17, 20] Rituximab, an anti-CD20 monoclonal antibody, was reported to be effective in one patient [21] but unsuccessful in another. [2] Reports of using chloroquine, chlorambucil, cyclophosphamide, azathioprine, plasmapheresis, and high-dose intravenous immunoglobulin have indicated no response. Psoralen plus UV light (PUVA) may reduce the intensity of the rash in some patients.

NSAIDs have proved to be of some benefit for the bone pain and fever, but not for the urticaria. Systemic steroids may be somewhat effective at controlling the cutaneous eruption, but usually at doses that can cause significant long-term adverse effects.

Pefloxacin mesylate administered at a dose of 800 mg/d may be a therapeutic option. In a case series of 11 patients, it was shown to significantly reduce the intensity and frequency of many of the manifestations in a majority of the group, and it provided a steroid-sparing effect for some patients being treated with systemic corticosteroids. [1] It was less active on the osteoarticular component of Schnitzler syndrome.

Anakinra, a recombinant form of the naturally occurring IL-1 receptor antagonist, and rilonacept, a dimeric fusion protein that acts as a decoy IL-1 receptor, are two agents with reported benefit in Schnitzler syndrome. [22] Anakinra has been shown to induce sustained dramatic improvements in patients with Schnitzler syndrome, allowing for improved quality of life and steroid-sparing effects. Interestingly, it was not shown to impact the levels of monoclonal gammopathy in a series of 29 patients followed over 3 years. [19]

Canakinumab, a selective monoclonal antibody specific to IL-1β, has also been shown effective in improving symptoms and decreasing markers of inflammation. [3, 23]

Ibrutinib has also been used. [24]

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Consultations

Consultation with hematologic oncologist should be considered for the monoclonal gammopathy.

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Long-Term Monitoring

Schnitzler syndrome requires long-term follow-up because of the potential for the development of lymphoproliferative disorders, especially Waldenström macroglobulinemia. Monitoring patients with periodic serum protein electrophoresis and reevaluation for lymphadenopathy and bone marrow involvement, if clinically indicated, is important. It is recommended that patients’ leukocyte counts and C-reactive protein values be followed every 3 months while on treatment, and then at least twice yearly once stable at normal levels. The monoclonal gammopathy should be monitored as usually recommended for monoclonal gammopathy of undetermined significance, as determined by the serum levels.

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