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Anakinra and Schnitzler syndrome

Question:

1/18/2018
I have a 32 year-old female with chronic severe hives, not responsive to antihistamine or xolair. Labs: increased WBC, sed rate, CRP. High IgM with a IgM monoclonal gammopathy on immunofixation. No other symptoms. I have made the diagnosis of Schnitzler syndrome. I have ordered Rx for anakinra. In the mean time I am having trouble answering pt questions: Will taking anakinra lower the lab abnormalities (WBC, sed rate)? Does anakinra help to prevent other diagnoses down the road (such as lymphoma or amyloidosis)?
 

Answer:

Dr David Khan has kindly provided a thoughtful response as follows:
This patient appears to meet both obligate criteria (chronic urticaria and monoclonal IgM) as well as one minor criteria (leukocytosis and/or elevated CRP) of the Strasbourg diagnostic criteria of Schnitzler’s syndrome.(1) This would indicate a probable diagnosis. Having another minor criteria such as: 1) recurrent fever, 2) objective findings of bone remodeling, or 3) neutrophilic dermal infiltrate on skin biopsy would indicate a definite diagnosis.

In regards to treatment of Schnitzler’s syndrome with anakinra, markers of inflammation should normalize (e.g. leukocytosis and CRP), often within weeks. While it has been speculated that control of inflammation may reduce the risk of development of amyloidosis, this is in fact a rare complication and it is unknown if IL-1 blockade truly reduces this risk.(2) IL-1 blockade does not affect the monoclonal gammopathy of Schnitzler’s syndrome and whether it reduces the risk of hematologic malignancies including Waldenström’s macroglobulinemia is also unknown.(3)

References:

1. Simon A, Asli B, Braun-Falco M, De Koning H, Fermand JP, Grattan C, et al. Schnitzler's syndrome: diagnosis, treatment, and follow-up. Allergy. 2013;68(5):562-8.
2. Gusdorf L, Lipsker D. Schnitzler Syndrome: a Review. Curr Rheumatol Rep. 2017;19(8):46.
3. Neel A, Henry B, Barbarot S, Masseau A, Perrin F, Bernier C, et al. Long-term effectiveness and safety of interleukin-1 receptor antagonist (anakinra) in Schnitzler's syndrome: a French multicenter study. Autoimmun Rev. 2014;13(10):1035-41.

I hope this helps with your patient.

Jacqueline A. Pongracic, MD, FAAAAI