Table 1.

Schnitzler syndrome patients treated with the Bruton tyrosine kinase (BTK) inhibitor ibrutinib.

Cases	Age (years)	Gender	Clinical manifestations	Monoclonal protein and underlying B-cell malignancy	Systemic treatments before BTK inhibitor	Current treatment	Outcomes
Jani et al. (14)	86	Male	Urticarial rashes	IgM (light-chain subtype not specified); lymphoplasmacytic lymphoma	Anakinra, rituximab	Ibrutinib 420 mg daily	Significant improvement of urticarial rashes (<10% of body surface involvement, much less than his presenting baseline)
Claves et al. (15)	60	Female	Fever, urticarial rashes, polyarthritis, weight loss, asthenia	IgMλ and IgGκ; splenic diffuse red pulp small B-cell lymphoma	Corticosteroids, anakinra, rituximab, canakinumab, chlorambucil, and splenectomy	Ibrutinib 420 mg daily	Complete disappearance of fever, urticarial rashes, arthritis, and monoclonal IgM. Her indolent lymphoma remained stable.
Current case	71	Female	Fever, urticarial rashes, and fatigue	IgMκ; IgM MGUS	Corticosteroids	Ibrutinib 420 mg daily with prednisone 25 mg daily	Resolution of fever and urticarial rashes, as well as improvement of fatigue.