piperidines and Schnitzler-Syndrome

We report a case of late-onset Schnitzler syndrome successfully treated with Janus-activated kinase (JAK) inhibitors and colchicine. Schnitzler syndrome should be considered for recurrent chronic urticaria when accompanied by fever, fatigue, rapid weight loss, and poor response to antihistamine treatment. Skin biopsy, bone marrow biopsy, and electrophoresis help confirm the diagnosis. Early diagnosis and treatment can lead to complete resolution of symptoms. Besides interleukin (IL)-1 and IL-6 inhibitors, JAK inhibitors and colchicine may be considered as other choices of treatment.

Topics: Colchicine; Humans; Interleukin-1; Piperidines; Schnitzler Syndrome; Urticaria

Schnitzler syndrome is a rare adult-onset acquired autoinflammatory disorder typically characterized by chronic urticarial rash and immunoglobulin M (IgM) (rarely IgG) monoclonal gammopathy. Its clinical symptoms usually respond well to interleukin-1 blockade therapy, which, however, does not impact the underlying monoclonal gammopathy. Herein, we described a female patient who presented with urticarial rash, recurrent fevers, and fatigue for 7 years. Laboratory investigations revealed IgMκ monoclonal protein and

Topics: Adenine; Adult; Antibodies, Monoclonal; Exanthema; Female; Humans; Piperidines; Schnitzler Syndrome; Urticaria

Topics: Adenine; Female; Humans; Interleukin-1; Lymphoma; Middle Aged; Piperidines; Schnitzler Syndrome

Topics: Adenine; Agammaglobulinaemia Tyrosine Kinase; Humans; Immunoglobulin M; Inflammasomes; NLR Family, Pyrin Domain-Containing 3 Protein; Piperidines; Protein Kinase Inhibitors; Schnitzler Syndrome

Topics: Adenine; Aged, 80 and over; Exanthema; Histocytochemistry; Humans; Male; Piperidines; Protein Kinase Inhibitors; Pyrazoles; Pyrimidines; Schnitzler Syndrome; Skin; Treatment Outcome