Schnitzler's syndrome treated successfully with intravenous pulse cyclophosphamide.
Schnitzler's syndrome is a rare clinical condition characterized by chronic urticaria, intermittent fever, bone pain, arthralgia or arthritis, and monoclonal immunoglobulin M (IgM) gammopathy. Here we describe the case of a 48-year-old Italian female with a long history of arthralgia, leucocytosis, spiking fever, and chronic urticaria with severe pruritus. The IgM-kappa monoclonal component in the serum and bone densification on conventional X-ray with hyperfixation on bone technetium scanning at the distal part of the femurs and at the proximal part of the tibias were detected 4 years after the onset of the symptoms. After many ineffective treatments, the use of pulse cyclophosphamide (CPX) resulted in complete remission of the disease that is still lasting after a 2-year follow-up.
Peterlana
D
D
Department of Clinical and Experimental Medicine, Section of Internal Medicine, University of Verona, Italy.
Puccetti
A
A
Tinazzi
E
E
Simeoni
S
S
Lunardi
C
C
D003520:Cyclophosphamide / Q000008:administration & dosage*; D004305:Dose-Response Relationship, Drug; D004334:Drug Administration Schedule; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007262:Infusions, Intravenous; D008875:Middle Aged; D020551:Pulse Therapy, Drug; D011877:Radionuclide Imaging; D035583:Rare Diseases; D018570:Risk Assessment; D019873:Schnitzler Syndrome / Q000175:diagnosis / Q000000981:diagnostic imaging* / Q000188:drug therapy*; D012720:Severity of Illness Index; D016896:Treatment Outcome
D003520:Cyclophosphamide