Schnitzler's syndrome treated successfully with intravenous pulse cyclophosphamide.

Abstract

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.

• Authors: D Peterlana, A Puccetti, E Tinazzi, S Simeoni, C Lunardi
• Journal: Scandinavian journal of rheumatology (Scand J Rheumatol) 2005 Jul-Aug Vol. 34 Issue 4 Pg. 328-30 ISSN: 0300-9742 [Print] England
• PMID: 16195169 (Publication Type: Case Reports, Journal Article)

Chemical References

• Cyclophosphamide

Topics

• Cyclophosphamide (administration & dosage)
• Dose-Response Relationship, Drug
• Drug Administration Schedule
• Female
• Follow-Up Studies
• Humans
• Infusions, Intravenous
• Middle Aged
• Pulse Therapy, Drug
• Radionuclide Imaging
• Rare Diseases
• Risk Assessment
• Schnitzler Syndrome (diagnosis, diagnostic imaging, drug therapy)
• Severity of Illness Index
• Treatment Outcome