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Schnitzler's Syndrome

Schnitzler's syndrome is an extremely rare disease which is characterised by a combination of chronic urticarial skin rashes and haematological abnormalities (monoclonal gammopathy, paraproteinaemia). Monoclonal gammopathy develops from the uncontrolled growth of a single clone of plasma cell precursors . The antibodies (immunoglobulins) produced are belonging to an immunoglobulin class, usually presenting as a monoclonal gammopathy of the IgM sub-type.

Inflammatory processes to various organ systems occur - which similar to CAPS – manifest themselves in the form of joint pain, bone and muscle pain, fever bouts and fatigue.

Only some 150 cases of this non-hereditary disease have been reported so far; disease onset occurs in the middle-aged and the disease persists for life. Here again the number of undetected cases is presumably clearly higher.

Therapy for Schnitzler's Syndrome is currently most difficult and usually does not lead to a total relief of symptoms for the patient despite continuous administration of drugs. Customary therapies include cortisone and other anti-inflammatory drugs, often with only moderate effectiveness. Currently a number of Interleukin-1ß neutralising drugs are being investigated in Schnitzler's Syndrome undefinedStudies for their efficacy and tolerability.

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