Increased angiogenesis and enhanced bone formation in patients with IGM monoclonal gammopathy and urticarial skin rash: new insight into the biology of the Schnitzler syndrome

نویسندگان

  • Evangelos Terpos
  • Bouchra Asli
  • Dimitrios Christoulas
  • Jean-Claude Brouet
  • Efstathios Kastritis
  • Michel Rybojad
  • Djaouida Bengoufa
  • Meletios A. Dimopoulos
  • Jean Paul Fermand
چکیده

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Increased angiogenesis and enhanced bone formation in patients with IgM monoclonal gammopathy and urticarial skin rash: new insight into the biology of Schnitzler syndrome.

Schnitzler syndrome is a rare plasma cell disorder the pathogenesis of which is still not fully understood. We evaluated the circulating levels of four major angiogenic cytokines (VEGF, angiogenin, angiopoietin-1 and angiopoietin-2) and six bone remodeling markers (sRANKL, osteoprotegerin, dickkopf-1, CTX, osteocalcin and bone-specific alkaline phosphatase-bALP) in 13 patients with Schnitzler s...

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Chronic urticaria and monoclonal IgM gammopathy (Schnitzler syndrome): report of 11 cases treated with pefloxacin.

BACKGROUND Schnitzler syndrome is characterized by chronic urticarial rash and monoclonal IgM gammopathy and is sometimes associated with periodic fever, arthralgias, and bone pain. Current treatment is unsatisfactory. OBSERVATIONS Eleven patients with Schnitzler syndrome were treated with oral pefloxacin mesylate (800 mg/d). In 10 patients, we observed a dramatic and sustained improvement of...

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The Schnitzler syndrome

The Schnitzler syndrome is a rare and underdiagnosed entity which is considered today as being a paradigm of an acquired/late onset auto-inflammatory disease. It associates a chronic urticarial skin rash, corresponding from the clinico-pathological viewpoint to a neutrophilic urticarial dermatosis, a monoclonal IgM component and at least 2 of the following signs: fever, joint and/or bone pain, ...

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Schnitzler’s syndrome: lessons from 281 cases

Schnitzler’s syndrome is an autoinflammatory disorder characterized by the association of a monoclonal IgM (or IgG) gammopathy, a chronic urticarial rash, and signs and symptoms of systemic inflammation, including fever, arthralgias and bone pain. It was first described in 1972. This review summarizes the clinical features, efficacy of therapies, and follow-up data of the 281 cases that have be...

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Long-term control of refractory Schnitzler syndrome with anakinra: a case report

Schnitzler syndrome is a rare inflammatory disorder characterized by chronic urticarial rash, monoclonal gammopathy, periodic fever, arthralgia/arthritis and bone pain. However, the results of management of Schnitzler syndrome are often disappointing and its treatment remains a challenge. No cases of spontaneous complete remission have been reported. Anakinra is an interleukin 1 receptor antago...

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تاریخ انتشار 2012