Schnitzler syndrome

Schnitzler-Syndrom

Abstract

Schnitzler syndrome is a very rare acquired systemic disease with many similarities to hereditary autoinflammatory syndromes. The main characteristics are generalized exanthema and IgM monoclonal gammopathy. Other clinical features include fever, muscle, bone, and/or joint pain, and lymphadenopathy. About 15–20% of patients with Schnitzler syndrome develop lymphoproliferative diseases and, in rare cases, amyloid A (AA) amyloidosis can occur if the disease is not treated. Activation of the innate immune system, especially interleukin (IL)-1β, is central to the pathogenesis of disease. Consequently, complete control of disease symptoms can be achieved in 80% of patients by treatment with the IL-1 receptor antagonist anakinra.

Zusammenfassung

Das Schnitzler-Syndrom ist eine sehr seltene, erworbene Systemerkrankung, die viele Gemeinsamkeiten mit den hereditären autoinflammatorischen Syndromen aufweist. Das Exanthem und eine monoklonale Gammopathie mit IgM sind die Charakteristika der Erkrankung. Zu den klinischen Hauptmerkmalen gehören Fieber, urtikarielles Exanthem, Muskel‑, Knochen- und/oder Gelenkschmerzen und eine Lymphadenopathie. Etwa 15–20 % der Patienten mit Schnitzler-Syndrom entwickeln eine lymphoproliferative Erkrankung, und selten kann es zum Auftreten einer AA-Amyloidose kommen, wenn die Erkrankung nicht behandelt wird. Eine Aktivierung des angeborenen Immunsystems, speziell des Interleukin(IL)-1β, ist zentral in der Pathogenese der Erkrankung. Folgerichtig kann bei 80 % der Patienten eine komplette Kontrolle der Krankheitssymptome durch Behandlung mit dem IL-1-Rezeptorantagonisten Anakinra erreicht werden.

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Correspondence to F. F. Gellrich.

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F. F. Gellrich and C. Günther declare that they have no competing interests.

For this article, no studies with human participants or animals were performed by any of the authors. All studies performed were in accordance with the ethical standards indicated in each case.

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Gellrich, F.F., Günther, C. Schnitzler syndrome. Hautarzt (2019). https://doi.org/10.1007/s00105-019-4434-4

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Keywords

  • Hereditary autoinflammatory syndrome
  • Urticarial rash
  • Interleukin-1
  • Anakinra
  • Systemic disease

Schlüsselwörter

  • Hereditäres autoinflammatorisches Syndrom
  • Urtikarielles Exanthem
  • Interleukin-1
  • Anakinra
  • Systemerkrankung