JOURNAL OF BIOLOGICAL REGULATORS & HOMEOSTATIC AGENTS
Vol. 29, no. 2 (S), 0-0 (2015)
SCHNITZLER SYNDROME RESPONDING TO INTERLEUKIN-1 ANTAGONIST ANAKINRA A. KOCH1, G. TCHERNEV2 ,CHOKOEVA A.A.3, T. LOTTI4 and UWE WOLLINA1 Department of Dermatology and Allergology, Academic Teaching Hospital Dresden-Friedrichstadt, Dresden, Germany; 2Policlinic for Dermatology and Venereology, Saint Kliment Ohridski University, Medical Faculty, University Hospital Lozenetz, Sofia, Bulgaria; Onkoderma-Policlinic for Dermatology and Dermatologic Surgery, General Skobelev 26, Sofia, Bulgaria; 4Professor and Chair of Dermatology University of Rome “G.Marconi” Rome, Italy 1
F O O R P
Since 2005, an 82-year-old female patient suffered from relapsing, generalized urticarial rash with relapsing fever episodes, arthralgias and burning tibial pain sensations. She unintentionally lost about 10 kg of body weight within 9 months. Systemic treatment with desloratadine, ranitidine, prednisolone and later, ciclosporin A, started. All these efforts could not control the symptoms but caused adverse effects including glaucoma and hypertension. Several years later a therapy with interleukin-1 inhibitor anakinra 100 mg s.c. once daily was started. The patient became symptom-free within 24 h after the first injection. Later, the dosage was reduced to 100 mg s.c. every other day. With a follow up of three years the patient remained completely symptom-free with no fever or burning sensations. Later on, the dosage was reduced to 100 mg s.c. every other day. With a follow up of three years the patient remained completely symptom-free with no fever or burning sensations. Anakinra reduces pro-inflammatory cytokine release due to lipopolysaccharide stimulation. In a long-term use the drug does not loose effectiveness. In 1974 a combination of urticarial, bony lesions and IgM macroglobulinemia was been observed by Schnitzler who gave this syndrome her name (1). Schnitzler syndrome today is considered as part of the spectrum of autoinflammatory diseases with cutaneous involvement (2). We report on successful and long-term treatment with interleukin-1 anatagonist anakinra. Case report An 82-year-old female patient, since 2005, suffered from relapsing, generalized urticarial rash with relapsing fever episodes, arthralgias, and burning tibial pain sensations. She unintentionally
lost about 10 kg of body weight within 9 months. In 2008 we confirmed the diagnosis of a Schnitzler syndrome and initiated a systemic treatment with desloratadine, ranitidine, and prednisolone and later, ciclosporin A. All these efforts could not control the symptoms and caused adverse effects including glaucoma and hypertension (170/ 90 mm Hg). In 2012 the patient returned with a relapse of urticarial rash (Fig. 1). A skin biopsy was taken. There was a normal epidermis. Numerous angiectasias were noted in papillary bodies and upper corium surrounded by perivascular and interstitial mixed mononuclear infiltrate composed of lymphocytes, neutrophils, a few eosinophils and mast cells. Vessel
Key words: Anakinra, IL-1, autoinflammatory disorders, macroglobulinemia, Schnitzler Mailing address: Prof. Dr. Uwe Wollina, Department of Dermatology and Allergology, Academic Teaching Hospital Dresden-Friedrichstadt, Friedrichstrasse 41, 01067 Dresden, Germany Tel.: +49 351 4801685 e-mail:
[email protected]
0393-974X (2015)
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µmol/l. Ultrasound imaging of the abdomen was unremarkable. Treatment and course: Considering adverse side effects and insufficient control of the disease, ciclosporin A therapy was stopped. We started with interleukin-1 inhibitor anakinra 100 mg s.c. once a day. The patient became symptom-free within 24 h after the first injection. Later on the dosage was reduced to 100 mg s.c. every other day. With a follow up of three years the patient remained completely symptom-free with no fever or burning sensations. Urticarial rash did not relapse.
F O DISCUSSION
Schnitzler syndrome is a rare disorder characterized by the combination of chronic relapsing urticarial and monoclonal IgM. The disease is further characterized by systemic symptoms such as arthralgias, bone pain, neutrophilia, thrombocytosis, lymphadenopathy and hepato-splenomegalia; fever of
