Schnitzler syndrome: case report, the experience with glucocorticoid and anakinra (Kineret^TM) therapies and monitoring of systemic cytokine response

Czech version

Authors: P. Szturz ¹; Z. Adam ¹; M. Klabusay ¹; Z. Fojtík ¹; Z. Kadaňka ²; O. Stehlíková ¹; J. Chovancová ³; L. Kalvodová ¹; D. Čorbová ⁴; K. Starý ⁵; J. Neubauer ⁶; J. Prášek ⁷; R. Koukalová ⁸; Z. Řehák ⁸; R. Hájek ¹; J. Mayer ¹
Authors‘ workplace: Interní hematoonkologická klinika Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednosta prof. MU Dr. Jiří Vorlíček, CSc. ¹; Neurologická klinika Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednosta prof. MU Dr. Josef Bednařík, CSc. ²; Lékařská fakulta MU Brno, děkan prof. MU Dr. Jiří Mayer, CSc. ³; Dermatovenerologická klinika Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednostka prof. MU Dr. Alena Pospíšilová, CSc. ⁴; Interní hepatogastroenterologická klinika Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednosta prof. MU Dr. Jan Lata, CSc. ⁵; Radiologická klinika Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednosta prof. MU Dr. Vlastimil A. Válek, CSc. ⁶; Klinika nukleární medicíny Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednosta doc. MU Dr. Jiří Prášek, CSc. ⁷; Oddělení nukleární medicíny, PET Centrum Masarykova onkologického ústavu Brno, přednosta prim. MU Dr. Karol Bolčák ⁸
Published in: Vnitř Lék 2011; 57(1): 97-112
Category: Case Reports

Overview

Schnitzler syndrome is a rare idiopathic disease characterized by chronic urtica, presence of monoclonal IgM immunoglobuline and further, less common symptoms. This case report describes another case of this disease affecting a male adult born in 1963. The first symptoms, eruptions of non‑pruritic urticarial rash, appeared in this patient at the age of 43. In addition, bone pains (mainly tibias) and joint pains (mainly knees) were present. Later on however, severe attacks of fever, chills and shaking together with bone and joint pains were added to during which new urticarial eruptions appeared. Primarily, the man was followed up without any substantial therapeutic results at a department of dermatovenerology, subsequently, due to a finding of monoclonal IgM κ immunoglobulin (serum concentration 1.9 g/ l) he was referred to our department for the reason of gammopathy being a differential diagnosis. On a CT scan hyperostosis in claviculae and pelvic bones was identified. Also on the CT, an increase in cortical thickness was described in the long bones of the lower extremities, where areas of technetium pyrophosphate accumulation were indentified on a bone scintigraphy. These areas were found in the chest and sacral regions as well. From the blood exams, the proinflammatory status of the organism was apparent (CRP 35.9 mg/ l, erythrocyte sedimentation rate 92 mm/ h, leukocytes 12.4 × 10⁹/ l). After excluding other differential diagnoses, the patient was diagnosed with Schnitzler syndrome. As regards therapy, we made initial use of the effect of corticoids which abated the symptoms, however, these were causing serious adverse reactions in the form of iatrogenous Cushing’s syndrome. The therapy took a turn only after biologic therapy with anakinra (interleukin‑1 receptor antagonist) had started, which minimized the Schnitzler symptoms with very good drug tolerance. In the work we measured serum levels of interleukins for disease activity monitoring. The most sensitive were interleukins IL‑6 and especially IL‑18 the levels of which were the highest at the time of clinical exacerbation of the disease, whereas the levels of IL‑1β and TNF‑α (tumour necrosis factor) were during all measurements below the limit of detection. Concerning the growing numbers of the reports on successful biological therapy with anakinra and our positive experience, we propose that the therapeutic response to anakinra should be included within the diagnostic criteria of Schnitzler syndrome, which is significant above all in differential diagnosis thereof.

Key words:
Schnitzler syndrome – autoinflammatory diseases – monoclonal gammopathy – multiple myeloma – Cushing’s syndrome – interleukins

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