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 kappa
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 identified 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 x 10(9)/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-1beta and
TNF-alpha (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.