SCHNITZLER SYNDROME: FAMILIAR URTICARIA IN UNFAMILIAR AUTOINFLAMMATORY DISEASES. EXPERIENCE OF TREATING A CHILD WITH SCHNITZLER SYNDROME IN THE PEDIATRIC DEPARTMENT KNP «OKDL KOR» KROPYVNYTSKYI
Miroshnychenko M.V., Shishkanova N.V., Ivanova V.G.
Summary. Schnitzler syndrome (SchS) is an auto-inflammatory disease associated with IgM (rarely IgG) monoclonal gammopathy and is manifested by chronic urticaria, signs of systemic inflammation in the form of fever, arthralgia and/or bone pain, as well as increased serum markers of systemic inflammation. Despite the fact that the disease has been known since 1972, when it was first described by Lilian Schnitzler, this pathology is rarely diagnosed in the world [1]. The highest effectiveness of treatment was found when using interleukin-1 (IL-1) inhibitors [2] [3], but unfortunately, it should be noted that against the background of discontinuation of medication, the return of symptoms was noted [4], so patients need lifelong treatment. Purpose: to increase the awareness of the medical community about the clinical manifestations, methods of diagnosis and treatment of SchS. To acquaint the community with the clinical results of the use of IL-1 inhibitors for the treatment of this syndrome in a child in the pediatric department of the KNP «OKDL KOR» in Kropyvnytskyi. Object and methods: Modern literary data were processed. A clinical case of Schnitzler syndrome in a child is presented, which was diagnosed by using of the Strasbourg criteria at the Kirovohrad Regional Children’s Hospital. The effectiveness of the treatment of SchS with an IL-1 inhibitor and side effects during a year of use were analyzed. Results: Against the background of treatment with an IL-1 inhibitor for 1 year, the patient had a significant improvement in her general well-being: it was noted a decrease in skin manifestations, up to their complete disappearance, absence of fever, arthralgia/osalgia, which were part of the symptom complex at the time of diagnosis. Against the background of drug administration, adverse reactions were noted: local manifestations in the form of redness and edema of the injection site, twice — systemic manifestations in the form of laryngospasm in combination with severe urticaria, which were treated with 1 dose of antihistamines. Conclusions: Schnitzler syndrome is a rare and underdiagnosed disease. A high vigilance regarding SchS should be observed during examining patients with chronic urticaria-like dermatoses, periodic fever, and arthralgias. To clarify the diagnosis, it is necessary to carry out additional tests — electrophoresis of serum proteins with immunofixation. It is important to recognize the disease in time, because Schnitzler syndrome is associated with the development of hematological malignancy. About 20% of patients develop a lymphoproliferative disorder an average of 7.6 years after the onset of symptoms. Thus, these patients require constant and long-term monitoring. IL-1 inhibitors are extremely effective in relieving symptoms and are considered first-line therapy.
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