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Abstract
Background Schnitzler syndrome (SchS) is a rare and easily misdiagnosed autoinflammatory disease. The misdiagnosis not only delays proper treatment, but also leads to the unreasonable use of immunosuppressants or even chemotherapeutic agents which results in needless financial and physical burdens.
Objectives The objective of this study was to summarize the clinical features and help physicians to avoid more misdiagnosis.
Methods Here we report a 52-year-old female with SchS, who suffered from chronic pruritic urticaria, fever, and arthralgia for over 10 years. Then we systemically reviewed 471 patients, 470 from 138 previous case reports and 1 from our case report.
Results Most of the patients were middle-aged or elderly women from European and American countries. There were limited number of cases coming from Asia, and no cases from Africa had yet been identified. The main clinical manifestations included chronic urticaria with or without pruritus, fever, arthralgia, ostealgia/myalgia, fatigue, and weight loss. Some cases also presented with lymphadenopathy, hepatomegaly, and splenomegaly. The presence of monoclonal immunoglobulin was the main diagnostic criteria, and the most common was IgMκ. Laboratory examinations also showed elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). Neutrophil infiltration was the most common pathological results in the skin tissue of SchS patients. Currently, anti-IL-1 therapy seems to be the best choice, followed by anti-IL-6 therapy. Glucocorticoid and traditional immunosuppressants seemed to be ineffective to SchS.
Conclusion As the diversity and variability of clinical symptoms, clinicians should pay more attention to the features of SchS to avoid misdiagnosis. Nearly half a century has passed since the first case of SchS was reported. Exploring its pathogenesis is still on the way. More Research is warranted for individualized therapy.
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Abnormalities in appearance. A: Urticaria on the face and thigh; B: Acropachy of both hands and feet.
A: X-ray showed bony hyperplasia “burr sign” at the acral areas of fingers (A) and toes (B).
Number of reported cases sorted by country. Most of the patients with Schnitzler syndrome were from the reports of European and American countries, while limited cased were from Asia and none from Africa.
Acknowledgements: NIL.
Disclosure of Interests None Declared.
- bDMARD
- Imaging
- Inflammatory arthritides