Kidney involvement in the Schnitzler syndrome, a rare disease
| Autor: | Luigi Rossi, Carlo Basile, Carlo Lomonte, Pasquale Libutti, Piero Lisi, Raffaele Manna, Francesco Casucci, Annalisa Teutonico |
|---|---|
| Rok vydání: | 2017 |
| Předmět: |
medicine.medical_specialty
membranoproliferative glomerulonephritis 030232 urology & nephrology monoclonal IgM gammopathy Disease Acute kidney injury Auto-inflammatory diseases Chronic urticarial rash Membranoproliferative glomerulonephritis Monoclonal IgM gammopathy Schnitzler syndrome Nephropathy 03 medical and health sciences Rare Diseases 0302 clinical medicine Gammopathy medicine auto-inflammatory diseases 030203 arthritis & rheumatology Transplantation Anakinra business.industry chronic urticarial rash Settore MED/09 - MEDICINA INTERNA Genetic disorder medicine.disease Dermatology acute kidney injury Nephrology business medicine.drug Rare disease |
| Zdroj: | Clinical Kidney Journal |
| ISSN: | 2048-8513 2048-8505 |
| DOI: | 10.1093/ckj/sfx077 |
| Popis: | The Schnitzler syndrome (SS) is a rare and underdiagnosed entity that associates a chronic urticarial rash, monoclonal IgM (or sometimes IgG) gammopathy and signs and symptoms of systemic inflammation. During the past 45 years, the SS has evolved from an elusive little-known disorder to the paradigm of a late-onset acquired auto-inflammatory syndrome. Though there is no definite proof of its precise pathogenesis, it should be considered as an acquired disease involving abnormal stimulation of the innate immune system, which can be reversed by the interleukin-1 receptor antagonist anakinra. It clearly expands our view of this group of rare genetic diseases and makes the concept of auto-inflammation relevant in polygenic acquired diseases as well. Increasing numbers of dermatologists, rheumatologists, allergologists, haematologists and, more recently, nephrologists, recognize the SS. The aim of this review is to focus on kidney involvement in the SS. Although the literature regarding kidney involvement in the SS is very poor it can be severe, as in our own case here reported, leading us to recommend the systematic search for nephropathy markers in the SS. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|