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Efficacy of interleukin-1 blockade in Schnitzler's syndrome without detectable monoclonal gammopathy: a case-based review
- Source :
- Clinical Rheumatology
- Publication Year :
- 2020
-
Abstract
- Schnitzler’s syndrome (SchS) is a rare autoinflammatory disorder characterized by urticarial rash and monoclonal gammopathy which is currently regarded as IL-1 mediated disease. We present the case of a 21-year-old woman presenting with urticarial rash, arthralgias, and elevated inflammatory markers. She has been suffering these symptoms for 2 years and was treated with antihistamines, omalizumab, steroids, and non-steroidal anti-inflammatory drugs (NSAIDs) without success. After an extensive diagnostic workout, we suspected SchS even without monoclonal gammopathy, and started Anakinra 100 mg daily with a dramatic response and achieving complete remission after 48 h of the beginning of the treatment, so we decided to confirm SchS diagnosis. We performed a search of the literature and found seven more cases of patients diagnosed with SchS without monoclonal gammopathy at the presentation. Five were treated with IL-1 blocking therapies and all achieved remission. We, therefore, prompt the possible role of IL-1 blockade therapy remission as support in diagnosing SchS without monoclonal gammopathy.
- Subjects :
- Adult
medicine.medical_specialty
Urticaria
Canakinumab
Paraproteinemias
Disease
Omalizumab
Monoclonal Gammopathy of Undetermined Significance
IL-1 blockade
03 medical and health sciences
Young Adult
0302 clinical medicine
Rheumatology
Internal medicine
medicine
Humans
Schnitzler Syndrome
030203 arthritis & rheumatology
Anakinra
business.industry
Schnitzler’s syndrome
Monoclonal gammopathy
Interleukin
General Medicine
Dermatology
Blockade
Interleukin 1 Receptor Antagonist Protein
Case Based Review
Paraprotein
Female
medicine.symptom
business
030215 immunology
medicine.drug
Interleukin-1
Subjects
Details
- ISSN :
- 14349949
- Volume :
- 40
- Issue :
- 7
- Database :
- OpenAIRE
- Journal :
- Clinical rheumatology
- Accession number :
- edsair.doi.dedup.....12250aa584a5a0ec5a6ada0cd34a4558