| Title |
Renal involvement in secondary amyloidosis of Muckle-Wells syndrome: marked improvement of renal function and reduction of proteinuria after therapy with human anti-interleukin-1β monoclonal antibody canakinumab
|
|---|---|
| Published in |
Clinical Rheumatology, February 2014
|
| DOI | 10.1007/s10067-013-2481-2 |
| Pubmed ID | |
| Authors |
Roberto Scarpioni, Donato Rigante, Luca Cantarini, Marco Ricardi, Vittorio Albertazzi, Luigi Melfa, Antonio Lazzaro |
| Abstract |
Muckle-Wells syndrome (MWS) is a rare hereditary autoinflammatory disorder characterized by recurrent urticaria-like skin rashes, arthralgias, conjunctivitis, hypoacusia, and risk of reactive AA amyloidosis due to the progressive accumulation of amyloid fibrils in different organs. Its genetic defect lies in mutations in the NLRP3 gene, encoding the cryopyrin protein, and resulting in interleukin (IL)-1β oversecretion. Renal involvement, in terms of proteinuria or renal insufficiency, can be observed in up to 25 % of patients. Herein, we describe our experience with two Caucasian patients, father and son, aged 52 and 26 years, respectively, heterozygous for both V198M and R260W NLRP3 mutations who had AA amyloid deposits on renal biopsy. The fully human monoclonal antibody canakinumab, providing selective and prolonged IL-1β blockade, was administered in both patients every 60 days over a period of 18 months. This treatment allowed to obtain amazing results: a rapid disappearance of any clinical symptoms, the stable normalization of serum amyloid-A and, furthermore, a marked improvement of glomerular filtration rate and proteinuria with no adverse events. Our data, though limited to only two patients, emphasize that therapeutic intervention with canakinumab, suppressing both inflammation and IL-1β-mediated manifestations, can contribute to improve kidney function in MWS with overt renal amyloidosis. |
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Geographical breakdown
| Country | Count | As % |
|---|---|---|
| Unknown | 1 | 100% |
Demographic breakdown
| Type | Count | As % |
|---|---|---|
| Members of the public | 1 | 100% |
Mendeley readers
The data shown below were compiled from readership statistics for 38 Mendeley readers of this research output. Click here to see the associated Mendeley record.
Geographical breakdown
| Country | Count | As % |
|---|---|---|
| South Africa | 1 | 3% |
| Unknown | 37 | 97% |
Demographic breakdown
| Readers by professional status | Count | As % |
|---|---|---|
| Researcher | 7 | 18% |
| Student > Postgraduate | 4 | 11% |
| Student > Doctoral Student | 4 | 11% |
| Professor | 4 | 11% |
| Student > Bachelor | 3 | 8% |
| Other | 13 | 34% |
| Unknown | 3 | 8% |
| Readers by discipline | Count | As % |
|---|---|---|
| Medicine and Dentistry | 17 | 45% |
| Agricultural and Biological Sciences | 4 | 11% |
| Biochemistry, Genetics and Molecular Biology | 2 | 5% |
| Nursing and Health Professions | 2 | 5% |
| Chemistry | 2 | 5% |
| Other | 5 | 13% |
| Unknown | 6 | 16% |
Attention Score in Context
This research output has an Altmetric Attention Score of 2. This is our high-level measure of the quality and quantity of online attention that it has received. This Attention Score, as well as the ranking and number of research outputs shown below, was calculated when the research output was last mentioned on 02 December 2016.
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#14,773,697
of 22,743,667 outputs
Outputs from Clinical Rheumatology
#1,888
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#184,022
of 310,260 outputs
Outputs of similar age from Clinical Rheumatology
#51
of 82 outputs
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