Skip to main content
Key Specialties
Cardiology Diabetology Endocrinology Gastroenterology Geriatrics and Gerontology Gynecology and Obstetrics Hematology Infectious Disease Internal Medicine Nephrology Neurology Oncology Pediatrics Respiratory Medicine Rheumatology Surgery
Congress Coverage
2024 ACC Congress 2023 ESMO Congress 2023 EASD Congress 2023 ERS Congress 2023 ESC Congress
Clinical Collections
Advances in Alzheimer’s

At a glance: The STEP trials

A round-up of the STEP phase 3 clinical trials evaluating semaglutide for weight loss in people with overweight or obesity.
ADVANCED SEARCH
Log in
Top
Clinical Rheumatology
Published in: Clinical Rheumatology 2/2008

01-02-2008 | Case Report

Extreme efficiency of anti-interleukin 1 agent (anakinra) in a Japanese case of CINCA syndrome

Authors: Yasuyo Kashiwagi, Hisashi Kawashima, Shigeo Nishimata, Ryuhei Nagao, Kiyoko Watanabe, Kouji Takekuma, Akinori Hoshika

Published in: Clinical Rheumatology | Issue 2/2008

Login to get access

Abstract

We describe a case of severe CINCA syndrome in a Japanese male infant. We recently managed with anti-interleukin 1 agent (anakinra). Anakinra immediately led to improvement of both the clinical symptoms and the laboratory findings.
Literature
1.
Lorber J (1973) Syndrome for diagnosis: dwarfing, persistently open fontanelle; recurrent meningitis; recurrent subdural effusions with temporary alternate-sided hemiplegia; high-tone deafness; visual defect with pseudopapilloedema; slowing intellectual development; recurrent acute polyarthritis; erythema marginatum, splenomegaly and iron-resistant hypochromic anaemia. Proc R Soc Med 66:1070–1071PubMed
2.
Prieur AM (2001) A recently recognized chronic inflammatory disease of early onset characterized by the triad of rash, central nervous system involvement and arthropathy. Clin Exp Rheumatol 19:103–106PubMed
3.
Kawashima H, Sato A, Nishimata S, Yamada N, Kashiwagi Y, Watanabe K, Takekuma K, Hoshika A, Ozawa T (2007) A case report of neonatal onset multisystemic inflammatory disease in Japan treated with continuous hemodiafiltration and steroid pulse therapy. Therapeutic Apheresis and Dialysis 11(3):233–235CrossRef
4.
Hannum CH, Wilcox CJ, Arend WP, Joslin FG, Dripps DJ, Heimdal PL et al (1990) Interleukin-1 receptor antagonist activity of a human interleukin-1 inhibitor. Nature 343:336–340PubMedCrossRef
5.
Hawkins PH, Lachmann HJ, Aganna E, McDermott MF (2004) Spectrum of clinical features in Muckle-Wells syndrome and response to anakinra. Arthritis Rheum 50:607–612PubMedCrossRef
6.
Ramos E, Arostegui JI, Campuzano S, Rius J, Bousono C, Yague J (2005) Positive clinical and biochemical responses to anakinra in a 3-yr-old patient with cryopyrin-associated periodic syndrome (CAPS). Rhematology (Oxford) 44:1072–1073CrossRef
7.
Granel B, Serratrice J, Disdier P, Weiller PJ (2005) Dramatic improvement with anakinra in a case of chronic infantile neurological cutaneous and articular (CINCA) syndrome. Rheumatology (Oxford) 44:689–690CrossRef
8.
Lovell DJ, Bowyer SL, Solinger AM (2005) Interleukin-1 blockade by anakinra improves clinical symptoms in patients with neonatal onset multisystem inflammatory disease. Arthritis Rheum 52:1283–1286PubMedCrossRef
9.
Aksentijevich I, Nowak M, Mallah M et al (2002) De Novo CIAS1 mutations, cytokine activation, and evidence for genetic heterogeneity in patients with neonatal-onset multisystem inflammatory disease (NOMID). Arthritis Rheum 46:3340–3348PubMedCrossRef
10.
Feldmann J, Prieur AM, Quartier P et al (2002) Chronic infantile neurological cutaneous and articular syndrome is caused by mutations in CIAS1, a gene highly expressed in polymorphonuclear cells and chondrocytes. Am J Hum Genet 71:198–203PubMedCrossRef
11.
Frenkel J et al (2004) Anakinra in mutation-negative NOMID/CINCA syndrome: comment on the articles by Hawkins et al and Hoffman and Patel. Arthritis Rheum 50:3738–3739PubMedCrossRef
12.
Callejas JL et al (2006) Anakinra in mutation-negative CINCA syndrome. Clin Rheumatol 27:1–2CrossRef
13.
Matsubayashi T, Sugiura H, Arai T, Oh-ishi T, Inamo Y (2006) Anakinra therapy for CINCA syndrome with a novel mutation in exon 4 of the CIAS1 gene. Acta Paediatrica 95:246–249PubMedCrossRef
14.
Matsubara T, Hasegawa M, Shiraishi M, Hoffman HM, Ichiyama T, Tanaka T, Ueda H, Ishihara T, Furukawa S (2006) A severe case of chronic infantile neurologic, cutaneous, articular syndrome treated with biologic agents. Arthritis Rheum 54(7):2314–2320PubMedCrossRef
Metadata
Title
Extreme efficiency of anti-interleukin 1 agent (anakinra) in a Japanese case of CINCA syndrome
Authors
Yasuyo Kashiwagi
Hisashi Kawashima
Shigeo Nishimata
Ryuhei Nagao
Kiyoko Watanabe
Kouji Takekuma
Akinori Hoshika
Publication date
01-02-2008
Publisher
Springer-Verlag
Published in
Clinical Rheumatology / Issue 2/2008
Print ISSN: 0770-3198
Electronic ISSN: 1434-9949
DOI
https://doi.org/10.1007/s10067-007-0734-7

Other articles of this Issue 2/2008

Clinical Rheumatology 2/2008 Go to the issue

Case Report

Multiple peripheral arterial and aortic aneurysms in Behcet’s syndrome—a case report

Case Report

Multifocal avascular necrosis revealing antiphospholipid syndrome

Brief Report

Familial autoimmunity: maternal parent-of-origin effect in juvenile idiopathic arthritis

Original Article

Treatment of venous thrombosis associated with Behcet’s disease: immunosuppressive therapy alone versus immunosuppressive therapy plus anticoagulation

Case Report

Severe invalidating pain syndrome associated with benznidazole therapy for Chagas’ disease

Brief Report

Rare copresent rheumatoid arthritis and gout: comparison with pure rheumatoid arthritis and a literature review
Obesity Clinical Trial Summary

At a glance: The STEP trials

Read more

A round-up of the STEP phase 3 clinical trials evaluating semaglutide for weight loss in people with overweight or obesity.

Developed by: Springer Medicine

Highlights from the ACC 2024 Congress

Year in Review: Pediatric cardiology

Pediatric Cardiology Video

Watch Dr. Anne Marie Valente present the last year's highlights in pediatric and congenital heart disease in the official ACC.24 Year in Review session.

Year in Review: Pulmonary vascular disease

Cardiopulmonary Comorbidity Video

The last year's highlights in pulmonary vascular disease are presented by Dr. Jane Leopold in this official video from ACC.24.

Year in Review: Valvular heart disease

Valvular Heart Disease Video

Watch Prof. William Zoghbi present the last year's highlights in valvular heart disease from the official ACC.24 Year in Review session.

Year in Review: Heart failure and cardiomyopathies

Heart Failure Video

Watch this official video from ACC.24. Dr. Biykem Bozkurt discuss last year's major advances in heart failure and cardiomyopathies.

ACC 2024 Congress Coverage

Heart Failure Video

Year in Review: Heart failure and cardiomyopathies

Watch now

Watch this official video from ACC.24. Dr. Biykem Bozkurt discuss last year's major advances in heart failure and cardiomyopathies.

Semaglutide benefits people with type 2 diabetes and obesity-related heart failure

16-04-2024 Type 2 Diabetes News

Incentivised to exercise

11-04-2024 Lifestyle Modifications News

New intervention for STEMI-related cardiogenic shock

10-04-2024 Myocardial Infarction News

» View more highlights on the ACC 2024 congress hub page

Image Credits