Evidence Based Recommendations for Diagnosis and Management of Tumor Necrosis Factor Receptor-1 Associated Periodic Syndrome (TRAPS)

Nienke ter Haar¹, Paul Brogan², Gilles Grateau³, Jordi Anton⁴, Karyl Barron⁵, Luca Cantarini⁶, Joost Frenkel⁷, Caroline Galeotti⁸, Veronique Hentgen⁹, Michael Hofer¹⁰, Tilmann Kallinich¹¹, Isabelle Kone-Paut¹², Jasmin Kuemmerle-Deschner¹³, Huri Ozdogan¹⁴, Seza Ozen¹⁵, Ricardo Russo¹⁶, Anna Simon¹⁷, Yosef Uziel¹⁸, Carine Wouters¹⁹, Brian Feldman²⁰, Bas Vastert⁷, Nico Wulffraat²¹, Helen Lachmann²² and Marco Gattorno²³, ¹Laboratory for Translational Immunology, University Medical Center Utrecht, Utrecht, Netherlands, ²Rheumatology Unit, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom, ³Service De Médecine Interne, Hopital Tenon, Paris, France, ⁴Pediatric Rheumatology Unit. Hospital Sant Joan de Déu. Universitat de Barcelona, Barcelona, Spain, ⁵NIH, Bethesda, MD, ⁶University of Siena, Siena, Italy, ⁷University Medical Center Utrecht, Utrecht, Netherlands, ⁸Bicêtre Hospital, University of Paris SUD, Paris, France, ⁹Versailles Hospital, Le Chesnay Cedex, France, ¹⁰Centre Multisite Romand de Rhumatologie Pediatrique, Lausanne, Switzerland, ¹¹Charite, University Medicine Berlin, Berlin, Germany, ¹²Department of Pediatric Rheumatology, Reference Centre for Autoinflammatory Disorders CEREMAI, Bicêtre Hospital, University of Paris SUD, Paris, France, ¹³Pediatrics, University Hospital Tuebingen, Tuebingen, Germany, ¹⁴Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical Faculty, University of Istanbul, Istanbul, Turkey, ¹⁵Deptartment. of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey, ¹⁶Immunology & Rheumatology, Hospital De Pediatria, Buenos Aires, Argentina, ¹⁷Radboudumc, Nijmegen, Netherlands, ¹⁸Tel-Aviv University, Sackler School of Medicine, Tel-Aviv, Israel, ¹⁹University of Leuven, Laboratory of Pediatric Immunology, University Hospital Leuven, Leuven, Belgium, ²⁰Rheumatology, The Hospital for Sick Children, Toronto, ON, Canada, ²¹Pediatric rheumatology, Wilhelmina Children's Hospital/ UMC Utrecht, Utrecht, Netherlands, ²²UK National Amyloidosis Centre, University College London Medical School, London, United Kingdom, ²³Istituto Giannina Gaslini, Genova, Italy

Meeting: 2014 ACR/ARHP Annual Meeting

Keywords: diagnosis, evidence-based practice, treatment and tumor necrosis factor (TNF)

Session Information

Title: Pediatric Rheumatology - Clinical and Therapeutic Aspects: Systemic Juvenile Idiopathic Arthritis, Spondyloarthropathy and Miscellaneous Pediatric Rheumatic Diseases

Session Type: Abstract Submissions (ACR)

Background/Purpose Tumor necrosis factor receptor-1 associated periodic syndrome (TRAPS) is a rare hereditary autoinflammatory syndrome that can lead to significant morbidity. Evidence-based guidelines are lacking and management is mostly based on physician’s experience. Consequently, treatment regimens differ throughout Europe. In 2012, a European initiative called SHARE (Single Hub and Access point for pediatric Rheumatology in Europe) was launched to optimize and disseminate diagnostic and management regimens in Europe for children and young adults with rheumatic diseases. One of the aims of SHARE was to provide evidence based recommendations for diagnosis and treatment of TRAPS.

Methods Evidence based recommendations were developed using the European League Against Rheumatism (EULAR) standard operating procedure. An expert committee was instituted, consisting of pediatric and adult rheumatologists. The expert committee defined search terms for the systematic literature review. Two independent experts scored articles for validity and level of evidence. Recommendations derived from the literature were evaluated by an online survey. Those with less than 80% agreement on the online survey or with relevant comments of the experts were reformulated. Subsequently, all recommendations were discussed at a consensus meeting using the nominal group technique. Recommendations were accepted if more than 80% agreement was reached.

Results The literature search yielded 523 articles, of which 22 were considered relevant and therefore scored for validity and level of evidence. Eighteen were scored valid and used in the formulation of the recommendations. Seventeen recommendations were suggested in the online survey and discussed during the consensus meeting. Five general recommendations on management, two recommendations for diagnosis, seven for monitoring and eight for treatment were accepted with more than 80% agreement. Topics covered are the following:

general recommendations: use of the multidisciplinary team, treatment goals and vaccinations
diagnosis: TNFRSF1A screening, interpretation of R92Q and P46L variants
monitoring: monitoring frequency and minimal assessments in TRAPS patients, the use of AIDAI score in clinical studies and risk assessment of amyloidosis
treatment: NSAIDS and/or glucocorticoids during attacks, IL-1 blockade, etanercept, anti-TNF monoclonal antibodies, switching between biologicals

Conclusion The SHARE initiative provides recommendations for diagnosis and treatment for TRAPS and thereby facilitates improvement and uniformity of care throughout Europe.

Disclosure:

N. ter Haar,
None;

P. Brogan,

Novartis, Roche,

Novartis Pharmaceutical Corporation,

G. Grateau,
None;

J. Anton,
None;

K. Barron,
None;

L. Cantarini,

Novartis Pharma AG, SOBI,

J. Frenkel,

European Union ERANET,

Novartis Pharmaceutical Corporation,

SOBI,

C. Galeotti,

Novartis Pharmaceutical Corporation,

V. Hentgen,

Novartis Pharmaceutical Corporation,

Novartis, Pfizer, Roche,

M. Hofer,
None;

T. Kallinich,

Novartis, SOBI,

Novartis Pharmaceutical Corporation,

I. Kone-Paut,
None;

J. Kuemmerle-Deschner,

Novartis Pharmaceutical Corporation,

SOBI,

H. Ozdogan,
None;

S. Ozen,
None;

R. Russo,
None;

A. Simon,

Servier,

Novartis, SOBI, Xoma,

Y. Uziel,

Novartis Pharmaceutical Corporation,

Abbvie, Neopharm, Novartis, Roche,

C. Wouters,
None;

B. Feldman,
None;

B. Vastert,
None;

N. Wulffraat,
None;

H. Lachmann,
None;

M. Gattorno,
None.

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