Ongoing Task Forces

Autoinflammatory syndromes in Allergology and Clinical Immunology

TF members:
Clive Grattan, Anna Simon, Helen Lachmann, Tilmann Kallinich, Jasmin Kümmerle-Deschner, Marcus Maurer, Claudia Traidl-Hoffmann, Eugen Feist, Karoline Krause, Marco Gattorno, Alexander Navarini, Helen De Koning, Carsten Bindslev-Jensen, Dan Lipsker

Start date: 2011

Proposed end date: 2014

Rationale:
The term "autoinflammation" was first used for a group of hereditary syndromes characterized by periodically recurring episodes of fever. Autoinflammatory diseases show systemic inflammatory processes without evidence of an underlying infection, immunodeficiency, allergy or autoimmune disorder. Lately, the concept of autoinflammation has been extended to diseases in which there is not necessarily a periodicity of symptoms and the occurrence of fever is not a mandatory appearance.In general, autoinflammatory disorders are very rare entities with limited awareness even among specialized health care professionals. Thus, they are often recognized with a diagnostic delay of many years or even decades. Although there have been important advances in the recent past, diagnosis and treatment of autoinflammatory syndromes remain to be a major challenge in a lot of cases. As skin manifestation such as urticarial or maculo-papular rash is often the earliest and one of the most common and prominent symptoms in these disorders allergologists and clinical immunologists should be aware of their existence and consider them as a potential differential diagnosis.

Objectives:
We aim at increasing the awareness of autoinflammatory diseases in the field of allergology and clinical immunology. Therefore, we will build a network, review the literature and create a position paper on the current status of diagnosis and treatment of autoinflammatory disorders.

Outcome and benefits:
With the help of the Task Force we will pool our knowledge on autoinflammatory syndromes and work on standard operating procedures to ensure an early recognition of autoinflammatory diseases and best medical care of these patients. Furthermore we will work on patient education, information and prevention programs.

Progress update:
Publication of two TF papers:
Krause K, Grattan CE, Bindslev-Jensen C, Gattorno M, Kallinich T, de Koning HD, Lachmann HJ, Lipsker D, Navarini AA, Simon A, Traidl-Hoffmann C, Maurer M. How not to miss autoinflammatory diseases masquerading as urticaria. Allergy. 2012: 67:1465-1474.

Kallinich T, Gattorno M, Grattan CE, de Koning HD, Traidl-Hoffmann C, Feist E, Krause K, Lipsker D, Navarini AA, Maurer M, Lachmann HJ, Simon A. Unexplained recurrent fever: When is the explanation autoinflammation? Allergy 2013; 68:285-96.

Plan 2014:
EAACI TF paper on unmet needs of autoinflammatory diseases.
Last updated 07 November 2014