Registry Working Party
Information on ongoing studies using the ESID Registry Database.
For completed studies please refer to the list of Registry publications.
If you want to propose a study to the ESID Registry, please use the following Research Proposal Form:
ESID registry Working Party is inviting the ESID registry participants to take part in the "Malignancies in IEI" study latest by August 31st 2022.
The IDDA score has already been available as optional module (tab) in the level 1 for your own use at the initial and follow up patient entries and is designed for all IEI with immune dysregulation. Now, I am inviting your active contribution to a prospective study until end of 2023 (prolongation possible), aiming to implement a powerful tool for assessment and monitoring of disease activity and phenotype patterns!
M. Seidel, March 2022
Familial hemophagocytic lymphohistiocytoses (FHL) are a group of genetic disorders of cytotoxicity, which predispose to the life-threatening syndrome of HLH. FHL2 (Perforin deficiency) and FHL3 (MUNC13-4 deficiency) are the most frequent forms of the disease. Current therapies include control of the aberrant immune activation by immune-/chemotherapy followed by HSCT still show about 20 to 40% overall mortality. A collaborative project between ESID, Histiocyte Society and inborn errors working party of EBMT MORE HERE
How can I report a patient for the FHL2/3 study? Click Here
Extention to Level 1 for patients with ILD.
Centres that have fully registered eligible patients in the ESID-registry, have already been contacted directly and asked for approval to use the documented data.
Data has been extracted and provided to the study team.
An ESID Registry level 3 project.
Released for recruitment!
If you treat a SOCS1 patient at your center and want to include her/him into the registry please send an email to
We then will provide individual information to you regarding the next steps.
GAIN is a network of physicians and scientists interested on collecting data on patients with multiorgan autoimmunity with and without immunodeficiency. The network started in Germany, this is why the dataset is called German multi-organ Auto Immunity Network (GAIN, https://www.research4rare.de/forschungsverbuende/gain/ )
GAIN website: www.g-a-i-n.de
The first ESID Registry level 3 project.
The first journal publication resulting from this study has been published in 2018!
Please refer to the ESID Registry Publication list for further details.
Retrospective analysis of X-linked agammaglobulinemia (XLA) and autosomal recessive agammaglobulinemia (ARA) patients: Does early diagnosis and treatment result in substantial health gain?
Risk factors for mortality in cartilage-hair hypoplasia