Hereditary pediatric Behçet-like disease

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Hereditary pediatric Behçet-like disease

Synonyms: Behçet-like disease due to HA20 | Behçet-like disease due to haploinsufficiency of A20

A rare autosomal dominant autoinflammatory syndrome characterized by early onset systemic inflammation with autoimmune manifestations and more rarely humoral immune deficiency and increased production of circulating proinflammatory cytokines variably manifesting with recurrent oral aphthous ulcers genital ulcers arthralgia or arthritis periodic fever uveitis and severe gastrointestinal involvement (pain diarrhea vomiting rectal bleeding).

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview.

Reference: Access aggregated data from Orphanet at Orphadata.

Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version March 2024

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Hereditary pediatric Behçet-like disease?

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Advocacy Organizations

Autoimmune Registry Inc.

Autoimmune Registry, Inc., ARI, is a 501(c)(3) non-profit umbrella organization that provides a hub for research, statistics, and patient data on all autoimmune diseases. Since 2016 we have operated a database for patients who suffer from any autoimmune disease. Our mission is to reduce the time of diagnosis, support research, compute prevalence statistics, and establish autoimmune disease as a major class of disease so that it receives the awareness of the public, the attention of healthcare providers, and the appropriate funding needed to improve upon existing treatment protocols and disease management strategies.

Ukrainian Association of Pediatric Immunology

Development of pediatric and clinical immunology in Ukraine. Raising awareness of inborn errors of immunity in Ukraine and rare immune diseases; Advocacy campaigns supporting patients with rare immune diseases and their treatment plans; Scientific research; Sharing knowledge about immunoprophylaxis; Advocacy campaigns supporting immunoprophylaxis.

Clinical Trials

For a list of clinical trials in this disease area, please click here.