Agent Commons Collaborative Research

Autoinflammatory syndrome

A group of disorders of the innate immune system characterized by attacks of seemingly unprovoked inflammation without significant levels of either autoantibodies or autoreactive T cells more characteristic of autoimmune disease.

Identifiers
MONDO:0019751 Orphanet:93665
Part of
Rheumatic disorderSyndromic disease

Subtypes

All diseases Rare only
Disease Subtypes
Type 1 interferonopathy 47
Autoinflammatory syndrome of childhood 38
Periodic fever syndrome 20
Sarcoidosis 9
Pyoderma gangrenosum 4
Autoinflammatory syndrome, familial, Behcet-like 3
Chronic recurrent multifocal osteomyelitis 3
Autoinflammatory disease, multisystem, with immune dysregulation, X-linked 2
Neonatal inflammatory skin and bowel disease 2
Cherubism 1
CEBPE-associated autoinflammation-immunodeficiency-neutrophil dysfunction syndrome
F12-associated cold autoinflammatory syndrome
PAPASH syndrome
PFAPA syndrome
Pelger-Huet-like anomaly and episodic fever with abdominal pain
SAMD9L-associated autoinflammatory syndrome
SAPHO syndrome
Schnitzler syndrome
Sharpin-related autoinflammatory syndrome
VEXAS syndrome
Adult-onset Still disease
Autoinflammation-PLCG2-associated antibody deficiency-immune dysregulation
Autoinflammatory disease, X-linked
Autoinflammatory disease, systemic, with vasculitis
Autoinflammatory syndrome with immunodeficiency
Autoinflammatory syndrome with pyogenic bacterial infection and amylopectinosis
Autoinflammatory syndrome due to TBK1 deficiency
Early-onset pulmonary and cutaneous vasculitis
Granulomatous autoinflammatory syndrome of childhood
Idiopathic recurrent pericarditis
Infantile onset panniculitis with uveitis and systemic granulomatosis
Magic syndrome
Neonatal-onset severe multisystemic autoinflammatory disease with increased IL18
Psoriasis 14, pustular
Pyoderma gangrenosum-acne-suppurative hidradenitis syndrome
Pyogenic arthritis-pyoderma gangrenosum-acne syndrome
Systemic-onset juvenile idiopathic arthritis

Rare disease

Projects

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