Autoinflammatory syndromes

被引：0
作者：
Bonnekoh, Hanna [1 ,2 ]
Krusche, Martin [3 ]
Feist, Eugen [4 ]
Wagner, Annette Doris [5 ]
Pankow, Anne [5 ,6 ]
机构：
[1] Charite Univ Med Berlin, Inst Allergieforschung, Hindenburgdamm 27, D-12203 Berlin, Germany
[2] Fraunhofer Inst Translationale Med & Pharmakol ITM, Allergol & Immunol, Berlin, Germany
[3] Univ Klinikum Hamburg Eppendorf, Med Klin & Poliklin Nephrol 3, Rheumatol & Endokrinol, Martinistr 52, D-20251 Hamburg, Germany
[4] Helios Fachklin Vogelsang Gommern, Klin Rheumatol, Sophie-von-Boetticher-Str 1, D-39245 Vogelsang, Germany
[5] Hannover Med Sch, Abt Nieren & Hochdruckerkrankungen, Ambulanz Seltene Entzundl Systemerkrankungen Niere, Carl-Neuberg-Str 1, D-30625 Hannover, Germany
[6] Charite Univ Med Berlin, Med Klin Schwerpunkt Rheumatol & Klin Immunol, Charitepl 1, D-10117 Berlin, Germany
来源：
INNERE MEDIZIN | 2023年 / 64卷 / 05期
关键词：
Autoinflammatory diseases; Familial Mediterranean fever; VEXAS syndrome; Schnitzler syndrome; Still's disease; adult-onset; FAMILIAL MEDITERRANEAN FEVER; ONSET STILLS-DISEASE; SCHNITZLER-SYNDROME; MUTANT UBA1; AMYLOIDOSIS; EFFICACY;
D O I：
10.1007/s00108-023-01505-1
中图分类号：
R5 [内科学];
学科分类号：
1002 ; 100201 ;
摘要：
The concept of autoinflammation includes a heterogeneous group of monogenic and polygenic diseases. These are characterized by excessive activation of the innate immune system without antigen-specific T cells or autoantibodies. The diseases are characterized by periodic episodes of fever and increased inflammation parameters. Monogenic diseases include familial Mediterranean fever (FMF) and the newly described VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome. Heterogeneous diseases include adult-onset Still's disease and Schnitzler syndrome. Treatment is aimed at preventing the excessive inflammatory reaction in order to avoid long-term damage, such as amyloid A (AA) amyloidosis.
引用
收藏
页码：442 / 451
页数：10
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