Adult-onset Still's disease pathophysiology
Template:Adult-onset Still's disease
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
Overview
Pathophysiology
Adult-onset Still's disease is an automminue inflammatory arthritis that typically affects adolescents and adults ranging from age 16-40 years. Major etiological mechanisms behind cause a dysfunction of the innate and cellular immunity (limited) leading to activation of effector cells of the disease.
Putative triggers
Although the pathogenesis of adult-onset Still's disease is largerly idiopathic. Triggers of ASOD lead to activation of toll-like receptors (TLR) and activation of immune system. The following triggers may be implicated as factors responsible for generating key pathological processes occurring in adult-onset Still's disease (ASOD):[1][2][3][4]
Pathogen-associated molecular patterns (PAMPs)
- Bacteria
- Yersinia enterocolitica
- Chlamydophila pneumoniae
- Brucella abortus
- Borrelia burgdorferi
- Viruses
- Rubella
- Echovirus 7
- Mumps
- Cytomegalovirus (CMV)
- Fungi
Danger-associated molecular patterns (DAMPs)
Immune dysfunction
Role of interleukin-1 beta
Interleukin-i beta plays a key role in producing major characteristic features of adult-onset Still's disease. The following processes are affected by an increased production of this key interleukin:
Hypothalamic-pituitary axis influence
Liver synthesis and secretion of acute phase proteins
Osteoclasts activation and matrix metalloproteinases (MMPs) synthesis
Innate immune system cells activation
Increased gene transcription of proinflammatory molecules
Role of interleukin-18
Role of interleukin-6
Role of interleukin-17
Role of interferon gamma
Role of tumor necrosis factor-alpha (TNF-alpha)
Reactive hemophagocytic lymphohistiocytosis
Genetics
Associated Conditions
Gross Pathology
Microscopic Pathology
References
- ↑ Perez C, Artola V (March 2001). "Adult Still's disease associated with Mycoplasma pneumoniae infection". Clin. Infect. Dis. 32 (6): E105–6. doi:10.1086/319342. PMID 11247732.
- ↑ Dua J, Nandagudi A, Sutcliffe N (December 2012). "Mycoplasma pneumoniae infection associated with urticarial vasculitis mimicking adult-onset Still's disease". Rheumatol. Int. 32 (12): 4053–6. doi:10.1007/s00296-011-2107-4. PMID 21918897.
- ↑ Escudero FJ, Len O, Falcó V, de Sevilla TF, Sellas A (June 2000). "Rubella infection in adult onset Still's disease". Ann. Rheum. Dis. 59 (6): 493. PMC 1753159. PMID 10885978.
- ↑ Efthimiou P, Georgy S (December 2006). "Pathogenesis and management of adult-onset Still's disease". Semin. Arthritis Rheum. 36 (3): 144–52. doi:10.1016/j.semarthrit.2006.07.001. PMID 16949136.