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:

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):

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)

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