Adult-onset Still's disease natural history, complications and prognosis
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Adult-onset Still's disease |
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Differentiating Adult-onset Still’s Disease from other Diseases |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
Overview
Natural History
- If left untreated, adult-onset Still's disease (AOSD) follows a relapsing and remitting course.
- Initial presentation of AOSD may be between 16 to 35 years of age.
- Symptoms usually evolve over weeks to months.
- AOSD exhibits a variable clinical course:
- 20% with long-term remission
- 30% remit-relapse
- 50% chronic arthritis
- May progress to develop macrophage activating syndrome (MAS), also known as hemophagocytic syndrome.
Complications
Life threatening complications
Adult-onset Still's disease may lead to the development of the following life-threatening complications:[1]
- Macrophage activation syndrome (MAS)
- Disseminated intravascular coagulation (DIC)
- Thrombotic thrombocytopenic purpura (TTP)
- Diffuse alveolar hemmorrhage
- Pulmonary arterial hypertension (PAH)
Prognosis
References
- ↑ Efthimiou P, Kadavath S, Mehta B (March 2014). "Life-threatening complications of adult-onset Still's disease". Clin. Rheumatol. 33 (3): 305–14. doi:10.1007/s10067-014-2487-4. PMID 24435354.