Prognosis

Clinical course

Three broad patterns of disease have traditionally been recognized:[5][42]​​

  • Monocyclic systemic (21% to 64% of cases): typically consists of one episode lasting a few months followed by remission

  • Polycyclic systemic (9% to 50%): intermittent episodes or flares

  • Chronic articular (12% to 56%): persisting and progressive symptoms, particularly affecting the joints.

The advent of targeted and biologic agents is likely to have a significant impact on the distribution of these clinical courses. Though chronic and progressive AOSD is the most frequent pattern, more prompt recognition and induction of therapy will allow a large number of these patients to achieve rapid and sustained disease control, bringing parity with the other disease patterns.[91]​ With all patterns of AOSD there is a high likelihood of rapid response to corticosteroids as well as biologic agents.[74] Nonetheless, observational studies report that many patients require medications for symptom control for months and years, highlighting the heterogeneity of the disease course and the need to take an individual approach with each patient.[5][91]​​​

Prognosis

Mortality data for AOSD is scarce and varies widely between studies.[5] One 5-year retrospective study of 5820 people hospitalized for ASOD in the US found an inpatient mortality rate of 2.6%.[12]​ The specific mortality rate has been estimated at 1% to 3%.[4]

  • Comprehensive analysis of risk factors, comorbidities, and the burden of disease in patients remains an unmet research need.

Evidence suggests that a delay in diagnosis of more than 6 months is associated with an increased likelihood of patients developing relapses or chronic articular patterns of disease.[5]

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