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. 2021 Apr 20;5(1):12.
doi: 10.1186/s41927-021-00183-6.

Adult onset Still's disease in the elderly: a case-based literature review

Arash Mollaeian  1 , Jingjing Chen  2 , Nina N Chan  2 , Gregory A Nizialek  2   3 , Christopher J Haas  2   3
Affiliations

Adult onset Still's disease in the elderly: a case-based literature review

Arash Mollaeian et al. BMC Rheumatol. .

Erratum in

Abstract

Background: Adult onset Still's disease (AOSD) is a rare inflammatory disorder that classically presents with high spiking fevers, evanescent rash, and arthritis. The diagnosis is one of exclusion and can be further complicated by atypical presentations, particularly in elderly patients in whom AOSD is very rare.

Case presentation: A case of AOSD in a 73-year-old woman with a non-classic presentation, leading to delayed diagnosis and management, is presented along with a review of the English literature for AOSD cases in elderly people over 70 years of age. Thirty nine case reports and series were identified and the current case was added, totaling 42 individual cases. Significant findings included a four-times higher prevalence in females, a higher prevalence of macrophage activation syndrome despite lower mortality, the presence of pruritic rash in almost one fifth of the cases, and high prevalence of delayed diagnosis.

Conclusions: AOSD in the elderly may vary from the classic criteria described in the medical literature and may lead to delayed diagnosis and management. Further evaluation and better characterization of AOSD in the elderly remains an area of interest.

Keywords: Adult onset Still’s disease; Arthritis; Autoinflammatory disorders; Case report; Elderly; Hyperferritinemia.

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Conflict of interest statement

Authors decline any conflicts of interest.

Figures

Fig. 1
Fig. 1
Computed tomography scan of cervical and lumbar spines. a-b Diffuse degenerative disc disease and spondylosis significantly at C5-C6 and C6-C7. Anterior offset of C4 on C5. c-d Degenerative disc disease and diffuse spondylotic changes at L4-L5, L5-S1 levels in lumbar spine along with disc space narrowing at all levels. Grade 1 anterolisthesis of L4 on L5
Fig. 2
Fig. 2
Magnetic resonance imaging of the cervical and lumbar spines. a-b T1 and T2 images of cervical spine showing endplate edema at C6-C7 level. Chronic degenerative changes without cord compression. c-d T1 and T2 images of cervical spine showing type I Modic change and enhancement along the endplates involving L2-L3 through L4–5, slightly increased at the L4-L5. Fluid signal intensity within the L3-L4 and L5-S1 disc levels with lesser involvement of L4-L5, L1-L2, and L2–3
Fig. 3
Fig. 3
a Temperature and white blood cell trend during 1st and 2nd admissions and their associations with steroids administration. b Ferritin and CRP trend throughout the disease course and their association with different therapies
Fig. 4
Fig. 4
Computed tomography scan of chest. Multiple mediastinal lymph nodes are present with the largest one measuring 1.75 × 1.0 cm
Fig. 5
Fig. 5
Pruritic confluent macular rash involving the chest and back
Fig. 6
Fig. 6
Literature review flow chart
See this image and copyright information in PMC

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