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

Mollaeian, Arash; Chen, Jingjing; Chan, N.; Nizialek, Gregory; Haas, Christopher

doi:10.1186/s41927-021-00183-6

Cited by 17 publications

(13 citation statements)

References 44 publications

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“…In a recent Dutch case series of VEXAS, among patients with unclassified autoinflammation, two among the twelve retrospectively identified were initially suspected with AOSD [ 28 ]. Thus, elderly patients with late AOSD diagnosis or suspicion as described in some series [ 29 , 30 ] could also benefit from UBA1 mutation screening.…”

Section: Discussionmentioning

confidence: 99%

USAID Associated with Myeloid Neoplasm and VEXAS Syndrome: Two Differential Diagnoses of Suspected Adult Onset Still’s Disease in Elderly Patients

Delplanque

Aouba

Hirsch

et al. 2021

JCM

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Background: Patients with solid cancers and hematopoietic malignancy can experience systemic symptoms compatible with adult-onset Still’s disease (AOSD). The newly described VEXAS, associated with somatic UBA1 mutations, exhibits an overlap of clinical and/or biological pictures with auto inflammatory signs and myelodysplastic syndrome (MDS). Objectives: To describe a cohort of patients with signs of undifferentiated systemic autoinflammatory disorder (USAID) concordant with AOSD and MDS/chronic myelomonocytic leukemia (CMML) and the prevalence of VEXAS proposed management and outcome. Methods: A French multicenter retrospective study from the MINHEMON study group also used for other published works with the support of multidisciplinary and complementary networks of physicians and a control group of 104 MDS/CMML. Results: Twenty-six patients were included with a median age at first signs of USAID of 70.5 years with male predominance (4:1). Five patients met the criteria for confirmed AOSD. The most frequent subtypes were MDS with a blast excess (31%) and MDS with multilineage dysplasia (18%). Seven patients presented with acute myeloid leukemia and twelve died during a median follow-up of 2.5 years. Six out of 18 tested patients displayed a somatic UBA1 mutation concordant with VEXAS, including one woman. High-dose corticosteroids led to a response in 13/16 cases and targeted biological therapy alone or in association in 10/12 patients (anakinra, tocilizumab, and infliximab). Azacytidine resulted in complete or partial response in systemic symptoms for 10/12 (83%) patients including 3 VEXAS. Conclusion: Systemic form of VEXAS syndrome can mimic AOSD. The suspicion of USAID or AOSD in older males with atypia should prompt an evaluation of underlying MDS and assessment of somatic UBA1 mutation.

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Section: Discussionmentioning

confidence: 99%

USAID Associated with Myeloid Neoplasm and VEXAS Syndrome: Two Differential Diagnoses of Suspected Adult Onset Still’s Disease in Elderly Patients

Delplanque

Aouba

Hirsch

et al. 2021

JCM

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“…1). Suda et al [3] and Mollaeian et al [42] reported 1 case of EOSD and reviewed, from the literature, 24 and 38 cases, respectively.…”

Section: Methodsmentioning

confidence: 99%

Elderly-onset adult Still’s disease

et al. 2021

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Adult-onset Still's disease is a rare inflammatory disorder usually affecting young adults. Elderly-onset Still's disease (EOSD) is reported in some cases, commonly in Japan, the USA and Europe. One of the most commonly used criteria for diagnosing EOSD is Yamaguci criteria. In elderly patients more severe course of the disease and more complications may be expected than in the younger group of patients with Still's disease. The lungs involvement is rather rare manifestation of this disease. In our article we discuss the problem of both the development of Still's disease in the elderly and interstitial lung changes in the course of the disease, based on available literature and own cases from one centre.

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“…The typical skin rash is an evanescent salmon-pink erythema, predominantly on the extremities, which is one of the diagnostic criteria. However, atypical skin rashes have been reported to be common in elderly-onset AOSD ( 29 , 30 ). The most frequent lesions of atypical skin rashes are persistent papules, plaques, or erythema ( 30 , 32 , 33 ).…”

Section: Discussionmentioning

confidence: 99%

Case Report: A Rare Case of Elderly-Onset Adult-Onset Still’s Disease in a Patient With Systemic Lupus Erythematosus

et al. 2022

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The rare systemic inflammatory disorder ‘adult-onset Still’s disease (AOSD)’ is characterized by recurrent fever, evanescent rash, arthralgia, and leukocytosis with neutrophilia. The Yamaguchi criteria are widely used to diagnose AOSD; these criteria can be used for diagnosis after a wide range of infectious, rheumatic, and neoplastic diseases have been excluded. AOSD generally does not overlap with other rheumatic diseases. We present the rare case of an 80-year-old Japanese woman who presented with arthralgia, fever, and skin rash during treatment for systemic lupus erythematosus (SLE), which was finally diagnosed as an overlap of AOSD. Blood tests revealed leukocytosis with neutrophilia, high C-reactive protein (CRP), and liver dysfunction. Her anti-ds-DNA antibody titer and serum complement titer were at the same level as before and remained stable. We suspected AOSD based on the high serum ferritin level but hesitated to diagnose AOSD because of the patient’s SLE history. We measured serum interleukin (IL)-18; it was extremely high at 161,221 pg/mL, which was strongly suggestive of AOSD. We thus diagnosed AOSD complicated during the course of treatment for SLE. The patient’s arthralgia and high CRP level persisted after we increased her oral prednisolone dose and added oral methotrexate, but her symptoms eventually improved with the addition of intravenous tocilizumab. We note that the presence of autoantibodies or other rheumatic diseases cannot be absolutely ruled out in the diagnosis of AOSD. Although high serum IL-18 levels are not specific for AOSD, the measurement of serum IL-18 may aid in the diagnosis of AOSD in similar rare cases.

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

Cited by 17 publications

References 44 publications

USAID Associated with Myeloid Neoplasm and VEXAS Syndrome: Two Differential Diagnoses of Suspected Adult Onset Still’s Disease in Elderly Patients

USAID Associated with Myeloid Neoplasm and VEXAS Syndrome: Two Differential Diagnoses of Suspected Adult Onset Still’s Disease in Elderly Patients

Elderly-onset adult Still’s disease

Case Report: A Rare Case of Elderly-Onset Adult-Onset Still’s Disease in a Patient With Systemic Lupus Erythematosus

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