Adult Still's disease despite the presence of positive antinuclear antibodies

Awad, Jamal; Farah, Raymond; Horn, Ivona

doi:10.1016/j.ejim.2006.07.025

Cited by 5 publications

(5 citation statements)

References 5 publications

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“…As in the Yamaguchi criteria, autoantibodies are generally negative in AOSD. However, there have been several reports of autoantibody-positive AOSD cases ( 3 – 5 ). Zeng et al.…”

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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“…As in the Yamaguchi criteria, autoantibodies are generally negative in AOSD. However, there have been several reports of autoantibody-positive AOSD cases ( 3 – 5 ). Zeng et al.…”

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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“…ANA in this patient was positive, with a titer of 1/80 without any feature or other antibodies for SLE. It is very rare to have positive ANA with ASOD, and the diagnosis of ASOD should not be excluded based on the rheumatoid factor (RF) or ANA [ 9 - 10 ].…”

Section: Discussionmentioning

confidence: 99%

A Case of Adult-Onset Still's Disease with Positive Antinuclear Antibodies

Lakshman

Athwal

Gondi

et al. 2020

Cureus

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Adult-onset Still's disease (AOSD) is a rare inflammatory disorder involving multiple systems. It can present a wide range of symptoms like maculopapular rash, fever, and arthralgia, which may overlap with many other disorders, making it difficult to diagnose. Unknown etiology and no diagnostic tests further make it complex to establish the diagnosis of AOSD. We report the case of a 30-year-old female who presented with persistent rash, joint pain, and fever, along with positive antinuclear antibodies (ANA), diagnosed with this condition. The patient improved with corticosteroids and the plan is to start disease-modifying antirheumatic drugs (DMARDs) after tapering off steroids.

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“…However, unlike many other autoimmune disorders, AOSD is an auto-inflammatory disease characterized by dysregulation of innate immune system and over expression of chemokines such as IL-1, IL-6 and IL-17 [11]. It is not associated with autoantibody production, although rare cases of AOSD with positive ANA [12] and positive anti-golgi antibodies [13] have been reported. Dysregulated innate system and over-expressed chemokines can activate memory B cells previously sensitized against melioidosis to produce antibodies.…”

Section: Discussionmentioning

confidence: 99%

Positive melioidosis serology in a patient with adult onset Still’s disease: a case report of a diagnostic dilemma

et al. 2018

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Background Autoimmune disorders are known to produce false positives in serological tests for infections. Aetiological association between infections and autoimmunity, increased susceptibility to infectious and autoimmune disorders with immune dysregulation and non-specific polyclonal expansion of B cells with autoimmunity may cause confusion in diagnosis and patient management. We report a patient with Adult Onset Still’s Disease (AOSD) presenting with rising melioidosis antibody titres that caused diagnostic confusion. Case presentation A forty-nine-year-old female presented with prolonged fever, sore-throat, large joint arthritis, lymphadenopathy, hepatomegaly and transient rash. She had elevated inflammatory markers and a rising melioidosis antibody titre. The patient responded poorly to prolonged course of appropriate antimicrobials but showed rapid and sustained improvement with glucocorticoids. Conclusion Positive melioidosis serology could have been due to a co-infection or false positive antibody reaction due to non-specific B cell expansion or an indicator of true infection that triggered the immune dysregulation to develop AOSD.

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Adult Still's disease despite the presence of positive antinuclear antibodies

Cited by 5 publications

References 5 publications

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

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

A Case of Adult-Onset Still's Disease with Positive Antinuclear Antibodies

Positive melioidosis serology in a patient with adult onset Still’s disease: a case report of a diagnostic dilemma

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