Adult Still's Disease with Sjoegren's Syndrome Successfully Treated with Intravenous Pulse Methylprednisolone and Oral Cyclophosphamide.

Sato, Masaki; Takeda, Atsushi; Honzu, Hiromi; Saku, Naohiko; Minato, Nagahiro; Kano, Shogo

doi:10.2169/internalmedicine.32.730

Cited by 8 publications

(3 citation statements)

References 14 publications

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“…To date, 40 cases of patients with elderly AOSD over 65 years of age, including our case, have been reported. [19–56] The clinical and laboratory characteristics of the 40 patients are shown in Table 1. Only 5 of 40 cases were reported between 1991 and 2000, 8 between 2001 and 2010, and the remaining 25 between 2011 and 2020 (Table 1).…”

Section: Discussionmentioning

confidence: 99%

Fulminant elderly adult-onset Still disease effectively treated with tocilizumab and methotrexate: A case report

et al. 2022

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Rationale: Adult-onset Still disease (AOSD) is a rare inflammatory disease of unknown etiology. AOSD is common in young or middle-aged adults; however, in recent years, there have been increasing reports of elderly AOSD. Differentiating AOSD from diseases such as infections and malignancies is difficult. Moreover, rare fulminant AOSD cases with resistance to corticosteroids and immunosuppressive drugs have been reported.Patient concerns: An 80-year-old woman presented with flaccid fever, generalized arthralgia, and erythema of the anterior chest for 2 weeks. On day 5 of hospitalization, the patient developed pleural effusion with hypoxemia and her vital signs indicated rapid progression to shock. During the clinical course, the levels of inflammatory markers, including maximum level of ferritin and white blood cells (WBCs) were elevated (252,796 ng/mL and 86,500/μV, respectively) with disseminated intravascular coagulation syndrome (DIC) and macrophage activation syndrome (MAS). Diagnosis:The patient was diagnosed with elderly AOSD as per the Yamaguchi criteria for AOSD. The state of disease was extremely severe with rapid progression and was, thus, categorized as a fulminant form of elderly AOSD. Interventions:The patient was treated with prednisolone (PSL) pulse therapy (1000 mg/d) twice and plasma exchange in the intensive care unit for the primary disease and shock. Although she recovered from shock, she developed DIC and MAS. Methotrexate (MTX; 10 mg/d) improved the DIC and MAS. However, severe pleuritis recurred and the patient developed pericarditis; her primary disease was poorly controlled. Finally, tocilizumab (TCZ) was introduced using interleukin-18 (IL-18) as a surrogate marker. The IL-18 level was measured repeatedly following admission, with the peak level (170,000 pg/mL) recorded on the 75th day of hospitalization, immediately prior to introducing TCZ.Outcomes: The combined use of MTX, TCZ, and PSL was effective in suppressing elderly AOSD, which was unsuccessfully controlled with MTX and PSL. Frequent monitoring of IL-18 levels proved useful for differentiating elderly AOSD from other diseases.Lessons: A fulminant form of elderly AOSD was treated with a combination of MTX, TCZ, and PSL. Repeated monitoring of IL-18 levels can be useful for decision-making in treating elderly AOSD.

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Section: Discussionmentioning

confidence: 99%

Fulminant elderly adult-onset Still disease effectively treated with tocilizumab and methotrexate: A case report

et al. 2022

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“…Other immunological abnormalities described in patients with AOSD include rheumatoid factor, anti-cyclic citrullinated peptide antibody, anti-SS-A/Ro antibodies, anti-dsDNA antibodies, and anti-neutrophil cytoplasmic antibodies ( 4 , 7 – 10 ). Although rare, there have been reports of an overlap of AOSD with other rheumatic diseases, such as Sjogren’s syndrome and systemic scleroderma ( 11 – 16 ). Although there have been reports of positive anti-dsDNA antibodies in patients with AOSD ( 8 ), there are no reports of AOSD cases with an overlap with SLE.…”

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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“…Rheumatoid factor, anti-cyclic citrullinated peptide antibody, anti-SS-A/Ro antibody, anti-dsDNA antibody, and anti-neutrophil cytoplasmic antibodies are among the other immunological abnormalities that have been reported in individuals with AOSD [6,15,16]. There have been instances of AOSD's overlap with other rheumatic disorders, including systemic sclerosis and Sjogren's syndrome [17][18][19].…”

Section: Minor Criteriamentioning

confidence: 99%

Chronic Adult-Onset Still’s Disease With Positive Antinuclear Antibodies: Navigating Diagnostic Dilemmas and Clinical Implications

Nagpure,

Raju,

Dube

et al. 2024

Cureus

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Adult-onset Still's disease (AOSD) is a rare systemic autoinflammatory disorder characterized by fever, rash, and joint pain. Despite primarily affecting young adults, it can occur at any age, presenting diagnostic challenges due to its heterogeneous nature and lack of specific laboratory findings. The subset of AOSD with positive antinuclear antibody (ANA) adds complexity, potentially overlapping with other autoimmune conditions. We describe a case of a 30-year-old female with a two-year history of fever, weight loss, and joint pain, initially misdiagnosed as seronegative arthritis with hypothyroidism. Further evaluation revealed severe anemia, leucocytosis, and hepatosplenomegaly. Despite a strongly positive ANA, the absence of systemic lupus erythematosus (SLE) features led to a diagnosis of chronic AOSD. Treatment with steroids and disease-modifying antirheumatic drugs (DMARDs) resulted in clinical improvement, highlighting the importance of accurate disease classification for tailored management in ANA-positive AOSD. This case underscores the diagnostic challenges of AOSD and emphasizes the need for precise classification for optimal treatment strategies.

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Adult Still's Disease with Sjoegren's Syndrome Successfully Treated with Intravenous Pulse Methylprednisolone and Oral Cyclophosphamide.

Cited by 8 publications

References 14 publications

Fulminant elderly adult-onset Still disease effectively treated with tocilizumab and methotrexate: A case report

Fulminant elderly adult-onset Still disease effectively treated with tocilizumab and methotrexate: A case report

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

Chronic Adult-Onset Still’s Disease With Positive Antinuclear Antibodies: Navigating Diagnostic Dilemmas and Clinical Implications

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