Adult-onset Still's Disease with Acute Kidney Injury Requiring Hemodialysis: A Case Report and Literature Review

Saito, Kenji; Temmoku, Jumpei; Sumichika, Yuya; Yoshida, Shuhei; Takano, Eisuke; Watanabe, Shuhei; Matsumoto, Haruki; Fujita, Yuya; Matsuoka, Naoki; Asano, Tomohiko; Sato, Shuzo; Watanabe, Hiroshi; Migita, Kiyoshi

doi:10.2169/internalmedicine.1026-22

Cited by 3 publications

(3 citation statements)

References 28 publications

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“…This is a rare systemic autoinflammatory disorder characterized by recurrent high fever, a fading salmon-pink rash, and arthritis. These signs/symptoms are often associated with pharyngodynia, myalgias, lymphadenopathy, splenomegaly, and neutrophilic leukocytosis (Table S5) [17,[20][21][22][23][24][25][26][27][28][29][30][31].…”

Section: Discussionmentioning

confidence: 99%

Unveiling the Mystery of Adult-Onset Still’s Disease: A Compelling Case Report

Sola,

Smirne,

Bruggi

et al. 2024

Life

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Adult-onset Still’s disease (AOSD) is a rare systemic inflammatory disorder. Diagnosis can take a long time, especially in the presence of confounding factors, and it is, to some extent, a process of exclusion. AOSD has life-threating complications ranging from asymptomatic to severe, such as macrophage activation syndrome (MAS), which is also referred to as hemophagocytic lymphohistocytosis (HLH). This condition is correlated with cytokine storm production and monocyte/macrophage overactivation and typically occurs with rash, pyrexia, pancytopenia, hepatosplenomegaly and systemic involvement. Exitus occurs in approximately 10% of cases. For the treatment of MAS-HLH, the Histiocyte Society currently suggests high-dose corticosteroids, with the possible addition of cyclosporine A, anti-interleukin (IL)-1, or IL-6 biological drugs; the inclusion of etoposide is recommended for the most severe conditions. In all cases, a multidisciplinary collaboration involving the resources and expertise of several specialists (e.g., rheumatologist, infectiologist, critical care medicine specialist) is advised. Herein, we provide a detailed description of the clinical case of a previously healthy young woman in which MAS developed as a dramatic onset manifestation of AOSD and whose diagnosis posed a real clinical challenge; the condition was finally resolved by applying the HLH-94 protocol (i.e., etoposide in combination with dexamethasone).

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Section: Discussionmentioning

confidence: 99%

Unveiling the Mystery of Adult-Onset Still’s Disease: A Compelling Case Report

Sola,

Smirne,

Bruggi

et al. 2024

Life

View full text Add to dashboard Cite

show abstract

“…This is a rare systemic autoinflammatory disorder characterized by recurrent high fever, a fading salmon-pink rash, and arthritis. These signs/symptoms are often associated with pharyngodynia, myalgias, lymphadenopathy, splenomegaly, and neutrophilic leukocytosis (Table 5) [14][15][16][17][18][19][20][21][22][23][24][25][26].…”

Section: Discussionmentioning

confidence: 99%

“…Gastrointestinal symptoms [19,23] The presence of abdominal pain is highly variable in AOSD, generally in relation to the onset of fever; however, there are reports of pancreatitis and aseptic peritonitis. Others [19,[24][25][26] Rare manifestations include: conjunctivitis, uveitis, aseptic meningitis, interstitial nephritis, glomerulonephritis, and secondary amyloidosis.…”

Section: Table 5 Characteristics Of the Clinical Manifestations Of Ad...mentioning

confidence: 99%

Unveiling the Mystery of Adult-Onset Still's Disease: a Compelling Case Report

Sola†,

Smirne,

Bruggi

et al. 2024

Preprint

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Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disorder. Diagnosis can take a long time, especially in the presence of confounding factors, and it is, at least in part, of exclusion. AOSD is generally mild, however a small percentage of patients can develop a fearsome as well potentially fatal complication, the macrophage activation syndrome (MAS), which is also referred to as hemophagocytic lymphohistocytosis (HLH). This condition is correlated with a cytokine storm production and monocyte/macrophage overactivation, and typically occurs with rash, pyrexia, pancytopenia, hepatosplenomegaly and systemic involvement. Exitus occurs in approximately 10% of cases. The Histiocyte Society, which is a nonprofit organization committed to improving the lives of patients with histiocytic disorders, currently suggests etoposide in combination with dexamethasone for the treatment of HLH/MAS, although a multidisciplinary collaboration using the resources and expertise of several specialists (e.g. rheumatologist, infectiologist, critical care medicine specialist) is always recommended. Hereby we propose the detailed description of the clinical case of a previously healthy young woman in which MAS was the dramatic onset manifestation of AOSD and whose diagnosis posed a real clinical challenge.

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