Adult-Onset Still's Disease

46 papers & discussions

A rare form of inflammatory arthritis characterised by spiking fevers, rash, and joint pain.

✦ AI summary

Adult-Onset Still's Disease (AOSD) is described as a rare inflammatory condition marked by high daily fevers, joint pain or arthritis, rash, sore throat, and very high ferritin levels in the blood, with diagnosis often difficult because doctors first need to rule out infections, other autoimmune diseases, and cancers [1][4]. Case reports describe a range of complications, including a perforation in the nasal septum [4], a type of eye damage called Purtscher-like retinopathy that can threaten vision [7], and a serious complication called macrophage activation syndrome (or hemophagocytic syndrome), where the immune system becomes overactive and can be life-threatening, sometimes occurring alongside sepsis in frail patients [6][12]. AOSD has also come up as a possible cause of "fever of unknown origin" in broader studies from Colombia and elsewhere, alongside conditions like tuberculosis, lymphoma, and lupus [9][3]. One case report notes AOSD occurring in a patient later found to have a serious bacterial infection (Rhodococcus equi) after long-term immune-suppressing treatment [11], and another set of sources notes that suspected AOSD together with low antib

This is an AI-generated summary of the sources below — not medical advice. Always talk to a doctor or pharmacist about your own situation.

Daily fever spikesSalmon-coloured rashJoint painSore throatFatigueLymphadenopathy

Research

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PubMed

Adult-onset Still's Disease Presenting with Prolonged Fever, Polyarthralgia, and Hyperferritinemia: A Case Report.

Adult-onset still's disease (AOSD) is a rare systemic inflammatory disorder characterized by quotidian fever, inflammatory arthritis, rash, and markedly elevated serum ferritin levels. Due to its nonspecific presentation and the need to exclude infectious, autoimmune, and malignant etiologies, diagn…

raadult stills
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PubMed

Association of elevated serum active IL-18 levels with cytokine profiles and clinical features in adult-onset still's disease.

Inflammasome-mediated activation of interleukin (IL)-1β and IL-18 plays a key role in the pathogenesis of adult-onset Still's disease (AOSD), a systemic autoinflammatory disorder. The cleaved free active form of IL-18 may more accurately reflect inflammasome activity than total IL-18, which includes…

raadult stillsperiodic fever
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PubMed

Clinical, genetic, and functional characterization of novel NFKB1 variants in Chinese patients with primary immunodeficiency.

The nuclear factor κB (NF-κB)-related disorders encompassed not only common variable immunodeficiency but also manifestations of autoinflammation, autoimmunity, and malignancies. While most cases have been reported in European populations, reports in the Chinese population are sparse. Clinical data…

adult stillsprimary immunodeficiency
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PubMed

A Rare Differential for Myalgia and Fever Associated With Cervical and Axillary Lymphadenopathy Presenting via Same Day Emergency Care.

Kikuchi-Fujimoto disease (KFD) is a rare, self-limiting necrotising lymphadenitis, mainly affecting young adults, and commonly presenting with fevers and lymphadenopathy. Diagnosis is confirmed by histopathological examination via lymph node biopsy, and management is primarily supportive with non-st…

lupussarcoidosisthoracic outlet syndromehereditary angioedema
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PubMed

Nasal septal perforation in adult Still's disease.

Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disorder characterized by fever, rash, arthritis, and pharyngitis. Ear, nose, and throat (ENT) complications beyond pharyngitis are uncommon. A 31-year-old female presented with fever, sore throat, maculopapular rash, and arthritis.…

thoracic outlet syndromeadult stills
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PubMed

Contribution to the debate on the origin of autoimmune joint diseases in Europe through an archaeological case of still's disease.

The objective of this study is twofold: (i) to evaluate the bony changes of an early medieval skeleton (UF2) from Barcelona, which presents lesions suggestive of Still's disease and (ii) to contribute to the debate concerning the existence of autoimmune joint diseases in Europe prior to Columbus's v…

hereditary angioedemaadult stills
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PubMed

[A rare differential diagnosis of fever of unknown origin].

A 22-year-old male patient with a clinical picture similar to that of sepsis was diagnosed with macrophage activation syndrome in adult Still's disease on the basis of clinical and laboratory criteria. The diagnostic work-up included the differentiated clarification of a persistent fever syndrome an…

thoracic outlet syndromeadult stills
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PubMed

Purtscher-like retinopathy as the manifestation of adult Still's disease.

Adult Still's disease (ASD) is a rare systemic inflammatory disorder for which ocular manifestations have rarely been described. We report a case of 38-year-old Brazilian woman with Purtscher-like retinopathy as a manifestation of ASD. She was diagnosed with Purtscher-like retinopathy based on fundo…

adult stillsvasculitis
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