Doença multissistêmica inflamatória rara caracterizada clinicamente por febre de origem desconhecida, artralgia ou artrite, hiperleucocitose e erupção cutânea típica.
Introdução
O que você precisa saber de cara
Doença multissistêmica inflamatória rara caracterizada clinicamente por febre de origem desconhecida, artralgia ou artrite, hiperleucocitose e erupção cutânea típica.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
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+ 26 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 59 características clínicas mais associadas, ordenadas por frequência.
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Os sinais que médicos procuram e os exames que confirmam
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Outros ensaios clínicos
24 ensaios clínicos encontrados, 8 ativos.
Publicações mais relevantes
Case Report: Flagellate eruption of adult-onset still disease in an Afro-Caribbean woman: highlighting atypical cutaneous disease in richly pigmented skin.
Adult-onset Still disease (AOSD) is an uncommon autoinflammatory disorder characterized by high spiking fevers, arthralgia, and, classically a transient, salmon-colored rash. Atypical cutaneous variants, such as persistent erythematous papules and plaques often arranged in a flagellate pattern, are increasingly reported; however, few cases illustrate this morphology in individuals with richly pigmented skin. We report a 70-year-old Afro-Caribbean woman (Fitzpatrick skin type VI) with persistent high-grade fevers, polyarthritis and diarrhea of unknown origin which persisted despite multiple courses of antibiotics. Laboratory evaluation revealed leukocytosis and transaminitis. Dermatology was consulted for a cutaneous eruption present for three weeks prior to hospitalization. Examination revealed subtle red-brown papules, plaques and patches on the trunk and extremities, some in a flagellate arrangement. Skin biopsy demonstrated hyperkeratosis, individually necrotic keratinocytes in the upper epidermis and a perivascular neutrophilic infiltrate characteristic of the atypical eruption of AOSD. Further blood work confirmed marked hyperferritinemia. Treatment with systemic and topical corticosteroids resulted in improvement. This case underscores the importance of recognizing atypical cutaneous variants of AOSD in persons with richly pigmented skin to prevent diagnostic delay and improve clinical outcomes.
A multifaceted interplay between hemophagocytosis, IL-18 and type I IFN distinguishes Still disease from other autoinflammatory diseases.
The unknown pathophysiology and the lack of specific features for systemic Juvenile Idiopathic arthritis and adult-onset Still disease (collectively Still disease/SD) delays diagnosis and appropriate treatment. The goal of this study was to identify features and mechanisms that distinguish SD from other systemic autoinflammatory diseases (SAID). We determined by SomaScan assay the plasma proteomes and by RNAseq the immune cell miRNA and RNA transcriptomes of 372 SAID patients. Proteomic findings were validated by ELISA assays. SD (n=72) and non-SD SAIDs (n=300) were compared to identified distinguishing features of SD. We performed integrated and unbiased analysis of all datasets using Weighted Gene Correlation Network Analysis (WGCNA) to identify feature modules that characterize SD and stratify patients. Elevated plasma heme oxygenase 1 (HO-1) and IL-18 strongly correlate and characterize SD, but do not associate with general inflammation. SD was characterized by ferroptosis in plasma, type I IFN signaling in monocyte transcriptomes and elevated NK cell miRNA-146a-5p, an IL-18 induced miRNA. Finally, we identified feature modules that distinguish SD from other SAIDs and stratify SD patients into two distinct subgroups not attributable to disease activity or inflammation, but hemophagocytosis. This unprecedented large omics dataset of SAIDs, revealed that complex interactions between hemophagocytosis, IL-18 and type I IFN signaling characterize SD. Furthermore, two distinct subgroups in SD patients are distinguished by the degree of hemophagocytic activity. Finally, the large proteomics and RNAseq datasets generated in this study can serve as an invaluable resource for the further investigation SD and other SAIDs.
New-Onset and Flare Episodes of Adult-Onset Still's Disease Following COVID-19 Vaccination: A Systematic Review of Published Case Reports.
This systematic review provides a descriptive synthesis of published case reports documenting new-onset or flare episodes of adult-onset Still's disease (AOSD) temporally occurring after COVID-19 vaccination. A comprehensive search of PubMed, Scopus, Web of Science, and Google Scholar identified 13 eligible case reports published between 2020 and 2024. Because all available evidence consisted solely of individual case descriptions without comparator groups, the review followed PRISMA 2020 guidelines and employed qualitative narrative synthesis rather than meta-analysis. Across the included cases, patients consistently presented with hallmark features of AOSD, including high spiking fever, arthritis or arthralgia, markedly elevated ferritin levels, and, in several instances, the characteristic salmon-colored rash. Symptom onset typically occurred within four to fifteen days following vaccination. Although these cases demonstrate recognisable clinical patterns, the absence of denominator data, lack of population-based studies, and inherent publication bias prevent estimation of incidence or risk, and no causal relationship with vaccination can be inferred. All reported patients responded favorably to corticosteroids, with some requiring biologic therapy for disease control. These findings highlight the importance of clinician awareness when evaluating persistent febrile or inflammatory symptoms in recently vaccinated individuals, while emphasising that COVID-19 vaccination remains overwhelmingly safe. Larger registries, pharmacovigilance data, and controlled studies are needed to clarify potential risk factors and guide future revaccination decisions.
Trends in fatigue in inflammatory rheumatic diseases: Annual data and trajectory analysis of the German National Database 2007-2023.
To explore the development of fatigue over the past 17 years and its relationship to physician- and patient-reported outcomes and social factors in inflammatory rheumatic diseases. Data from ≈9300 patients per year from the German National Database (2007-2023) were included, considering arthritides, spondyloarthritides, connective tissue diseases and vasculitides. Fatigue was assessed on a numeric rating scale (0-10) with >2 defined as present and >6 as severe. Presence and severity were compared by diagnosis, gender and year. Fatigue clusters were identified based on trajectory patterns over three consecutive visits. Fatigue affected 55 % (adult onset Still disease) to 67 % (systemic sclerosis) of patients, with severe fatigue in up to 26 % (systemic sclerosis). Substantial proportions of women (47-61 %) and men (35-52 %) experienced moderate-to-severe fatigue. Despite marked improvements in inflammation-responsive outcomes (CRP -40 %, tender joints -50 %, physician disease activity -42 %) and employment (52 %→70 %), mean fatigue remained stable. Trajectory analysis identified 35 % with persistent low, 23 % persistent high, 24 % worsening, and 19 % improving fatigue. Tender joints and morning stiffness effectively discriminated between persistent high versus low fatigue clusters. Emotional well-being, physical functioning, coping, and sleep quality showed stronger associations with fatigue trajectories than inflammatory markers. Differences across fatigue clusters substantially exceeded those between diagnostic groups. Fatigue affected a large proportion of both women and men across diagnoses. Fatigue trajectories reflect complex interplay of clinical and psychosocial factors. Management should incorporate multidimensional interventions addressing emotional well-being, physical function and social support beyond traditional inflammatory control.
Examination of HLA-DRB1*15-linked Candidate Antigens in Still Disease With and Without Lung Disease and Drug-Associated Immune Reactions.
Lung disease (LD) in systemic juvenile idiopathic arthritis and adult-onset Still disease (Still-LD) is a severe manifestation strongly associated with HLA-DRB1*15 alleles and drug-associated immune reactions (DAIR), including eosinophilia, non-Still disease rashes, and elevated liver function tests. Despite the high morbidity and mortality of these phenomena, pathogenesis remains poorly understood. This study investigates whether Still-LD and DAIR are associated with pathogenic antigens through hypersensitivity reactions to Aspergillus fumigatus, for which HLA-DRB1*15 is a known risk allele, or drug reaction with eosinophilia and systemic symptoms (DRESS), which is frequently associated with human herpesvirus (HHV) reactivation. Pediatric and adult subjects were drawn from the National Institutes of Health Still disease cohort. Subjects with Still-LD and/or DAIR were identified by chart review. Serum samples were analyzed for anti-A. fumigatus IgE using ImmunoCap assay, and Epstein-Barr virus (EBV), cytomegalovirus (CMV), and HHV 6 (HHV-6) antibodies were analyzed by luciferase immunoprecipitation systems. Subjects were screened for EBV, CMV, and HHV-6 by nucleic acid amplification tests and/or Viral Transcript Usage Sensor (VIRTUS) analysis of whole-blood RNA sequencing data. Fifty-four subjects were included in the study, 11 had LD and DAIR, and 8 had DAIR alone. Thirty-three subjects were tested for anti-A. fumigatus antibodies and all were negative. Forty-nine subjects were tested for CMV, EBV, and HHV-6; 2 were positive for EBV, both of whom did not have Still-LD or DAIR. The absence of anti-A. fumigatus IgE antibodies and detectable herpesvirus nucleic acids in subjects with Still-LD and DAIR does not support a mechanistic association with hypersensitivity to A. fumigatus or with HHV reactivation.
Publicações recentes
Case Report: Flagellate eruption of adult-onset still disease in an Afro-Caribbean woman: highlighting atypical cutaneous disease in richly pigmented skin.
🥈 ObservacionalA Multifaceted Interplay Among Hemophagocytosis, Interleukin-18, and Type I Interferon Distinguishes Still Disease From Other Autoinflammatory Diseases.
🥇 Meta-análiseA Case of Refractory Adult-Onset Still Disease With Macrophage Activation Syndrome and Pulmonary Alveolar Proteinosis.
📖 RevisãoNew-Onset and Flare Episodes of Adult-Onset Still's Disease Following COVID-19 Vaccination: A Systematic Review of Published Case Reports.
Trends in fatigue in inflammatory rheumatic diseases: Annual data and trajectory analysis of the German National Database 2007-2023.
📚 EuropePMC161 artigos no totalmostrando 177
Case Report: Flagellate eruption of adult-onset still disease in an Afro-Caribbean woman: highlighting atypical cutaneous disease in richly pigmented skin.
Frontiers in immunologyA multifaceted interplay between hemophagocytosis, IL-18 and type I IFN distinguishes Still disease from other autoinflammatory diseases.
Arthritis & rheumatology (Hoboken, N.J.)A Case of Refractory Adult-Onset Still Disease With Macrophage Activation Syndrome and Pulmonary Alveolar Proteinosis.
The Journal of rheumatologyNew-Onset and Flare Episodes of Adult-Onset Still's Disease Following COVID-19 Vaccination: A Systematic Review of Published Case Reports.
CureusTrends in fatigue in inflammatory rheumatic diseases: Annual data and trajectory analysis of the German National Database 2007-2023.
Seminars in arthritis and rheumatismExamination of HLA-DRB1*15-linked Candidate Antigens in Still Disease With and Without Lung Disease and Drug-Associated Immune Reactions.
The Journal of rheumatologyDermatomyositis-Like Lesion: An Atypical Cutaneous Presentation in Adult-Onset Still Disease.
Dermatology practical & conceptualBrexpiprazole for Managing Mania After Methylprednisolone Pulse Therapy in a Patient With Adult-Onset Still Disease.
American journal of therapeuticsPruritic eruption in a young woman with fever.
Skin health and diseaseClinical Features and Outcome Measures Across Still Disease (Systemic Juvenile Idiopathic Arthritis and Adult-Onset Still Disease) Cohorts Worldwide: A Systematic Literature Review.
The Journal of rheumatologyChallenges on diagnosis and treatment of refractory adult-onset still disease in Sub-Saharan Africa: a case report.
Journal of medical case reportsCarpal tunnel syndrome with both AA amyloidosis and elderly-onset Still disease: A case report.
MedicineLow U-neck distribution of persistent pruritic eruptions in adult-onset Still disease: A retrospective cohort study with 65 patients.
Journal of the American Academy of DermatologyDiagnosis and outcomes of fever of unknown origin cases with an erythrocyte sedimentation rate of 100 mm/h or more: An International ID-IRI (Infectious Diseases - International Research Initiative) Observational Retrospective Cohort Study.
MedicineNasal septal perforation in adult Still's disease.
Joint bone spineMacrophage activation syndrome-associated adult onset Still disease treatment: a scoping review of case reports and case series.
Proceedings (Baylor University. Medical Center)TREATMENT OF HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS IN THE INTENSIVE CARE UNIT: TOO LATE AFTER RESORTING TO INVASIVE ORGAN SUPPORTS?
Shock (Augusta, Ga.)Ruxolitinib targets JAK-STAT signaling to modulate neutrophil activation in refractory macrophage activation syndrome.
BloodEndogenous Lipoid Pneumonia in Adult Autoinflammatory Disease.
Arthritis care & researchLong-Term Health Consequences of SARS-CoV-2- Trigger for Still Disease in Adults.
ACR open rheumatologyAchievement and Usefulness of Intermediate Treatment Targets for Still Disease Proposed by the European Alliance of Associations for Rheumatology and the Paediatric Rheumatology European Society.
The Journal of rheumatologyEvaluation of performance of the Still Activity Score for assessment of Adult-onset Still's Disease: Comparative study with Systemic Feature Score and Modified Pouchot-Activity Score.
Reumatologia clinicaAdult-onset Still disease.
CMAJ : Canadian Medical Association journal = journal de l'Association medicale canadienneRole of Positron Emission Tomography-Computed Tomography Scan in Reaching Definite Diagnosis in Patients With Fever of Unknown Origin and Inflammation of Unknown Origin in Rheumatology Outpatient Clinic.
Open access rheumatology : research and reviewsAccuracy and Performance Characteristics of Administrative Codes for the Diagnosis of Autoinflammatory Syndromes: A Discovery and Validation Study.
Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseasesNon-Classical Complications of Adult-Onset Still's Disease: A Multicenter Spanish Study.
Journal of clinical medicineAcquired autoinflammatory disorders: a dermatologist's perspective.
Clinical and experimental dermatologyOverlap of Adult-Onset Still Disease and Kikuchi-Fujimoto Disease: A Case Report and Literature Review.
CureusEvaluation of Myocarditis in Patients With Still Disease: Clinical Findings From the Multicenter International AIDA Network Still Disease Registry.
The Journal of rheumatologyCharacterization of Genetic Landscape and Novel Inflammatory Biomarkers in Patients With Adult-Onset Still's Disease.
Arthritis & rheumatology (Hoboken, N.J.)Mitochondrial DNA copy number and the risk of autoimmune diseases: A Mendelian randomization study with meta-analysis.
Journal of translational autoimmunityAdult-Onset Still's Disease and Pulmonary Embolism: A Case Report.
CureusIndices and ferritin level that predict organ involvement in adult-onset Still's disease.
Biomarkers in medicineCase Report: Adult Onset Still Disease in a 26-year-old Male.
South Dakota medicine : the journal of the South Dakota State Medical AssociationEmapalumab Treatment in Patients With Rheumatologic Disease-Associated Hemophagocytic Lymphohistiocytosis in the United States: A Retrospective Medical Chart Review Study.
Arthritis & rheumatology (Hoboken, N.J.)Criteria for Cytokine Storm Syndromes.
Advances in experimental medicine and biologyThe History of Macrophage Activation Syndrome in Autoimmune Diseases.
Advances in experimental medicine and biologyAssociation between Relative Bradycardia and Adult-onset Still Disease.
JMA journalA case of adult-onset Still disease with eyelid xanthogranuloma mimicking Erdheim-Chester disease.
Clinical and experimental dermatologyNationwide Analysis of Adult-Onset Still Disease With and Without Hemophagocytic Lymphohistiocytosis.
Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseasesThree Case Reports of Adult-onset Still Disease Associated with Breast Implantation.
Plastic and reconstructive surgery. Global openThe Systemic Score May Identify Life-Threatening Evolution in Still Disease: Data from the GIRRCS AOSD-Study Group and the AIDA Network Still Disease Registry.
Arthritis & rheumatology (Hoboken, N.J.)Systematic Review and Metaanalysis of Pharmacological Interventions in Adult-Onset Still Disease and the Role of Biologic Disease-Modifying Antirheumatic Drugs.
The Journal of rheumatologyMultidisciplinary approach to diagnosis and management of fever of unknown origin: A case report.
MedicineTreatment of systemic juvenile idiopathic arthritis.
Nature reviews. RheumatologyNeutrophilic urticarial dermatosis preceding adult-onset Still's disease.
Dermatology online journalAutoimmune and Autoinflammatory Connective Tissue Disorders Following COVID-19.
JAMA network openCigarette smoking and risk of adult-onset Still disease: a propensity score matching analysis.
Internal medicine journalIL-18 in Autoinflammatory Diseases: Focus on Adult Onset Still Disease and Macrophages Activation Syndrome.
International journal of molecular sciencesTocilizumab-induced mucosal injury in the terminal ileum mimicking intestinal Behçet's disease: A case report.
MedicineHepatic Superscan in Methotrexate-Associated Lymphoproliferative Disorder.
Clinical nuclear medicinePersistent, Pruritic, Reddish-Brown Papules and Plaques: An Atypical Presentation of Adult-Onset Still's Disease.
CureusExtremely high erythrocyte sedimentation rate revisited in rheumatic diseases: a singlecenter experience.
Turkish journal of medical sciencesDermatologic Manifestations of Noninflammasome-Mediated Autoinflammatory Diseases.
JID innovations : skin science from molecules to population healthBrain abscess due to Nocardia asiatica in a 63-year-old patient with adult-onset still disease: A case report.
Clinical case reportsFerritin-induced NETs lead to cytokine storm in AOSD.
Nature reviews. RheumatologyAdult-Onset Still's Disease-A Complex Disease, a Challenging Treatment.
International journal of molecular sciencesCOVID-19 in a patient with new adult-onset Still disease: A case report.
MedicineEvanescent, episodic salmon-colored macules in a young woman.
JAAD case reportsActivation mechanisms of monocytes/macrophages in adult-onset Still disease.
Frontiers in immunologyDiagnostic utility of clinical characteristics, laboratory tests, and serum ferritin in diagnosis of adult-onset Still disease.
MedicineIdiopathic multicentric Castleman disease and associated autoimmune and autoinflammatory conditions: practical guidance for diagnosis.
Rheumatology (Oxford, England)Development of Pulmonary Alveolar Proteinosis in a Patient With Adult-Onset Still Disease Treated With Tocilizumab.
The Journal of rheumatologyAdult-Onset Still Disease After ChAdOx1 nCOV-19 Vaccination.
The Journal of rheumatologyValidation of the neutrophil-to-lymphocyte ratio as a new simple biomarker of adult onset Still's disease: A STROBE-Compliant prospective observational study.
MedicineGlucose metabolism in systemic juvenile idiopathic arthritis.
Pediatric rheumatology online journalFulminant elderly adult-onset Still disease effectively treated with tocilizumab and methotrexate: A case report.
MedicineAssessment of health-related quality of life in patients with adult onset Still disease: Results from a multicentre cross-sectional study.
MedicineBibliometrics and Visual Analysis of Adult-onset Still Disease (1976-2020).
Frontiers in public healthStill disease with persistent atypical dermatomyositis-like skin eruption: two cases associated with macrophage activation syndrome.
Clinical and experimental dermatologyCOVID-19 infection among patients with autoinflammatory diseases: a study on 117 French patients compared with 1545 from the French RMD COVID-19 cohort: COVIMAI - the French cohort study of SARS-CoV-2 infection in patient with systemic autoinflammatory diseases.
RMD openHeliotrope-like manifestation of adult-onset Still disease with macrophage activation syndrome: A case-based review.
The Journal of dermatologyAdult-onset Still disease and adenovirus vector COVID-19 vaccination.
Internal medicine journalPrognostic factors for adult-onset Still disease: a retrospective analysis of 90 patients in China.
European journal of dermatology : EJDBibliometric analysis of the global literature productivity of adult-onset Still's disease (2011-2020).
Rheumatology internationalAdult-onset Still disease post-adenovirus vector COVID-19 vaccine.
Internal medicine journalErythroderma and persistent erythema in adult-onset Still disease associated with macrophage activation syndrome.
JAAD case reportsDermato-Pathologic Clues To Diagnosis Of Adult Onset Still Disease: A Case Report.
Indian dermatology online journalFever and flagellate dermatosis in an otherwise healthy woman.
Clinical and experimental dermatologyClinical manifestations and treatment outcomes in prurigo pigmentosa (Nagashima disease): A systematic review of the literature.
JAAD internationalEfficacy of tight control strategy in the treatment of adult-onset Still disease.
Clinical rheumatology[Adult-onset Still's disease and the neutrophil-lymphocyte index in the Mexican population. A case series report].
Revista alergia Mexico (Tecamachalco, Puebla, Mexico : 1993)Immune cartography of macrophage activation syndrome in the COVID-19 era.
Nature reviews. RheumatologyThe JANUS of chronic inflammatory and autoimmune diseases onset during COVID-19 - A systematic review of the literature.
Journal of autoimmunityHemophagocytic Lymphohistiocytosis Hospitalizations in Adults and Its Association With Rheumatologic Diseases: Data From Nationwide Inpatient Sample.
Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseasesRisk factors associated with relapse of adult-onset Still disease in Korean patients.
MedicineSingle-arm, open-label pilot intervention study to investigate an effect of oral 5-aminolevulinic acid plus sodium ferrous citrate on glucocorticoid reduction in patients with adult-onset Still disease: Study protocol for clinical trial (SPIRIT compliant).
MedicineEtiologies and management of haemophagocytic lymphohistiocytosis: is it time for an updated protocol and targeted treatments?
Rheumatology (Oxford, England)Schnitzler Syndrome: the paradigm of an acquired adult-onset auto-inflammatory disease.
Giornale italiano di dermatologia e venereologia : organo ufficiale, Societa italiana di dermatologia e sifilografiaLevels of Carbohydrate Antigen 125 in Patients With Adult Onset Still Disease: A Case Report.
CureusSuccessful use of short-term add-on tocilizumab for refractory adult-onset still's disease with macrophage activation syndrome despite treatment with high-dose glucocorticoids, cyclosporine, and etoposide.
Modern rheumatology case reportsCurrent treatment options and safety considerations when treating adult-onset Still's disease.
Expert opinion on drug safetyCase Report: Adult Still's Disease in an Alemtuzumab-Treated Multiple Sclerosis Patient.
Frontiers in immunologyA Case Report on Adult-Onset Still's Disease Successfully Treated With Tocilizumab: A Brief Review on its Safety and Efficacy.
CureusElevated Granulocyte Colony-stimulating Factor Levels in Patients With Active Phase of Adult-onset Still Disease.
The Journal of rheumatologyThe Role of Tocilizumab in the Treatment of Adult-Onset Still Disease-Report of a Tertiary Single-Center Experience.
Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseasesParvovirus-Induced Adult-Onset Still Disease Presenting With Elevated Liver Transaminases.
Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseasesAdult-Onset Still Disease as a Novel Autoimmune Complication of Alemtuzumab Therapy: A Case Report and Review of Alemtuzumab-Associated Autoimmune Disease.
Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseasesTreating Adult-Onset Still Disease With Tocilizumab: A Case-Based Review.
Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseasesSuccessful treatment of adult-onset still disease caused by pulmonary infection-associated hemophagocytic lymphohistiocytosis: A case report.
World journal of clinical casesUnprovoked Pulmonary Embolism Identified on Initial Presentation of Adult-Onset Still Disease in an Elderly Patient With No Malignancy.
Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseasesUp-regulation of CD64 Expression on Monocytes in Patients With Active Adult-Onset Still Disease: A Possible Biomarker of Disease Activity.
Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseasesAutoimmune polyglandular syndrome type III associated with antineutrophil cytoplasmic autoantibody-mediated crescentic glomerulonephritis: A case report and literature review.
MedicineDyskeratotic cells in persistent pruritic skin lesions as a prognostic factor in adult-onset Still disease.
Medicine[Purtscher-like retinopathy associated with adult onset still disease: Case report and review of the literature].
La Revue de medecine interneFluorodeoxyglucose PET/CT of Arthritis in Rheumatic Diseases: A Pictorial Review.
Radiographics : a review publication of the Radiological Society of North America, IncAdult-Onset Still Disease After Human Herpesvirus 6 Infection in an Elderly Patient: A Case Report.
Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseasesAre there new targets for juvenile idiopathic arthritis?
Seminars in arthritis and rheumatismGastric mucormycosis complicated by a gastropleural fistula: A case report and review of the literature.
MedicineAdult-Onset Still Disease Presenting With Insterstitial Lung Disease and Dermatomyositis-Like Atypical Persistent Eruption Successfully Treated With Mycophenolate Mofetil.
Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseasesMacrophage activation syndrome and pulmonary arterial hypertension in a patient with adult-onset Still disease: A case report.
MedicineAdult-Onset Still Disease Presenting With Dermatomyositis-Like Persistent Pruritic Lesions.
The American Journal of dermatopathologyThe Role of Natural Killer Cells in Inflammation in Active Adult-onset Still Disease.
The Journal of rheumatologyNeutrophilic urticarial dermatosis: an entity bridging monogenic and polygenic autoinflammatory disorders, and beyond.
Journal of the European Academy of Dermatology and Venereology : JEADVAnakinra Drug Retention Rate and Predictive Factors of Long-Term Response in Systemic Juvenile Idiopathic Arthritis and Adult Onset Still Disease.
Frontiers in pharmacologyAtypical Findings in Adult-Onset Still Disease.
Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseasesRefractory adult-onset Still disease treated by tocilizumab combined with methotrexate: A STROBE-compliant article.
MedicineJoint pain with violaceous papules and plaques.
JAAD case reportsNeutrophil Extracellular Traps May Contribute to the Pathogenesis in Adult-onset Still Disease.
The Journal of rheumatologyRefractory macrophage activation syndrome in the setting of adult-onset Still disease with hemophagocytic lymphohistiocytosis detected on skin biopsy treated with canakinumab and tacrolimus.
Journal of cutaneous pathologyThe changing pattern of fever of unknown origin in the Republic of North Macedonia.
Romanian journal of internal medicine = Revue roumaine de medecine interneAdult onset still disease associated with endogenous lipoid pneumonia.
Advances in respiratory medicineTocilizumab for the treatment of adult-onset Still's disease.
Expert opinion on biological therapyCharacteristics of Circulating Natural Killer Cells and Their Interferon-γ Production in Active Adult-onset Still Disease.
The Journal of rheumatologyDiagnosis and treatment of autoinflammatory diseases in adults: a clinical approach from rheumatologists.
Immunological medicineCytokine levels in persistent skin lesions of adult-onset Still disease.
Journal of the American Academy of DermatologyEndocarditis in Adult-onset Still Disease: A Case Report and Literature Review.
The Journal of rheumatologySuccessful slow tocilizumab desensitization in a patient with adult onset Still disease.
Biologicals : journal of the International Association of Biological Standardization[Non-infective endocarditis].
La Revue de medecine interneClinical analysis of 215 consecutive cases with fever of unknown origin: A cohort study.
MedicineIncreased severity and epidermal alterations in persistent versus evanescent skin lesions in adult-onset Still disease.
Journal of the American Academy of DermatologyAtypical Still disease with necrotic keratinocytes: A histologic mimicker of erythema multiforme.
JAAD case reportsMacrophage Activation Syndrome in Patients Affected by Adult-onset Still Disease: Analysis of Survival Rates and Predictive Factors in the Gruppo Italiano di Ricerca in Reumatologia Clinica e Sperimentale Cohort.
The Journal of rheumatologyDiagnostic and management of life-threatening Adult-Onset Still Disease: a French nationwide multicenter study and systematic literature review.
Critical care (London, England)Selective IgM deficiency associated with adult-onset Still disease.
Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & ImmunologyBreast Implant, a Mimicker of Adult-Onset Still Disease.
Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseasesAdult-Onset Still Disease and Macrophage Activation Syndrome Following Chikungunya and Hepatitis E Coinfection.
Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases[Systemic juvenile onset idiopathic arthritis and adult onset still disease].
Revue medicale suisseCrohn disease-associated neutrophilic urticarial dermatosis: report and literature review of neutrophilic urticarial dermatosis.
Dermatology online journalAdult-Onset Still Disease Masquerading as Multiple Organ Failure: Neither Benign Nor So Rare.
Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseasesAcute appendicitis complicated with necrotizing fasciitis in a patient with adult-onset Still's disease: A case report.
MedicineApproaches to sample size calculation for clinical trials in rare diseases.
Pharmaceutical statisticsSevere Adult-onset Still Disease with Constrictive Pericarditis and Pleuritis That Was Successfully Treated with Tocilizumab in Addition to Corticosteroids and Cyclosporin A.
Internal medicine (Tokyo, Japan)Aspergillus Pneumonia in a Patient With Adult-Onset Still Disease Successfully Treated With Anakinra.
Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseasesAn Adult Patient With a Novel Mutation in NLRP3 Gene Associated With Cryopyrin-Associated Periodic Syndrome Mimicking Adult-Onset Still Disease.
Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseasesInterleukin-1 Inhibitors and Dacryoadenitis in Adult-Onset Still Disease.
Annals of internal medicineImaging Characteristics of Chemotherapy Related Adult-Onset Still Disease.
Clinical nuclear medicinePulmonary involvement in adult Still's disease: Case report and brief review of literature.
Respiratory medicine case reportsTocilizumab for uncontrollable systemic inflammatory response syndrome complicating adult-onset Still disease: Case report and review of literature.
MedicineAutoimmune meningitis and encephalitis in adult-onset still disease - Case report.
Neurologia i neurochirurgia polskaRefractory adult-onset Still disease complicated by macrophage activation syndrome and acute myocarditis: A case report treated with high doses (8 mg/kg/d) of anakinra.
MedicineCoexistence of sarcoidosis and adult onset Still disease.
Reumatologia clinicaTocilizumab-Induced Drug Reaction With Eosinophilia and Systemic Symptoms Syndrome in Adult-Onset Still Disease.
Annals of internal medicineElevated Expression of the NLRP3 Inflammasome and Its Correlation with Disease Activity in Adult-onset Still Disease.
The Journal of rheumatologyApplication of the 2016 EULAR/ACR/PRINTO Classification Criteria for Macrophage Activation Syndrome in Patients with Adult-onset Still Disease.
The Journal of rheumatologyClinical and histopathological features of cutaneous manifestations of adult-onset Still disease.
Journal of cutaneous pathology[Adult-onset Still disease after hematopoietic stem cell transplantation].
Medicina clinicaPURTSCHER-LIKE RETINOPATHY ASSOCIATED WITH ADULT-ONSET STILL DISEASE.
Retinal cases & brief reportsVisceral leishmaniasis triggering (mimicking) macrophage activation syndrome in a patient with adult onset Still disease.
Internal medicine journalOccasional detection of thymic epithelial tumor 4 years after diagnosis of adult onset Still disease: A challenging case report and immuno-oncological considerations coming from pertinent literature review.
Medicine18F-FDG PET/CT for identifying the potential causes and extent of secondary hemophagocytic lymphohistiocytosis.
Diagnostic and interventional radiology (Ankara, Turkey)Adult-Onset Still Disease Is on the Move.
SkinmedParenchymal lung involvement in adult-onset Still disease: A STROBE-compliant case series and literature review.
MedicineBenefit of Tocilizumab Therapy for Adult-Onset Still Disease Complicated With Acute Respiratory Distress Syndrome.
Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseasesOnce-weekly teriparatide improves glucocorticoid-induced osteoporosis in patients with inadequate response to bisphosphonates.
SpringerPlusRefractory Genital HPV Infection and Adult-Onset Still Disease: A Case Report and Literature Review.
MedicineAtypical Cutaneous Presentation Of Adult Onset Still Disease.
Journal of Ayub Medical College, Abbottabad : JAMCSafety profile of anakinra in the management of rheumatologic, metabolic and autoinflammatory disorders.
Clinical and experimental rheumatologyEpidemiology and outcome of articular complications in adult onset Still's disease.
The Pan African medical journal[A case of anti-PL7 antibody positive myositis and a clinical and pathological review of the anti-synthetase syndrome].
Rinsho shinkeigaku = Clinical neurology[Pseudotumoral thoracic splenosis in a patient with adult-onset Still disease and reactive hemophagocytic syndrome].
Presse medicale (Paris, France : 1983)[Pseudo-adult Still's disease, anasarca, thrombotic thrombocytopenic purpura and dysautonomia: An atypical presentation of multicentric Castleman's disease. Discussion of TAFRO syndrome].
La Revue de medecine interneEstimation of the symptoms for GERD by GerdQ in the patients with rheumatic diseases.
Modern rheumatologyUpdates in adult-onset Still disease: Atypical cutaneous manifestations and associations with delayed malignancy.
Journal of the American Academy of DermatologyThe pathologic findings of skin, lymph node, liver, and bone marrow in patients with adult-onset still disease: a comprehensive analysis of 40 cases.
MedicineMyocarditis and Adult onset Still disease.
Revista clinica espanolaReactive hemophagocytic syndrome in adult-onset Still disease: clinical features, predictive factors, and prognosis in 21 patients.
MedicineAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
Ainda não temos associações cadastradas para Doença de Still de início no adulto.
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Comunidades
Grupos ativos de quem convive com esta doença aqui no Raras
Ainda não existe comunidade no Raras para Doença de Still de início no adulto
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- Case Report: Flagellate eruption of adult-onset still disease in an Afro-Caribbean woman: highlighting atypical cutaneous disease in richly pigmented skin.
- A multifaceted interplay between hemophagocytosis, IL-18 and type I IFN distinguishes Still disease from other autoinflammatory diseases.
- New-Onset and Flare Episodes of Adult-Onset Still's Disease Following COVID-19 Vaccination: A Systematic Review of Published Case Reports.
- Trends in fatigue in inflammatory rheumatic diseases: Annual data and trajectory analysis of the German National Database 2007-2023.
- Examination of HLA-DRB1*15-linked Candidate Antigens in Still Disease With and Without Lung Disease and Drug-Associated Immune Reactions.
- A Case of Refractory Adult-Onset Still Disease With Macrophage Activation Syndrome and Pulmonary Alveolar Proteinosis.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:829(Orphanet)
- MONDO:0019355(MONDO)
- GARD:436(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Artigo Wikipedia(Wikipedia)
- Q1187697(Wikidata)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
