Raras
Buscar doenças, sintomas, genes...
Doença de Still de início no adulto
ORPHA:829CID-10 · M06.1CID-11 · FA23DOENÇA RARA

Doença multissistêmica inflamatória rara caracterizada clinicamente por febre de origem desconhecida, artralgia ou artrite, hiperleucocitose e erupção cutânea típica.

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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.

Pesquisas ativas
8 ensaios
24 total registrados no ClinicalTrials.gov
Publicações científicas
277 artigos
Último publicado: 2026

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
1-9 / 100 000
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
3.9
Japan
Início
Adult
+ elderly
🏥
SUS: Sem cobertura SUSScore: 0%
3 medicamentos CEAFCID-10: M06.1
Você se identifica com essa condição?
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Entender a doença

Do básico ao detalhe, leia no seu ritmo

Preparando trilha educativa...

Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

🩸
Sangue
7 sintomas
🫃
Digestivo
7 sintomas
🫁
Pulmão
3 sintomas
❤️
Coração
3 sintomas
📏
Crescimento
2 sintomas
🛡️
Imunológico
2 sintomas

+ 26 sintomas em outras categorias

Características mais comuns

90%prev.
Eritema
Muito frequente (99-80%)
90%prev.
Taxa de sedimentação de eritrócitos elevada
Muito frequente (99-80%)
90%prev.
Leucocitose
Muito frequente (99-80%)
90%prev.
Inchaço articular
Muito frequente (99-80%)
90%prev.
Erupção cutânea
Muito frequente (99-80%)
90%prev.
Fadiga
Muito frequente (99-80%)
59sintomas
Muito frequente (14)
Frequente (18)
Ocasional (18)
Muito raro (9)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 59 características clínicas mais associadas, ordenadas por frequência.

EritemaErythema
Muito frequente (99-80%)90%
Taxa de sedimentação de eritrócitos elevadaElevated erythrocyte sedimentation rate
Muito frequente (99-80%)90%
LeucocitoseLeukocytosis
Muito frequente (99-80%)90%
Inchaço articularJoint swelling
Muito frequente (99-80%)90%
Erupção cutâneaSkin rash
Muito frequente (99-80%)90%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico277PubMed
Últimos 10 anos177publicações
Pico202523 papers
Linha do tempo
2026Hoje · 2026🧪 2004Primeiro ensaio clínico📈 2025Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

🧬

Nenhum gene associado encontrado

Os dados genéticos desta condição ainda estão sendo catalogados.

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
3Fase 35
2Fase 22
1Fase 12
·Pré-clínico11
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 20 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Doença de Still de início no adulto

🗺️

Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

🟢 Recrutando agora

5 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.

Outros ensaios clínicos

24 ensaios clínicos encontrados, 8 ativos.

Distribuição por fase
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

🥇Melhor nível de evidência: Meta-análise
Timeline de publicações
180 papers (10 anos)
#1

Case Report: Flagellate eruption of adult-onset still disease in an Afro-Caribbean woman: highlighting atypical cutaneous disease in richly pigmented skin.

Frontiers in immunology2026

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.

#2

A multifaceted interplay between hemophagocytosis, IL-18 and type I IFN distinguishes Still disease from other autoinflammatory diseases.

Arthritis &amp; rheumatology (Hoboken, N.J.)2026 Feb 23

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.

#3

New-Onset and Flare Episodes of Adult-Onset Still's Disease Following COVID-19 Vaccination: A Systematic Review of Published Case Reports.

Cureus2026 Jan

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.

#4

Trends in fatigue in inflammatory rheumatic diseases: Annual data and trajectory analysis of the German National Database 2007-2023.

Seminars in arthritis and rheumatism2026 Feb

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.

#5

Examination of HLA-DRB1*15-linked Candidate Antigens in Still Disease With and Without Lung Disease and Drug-Associated Immune Reactions.

The Journal of rheumatology2026 Feb 15

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

Ver todas no PubMed

📚 EuropePMC161 artigos no totalmostrando 177

2026

Case Report: Flagellate eruption of adult-onset still disease in an Afro-Caribbean woman: highlighting atypical cutaneous disease in richly pigmented skin.

Frontiers in immunology
2026

A multifaceted interplay between hemophagocytosis, IL-18 and type I IFN distinguishes Still disease from other autoinflammatory diseases.

Arthritis &amp; rheumatology (Hoboken, N.J.)
2026

A Case of Refractory Adult-Onset Still Disease With Macrophage Activation Syndrome and Pulmonary Alveolar Proteinosis.

The Journal of rheumatology
2026

New-Onset and Flare Episodes of Adult-Onset Still's Disease Following COVID-19 Vaccination: A Systematic Review of Published Case Reports.

Cureus
2026

Trends in fatigue in inflammatory rheumatic diseases: Annual data and trajectory analysis of the German National Database 2007-2023.

Seminars in arthritis and rheumatism
2026

Examination of HLA-DRB1*15-linked Candidate Antigens in Still Disease With and Without Lung Disease and Drug-Associated Immune Reactions.

The Journal of rheumatology
2025

Dermatomyositis-Like Lesion: An Atypical Cutaneous Presentation in Adult-Onset Still Disease.

Dermatology practical &amp; conceptual
2025

Brexpiprazole for Managing Mania After Methylprednisolone Pulse Therapy in a Patient With Adult-Onset Still Disease.

American journal of therapeutics
2025

Pruritic eruption in a young woman with fever.

Skin health and disease
2026

Clinical 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 rheumatology
2025

Challenges on diagnosis and treatment of refractory adult-onset still disease in Sub-Saharan Africa: a case report.

Journal of medical case reports
2025

Carpal tunnel syndrome with both AA amyloidosis and elderly-onset Still disease: A case report.

Medicine
2025

Low 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 Dermatology
2025

Diagnosis 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.

Medicine
2025

Nasal septal perforation in adult Still's disease.

Joint bone spine
2025

Macrophage activation syndrome-associated adult onset Still disease treatment: a scoping review of case reports and case series.

Proceedings (Baylor University. Medical Center)
2025

TREATMENT OF HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS IN THE INTENSIVE CARE UNIT: TOO LATE AFTER RESORTING TO INVASIVE ORGAN SUPPORTS?

Shock (Augusta, Ga.)
2025

Ruxolitinib targets JAK-STAT signaling to modulate neutrophil activation in refractory macrophage activation syndrome.

Blood
2025

Endogenous Lipoid Pneumonia in Adult Autoinflammatory Disease.

Arthritis care &amp; research
2025

Long-Term Health Consequences of SARS-CoV-2- Trigger for Still Disease in Adults.

ACR open rheumatology
2025

Achievement 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 rheumatology
2025

Evaluation 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 clinica
2025

Adult-onset Still disease.

CMAJ : Canadian Medical Association journal = journal de l'Association medicale canadienne
2025

Role 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 reviews
2025

Accuracy 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 &amp; musculoskeletal diseases
2025

Non-Classical Complications of Adult-Onset Still's Disease: A Multicenter Spanish Study.

Journal of clinical medicine
2025

Acquired autoinflammatory disorders: a dermatologist's perspective.

Clinical and experimental dermatology
2024

Overlap of Adult-Onset Still Disease and Kikuchi-Fujimoto Disease: A Case Report and Literature Review.

Cureus
2025

Evaluation of Myocarditis in Patients With Still Disease: Clinical Findings From the Multicenter International AIDA Network Still Disease Registry.

The Journal of rheumatology
2025

Characterization of Genetic Landscape and Novel Inflammatory Biomarkers in Patients With Adult-Onset Still's Disease.

Arthritis &amp; rheumatology (Hoboken, N.J.)
2024

Mitochondrial DNA copy number and the risk of autoimmune diseases: A Mendelian randomization study with meta-analysis.

Journal of translational autoimmunity
2024

Adult-Onset Still's Disease and Pulmonary Embolism: A Case Report.

Cureus
2024

Indices and ferritin level that predict organ involvement in adult-onset Still's disease.

Biomarkers in medicine
2024

Case Report: Adult Onset Still Disease in a 26-year-old Male.

South Dakota medicine : the journal of the South Dakota State Medical Association
2025

Emapalumab Treatment in Patients With Rheumatologic Disease-Associated Hemophagocytic Lymphohistiocytosis in the United States: A Retrospective Medical Chart Review Study.

Arthritis &amp; rheumatology (Hoboken, N.J.)
2024

Criteria for Cytokine Storm Syndromes.

Advances in experimental medicine and biology
2024

The History of Macrophage Activation Syndrome in Autoimmune Diseases.

Advances in experimental medicine and biology
2024

Association between Relative Bradycardia and Adult-onset Still Disease.

JMA journal
2024

A case of adult-onset Still disease with eyelid xanthogranuloma mimicking Erdheim-Chester disease.

Clinical and experimental dermatology
2024

Nationwide Analysis of Adult-Onset Still Disease With and Without Hemophagocytic Lymphohistiocytosis.

Journal of clinical rheumatology : practical reports on rheumatic &amp; musculoskeletal diseases
2024

Three Case Reports of Adult-onset Still Disease Associated with Breast Implantation.

Plastic and reconstructive surgery. Global open
2024

The 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 &amp; rheumatology (Hoboken, N.J.)
2024

Systematic Review and Metaanalysis of Pharmacological Interventions in Adult-Onset Still Disease and the Role of Biologic Disease-Modifying Antirheumatic Drugs.

The Journal of rheumatology
2023

Multidisciplinary approach to diagnosis and management of fever of unknown origin: A case report.

Medicine
2023

Treatment of systemic juvenile idiopathic arthritis.

Nature reviews. Rheumatology
2023

Neutrophilic urticarial dermatosis preceding adult-onset Still's disease.

Dermatology online journal
2023

Autoimmune and Autoinflammatory Connective Tissue Disorders Following COVID-19.

JAMA network open
2024

Cigarette smoking and risk of adult-onset Still disease: a propensity score matching analysis.

Internal medicine journal
2023

IL-18 in Autoinflammatory Diseases: Focus on Adult Onset Still Disease and Macrophages Activation Syndrome.

International journal of molecular sciences
2023

Tocilizumab-induced mucosal injury in the terminal ileum mimicking intestinal Behçet's disease: A case report.

Medicine
2023

Hepatic Superscan in Methotrexate-Associated Lymphoproliferative Disorder.

Clinical nuclear medicine
2023

Persistent, Pruritic, Reddish-Brown Papules and Plaques: An Atypical Presentation of Adult-Onset Still's Disease.

Cureus
2022

Extremely high erythrocyte sedimentation rate revisited in rheumatic diseases: a singlecenter experience.

Turkish journal of medical sciences
2023

Dermatologic Manifestations of Noninflammasome-Mediated Autoinflammatory Diseases.

JID innovations : skin science from molecules to population health
2023

Brain abscess due to Nocardia asiatica in a 63-year-old patient with adult-onset still disease: A case report.

Clinical case reports
2023

Ferritin-induced NETs lead to cytokine storm in AOSD.

Nature reviews. Rheumatology
2022

Adult-Onset Still's Disease-A Complex Disease, a Challenging Treatment.

International journal of molecular sciences
2022

COVID-19 in a patient with new adult-onset Still disease: A case report.

Medicine
2022

Evanescent, episodic salmon-colored macules in a young woman.

JAAD case reports
2022

Activation mechanisms of monocytes/macrophages in adult-onset Still disease.

Frontiers in immunology
2022

Diagnostic utility of clinical characteristics, laboratory tests, and serum ferritin in diagnosis of adult-onset Still disease.

Medicine
2023

Idiopathic multicentric Castleman disease and associated autoimmune and autoinflammatory conditions: practical guidance for diagnosis.

Rheumatology (Oxford, England)
2023

Development of Pulmonary Alveolar Proteinosis in a Patient With Adult-Onset Still Disease Treated With Tocilizumab.

The Journal of rheumatology
2023

Adult-Onset Still Disease After ChAdOx1 nCOV-19 Vaccination.

The Journal of rheumatology
2022

Validation of the neutrophil-to-lymphocyte ratio as a new simple biomarker of adult onset Still's disease: A STROBE-Compliant prospective observational study.

Medicine
2022

Glucose metabolism in systemic juvenile idiopathic arthritis.

Pediatric rheumatology online journal
2022

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

Medicine
2022

Assessment of health-related quality of life in patients with adult onset Still disease: Results from a multicentre cross-sectional study.

Medicine
2022

Bibliometrics and Visual Analysis of Adult-onset Still Disease (1976-2020).

Frontiers in public health
2022

Still disease with persistent atypical dermatomyositis-like skin eruption: two cases associated with macrophage activation syndrome.

Clinical and experimental dermatology
2022

COVID-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 open
2022

Heliotrope-like manifestation of adult-onset Still disease with macrophage activation syndrome: A case-based review.

The Journal of dermatology
2022

Adult-onset Still disease and adenovirus vector COVID-19 vaccination.

Internal medicine journal
2022

Prognostic factors for adult-onset Still disease: a retrospective analysis of 90 patients in China.

European journal of dermatology : EJD
2022

Bibliometric analysis of the global literature productivity of adult-onset Still's disease (2011-2020).

Rheumatology international
2021

Adult-onset Still disease post-adenovirus vector COVID-19 vaccine.

Internal medicine journal
2021

Erythroderma and persistent erythema in adult-onset Still disease associated with macrophage activation syndrome.

JAAD case reports
2021

Dermato-Pathologic Clues To Diagnosis Of Adult Onset Still Disease: A Case Report.

Indian dermatology online journal
2022

Fever and flagellate dermatosis in an otherwise healthy woman.

Clinical and experimental dermatology
2021

Clinical manifestations and treatment outcomes in prurigo pigmentosa (Nagashima disease): A systematic review of the literature.

JAAD international
2021

Efficacy of tight control strategy in the treatment of adult-onset Still disease.

Clinical rheumatology
2020

[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)
2021

Immune cartography of macrophage activation syndrome in the COVID-19 era.

Nature reviews. Rheumatology
2021

The JANUS of chronic inflammatory and autoimmune diseases onset during COVID-19 - A systematic review of the literature.

Journal of autoimmunity
2022

Hemophagocytic Lymphohistiocytosis Hospitalizations in Adults and Its Association With Rheumatologic Diseases: Data From Nationwide Inpatient Sample.

Journal of clinical rheumatology : practical reports on rheumatic &amp; musculoskeletal diseases
2020

Risk factors associated with relapse of adult-onset Still disease in Korean patients.

Medicine
2020

Single-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).

Medicine
2021

Etiologies and management of haemophagocytic lymphohistiocytosis: is it time for an updated protocol and targeted treatments?

Rheumatology (Oxford, England)
2020

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 sifilografia
2020

Levels of Carbohydrate Antigen 125 in Patients With Adult Onset Still Disease: A Case Report.

Cureus
2020

Successful 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 reports
2020

Current treatment options and safety considerations when treating adult-onset Still's disease.

Expert opinion on drug safety
2020

Case Report: Adult Still's Disease in an Alemtuzumab-Treated Multiple Sclerosis Patient.

Frontiers in immunology
2020

A Case Report on Adult-Onset Still's Disease Successfully Treated With Tocilizumab: A Brief Review on its Safety and Efficacy.

Cureus
2021

Elevated Granulocyte Colony-stimulating Factor Levels in Patients With Active Phase of Adult-onset Still Disease.

The Journal of rheumatology
2021

The 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 &amp; musculoskeletal diseases
2021

Parvovirus-Induced Adult-Onset Still Disease Presenting With Elevated Liver Transaminases.

Journal of clinical rheumatology : practical reports on rheumatic &amp; musculoskeletal diseases
2021

Adult-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 &amp; musculoskeletal diseases
2021

Treating Adult-Onset Still Disease With Tocilizumab: A Case-Based Review.

Journal of clinical rheumatology : practical reports on rheumatic &amp; musculoskeletal diseases
2020

Successful treatment of adult-onset still disease caused by pulmonary infection-associated hemophagocytic lymphohistiocytosis: A case report.

World journal of clinical cases
2020

Unprovoked 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 &amp; musculoskeletal diseases
2020

Up-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 &amp; musculoskeletal diseases
2020

Autoimmune polyglandular syndrome type III associated with antineutrophil cytoplasmic autoantibody-mediated crescentic glomerulonephritis: A case report and literature review.

Medicine
2020

Dyskeratotic cells in persistent pruritic skin lesions as a prognostic factor in adult-onset Still disease.

Medicine
2020

[Purtscher-like retinopathy associated with adult onset still disease: Case report and review of the literature].

La Revue de medecine interne
2020

Fluorodeoxyglucose PET/CT of Arthritis in Rheumatic Diseases: A Pictorial Review.

Radiographics : a review publication of the Radiological Society of North America, Inc
2021

Adult-Onset Still Disease After Human Herpesvirus 6 Infection in an Elderly Patient: A Case Report.

Journal of clinical rheumatology : practical reports on rheumatic &amp; musculoskeletal diseases
2019

Are there new targets for juvenile idiopathic arthritis?

Seminars in arthritis and rheumatism
2019

Gastric mucormycosis complicated by a gastropleural fistula: A case report and review of the literature.

Medicine
2019

Adult-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 &amp; musculoskeletal diseases
2019

Macrophage activation syndrome and pulmonary arterial hypertension in a patient with adult-onset Still disease: A case report.

Medicine
2019

Adult-Onset Still Disease Presenting With Dermatomyositis-Like Persistent Pruritic Lesions.

The American Journal of dermatopathology
2019

The Role of Natural Killer Cells in Inflammation in Active Adult-onset Still Disease.

The Journal of rheumatology
2020

Neutrophilic urticarial dermatosis: an entity bridging monogenic and polygenic autoinflammatory disorders, and beyond.

Journal of the European Academy of Dermatology and Venereology : JEADV
2019

Anakinra Drug Retention Rate and Predictive Factors of Long-Term Response in Systemic Juvenile Idiopathic Arthritis and Adult Onset Still Disease.

Frontiers in pharmacology
2020

Atypical Findings in Adult-Onset Still Disease.

Journal of clinical rheumatology : practical reports on rheumatic &amp; musculoskeletal diseases
2019

Refractory adult-onset Still disease treated by tocilizumab combined with methotrexate: A STROBE-compliant article.

Medicine
2019

Joint pain with violaceous papules and plaques.

JAAD case reports
2019

Neutrophil Extracellular Traps May Contribute to the Pathogenesis in Adult-onset Still Disease.

The Journal of rheumatology
2019

Refractory 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 pathology
2019

The changing pattern of fever of unknown origin in the Republic of North Macedonia.

Romanian journal of internal medicine = Revue roumaine de medecine interne
2019

Adult onset still disease associated with endogenous lipoid pneumonia.

Advances in respiratory medicine
2019

Tocilizumab for the treatment of adult-onset Still's disease.

Expert opinion on biological therapy
2019

Characteristics of Circulating Natural Killer Cells and Their Interferon-γ Production in Active Adult-onset Still Disease.

The Journal of rheumatology
2018

Diagnosis and treatment of autoinflammatory diseases in adults: a clinical approach from rheumatologists.

Immunological medicine
2018

Cytokine levels in persistent skin lesions of adult-onset Still disease.

Journal of the American Academy of Dermatology
2018

Endocarditis in Adult-onset Still Disease: A Case Report and Literature Review.

The Journal of rheumatology
2018

Successful slow tocilizumab desensitization in a patient with adult onset Still disease.

Biologicals : journal of the International Association of Biological Standardization
2018

[Non-infective endocarditis].

La Revue de medecine interne
2018

Clinical analysis of 215 consecutive cases with fever of unknown origin: A cohort study.

Medicine
2018

Increased severity and epidermal alterations in persistent versus evanescent skin lesions in adult-onset Still disease.

Journal of the American Academy of Dermatology
2018

Atypical Still disease with necrotic keratinocytes: A histologic mimicker of erythema multiforme.

JAAD case reports
2018

Macrophage 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 rheumatology
2018

Diagnostic and management of life-threatening Adult-Onset Still Disease: a French nationwide multicenter study and systematic literature review.

Critical care (London, England)
2018

Selective IgM deficiency associated with adult-onset Still disease.

Annals of allergy, asthma &amp; immunology : official publication of the American College of Allergy, Asthma, &amp; Immunology
2018

Breast Implant, a Mimicker of Adult-Onset Still Disease.

Journal of clinical rheumatology : practical reports on rheumatic &amp; musculoskeletal diseases
2018

Adult-Onset Still Disease and Macrophage Activation Syndrome Following Chikungunya and Hepatitis E Coinfection.

Journal of clinical rheumatology : practical reports on rheumatic &amp; musculoskeletal diseases
2018

[Systemic juvenile onset idiopathic arthritis and adult onset still disease].

Revue medicale suisse
2017

Crohn disease-associated neutrophilic urticarial dermatosis: report and literature review of neutrophilic urticarial dermatosis.

Dermatology online journal
2018

Adult-Onset Still Disease Masquerading as Multiple Organ Failure: Neither Benign Nor So Rare.

Journal of clinical rheumatology : practical reports on rheumatic &amp; musculoskeletal diseases
2018

Acute appendicitis complicated with necrotizing fasciitis in a patient with adult-onset Still's disease: A case report.

Medicine
2018

Approaches to sample size calculation for clinical trials in rare diseases.

Pharmaceutical statistics
2018

Severe 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)
2018

Aspergillus Pneumonia in a Patient With Adult-Onset Still Disease Successfully Treated With Anakinra.

Journal of clinical rheumatology : practical reports on rheumatic &amp; musculoskeletal diseases
2018

An 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 &amp; musculoskeletal diseases
2018

Interleukin-1 Inhibitors and Dacryoadenitis in Adult-Onset Still Disease.

Annals of internal medicine
2017

Imaging Characteristics of Chemotherapy Related Adult-Onset Still Disease.

Clinical nuclear medicine
2017

Pulmonary involvement in adult Still's disease: Case report and brief review of literature.

Respiratory medicine case reports
2017

Tocilizumab for uncontrollable systemic inflammatory response syndrome complicating adult-onset Still disease: Case report and review of literature.

Medicine
2017

Autoimmune meningitis and encephalitis in adult-onset still disease - Case report.

Neurologia i neurochirurgia polska
2017

Refractory 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.

Medicine
2019

Coexistence of sarcoidosis and adult onset Still disease.

Reumatologia clinica
2017

Tocilizumab-Induced Drug Reaction With Eosinophilia and Systemic Symptoms Syndrome in Adult-Onset Still Disease.

Annals of internal medicine
2017

Elevated Expression of the NLRP3 Inflammasome and Its Correlation with Disease Activity in Adult-onset Still Disease.

The Journal of rheumatology
2017

Application of the 2016 EULAR/ACR/PRINTO Classification Criteria for Macrophage Activation Syndrome in Patients with Adult-onset Still Disease.

The Journal of rheumatology
2017

Clinical and histopathological features of cutaneous manifestations of adult-onset Still disease.

Journal of cutaneous pathology
2016

[Adult-onset Still disease after hematopoietic stem cell transplantation].

Medicina clinica
2018

PURTSCHER-LIKE RETINOPATHY ASSOCIATED WITH ADULT-ONSET STILL DISEASE.

Retinal cases &amp; brief reports
2016

Visceral leishmaniasis triggering (mimicking) macrophage activation syndrome in a patient with adult onset Still disease.

Internal medicine journal
2016

Occasional 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.

Medicine
2016

18F-FDG PET/CT for identifying the potential causes and extent of secondary hemophagocytic lymphohistiocytosis.

Diagnostic and interventional radiology (Ankara, Turkey)
2016

Adult-Onset Still Disease Is on the Move.

Skinmed
2016

Parenchymal lung involvement in adult-onset Still disease: A STROBE-compliant case series and literature review.

Medicine
2016

Benefit of Tocilizumab Therapy for Adult-Onset Still Disease Complicated With Acute Respiratory Distress Syndrome.

Journal of clinical rheumatology : practical reports on rheumatic &amp; musculoskeletal diseases
2016

Once-weekly teriparatide improves glucocorticoid-induced osteoporosis in patients with inadequate response to bisphosphonates.

SpringerPlus
2016

Refractory Genital HPV Infection and Adult-Onset Still Disease: A Case Report and Literature Review.

Medicine
2016

Atypical Cutaneous Presentation Of Adult Onset Still Disease.

Journal of Ayub Medical College, Abbottabad : JAMC
2016

Safety profile of anakinra in the management of rheumatologic, metabolic and autoinflammatory disorders.

Clinical and experimental rheumatology
2015

Epidemiology and outcome of articular complications in adult onset Still's disease.

The Pan African medical journal
2015

[A case of anti-PL7 antibody positive myositis and a clinical and pathological review of the anti-synthetase syndrome].

Rinsho shinkeigaku = Clinical neurology
2015

[Pseudotumoral thoracic splenosis in a patient with adult-onset Still disease and reactive hemophagocytic syndrome].

Presse medicale (Paris, France : 1983)
2016

[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 interne
2016

Estimation of the symptoms for GERD by GerdQ in the patients with rheumatic diseases.

Modern rheumatology
2015

Updates in adult-onset Still disease: Atypical cutaneous manifestations and associations with delayed malignancy.

Journal of the American Academy of Dermatology
2015

The pathologic findings of skin, lymph node, liver, and bone marrow in patients with adult-onset still disease: a comprehensive analysis of 40 cases.

Medicine
2015

Myocarditis and Adult onset Still disease.

Revista clinica espanola
2015

Reactive hemophagocytic syndrome in adult-onset Still disease: clinical features, predictive factors, and prognosis in 21 patients.

Medicine

Associações

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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Doenças relacionadas

Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico

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.

  1. Case Report: Flagellate eruption of adult-onset still disease in an Afro-Caribbean woman: highlighting atypical cutaneous disease in richly pigmented skin.
    Frontiers in immunology· 2026· PMID 41853279mais citado
  2. A multifaceted interplay between hemophagocytosis, IL-18 and type I IFN distinguishes Still disease from other autoinflammatory diseases.
    Arthritis &amp; rheumatology (Hoboken, N.J.)· 2026· PMID 41728940mais citado
  3. New-Onset and Flare Episodes of Adult-Onset Still's Disease Following COVID-19 Vaccination: A Systematic Review of Published Case Reports.
    Cureus· 2026· PMID 41658735mais citado
  4. Trends in fatigue in inflammatory rheumatic diseases: Annual data and trajectory analysis of the German National Database 2007-2023.
    Seminars in arthritis and rheumatism· 2026· PMID 41412093mais citado
  5. Examination of HLA-DRB1*15-linked Candidate Antigens in Still Disease With and Without Lung Disease and Drug-Associated Immune Reactions.
    The Journal of rheumatology· 2026· PMID 41397865mais citado
  6. A Case of Refractory Adult-Onset Still Disease With Macrophage Activation Syndrome and Pulmonary Alveolar Proteinosis.
    J Rheumatol· 2026· PMID 41692495recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:829(Orphanet)
  2. MONDO:0019355(MONDO)
  3. GARD:436(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Artigo Wikipedia(Wikipedia)
  6. 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

Doença de Still de início no adulto
Compêndio · Raras BR

Doença de Still de início no adulto

ORPHA:829 · MONDO:0019355
🇧🇷 Brasil SUS
CEAF
1ARilzabrutinibeCanaquinumabeAnacinra
Geral
Prevalência
1-9 / 100 000
Herança
Not applicable
CID-10
M06.1 · Doença de Still do adulto
CID-11
Ensaios
8 ativos
Início
Adult, Elderly
Prevalência
3.9 (Japan)
MedGen
UMLS
C0085253
EuropePMC
Wikidata
Wikipedia
Papers 10a
Evidência
🥇 Meta-análise
DiscussaoAtiva

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