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1.
Pan Afr Med J ; 32: 158, 2019.
Artigo em Francês | MEDLINE | ID: mdl-31303928

RESUMO

Renal amyloidosis is a rare complication of adult onset Still's disease. We here report three cases of renal amyloidosis in a series of 33 cases of adult onset Still's disease. The three patients enrolled had an average age of 43 years (with a range from 33 to 58 years). The diagnosis of Still's disease was retained on the basis of febrile polyarthritis (3 cases) associated with fleeting rush (1 case), biologic inflammatory syndrome in the absence of any infectious, inflammatory or neoplastic causes. All patients were treated with corticosteroids secondarily associated with methotrexate due to destructive polyarthritis (2 cases) and to a recurrence (1 case). Renal amyloidosis had occurred 4.9 years after Still's disease (with a range from 33 months to 7 years). Amyloidosis was revealed by nephrotic syndrome (3 cases) associated with renal failure (1 case). Diagnosis was based on renal puncture biopsy (3 cases) which showed AA amyloidosis (2 cases) and untyped amyloidosis (1 case). All patients received colchicine. Outcome was favorable in a female patient while in the other two patients the disease progressed to chronic renal failure. Renal amyloidosis uncommonly results from adult onset Still's disease. Once the disease gets established it can be life-threatening.


Assuntos
Amiloidose/etiologia , Síndrome Nefrótica/etiologia , Doença de Still de Início Tardio/complicações , Corticosteroides/administração & dosagem , Adulto , Amiloidose/diagnóstico , Amiloidose/tratamento farmacológico , Biópsia por Agulha , Colchicina/administração & dosagem , Progressão da Doença , Feminino , Humanos , Falência Renal Crônica/etiologia , Metotrexato/administração & dosagem , Pessoa de Meia-Idade , Insuficiência Renal/etiologia , Doença de Still de Início Tardio/diagnóstico , Doença de Still de Início Tardio/tratamento farmacológico
2.
Hautarzt ; 70(7): 530-534, 2019 Jul.
Artigo em Alemão | MEDLINE | ID: mdl-30937478

RESUMO

Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disease characterized by high spiking fever, arthritis, salmon-pink maculopapular rash and multiple organ involvement. We report a case of an adult-onset Still's disease that meets Yamaguchi's criteria and presented with typical clinical manifestations. AOSD is treated with anti-inflammatory medications. Standard therapy includes corticosteroids. Other medications like azathioprine, methotrexate or interleukin-1 or -6 blockers can be used when standard steroid treatment is not adequate.


Assuntos
Corticosteroides/uso terapêutico , Exantema/patologia , Doença de Still de Início Tardio/diagnóstico , Adulto , Idade de Início , Anti-Inflamatórios/uso terapêutico , Artrite/etiologia , Exantema/etiologia , Febre/etiologia , Humanos , Doença de Still de Início Tardio/tratamento farmacológico , Doença de Still de Início Tardio/imunologia , Síndrome
3.
BMC Infect Dis ; 19(1): 198, 2019 Feb 27.
Artigo em Inglês | MEDLINE | ID: mdl-30813923

RESUMO

BACKGROUND: Few studies have reported the long-term clinical outcome of patients discharged with undiagnosed fever of unknown origin (FUO). In this study, the clinical features and outcomes of patients with unexplained fever were explored to improve our understanding of FUO. METHOD: Patients diagnosed with FUO at admission and discharged without final diagnoses after systematic examination in the department of infectious diseases at Peking Union Medical College Hospital between 2004 and 2010 were followed up by telephone. Medical records were reviewed, and the clinical features and outcomes of patients for whom follow-up data were available were summarized. RESULTS: Between 2004 and 2010, 58 patients with follow-up data, who were diagnosed with FUO at admission and did not have a final diagnosis at discharge, were enrolled in this study. The median duration of follow-up was 518 (0.4-830) weeks, and the fever duration was 24.6 (6.7-763.2) weeks. Final diagnoses were established in 11 cases (19%), and the diagnostic methods included clinical diagnosis, diagnostic therapy, genetic screening and biopsy pathology. The fever in 35 patients (60%) subsided during hospitalization or after discharge. Their condition was stable and self-limited after long-term follow-up, and they were ultimately thought to be cured. Two patients had periodic fever during prolonged observation: one patient needed intermittent use of nonsteroidal antiinflammatory drugs (NSAIDs), and the other needed intermittent use of NSAIDs and a steroid. Ten patients died during follow-up, with 9 deaths being caused by severe and worsening conditions related to the febrile illness. CONCLUSIONS: Long-term follow-up should be performed for patients with undiagnosed FUO. Some patients can obtain a definitive diagnosis by repeated multiple invasive examinations and diagnostic treatment. Most patients have a self-limited illness, and their prognosis is good.


Assuntos
Febre de Causa Desconhecida/diagnóstico , Febre de Causa Desconhecida/etiologia , Adolescente , Adulto , Idoso , Anti-Inflamatórios não Esteroides/uso terapêutico , Doenças Transmissíveis , Feminino , Febre de Causa Desconhecida/tratamento farmacológico , Seguimentos , Hospitalização , Humanos , Masculino , Pessoa de Meia-Idade , Alta do Paciente , Exame Físico , Prognóstico , Estudos Retrospectivos , Esteroides/uso terapêutico , Doença de Still de Início Tardio/diagnóstico , Doença de Still de Início Tardio/etiologia , Resultado do Tratamento
4.
J Dtsch Dermatol Ges ; 17(1): 43-64, 2019 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-30615277

RESUMO

The coincidence of skin and joint inflammation poses a challenge for both dermatologists and rheumatologists. Adequate management of such disorders requires that physicians of both specialties have sound knowledge of the other discipline. In case of suspected joint involvement, familiarity with the diagnostic options available to rheumatologists enables dermatologists to selectively refer their patients for a rheumatology consult. The objective of the present review is to familiarize the reader with the stepwise diagnostic workup performed by rheumatologists today, including laboratory tests, musculoskeletal ultrasound, X-ray studies, and magnetic resonance imaging. Subsequently, we will discuss a number of disorders characterized by the concurrence of skin and joint inflammation, highlighting aspects of epidemiology, etiology and pathogenesis, clinical presentation, diagnosis and treatment. These disorders include psoriatic arthritis as well as autoinflammatory disorders such as SAPHO syndrome, Still's disease and Behçet's disease.


Assuntos
Síndrome de Hiperostose Adquirida , Artrite Psoriásica , Síndrome de Behçet , Doença de Still de Início Tardio , Síndrome de Hiperostose Adquirida/diagnóstico , Síndrome de Hiperostose Adquirida/etiologia , Artrite/complicações , Artrite Psoriásica/diagnóstico , Artrite Psoriásica/etiologia , Síndrome de Behçet/diagnóstico , Síndrome de Behçet/etiologia , Humanos , Inflamação , Doença de Still de Início Tardio/diagnóstico , Doença de Still de Início Tardio/etiologia , Sinovite/complicações
5.
Clin Exp Rheumatol ; 36(6 Suppl 115): 74-79, 2018 Nov-Dec.
Artigo em Inglês | MEDLINE | ID: mdl-30582502

RESUMO

OBJECTIVES: To perform unbiased analysis of fever patterns and to investigate their association with clinical manifestations and outcome of patients with adult-onset Still's disease (AOSD). METHODS: AOSD patients who were treated as in-patients from 2004 through 2015 were grouped according to 24-hour body temperature (BT) by hierarchical clustering using a Euclidean distance metric with complete linkage. The clinical and laboratory characteristics of the groups were then examined. RESULTS: Hierarchical clustering partitioned 70 AOSD patients into three distinct groups. Group 1 (n=14) had the highest mean BT (38.1± 0.4°C) and the widest variation in BT (2.7±0.9°C). Group 2 (n=35) had a lower mean BT (37.4±0.3°C) and a smaller variation (2.1±0.7°C). Group 3 (n=21) had the lowest mean BT (36.7±0.3°C) and the smallest variation (1.5±0.6°C). Clinical features and extent of organ involvement did not differ significantly between groups. However, Group 1 had lower platelet counts and higher lactate dehydrogenase, ferritin levels, and prothrombin time than the other groups. In addition, Group 1 exhibited higher risk of having a macrophage activation syndrome (MAS) and tended to require more intense treatment with corticosteroids and immunosuppressant to achieve clinical remission as compared to other groups. CONCLUSIONS: Hierarchical clustering identified three distinct fever patterns in patients with AOSD. Higher BT was associated with wider variations in diurnal temperature, higher risk of developing MAS, more intense treatment, and longer time to clinical remission, suggesting that fever pattern is a prognostic factor for AOSD.


Assuntos
Regulação da Temperatura Corporal , Ritmo Circadiano , Febre/etiologia , Doença de Still de Início Tardio/complicações , Corticosteroides/uso terapêutico , Adulto , Idoso , Biomarcadores/sangue , Análise por Conglomerados , Feminino , Febre/diagnóstico , Febre/tratamento farmacológico , Febre/fisiopatologia , Humanos , Imunossupressores/uso terapêutico , Síndrome de Ativação Macrofágica/etiologia , Masculino , Pessoa de Meia-Idade , Reconhecimento Automatizado de Padrão , Indução de Remissão , Estudos Retrospectivos , Doença de Still de Início Tardio/diagnóstico , Doença de Still de Início Tardio/tratamento farmacológico , Doença de Still de Início Tardio/fisiopatologia , Fatores de Tempo , Resultado do Tratamento , Aprendizado de Máquina não Supervisionado
8.
J Ayub Med Coll Abbottabad ; 30(2): 289-292, 2018 Apr-Jun.
Artigo em Inglês | MEDLINE | ID: mdl-29938438

RESUMO

Macrophage activation syndrome (MAS) is a potentially lethal complication of chronic rheumatological conditions like ankylosing spondylitis, rheumatoid arthritis, and adult-onset Still's disease (AOSD). It is a multisystem inflammatory syndrome caused by immense cytokine release from activated lymphocytes and macrophages. We give an account of the incidence of a twenty years old Asian girl suffering from non-remitting fever and an evanescent rash for last ten weeks. Physical examination and laboratory work-up suggested high grade fever, pancytopenia, hyperferritinemia, hypertriglyceridemia, hypofibrinogenemia and impaired liver function. Bone marrow biopsy was also done. It showed active hemophagocytosis. She was diagnosed as a case of Macrophage Activation Syndrome associated with Adult Onset Still's disease. She was treated with high dose steroids and cyclosporine and recovered completely.


Assuntos
Síndrome de Ativação Macrofágica/etiologia , Doença de Still de Início Tardio/complicações , Biópsia , Células da Medula Óssea/patologia , Citocinas/sangue , Feminino , Humanos , Síndrome de Ativação Macrofágica/sangue , Síndrome de Ativação Macrofágica/diagnóstico , Doença de Still de Início Tardio/diagnóstico , Adulto Jovem
9.
Gerodontology ; 35(2): 139-142, 2018 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-29733534

RESUMO

OBJECTIVE: To present a case of Adult-onset Still's disease (AOSD) initially suspected to be odontogenic inflammation. BACKGROUND: Adult-onset Still's disease is a rare, complex autoinflammatory disease and a known cause of fever of unknown origin. MATERIALS AND METHODS: The patient had both a fever and dental pain. Following meticulous examination, the patient was diagnosed with AOSD. CONCLUSION: Clinicians should keep in mind that a patient such as AOSD may visit their clinics.


Assuntos
Infecção Focal Dentária/diagnóstico , Doença de Still de Início Tardio/diagnóstico , Contagem de Células Sanguíneas , Testes de Coagulação Sanguínea , Diagnóstico Diferencial , Infecção Focal Dentária/diagnóstico por imagem , Infecção Focal Dentária/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Periodontite Periapical/diagnóstico , Periodontite Periapical/patologia , Radiografia Panorâmica , Doença de Still de Início Tardio/diagnóstico por imagem , Doença de Still de Início Tardio/patologia
10.
Clin Exp Rheumatol ; 36 Suppl 110(1): 10-24, 2018 Jan-Feb.
Artigo em Inglês | MEDLINE | ID: mdl-29742054

RESUMO

Fever of unknown origin (FUO) can be caused by a wide group of diseases, and can include both benign and serious conditions. Since the first definition of FUO in the early 1960's, several updates to the definition, diagnostic and therapeutic approaches have been proposed. This review outlines a case report of an elderly Italian male patient with high fever and migrating arthralgia who underwent many procedures and treatments before a final diagnosis of Adult-onset Still's disease was achieved. This case report highlights the difficulties in diagnosing certain causes of FUO that requires a very high index of suspicion. The main causes of FUO in paediatric and adult patients will be reviewed here, underlying the fact that a physician should also consider the possibility that a patient with FUO may have a monogenic autoinflammatory disease (AID). The identification of AIDs requires a careful evaluation of both history and clinical details that may reveal important clues to identify the correct aetiology. We also provide a comprehensive account of specific signs and symptoms that could suggest possible diagnoses and guide the work-up of FUO and non-genetic periodic fevers in children.


Assuntos
Febre de Causa Desconhecida/etiologia , Doença de Still de Início Tardio/diagnóstico , Adulto , Idoso , Algoritmos , Artralgia/etiologia , Criança , Diagnóstico Diferencial , Exantema/etiologia , Humanos , Masculino , Pseudolinfoma/etiologia , Doença de Still de Início Tardio/complicações
11.
An Bras Dermatol ; 93(2): 271-273, 2018 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-29723378

RESUMO

Adult-onset Still's disease is a systemic inflammatory disorder of unknown etiology, characterized by skin rash, spiking fever, arthralgias or arthritis, and leukocytosis. The typical skin rash is evanescent, salmon-pink, nonpruritic and maculopapular, predominantly on the extremities. It is considered one of the major Yamaguchi's criteria in adult-onset Still's disease. However, atypical skin lesions are also described. Here, a 61-year-old woman with sore throat, spiking fever, polyarthritis and evanescent salmon-pink nonpruritic maculopapular skin rash on the extremities was diagnosed with adult-onset Still's disease. In addition, atypical brown macules on oral mucosa, localized on the inner lips and tongue were also observed. Biopsy revealed a neutrophilic infiltrate. Despite treatment and improvement of the adult-onset Still's disease, the atypical oral mucosal lesions persisted.


Assuntos
Doenças da Boca/patologia , Mucosa Bucal/patologia , Doença de Still de Início Tardio/patologia , Biópsia , Exantema/patologia , Feminino , Humanos , Lábio/patologia , Pessoa de Meia-Idade , Doenças da Boca/diagnóstico , Infiltração de Neutrófilos , Doença de Still de Início Tardio/diagnóstico , Língua/patologia
12.
Med. clín (Ed. impr.) ; 150(9): 348-353, mayo 2018. ilus, tab
Artigo em Espanhol | IBECS | ID: ibc-173388

RESUMO

La enfermedad de Still del adulto es una entidad infrecuente a medio camino entre los síndromes autoinflamatorios y las enfermedades autoinmunes, con una gran heterogeneidad en su presentación clínica, gravedad y evolución. En este artículo se revisan las novedades y aspectos menos conocidos de esta enfermedad referidos a su espectro clínico (lesiones cutáneas atípicas, complicaciones distintas de las manifestaciones clásicas, síndrome de activación macrofágica, fenotipos de la enfermedad), a la controversia existente acerca de su posible asociación con neoplasias, la búsqueda de biomarcadores para su diagnóstico, la evaluación del pronóstico (factores clínicos, índices pronósticos y biomarcadores) y los factores determinantes en la elección del tratamiento biológico


Adult onset Still's disease is a rare systemic condition at the crossroads between auto-inflammatory syndromes and autoimmune diseases, with considerable heterogeneity in terms of clinical presentation, evolution and severity. This article reviews the main advances and lesser known aspects of this entity related to its clinical spectrum (atypical cutaneous lesions, unusual manifestations, macrophage activation syndrome, disease phenotypes), the emerging controversy around its association with delayed malignancy, the search for new biomarkers for its diagnosis, evaluation of prognosis (clinical factors, prognostic indexes and biomarkers to identify patients at risk of severe organ failure or life-threatening complications), and the determinants in the choice of biological treatment


Assuntos
Humanos , Adulto , Doença de Still de Início Tardio/complicações , Doença de Still de Início Tardio/diagnóstico , Doença de Still de Início Tardio/tratamento farmacológico , Prognóstico , Biomarcadores , Artrite Reumatoide
13.
Crit Care ; 22(1): 88, 2018 Apr 11.
Artigo em Inglês | MEDLINE | ID: mdl-29642928

RESUMO

BACKGROUND: Adult-onset Still disease (AOSD) is a rare systemic inflammatory disorder. A few patients develop organ complications that can be life-threatening. Our objectives were to describe the disease course and phenotype of life-threatening AOSD, including response to therapy and long-term outcome. METHODS: A multicenter case series of intensive care medicine (ICU) patients with life-threatening AOSD and a systematic literature review. RESULTS: Twenty patients were included. ICU admission mostly occurred at disease onset (90%). Disease manifestations included fever (100%), sore throat (65%), skin rash (65%), and arthromyalgia (55%). Serum ferritin was markedly high (median: 29,110 ng/mL). Acute respiratory failure, shock and multiple organ failure occurred in 15 (75%), 10 (50%), and 7 (35%) cases, respectively. Hemophagocytosis was demonstrated in eight cases. Two patients died. Treatment delay was significant. All patients received corticosteroids. Response rate was 50%. As second-line, intravenous immunoglobulins were ineffective. Anakinra was highly effective. After ICU discharge, most patients required additional treatment. Literature analysis included 79 cases of AOSD with organ manifestations, which mainly included reactive hemophagocytic syndrome (42%), acute respiratory failure (34%), and cardiac complications (23%). Response rate to corticosteroids was 68%. Response rates to IVIgs, cyclosporin, and anakinra were 50%, 80%, and 100%, respectively. CONCLUSIONS: AOSD should be recognized as a rare cause of sepsis mimic in patients with fever of unknown origin admitted to the ICU. The diagnosis relies on a few simple clinical clues. Early intensive treatment may be discussed. IVIgs should be abandoned. Long-term prognosis is favorable.


Assuntos
Doença de Still de Início Tardio/diagnóstico , Doença de Still de Início Tardio/terapia , Adolescente , Corticosteroides/uso terapêutico , Adulto , Ciclosporina/uso terapêutico , Feminino , França , Humanos , Imunoglobulinas/uso terapêutico , Unidades de Terapia Intensiva/organização & administração , Unidades de Terapia Intensiva/estatística & dados numéricos , Masculino , Pessoa de Meia-Idade , Insuficiência de Múltiplos Órgãos/etiologia , Insuficiência de Múltiplos Órgãos/mortalidade , Prognóstico , Escala Psicológica Aguda Simplificada , Estatísticas não Paramétricas , Doença de Still de Início Tardio/mortalidade , Tomografia Computadorizada por Raios X/métodos
14.
Mod Rheumatol ; 28(5): 736-757, 2018 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-29651907

RESUMO

OBJECTIVES: Using an expert- and data-driven methodology, we have constructed the first clinical practice guidelines (CPGs) for adult Still's disease (ASD) after complete systematic review (SR) of the literature based upon the Medical Information Network Distribution Service (Minds) procedure. METHODS: The CPG committee for ASD organized by the Research Team for Autoimmune Diseases, the Research Program for Intractable Disease of the Japanese Ministry of Health, Labour, and Welfare has developed CPG for ASD 2017, according to the procedure proposed by Minds. The CPG development process includes (1) clarification of the purpose of CPG, (2) organization of the steering committee, (3) organization of the CPG committee and secretariat, (4) defining the scope (setting of clinical questions (CQs)), (5) SR, (6) development of recommendations, (7) drafting the CPG, (8) external evaluation and public comments, and (9) release. Because we wanted to construct CPG for ASD to encompass both adult-onset Still's disease (AOSD) and adult patients with systemic juvenile idiopathic arthritis (sJIA), we also included SR data from sJIA in this study. RESULTS: Twenty-six CQs were selected and roughly divided into the following items: (1) clinical findings (CQs 1-4), (2) laboratory findings (CQs 5-8), (3) complications (CQs 9-13), (4) treatment with oral medicine (CQs 14-19), (5) treatment with biological reagents (CQs 20-23), and (6) treatments for sJIA (CQs 25-26). Recommendations and the strength of the recommendations for these CQs were decided by a modified Delphi method. CONCLUSION: We have developed the first published CPG for ASD including AOSD and sJIA, which includes 26 CQs and recommendations. This guideline will help rheumatologists, non-specialized physicians, other healthcare providers, medical and health-related students, and patients and their family members to understand and treat ASD.


Assuntos
Medicina Baseada em Evidências/métodos , Guias de Prática Clínica como Assunto , Doença de Still de Início Tardio/tratamento farmacológico , Medicina Baseada em Evidências/normas , Humanos , Doença de Still de Início Tardio/diagnóstico
15.
Medicine (Baltimore) ; 97(15): e0333, 2018 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-29642170

RESUMO

RATIONALE: Familial Mediterranean fever (FMF) and adult onset Still's disease (AOSD) have overlapping features, and are categorized as being on the spectrum of autoinflammatory diseases (AIDs). FMF is more prevalent in the Mediterranean region but rarely, described in the Chinese population. PATIENT CONCERNS: We present an interesting case of a Han Chinese AOSD patient with episodic fever, wheals, and polyarthritis for 2 years. DIAGNOSIS: Sequencing analysis found exon 2 mutations of the MEFV gene (c.329T>C [L110P], and c.442G>C [E148Q]). INTERVENTION: Her arthritis was well-controlled with colchicine treatment, but fever, and rashes were not. OUTCOMES: She eventually received tocilizumab, in addition to colchicine, and her symptoms completely disappeared. LESSONS: MEFV mutations may exist in AOSD patients, and treatment with colchicine might be helpful in such patients.


Assuntos
Anticorpos Monoclonais Humanizados/uso terapêutico , Colchicina/uso terapêutico , Análise Mutacional de DNA , Pirina/genética , Doença de Still de Início Tardio/tratamento farmacológico , Doença de Still de Início Tardio/genética , Adulto , Quimioterapia Combinada , Éxons/genética , Feminino , Humanos , Análise de Sequência de DNA , Doença de Still de Início Tardio/diagnóstico
16.
Pediatr Rheumatol Online J ; 16(1): 9, 2018 Jan 25.
Artigo em Inglês | MEDLINE | ID: mdl-29370863

RESUMO

BACKGROUND: Adult-onset Still's disease (AOSD) is an adult form of systemic juvenile idiopathic arthritis (JIA) that differs from the latter in its classification. This study evaluated the concordance between the International League Against Rheumatism (ILAR) criteria for systemic JIA and the Yamaguchi criteria and then compared their possible prognostic value in patients with AOSD. METHODS: In a retrospective review of 169 adults with suspected AOSD, patients were classified according to the Yamaguchi or ILAR criteria. Then the concordance in cross-referencing the other group with the different criteria was investigated and the sensitivity and specificity of each set of criteria were determined. Disease activity markers in AOSD patients were correlated with positivity according to both systems. RESULTS: Concordance was good in patients with suspected AOSD (k = 0.7144, p <  0.001) and low in those with a diagnosis of AOSD (k = 0.3787, p <  0.001). The sensitivity of the ILAR criteria in AOSD patients was 0.8864 (95% confidence interval (CI): 0.8322-0.9405), and the specificity was 0.7838 (0.6511, 0.9164). Positivity according to the ILAR criteria correlated with the systemic score (r = 0.763, p <  0.0001) and C-reactive protein levels (r = 0.183, p = 0.0356) and was associated with a relapse (odds ratio: 1.589, 95% CI: 1.043-2.421), macrophage activation syndrome (MAS; odds ratio: 1.993, 95% CI: 1.218-3.263) and care in the intensive care unit (ICU; odds ratio: 2.087, 95% CI: 1.086-4.011). CONCLUSIONS: In AOSD patients, there is fair concordance between the Yamaguchi and ILAR criteria for systemic JIA. Positive ILAR criteria may be useful for identifying AOSD patients at high risk for relapse, MAS and the need for ICU care. Further studies including larger populations from several centers are needed to confirm our results regarding the utility of the ILAR criteria in AOSD patients.


Assuntos
Artrite Juvenil/diagnóstico , Doença de Still de Início Tardio/diagnóstico , Adulto , Biomarcadores Tumorais , Diagnóstico Diferencial , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Sensibilidade e Especificidade , Doença de Still de Início Tardio/tratamento farmacológico
19.
Ocul Immunol Inflamm ; 26(2): 286-291, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-27599266

RESUMO

Adult-onset Still's disease (AOSD) is a rare multisystemic immune-mediated disease of unknown etiology with quotidian spiking fever, evanescent rash, arthralgia, and multiple organ involvement. The few AOSD cases that have been reported developed Purtscher's-like retinopathy associated with thrombotic microangiopathy (TMA). Here, we report Purtscher's-like retinopathy without TMA in a patient with AOSD. A 29-year-old-man who presented for evaluation of blurred vision was diagnosed with AOSD based on Yamaguchi criteria. He had Purtscher's-like retinopathy in his right eye. Lesions improved after steroid treatment. Although almost all reported AOSD cases with Purtscher's-like retinopathy are associated with TMA, in this case such a complication was not encountered.


Assuntos
Doenças Retinianas/diagnóstico , Doença de Still de Início Tardio/diagnóstico , Adulto , Corantes/administração & dosagem , Quimioterapia Combinada , Angiofluoresceinografia , Glucocorticoides/uso terapêutico , Humanos , Imunossupressores/uso terapêutico , Verde de Indocianina/administração & dosagem , Masculino , Prednisolona/uso terapêutico , Pregnenodionas/uso terapêutico , Doenças Retinianas/tratamento farmacológico , Doenças Retinianas/fisiopatologia , Doença de Still de Início Tardio/tratamento farmacológico , Doença de Still de Início Tardio/fisiopatologia , Tomografia de Coerência Óptica
20.
Semin Arthritis Rheum ; 47(4): 578-585, 2018 02.
Artigo em Inglês | MEDLINE | ID: mdl-28760536

RESUMO

OBJECTIVES: To validate the Fautrel classification criteria for adult-onset Still's disease (AOSD) and to compare the discriminative performance to that of the Yamaguchi criteria. METHODS: We retrospectively reviewed the medical charts of 426 patients who had serum ferritin level and percentage glycosylated ferritin assayed at the biochemistry laboratory of Bichat Hospital. Medical data were extracted by use of a standardized form. All clinical, biological, and imaging features were collected, as well, evidence favoring an alternative diagnosis, specifically symptoms suggestive of other immune-mediated inflammatory diseases (IMID) or active infections. Patients were classified as AOSD patients or controls according to a predefined procedure, including consultation with a multidisciplinary expert group. Algorithms corresponding to the Fautrel and Yamaguchi classification criteria were applied for each patient. RESULTS: In all, 54 AOSD and 278 control patients were included. For the Fautrel criteria, the sensitivity was 87.0%, specificity 97.8%, and positive and negative predictive values 88.7% and 97.5%, respectively. For the standard Yamaguchi set-without strict application of exclusion criteria-the sensitivity was 96.3%, specificity 98.9%, and positive and negative predictive values 94.5% and 99.3%, respectively. If we applied a stricter definition of exclusion criteria, the sensitivity of the Yamaguchi set decreased to 31.5%. As wall, 37 AOSD diagnoses were missed. CONCLUSION: This study validates the Fautrel classification criteria with a cohort independent of that used for the original publication. This criteria set demonstrates good sensitivity and specificity, overcomes exclusion criteria, and includes glycosylated ferritin level. It also confirms the high discriminative power of the Yamaguchi criteria, albeit substantially affected by how exclusion criteria are interpreted.


Assuntos
Doença de Still de Início Tardio/diagnóstico , Adulto , Diagnóstico Diferencial , Feminino , Ferritinas/sangue , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Sensibilidade e Especificidade , Doença de Still de Início Tardio/sangue , Adulto Jovem
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