RESUMEN
Renal involvement is underestimated as an extramuscular manifestation of dermatomyositis (DM). Here, we describe a 67-year-old woman with anti-glycyl-transfer ribonucleic acid synthetase (anti-EJ) antibody and anti-ribonucleoprotein antibody-positive DM complicated by systemic sclerosis, who developed nephrotic syndrome concurrently with the exacerbation of DM, as indicated by incremental serum creatine kinase levels, high-intensity lesions on muscle magnetic resonance imaging, and active interstitial pneumonitis on chest computed tomography. Renal biopsy revealed the presence of immune-deposition in the glomerulus by immunofluorescence. To our knowledge, this is the first report describing the coexistence of anti-EJ antibody-positive DM and nephrotic syndrome. More reports of similar cases are warranted to substantiate the association.
RESUMEN
BACKGROUND: Discordance between patient global assessment (PGA) and physician global assessment (PhGA) of rheumatoid arthritis (RA) disease activity is mainly determined by pain and functional disabilities. This study aimed to investigate the shift in PGA-PhGA discordance and the variables associated with future positive discordance (PGA > PhGA) based on the NinJa database in Japan. METHODS: We examined 7557 adults with RA registered in both NinJa 2014 and 2018, with a discordance cutoff of 3 on a 10-cm scale. The affected joint distribution was investigated using the joint indices x, y, and z, which were calculated as indices for the upper joint, lower joint, and large joint involvement, respectively. The variables in NinJa 2014 that were associated with positive discordance in NinJa 2018 were examined using binary stepwise logistic regression analysis. RESULTS: Due to the small number of patients with RA categorized as having negative discordance (PGA < PhGA), we focused on patients with RA categorized as having either concordance or positive discordance. Logistic regression analysis revealed that positive discordance in NinJa 2018 was associated with age, pain, modified Health Assessment Questionnaire (mHAQ) score, corticosteroid use, and existent positive discordance and was inversely associated with C-reactive protein (CRP) and x at baseline (NinJa 2014). The same findings were observed when patients with RA were divided based on the discordance status at baseline. Persistence (positive discordance to positive discordance) was associated with pain and mHAQ scores but inversely associated with CRP. CONCLUSIONS: Positive discordance may persist. Circumventing this requires adequate management of pain and functional impairment.
Asunto(s)
Artritis Reumatoide , Médicos , Adulto , Humanos , Artritis Reumatoide/diagnóstico , Artritis Reumatoide/epidemiología , Japón/epidemiología , Dolor , Índice de Severidad de la EnfermedadRESUMEN
We investigated the relationship between distal interphalangeal (DIP) joint involvement and disease activity in 10,038 patients with adult-onset rheumatoid arthritis (RA). The affected joint distribution was investigated using the joint indices (JI) x, y, and z, corresponding to the upper and lower joints, and the predominance of large-joint involvement, respectively. DIP joint involvement (defined by the presence of tenderness and/or swelling in DIP joints) was present in 206 (2.1%) of 10,038 patients with RA. Patients with RA exhibiting DIP joint involvement were significantly younger, and more frequently women. DIP joint involvement was positively associated with Disease Activity Score-28 using C-reactive protein, and clinical variables related to high RA disease activity, including JIs x and y, and was negatively associated with JI z. JI x was significantly higher than JI y in RA patients with DIP joint involvement. An odds ratio analysis revealed that small-to-medium sized and upper-extremity joints ranked first, second, and fourth among the eight variables significantly associated with DIP joint involvement. The correlation coefficients revealed that small-sized and upper-extremity joints ranked first and second among the five significant variables. DIP joint involvement, albeit rare, is significantly associated with high RA disease activity with predominance of small-sized and upper-extremity joints.
RESUMEN
Neuropsychiatric (NP) manifestations of systemic lupus erythematosus (SLE) are diverse, but involvement of basal ganglia is rare. We describe here a 28-year-old woman with NPSLE presenting aseptic meningitis accompanied by elevated interleukin-6 levels in the cerebrospinal fluid, who developed symmetrical basal ganglia lesions, containing a cytotoxic oedematous core, surrounded by vasogenic oedema upon magnetic resonance imaging. We were able to observe these lesions from a de novo appearance during the disease onset to its disappearance during immunosuppressive treatment. Reversibility upon immunosuppressive treatment indicated that autoimmune mediated mechanisms could contribute to the basal ganglia lesions in NPSLE.
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Ganglios Basales/patología , Edema/diagnóstico , Edema/etiología , Vasculitis por Lupus del Sistema Nervioso Central/diagnóstico , Adulto , Ganglios Basales/efectos de los fármacos , Edema/tratamiento farmacológico , Femenino , Humanos , Inmunosupresores/farmacología , Inmunosupresores/uso terapéutico , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/diagnóstico , Lupus Eritematoso Sistémico/tratamiento farmacológico , Vasculitis por Lupus del Sistema Nervioso Central/tratamiento farmacológico , Vasculitis por Lupus del Sistema Nervioso Central/etiología , Evaluación de SíntomasRESUMEN
We describe a case of eosinophilic temporal arteritis in a 61-year-old woman with hypereosinophilic syndrome, who developed subcutaneous nodules in the temporal areas and digital cyanosis with small nodules on the sides of her fingers. Ultrasound revealed occlusion and corkscrew-like changes of the temporal and digital arteries, respectively. Temporal artery biopsy revealed eosinophilic vasculitis without giant cell formation. Angiography showed occlusion of the ulnar and digital arteries. Administration of low-dose corticosteroid improved the temporal artery swelling and digital cyanosis. More reports of similar cases are required to characterize this type of non-giant cell eosinophilic vasculitis that affects the peripheral arteries.
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Arteritis de Células Gigantes/complicaciones , Síndrome Hipereosinofílico/complicaciones , Enfermedades Vasculares Periféricas/complicaciones , Biopsia , Femenino , Dedos/irrigación sanguínea , Arteritis de Células Gigantes/patología , Humanos , Síndrome Hipereosinofílico/patología , Persona de Mediana Edad , Enfermedades Vasculares Periféricas/patología , Arterias Temporales/patologíaRESUMEN
BACKGROUND: Previous studies suggest that RA activity is sensitive to seasonal changes. This study explored the influence of season on RA activity, particularly the distribution of affected joints, using a nationwide database in Japan. METHODS: We investigated 12,839 patients whose RA activity was recorded in spring (n = 3250), summer (n = 916), fall (n = 1021), and winter (n = 7652). Disease activity score (DAS) 28-CRP, simplified disease activity index (SDAI), and clinical disease activity index (CDAI) were used as indices of disease activity. Disease activity was also assessed according to DAS28-CRP scores (remission, low, moderate, or high). The affected joint distribution was investigated using novel joint indices (x, y, z), where x and y are indices for the upper and lower joints, respectively, and z is the index for large joint predominance. RESULTS: Mean DAS28-CRP and median SDAI and CDAI scores were highest in spring and lowest in fall. There was a significant difference in the DAS28-CRP for fall versus spring and winter. Fall was associated with a higher remission rate, and spring and winter with high and moderate RA activity, respectively. Significant differences in x, y, SDAI, and CDAI scores were found for spring versus summer, fall, and winter, in addition to fall versus winter (except in y). There was no seasonal difference in the z index. CONCLUSIONS: RA activity in the upper and lower extremities may be highest in spring, followed by winter. Seasonal changes should be considered in patients with RA to better understand their symptoms.
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Artritis Reumatoide/diagnóstico , Artritis Reumatoide/epidemiología , Estaciones del Año , Índice de Severidad de la Enfermedad , Anciano , Bases de Datos Factuales/tendencias , Progresión de la Enfermedad , Femenino , Humanos , Japón/epidemiología , Articulaciones/patología , Masculino , Persona de Mediana EdadRESUMEN
OBJECTIVES: The discordance between patient global assessment (PGA) and physician global assessment (PhGA) of rheumatoid arthritis (RA) disease activity may be problematic in clinical practice. The aim of this study was to identify determinants of this discordance using a nationwide RA database in Japan (NinJa) with special attention to large joint involvement. METHODS: We investigated 12 043 adults with RA and used a discordance cutoff of 3 cm. Large joint involvement was investigated using novel joint indices (x, y, z), where x and y were the indices for upper and lower joints, respectively, and z was for large joint predominance. Predictors of PGA-PhGA discordance and determinants of PGA and PhGA were analyzed by multivariate logistic and linear regression models, respectively. RESULTS: Multivariate logistic regression identified age, pain and high modified Health Assessment Questionnaire score as predictors of positive discordance (PGA ≥ PhGA), whereas parameters of disease activity in RA (C-reactive protein, x and y), class 3-4 functional status, and z were found to predict against positive discordance. Linear regression analysis revealed that PGA was mainly determined by pain, whereas PhGA was determined by various other factors. CONCLUSIONS: RA care providers should focus on pain and functional disability to decrease PGA-PhGA discordance. High disease activity and large joint involvement decreased PGA-PhGA discordance, indicating that the number and distribution of affected joints influenced the perception of disease activity by patients with RA and their physicians.
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Artritis Reumatoide/diagnóstico , Evaluación de la Discapacidad , Articulaciones/patología , Dimensión del Dolor , Pacientes/psicología , Médicos/psicología , Autoinforme , Anciano , Anciano de 80 o más Años , Artritis Reumatoide/patología , Artritis Reumatoide/psicología , Biomarcadores/sangre , Proteína C-Reactiva/análisis , Distribución de Chi-Cuadrado , Bases de Datos Factuales , Femenino , Humanos , Japón , Modelos Lineales , Modelos Logísticos , Masculino , Persona de Mediana Edad , Análisis Multivariante , Variaciones Dependientes del Observador , Oportunidad Relativa , Valor Predictivo de las Pruebas , Reproducibilidad de los Resultados , Índice de Severidad de la EnfermedadRESUMEN
AIM: To determine whether the age at onset of rheumatoid arthritis (RA) has increased in Japan using a nationwide database (National Database of Rheumatic Diseases by iR-net in Japan, NinJa). METHOD: We analyzed the data of RA patients who had been diagnosed with early RA (disease duration < 2 years) and newly registered in 2003, 2008 or 2013. RESULTS: The numbers of patients who developed RA in 2002-2003, 2007-2008, and 2012-2013 were 536, 812 and 1864, respectively. The mean age at RA onset increased significantly from 55.8 years in 2002-2003 and 57.0 years in 2007-2008 to 59.9 years in 2012-2013. The peak age shifted from the 50-59 years age group in 2002-2003 to the 60-69 years age group in 2012-2013. There was no apparent difference in the age at RA onset between male and female RA patients. Notably, in the period 2002-2003, the prevalence of RA was markedly higher in the age group of 50-59 years, which included the first 'baby boomers', than in the age groups of 30-39 and 40-49 years, even with consideration of the variations in the age composition of the general population. CONCLUSIONS: We have demonstrated that the age at RA onset in Japan has increased significantly over the last decade. This can be attributed to Japan's aging population. In addition, the high prevalence of RA among the first baby boomers suggests that environmental factors might also have contributed to the increase in age at RA onset in Japan.