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1.
Haemophilia ; 30 Suppl 3: 120-127, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38578694

RESUMO

Gene therapy and universal use of safer, more effective, and personalised prophylactic regimens (factor, and nonfactor) are expected to prevent joint bleeding and promote joint health in persons with haemophilia (PwH). Growing evidence suggests that subclinical bleeding, with active and inactive synovial proliferation, continues and haemophilic arthropathy remains a major morbidity in PwH despite early institution of joint prophylaxis. Joint health assessment is evolving with physical examination scores complementing imaging scores. Point-of-care ultrasound is emerging as a safe, cost-effective, and readily available tool for acute determination of musculoskeletal abnormalities, serial evaluation of joints for sonographic markers of haemophilic arthropathy, and in providing objective insight into the efficacy of new therapies. In acute haemarthrosis, arthrocentesis expedites recovery and prevent the vicious cycle of bleed-synovitis-rebleed. When synovial proliferation develops, a multidisciplinary team approach is critical with haematology, orthopaedics, and physiotherapy involvement. Synovectomy is considered for patients with chronic synovitis that fail conservative management. Non-surgical and minimally invasive procedures should always be offered and considered first. Careful patient selection, screening and early intervention increase the success of these interventions in reducing bleeding, pain, and improving joint function and quality of life. Chemical synovectomy is practical in developing countries, but radioactive synovectomy appears to be more effective. When surgical synovectomy is considered, arthroscopic/minimally invasive approach should be attempted first. In advanced haemophilic arthropathy, joint replacement and arthrodesis can be considered. While excited about the future of haemophilia management, navigating musculoskeletal challenges in the aging haemophilia population is equally important.


Assuntos
Artrite , Hemofilia A , Sinovite , Humanos , Hemofilia A/complicações , Hemofilia A/terapia , Hemofilia A/diagnóstico , Qualidade de Vida , Hemartrose/diagnóstico , Hemartrose/etiologia , Hemartrose/terapia , Sinovite/diagnóstico , Sinovite/etiologia , Sinovite/terapia , Envelhecimento , Artrodese
2.
BMJ Case Rep ; 17(3)2024 Mar 15.
Artigo em Inglês | MEDLINE | ID: mdl-38490701

RESUMO

We present the case of an elderly man with a small-joint polyarthritis, accompanied by pitting oedema, involving hands and feet, raising clinical suspicion of remitting seronegative symmetrical synovitis with pitting oedema (RS3PE). Treatment with corticosteroids was initiated with significant improvement, but unacceptable iatrogeny ensued, and tapering was not possible without disease flare-up. A trial of tocilizumab allowed disease activity control, slow weaning of corticosteroids and, ultimately, its suspension. RS3PE is a rare rheumatological entity, initially thought to be a variant of rheumatoid arthritis (RA), with shared traits with polymyalgia rheumatica (PMR), and other seronegative spondyloarthropathies, thereby implying a shared pathophysiological background. Elevated levels of interleukin 6 (IL-6) are found in patients with RA, have shown to mirror disease activity in PMR and have also been described in the serum and synovial fluid of patients with RS3PE. Tocilizumab, an anti-IL-6 receptor antibody, shows auspicious results in several other rare rheumatic diseases other than RA.


Assuntos
Artrite Reumatoide , Polimialgia Reumática , Sinovite , Masculino , Humanos , Idoso , Sinovite/diagnóstico , Sinovite/tratamento farmacológico , Sinovite/complicações , Polimialgia Reumática/complicações , Artrite Reumatoide/complicações , Artrite Reumatoide/tratamento farmacológico , Corticosteroides/uso terapêutico , Edema/tratamento farmacológico , Edema/complicações
3.
Curr Med Imaging ; 20: 1-9, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38389340

RESUMO

BACKGROUND: Rheumatoid Arthritis Magnetic Resonance Imaging Score (RAMRIS) is usually used for the semi-quantitative evaluation of joint changes in Rheumatoid Arthritis (RA). However, this method cannot evaluate early changes in bone marrow edema (BME). OBJECTIVE: To determine whether T1 mapping of wrist BME predicts early treatment response in RA. METHODS: This study prospectively enrolled 48 RA patients administered oral anti-rheumatic drugs. MRI of the most severely affected wrist was performed before and after 4 (48 patients) and 8 weeks of treatment (38 patients). Mean T1 values of BME in the lunate, triangular, and capitate bones; RAMRIS for each wrist; Erythrocyte-Sedimentation Rate (ESR); and 28-joint Disease Activity Score (DAS28)-ESR score were analyzed. Patients were divided into responders (4 weeks, 30 patients; 8 weeks, 32 patients) and non-responders (4 weeks, 18 patients; 8 weeks, 6 patients), according to EULAR response criteria. Receiver operating characteristic (ROC) curves were used to evaluate the efficacy of T1 values. RESULTS: ESR and DAS28-ESR were not correlated with T1 value and RAMRIS at each examination (P > 0.05). Changes in T1 value and DAS28-ESR relative to the baseline were moderately positively correlated with each other at 4 and 8 weeks (r = 0.555 and 0.527, respectively; P < 0.05). At 4 weeks, the change and rate of change in T1 value significantly differed between responders and non-responders (-85.63 vs. -19.92 ms; -12.89% vs. -2.81%; P < 0.05). The optimal threshold of the rate of change in T1 value at 4 weeks for predicting treatment response was -5.32% (area under the ROC curve, 0.833; sensitivity, 0.900; specificity, 0.667). CONCLUSION: T1 mapping provides a new imaging method for monitoring RA lesions; changes in wrist BME T1 values reflect early treatment response.


Assuntos
Artrite Reumatoide , Sinovite , Humanos , Sinovite/diagnóstico , Sinovite/patologia , Artrite Reumatoide/diagnóstico por imagem , Artrite Reumatoide/tratamento farmacológico , Imageamento por Ressonância Magnética/métodos , Articulação do Punho/diagnóstico por imagem , Articulação do Punho/patologia , Edema/diagnóstico , Edema/patologia , Espectroscopia de Ressonância Magnética
6.
Int J Rheum Dis ; 27(1): e14878, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37592395

RESUMO

Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) is a rare chronic inflammatory disease that develops in adults. We present a case of SAPHO syndrome in a 37-year-old male presenting with gradually worsening back and neck pain for a 7-year period. The episodes were preceded by a history of pustular skin eruptions, which first appeared on the upper trunk and then involved his face and were pustular and scarring. The purpose of presenting this case report from Iraq is to raise awareness about this rare condition, which is frequently misdiagnosed and under-recognized.


Assuntos
Acne Vulgar , Síndrome de Hiperostose Adquirida , Osteíte , Sinovite , Masculino , Adulto , Humanos , Síndrome de Hiperostose Adquirida/complicações , Síndrome de Hiperostose Adquirida/diagnóstico , Síndrome de Hiperostose Adquirida/tratamento farmacológico , Sinovite/diagnóstico , Dor nas Costas/diagnóstico , Dor nas Costas/etiologia , Pele , Acne Vulgar/diagnóstico
7.
Scand J Rheumatol ; 53(2): 85-93, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-36974977

RESUMO

OBJECTIVE: To investigate the performance and factors of influence of optical spectral transmission (OST) imaging as a new technique for measuring joint inflammation in rheumatoid arthritis (RA). METHOD: OST was performed in 24 RA patients and 37 controls. Mann-Whitney U-test was used to assess differences in OST score between RA patients and controls. Receiver operating characteristics (ROC), linear regression and generalized estimating equations analysis were used to assess the discriminative capability of OST and the association of OST score with clinical disease parameters, ultrasound, radiographic features and cardiovascular risk parameters. RESULTS: Median OST score was higher in RA patients than in controls [16.9 (interquartile range 12.77-19.7) vs 12.11 (10.32-14.93)]. At patient level, OST score was moderately associated with ultrasound [beta 0.38 (95% CI 0.16-0.60), p = 0.001] and clinical disease activity [28-joint Disease Activity Score-C-reactive protein beta 0.30 (95% CI 0.04- 0.57), p = 0.024] in RA patients. In controls, male sex, high body mass index, and hypertension were associated with higher OST scores, while these associations were absent in RA. At joint level, the area under the ROC curve for OST score, with ultrasound or clinical swelling as reference, ranged from 0.63 to 0.70. Joint-space narrowing and malalignment were associated with higher OST joint scores, and subchondral sclerosis with lower scores. CONCLUSION: OST provides an objective measure of synovitis and correlates moderately with other examined disease activity assessment tools. Clinical patient characteristics must be considered when interpreting the results.


Assuntos
Artrite Reumatoide , Sinovite , Humanos , Masculino , Artrite Reumatoide/diagnóstico , Ultrassonografia/métodos , Sinovite/diagnóstico , Curva ROC , Índice de Gravidade de Doença
8.
Arthritis Res Ther ; 25(1): 231, 2023 11 30.
Artigo em Inglês | MEDLINE | ID: mdl-38037132

RESUMO

OBJECTIVE: The objective of this study was to investigate the relation between swelling and tenderness of individual finger joints and grip force in patients with early rheumatoid arthritis (RA). METHODS: In an inception cohort of patients with early RA (symptom duration < 12 months), all patients were examined by the same rheumatologist, and grip force was measured using the Grippit instrument at inclusion, 1 and 5 years. The average grip force values of each hand were evaluated and expressed as % of expected values, based on age- and sex-specific reference values. Linear regression analyses were used to assess the cross-sectional relation between the involvement of individual finger joints and grip force. In generalized estimating equations, the impact of time-varying synovitis/tenderness on grip force over time was estimated. Analyses were adjusted for wrist involvement, erythrocyte sedimentation rate, and patient-reported pain. RESULTS: In 215 patients with early RA, grip force was 39% of expected at diagnosis, and increased to 56% after 5 years. Synovitis of the first metacarpophalangeal (MCP) joint (60% and 69% at baseline in the right and left hand) was associated with reduced grip force at inclusion (adjusted ß - 9.2 percentage unit of expected grip force; 95% CI - 13.6 to - 4.8 for both hands combined) and at all follow-up evaluations. Synovitis of MCP I and MCP IV (12% at baseline) was significantly associated with reduced grip force over time in both hands. Proximal interphalangeal (PIP) joint swelling, and tenderness of MCP or PIP joints, had less impact on grip force. CONCLUSION: MCP I synovitis is the major contributor to reduced grip force in patients with early RA. This underlines the importance of the involvement of the thumb for impaired hand function in RA. MCP IV synovitis, but not PIP involvement or finger joint tenderness, also has a substantial impact on grip force.


Assuntos
Artrite Reumatoide , Sinovite , Masculino , Feminino , Humanos , Articulações dos Dedos , Estudos de Coortes , Estudos Transversais , Sinovite/diagnóstico , Força da Mão
9.
Pediatr Rheumatol Online J ; 21(1): 142, 2023 Nov 24.
Artigo em Inglês | MEDLINE | ID: mdl-38001451

RESUMO

BACKGROUND: Limping is a common clinical symptom in childhood; different clinical conditions may lead to limping and the diagnosis of the underlying cause may often be a challenge for the pediatrician. CASE PRESENTATION: We describe the clinical manifestations, radiological pictures and disease course of other causes of limping in childhood, through a case series of seven cases and a brief discussion of each disease. CONCLUSIONS: although trauma is the most common cause of acute limping, when there is no history of traumatic events and the limping has a chronic course, Juvenile Idiopathic Arthritis is usually the most likely clinical diagnosis. However, other some rare conditions should be taken into account if JIA is not confirmed or if it presents with atypical clinical picture.


Assuntos
Artrite Juvenil , Sinovite , Humanos , Artrite Juvenil/complicações , Artrite Juvenil/diagnóstico , Sinovite/diagnóstico , Diagnóstico Diferencial
10.
Mil Med Res ; 10(1): 31, 2023 Jul 14.
Artigo em Inglês | MEDLINE | ID: mdl-37443101

RESUMO

Rheumatoid arthritis (RA) is a systemic autoimmune disease that is primarily manifested as synovitis and polyarticular opacity and typically leads to serious joint damage and irreversible disability, thus adversely affecting locomotion ability and life quality. Consequently, good prognosis heavily relies on the early diagnosis and effective therapeutic monitoring of RA. Activatable fluorescent probes play vital roles in the detection and imaging of biomarkers for disease diagnosis and in vivo imaging. Herein, we review the fluorescent probes developed for the detection and imaging of RA biomarkers, namely reactive oxygen/nitrogen species (hypochlorous acid, peroxynitrite, hydroxyl radical, nitroxyl), pH, and cysteine, and address the related challenges and prospects to inspire the design of novel fluorescent probes and the improvement of their performance in RA studies.


Assuntos
Artrite Reumatoide , Sinovite , Humanos , Corantes Fluorescentes/uso terapêutico , Artrite Reumatoide/diagnóstico por imagem , Artrite Reumatoide/tratamento farmacológico , Sinovite/diagnóstico , Biomarcadores , Espécies Reativas de Nitrogênio/uso terapêutico , Espécies Reativas de Oxigênio
11.
J Med Case Rep ; 17(1): 301, 2023 Jun 30.
Artigo em Inglês | MEDLINE | ID: mdl-37386644

RESUMO

BACKGROUND: Blau Syndrome (BS) is a rare autosomal dominant noncaseous granulomatous disease caused by mutations in the NOD2 gene. The disease is characterized by granulomatous dermatitis, symmetrical arthritis, and uveitis, which, if left untreated, can progress to blindness. The diagnosis of BS can be challenging because of its rarity and overlap with other rheumatologic disorders. Early detection of ocular involvement is critical to prevent vision loss and improve the prognosis of patients with BS. CASE PRESENTATION: In this report, we present a case of a five-year-old Chinese girl diagnosed with BS one year ago after presenting with a systemic rash and urinary calculi. Genetic testing was recommended by a physician, and a heterozygous mutation of the NOD2 gene c.1538T > C (p.M513T) was identified. Eight months ago, due to bilateral corneal punctate opacity, we had examined and diagnosed bilateral uveitis, bilateral corneal zonal degeneration, persistent fetal vasculature (PFV) in the right eye, and perivascular granuloma in the right eye. As a result, Vitrectomy was performed on the right eye, resulting in a significant improvement in visual acuity from 1/50 on the first day after surgery to 3/10 after 1 week. After 6 months, the visual acuity of the right eye was maintained at 3/20, but opacification of the lens posterior capsule was observed. Follow-up appointments are ongoing to monitor the condition of the affected eyes. Our report underscores the importance of prompt detection and management of ocular involvement in BS accompany with PFV to prevent vision loss and improve patient outcomes. CONCLUSIONS: This report details the case of a child diagnosed with BS who accompanied a periretinal granuloma and PFV in the right eye. Regrettably, the left eye was observed to have no light perception (NLP) with the fundus not being visible. The occurrence of ocular complications in patients with BS, must be closely monitored to prevent vision loss and enhance treatment outcomes. This case underscores the importance of prompt diagnosis and management of ocular complications in patients with BS to prevent further damage and optimize patient outcomes.


Assuntos
Artrite , Sarcoidose , Sinovite , Uveíte , Criança , Feminino , Humanos , Pré-Escolar , Artrite/diagnóstico , Sinovite/diagnóstico , Sinovite/cirurgia , Uveíte/diagnóstico , Sarcoidose/complicações , Sarcoidose/diagnóstico , Cegueira
12.
Int J Rheum Dis ; 26(10): 2080-2084, 2023 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-37246600

RESUMO

Blau syndrome (BS) is a rare genetic immune disease which commonly presents in childhood. Currently, the miss-rate of BS diagnosis is very high, and an effective clinical management of BS has not been well established. This case report depicts a 54-year-old male Chinese patient presenting with hand malformation, fever, skin rash and joint pain. His diagnosis was ultimately confirmed according to typical medical history and genetic analysis. This case report will further help clinicians to be aware of this rare clinical entity for correct diagnosis and proper treatment.


Assuntos
Artrite , Sarcoidose , Sinovite , Uveíte , Masculino , Humanos , Pessoa de Meia-Idade , Proteína Adaptadora de Sinalização NOD2/genética , Artrite/diagnóstico , Artrite/genética , Artrite/tratamento farmacológico , Sinovite/diagnóstico , Sinovite/genética , Sinovite/tratamento farmacológico , Uveíte/diagnóstico , Uveíte/tratamento farmacológico , Uveíte/genética , Sarcoidose/diagnóstico , Sarcoidose/genética , Mutação
13.
Rev Med Inst Mex Seguro Soc ; 61(3): 363-369, 2023 May 02.
Artigo em Espanhol | MEDLINE | ID: mdl-37216683

RESUMO

Background: RS3PE (remitting seronegative symmetrical synovitis with edema and pitting) is a rare entity of unknown etiology that has been related to genetic predisposition due to the presence of HLA-A2 in 50% of cases and less frequently HLA-B7. Its pathogenesis is unknown, but it has been related to growth factors, and some mediators (TNF, IL-6). It is common in elderly people and the course of this illness presents with acute symmetrical polyarthritis, accompanied by edema in hands and feet. The diagnosis requires a high index of suspicion and to differentiate it from other entities such as rheumatoid arthritis, complex regional pain syndrome, rheumatic polymyalgia, in addition to ruling out malignant neoplasms, since there are many reports of its association with both solid and hematological neoplasms, being of bad prognosis when there is association. When there is no association with cancer, it responds well to the use of low doses of steroids and its prognosis is usually favorable. Clinical case: 80-year-old woman with an acute onset with polyarthralgia, functional limitation associated with pitting edema in hands and feet. After approaching the patient and ruling out associated neoplasms, it was diagnosed RS3PE. It was managed with prednisone, observing a good response, with remission of the manifestations at 6 weeks and subsequent suspension of the steroid. Conclusions: RS3PE is a rare entity, and a high index of suspicion is required for the diagnosis. A complete approach is important to rule out cancer in patients affected with this syndrome. Prednisone continues to be the best therapeutic option.


Introducción: la RS3PE (sinovitis simétrica seronegativa remitente con edema y fóvea) es una entidad rara de etiología desconocida que se ha relacionado con predisposición genética por la presencia de HLA-A2 en el 50% de los casos y con menor frecuencia HLA-B7. Se desconoce su patogenia, pero se ha relacionado con factores de crecimiento y algunos mediadores (TNF, IL-6). Se presenta en personas de edad avanzada, cursa con poliartritis aguda simétrica, acompañada de edema en manos y pies. El diagnóstico requiere de un alto índice de sospecha y diferenciarlo de otras entidades como artritis reumatoide, síndrome de dolor regional complejo, polimialgia reumática, además de descartar neoplasias malignas, ya que existen muchos reportes de su asociación con neoplasias tanto sólidas como hematológicas y es de mal pronóstico cuando existe asociación. Cuando no existe asociación con cáncer tiene buena respuesta al uso de dosis bajas de esteroides y su pronóstico suele ser favorable. Caso clínico: mujer de 80 años con un cuadro de inicio agudo con poliartralgias, limitación funcional asociada a edema de manos y pies con fóvea. Después del abordaje y de descartar neoplasias asociadas, se diagnosticó RS3PE. Se manejó con prednisona y hubo buena respuesta, con remisión de las manifestaciones a las 6 semanas y suspensión posterior del esteroide. Conclusiones: la RS3PE es una entidad rara y para diagnosticarla se requiere un alto índice de sospecha. Es importante el abordaje completo para descartar cáncer en los pacientes afectados con este síndrome. La prednisona continúa siendo la mejor opción terapéutica.


Assuntos
Sinovite , Feminino , Humanos , Idoso , Idoso de 80 Anos ou mais , Prednisona/uso terapêutico , Sinovite/complicações , Sinovite/diagnóstico , Sinovite/tratamento farmacológico , Edema/etiologia , Edema/complicações , Síndrome , Mãos
14.
Am Fam Physician ; 107(5): 474-485, 2023 05.
Artigo em Inglês | MEDLINE | ID: mdl-37192073

RESUMO

A limp is a deviation from normal gait pattern, with pain as the presenting feature in about 80% of cases. The differential diagnosis is broad and includes congenital/developmental, infectious, inflammatory, traumatic (including nonaccidental), and, less commonly, neoplastic etiologies. Transient synovitis of the hip is the cause of a limp in the absence of trauma in 80% to 85% of children. It can be differentiated from septic arthritis of the hip by the absence of fever or ill-appearance and with laboratory testing that shows normal or only mildly elevated inflammatory markers and white blood cell count. If septic arthritis is suspected, joint aspiration should be performed urgently with ultrasound guidance and the aspirated fluid sent for Gram staining, culture, and cell count. Patient history, such as breech presentation at birth, and a leg-length discrepancy on physical examination may suggest developmental dysplasia of the hip. Pain reported primarily at night can occur with neoplasms. Hip pain in an adolescent who is overweight or has obesity may suggest slipped capital femoral epiphysis. Knee pain in an active adolescent may suggest Osgood-Schlatter disease. Radiography shows the degenerative femoral head changes in Legg-Calvé-Perthes disease. Abnormalities in bone marrow shown on magnetic resonance imaging indicate septic arthritis. A complete blood count with differential, erythrocyte sedimentation rate, and C-reactive protein should be obtained if infection or malignancy is suspected.


Assuntos
Artrite Infecciosa , Sinovite , Recém-Nascido , Adolescente , Criança , Humanos , Articulação do Quadril/diagnóstico por imagem , Articulação do Quadril/patologia , Sinovite/diagnóstico , Sinovite/patologia , Artrite Infecciosa/diagnóstico , Artrite Infecciosa/complicações , Dor/diagnóstico , Dor/etiologia , Marcha
15.
Am J Case Rep ; 24: e939650, 2023 Apr 26.
Artigo em Inglês | MEDLINE | ID: mdl-37185664

RESUMO

BACKGROUND Remitting seronegative symmetrical synovitis with pitting edema (RS3PE) is a rare condition with underlying polyarthritis, pitting edema, and negative rheumatoid factor. It can be associated with an underlying rheumatological condition or can present as a paraneoplastic syndrome with malignancy. We present a rare case of RS3PE associated with monoclonal gammopathy of undermined significance (MGUS). CASE REPORT A 62-year-old man presented in ambulatory medicine clinic with 3-month swelling of distal lower extremities that progressed to distal upper extremities. He had pain and morning stiffness in hands, left elbow, and left shoulder. Examination revealed 3+ pitting edema in bilateral hands, feet, legs, and thighs. Laboratory studies revealed normal blood counts and renal and liver functions. Erythrocyte sedimentation rate was normal; C-reactive protein was mildly elevated (0.7 mg/dL). Echocardiogram and computed tomography of chest, abdomen, and pelvis revealed mild splenomegaly (14.5 cm). Serum protein electrophoresis revealed IgG kappa monoclonal peak of 0.1 g/dL. Beta-2 microglobulin was elevated (7.4 mg/L); LDH was elevated (264 U/L). No lytic lesions were present in bones. RS3PE was diagnosed based on established diagnostic criteria. Prednisone produced significant improvement in swelling within 72 h of start; however, he required a longer duration of steroid treatment due to relapse and continued periodic MGUS surveillance. CONCLUSIONS Our case highlights the importance of awareness of this condition in general practice to help with timely diagnosis and intervention, as this condition is steroid responsive. Also, it is important to screen for underlying autoimmune condition, hematological, and solid organ malignancies with appropriate workup.


Assuntos
Artrite , Gamopatia Monoclonal de Significância Indeterminada , Sinovite , Masculino , Humanos , Pessoa de Meia-Idade , Sinovite/diagnóstico , Sinovite/tratamento farmacológico , Sinovite/complicações , Edema/etiologia , Prednisona , Anticorpos Monoclonais
16.
J Pediatr Orthop ; 43(8): e608-e613, 2023 Sep 01.
Artigo em Inglês | MEDLINE | ID: mdl-37253715

RESUMO

BACKGROUND: Although differentiating between transient synovitis and septic hip arthritis is challenging, clinical prediction rules such as the Kocher criteria (KC) have been shown to help with the diagnosis of septic hip arthritis in children. Their performance in septic arthritis due to less virulent pathogens such as Kingella Kingae , however is unknown. We aimed to describe the performance of these clinical prediction rules in pre-school children with septic hip arthritis due to different pathogens. We hypothesised that the number of KC or modified KC met would be lower in children with septic hip arthritis caused by K. kingae , compared to those caused by Staphylococcus aureus . METHODS: In this retrospective multicentre study conducted in Australia and New Zealand between 2012-2016, we included children with confirmed septic hip arthritis due to S. aureus (n=29), K. kingae (n=20), other pathogens (n=32), and no pathogen identified (n=48). We applied the KC (temperature, weight-bearing, erythrocyte sedimentation rate, white blood cell count) and the modified KC (C-reactive protein added) and assessed their sensitivity for septic hip arthritis, using cut offs of KC ≥ 3 and modified KC ≥ 4. RESULTS: The score of the KC and the modified KC was not lower in K. kingae compared to S. aureus ( P =0.27, P =0.21). In addition, both the sensitivity for the KC ( S. aureus 18/29 (62.1%); K. kingae 12/20 (60.0%)), and for the modified KC ( S. aureus 18/29 (62.1%); K. kingae 12/20 (60.0%)) did not differ between K. kingae and S. aureus . Of all children with septic hip arthritis, the sensitivity of both the KC and modified KC were 56.6% (95%CI 47.6-65.3). CONCLUSIONS: The clinical prediction rules had comparable performance in K. kingae infections to those caused by S. aureus . Concerningly, less than 60% of the children with confirmed septic hip arthritis met the cut-off values. These prediction rules lack sensitivity to rule-out septic hip arthritis in the early assessment of pre-school aged children with acute hip pain. LEVEL OF EVIDENCE: Level III Diagnostic.


Assuntos
Artrite Infecciosa , Infecções Estafilocócicas , Sinovite , Humanos , Pré-Escolar , Lactente , Criança , Staphylococcus aureus , Regras de Decisão Clínica , Artrite Infecciosa/diagnóstico , Sinovite/diagnóstico , Infecções Estafilocócicas/diagnóstico
17.
Int J Rheum Dis ; 26(10): 2060-2063, 2023 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-37021927

RESUMO

Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome is a rare chronic inflammatory disease. The main clinical manifestation of SAPHO syndrome is an osteoarthropathy with cutaneous involvement. Relapsing polychondritis (RP) characterized by chronic inflammation and cartilage degeneration is a rare systematic autoimmune disease. Here we report a RP case in a SAPHO syndrome patient, in which auricularitis happened 10 years after the diagnosed as SAPHO syndrome. Tofacitinib treatment can alleviate the symptoms.


Assuntos
Acne Vulgar , Síndrome de Hiperostose Adquirida , Osteíte , Policondrite Recidivante , Sinovite , Humanos , Síndrome de Hiperostose Adquirida/complicações , Síndrome de Hiperostose Adquirida/diagnóstico , Síndrome de Hiperostose Adquirida/tratamento farmacológico , Policondrite Recidivante/complicações , Policondrite Recidivante/diagnóstico , Policondrite Recidivante/tratamento farmacológico , Sinovite/diagnóstico , Acne Vulgar/diagnóstico
18.
Int J Rheum Dis ; 26(5): 922-929, 2023 May.
Artigo em Inglês | MEDLINE | ID: mdl-36944587

RESUMO

OBJECTIVES: The development of rheumatoid arthritis (RA) has been classified into 6 phases A-F according to the present risk factors in sequence of genes, environments, autoimmunity, arthralgia and joint swelling. To clarify the ultrasound synovitis scores in at-risk patients (phases C-E) and RA (phase F). METHODS: Patients who had been experiencing hand joint symptoms for at least 6 weeks and asymptomatic patients with positive rheumatoid factor and/or anti-cyclic citrullinated peptide antibodies were enrolled. A 40-joint ultrasonography with semiquantitative synovitis scoring for gray scale (GS) and power Doppler (PD) images was performed. RESULTS: A total of 139 patients were enrolled and classified into non-RA, phase C, phase D, phase E and phase F. Total GS scores in phases C (17.4 ± 7.0), D (16.0 ± 5.4), E (18.5 ± 7.7) and F (21.8 ± 9.1) were higher than those in non-RA (9.8 ± 4.0, P < 0.001). The total PD score in phase F (3.1 ± 4.6) was higher than that in phases C (0.2 ± 0.5), D (0.1 ± 0.4), and E (0.1 ± 0.3), as well as in non-RA (0.0 ± 0.0, P < 0.01). A total GS score ≥14 differentiated patients at risk for RA from patients with non-RA (area under curve [AUC] 0.82), while a total PD score ≥2 differentiated patients with RA from patients at risk for RA (AUC 0.71). CONCLUSION: Total GS score may differentiate patients at risk for RA from non-RA patients, while total PD score may differentiate patients with RA from those who are at risk for RA.


Assuntos
Artrite Reumatoide , Articulação da Mão , Sinovite , Humanos , Sinovite/diagnóstico , Artrite Reumatoide/diagnóstico , Ultrassonografia/métodos , Ultrassonografia Doppler/métodos , Articulação da Mão/diagnóstico por imagem
19.
Pediatr Rheumatol Online J ; 21(1): 23, 2023 Mar 13.
Artigo em Inglês | MEDLINE | ID: mdl-36915122

RESUMO

BACKGROUND: Blau syndrome is a rare autoinflammatory disease caused by autosomal dominant mutations in the CARD15/NOD2 gene. Vascular involvement is a rare phenotype in Blau syndrome patients. In this study, we aimed to describe a 20-year- old Chinese girl with Blau syndrome complicated by renal arteritis. In addition, we summarized a literature review of published cases of vascular involvement in patients with Blau syndrome. CASE PRESENTATION: We describe a 20-year-old girl who was initially misdiagnosed with juvenile idiopathic arthritis (JIA) almost 15 years prior. In October 2019, she developed renal arteritis at the age of 17 years and was eventually diagnosed with Blau syndrome. A de-novo M513T mutation was found in her gene testing. A review of the literature on patients with Blau syndrome and vasculitis showed that a total of 18 cases were reported in the past 40 years. The vast majority of them were predominantly involved medium and large vessel arteritis. Of the 18 patients included in our literature review, 14 patients had aorto-arteritis, and 4 of them had renal artery involvement. Two patients presented with renal artery stenosis, 1with a sinus of Valsalva aneurysm, and 1 with retinal vasculitis. CONCLUSION: A detailed medical history inquiry and a careful physical examination are helpful for the early identification of Blau syndrome, especially for infant onset refractory JIA. Medium-and large-vessel arteritis is a rare clinical manifestation in Blau syndrome patients. Careful examination of the peripheral pulse and measurement of blood pressure at every regular visit may be helpful in the early identification of Blau syndrome-arteritis. Early diagnosis and appropriate treatment may prevent or delay the occurrence of severe symptoms in patients to improve the patient's quality of life.


Assuntos
Arterite , Artrite , Sarcoidose , Sinovite , Uveíte , Feminino , Humanos , Artrite/etiologia , Artrite/genética , População do Leste Asiático , Mutação , Proteína Adaptadora de Sinalização NOD2/genética , Qualidade de Vida , Sarcoidose/complicações , Sarcoidose/diagnóstico , Sarcoidose/genética , Sinovite/diagnóstico , Sinovite/genética , Uveíte/etiologia , Uveíte/genética , Adulto Jovem
20.
Arthritis Res Ther ; 25(1): 47, 2023 03 25.
Artigo em Inglês | MEDLINE | ID: mdl-36964628

RESUMO

BACKGROUND: Optical spectral transmission (OST) is a modern diagnostic modality, able to assess the blood-specific absorption of light transmitted through a tissue, promising quantification of inflammation in the finger and wrist joints of patients with arthritis. To date, there are no adequate data regarding the diagnostic value of OST in the evaluation of inflammatory activity changes, during arthritis follow-up. Objectives of this study were therefore to examine the performance of OST in assessing response to anti-inflammatory therapy in patients with active arthritis and to explore OST associations with clinical, laboratory, and ultrasonographic (US) activity markers. METHODS: 1173 joints of 54 patients with arthritides of the wrist and finger joints were examined by OST before and after oral administration of glucocorticoids (GC), during a disease flare. For the same time-points patients underwent clinical, laboratory, and joint US [grayscale (GSUS), power-Doppler (PDUS)] examinations. The distribution of ΔOST-values between the two time-points was compared with the respective distributions of ΔPDUS and ΔGSUS by Bayesian statistical analyses. Moreover, the diagnostic performance of OST compared to a control group (2508 joints of 114 subjects) was examined by receiver operating characteristics and associations of OST values with clinical, laboratory, and arthrosonographic parameters were evaluated by correlation analyses. RESULTS: OST and US performed similarly in the assessment of inflammatory changes caused by GC (same value-change tendency in 83.2% of the cases). Bayesian statistics revealed no significant differences between ΔOST and ΔPDUS for all 3 examined joint categories (accuracy: metacarpophalangeal (MCP): 68.1%; proximal interphalangeal (PIP): 60.4%; wrists: 50.4%) and between ΔOST and ΔGSUS for MCP and PIP joints (accuracy: 51.1% and 78.7%, respectively). OST diagnostic performance (patients vs. controls) was excellent in both time-points [area under the curve (AUC) before GC=0.883(95%CI=0.83-0.94) and after GC=0.811(95%CI=0.74-0.881); p<0.001]. Furthermore, OST correlated significantly with all examined sonographic activity scores (all; p<0.001) and with swollen joint counts (p<0.01). CONCLUSIONS: OST was able to assess response to therapy in a similar way to joint US and correlated significantly with arthritis activity markers. Therefore, OST has proved to be a valuable tool to assist disease activity monitoring in the examined cohort. TRIAL REGISTRATION: German Registry of Clinical Trials, DRKS00016752.


Assuntos
Artrite Reumatoide , Sinovite , Humanos , Artrite Reumatoide/tratamento farmacológico , Teorema de Bayes , Articulações dos Dedos/diagnóstico por imagem , Seguimentos , Glucocorticoides/uso terapêutico , Índice de Gravidade de Doença , Sinovite/diagnóstico , Ultrassonografia , Ultrassonografia Doppler , Articulação do Punho/diagnóstico por imagem
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