ARP Rheumatology最新文献

Kikuchi-Fujimoto disease (KFD) is a rare and benign condition mainly characterized by fever and lymphadenopathies. Although many studies have been carried out over time, its aetiology remains unclear, with infectious and autoimmune processes being hypothesized as the main causes. We report three cases of Kikuchi-Fujimoto disease. All patients were female and presented with fever and cervical lymphadenopathies. Extensive work up was performed, in order to rule out infectious, autoimmune and lymphoproliferative diseases. The diagnosis was established through lymph node excisional biopsy and histopathological examination. All patients were followed-up in a medical appointment, with one developing systemic lupus erythematosus (SLE).

Introduction: Anakinra has dramatically improved the management of systemic juvenile idiopathic arthritis (SJIA) over the last decade. Nevertheless, management remains inconsistent; corticosteroids are still frequently used. We analyzed the course of SJIA in children treated with anakinra according to the time of treatment initiation after disease onset.

Method: Children with SJIA treated with anakinra between 2006 and 2020 were included in this single-center, retrospective observational study.

Results: Twenty-four children received anakinra at a median time of 58 (range 12-2940) days after SJIA onset, all after failure of nonsteroidal anti-inflammatory drug (NSAID) treatment. Eighteen were males and the median age at disease onset was 6.04 (range 0.8-13) years. The median follow-up time was 3.5 (range 0.5-10.8) years after treatment initiation. At the last follow-up, remission attributable to anakinra was observed in 18/24 (75%) children and treatment-free remission was observed in 12 (67%). For each child, the response to anakinra was the same at 3 months and at the last follow-up. The 15 children treated with anakinra within the first 3 months after disease onset exhibited better remission (93%) than did the 9 children treated after 3 months (44%) (p = 0.015) and the former received fewer corticosteroids (7% versus 67%) (p = 0.004). One child with long-standing disease died of the disease.

Conclusions: Early anakinra initiation within the first 3 months of SJIA onset after NSAID failure ensures long-term remission and reduces corticosteroid use. Anakinra should not be continued for more than 3 months in nonresponding children.

Aims: ultrasound (US) diagnosis of enthesitis is burdened of low specificity, especially when it is performed in patients with psoriasis (PsO) but without clinical psoriatic arthritis (PsA), because of mechanical, dysmetabolic and age-related concurrent enthesopatic changes. We propose a novel US score to quantify the cortical-entheseal bone remodeling burden of several peripheral entheses, aiming to improve the specificity of US for PsA-related enthesitis, and to evaluate its diagnostic value in PsO patients with subsequent diagnosis of psoriatic arthritis (PsO/PsA).

Methods: clinical and US data of 119 consecutive patients with moderate/severe PsO and nonspecific musculoskeletal symptoms, were included in this retrospective study. PsO patients underwent a multi-joint US examination and a subsequent rheumatologic visit to evaluate concurrent PsA clinical diagnosis, in a scenario of real clinical practice. The cortical-entheseal bone remodeling has been evaluated with a morphologic gray-scale US score named "CERTUS" (Cortical-Entheseal Remodeling Tuscany Ultrasonographic Score, range 0-36), grading the combination of both enthesophytes and erosions in a semiquantitative scale. A variant of CERTUS, with Power Doppler (PD), was calculated too (CERTUS-PD, range 0-48), scoring PD signals into erosions. The sum of the scores obtained for 12 peripheral entheses was used as global score for statistic aims. The new bone formation at extensor tendon entheses at distal inter-phalangeal (DIP) joints were also recorded.

Results: a clinical diagnosis of PsO/PsA was made in 48/119 PsO patients (40.3%), showing older age (p<0.001), higher BMI (p=0.015), prevalence of metabolic syndrome (p=0.014) and smoking habit (p<0.001). CERTUS (AUROC 0.814) showed a highest specificity cut-off=11 (sensitivity 41.4%, specificity 100%), whereas CERTUS-PD (AUROC 0.828) showed a highest specificity cut-off=13 (sensitivity 37.9%, specificity 100%). CERTUS and CERTUS-PD correlated with both other validated US scores as Belgrade Ultrasound Enthesitis Score (BUSES) (p<0.001), DACTylitis glObal Sonographic (DACTOS) score (p=0.05 and p=0.031 respectively), amount of synovitis (p=0.036 and p=0.04 respectively), enthesitis (p<0.001) and entheseal new bone formation on DIP joints (p=0.029 and p=0.031 respectively).

Conclusions: the scoring system named CERTUS (and its variant with PD) is a quick tool to quantify cortico-entheseal bone remodeling burden in PsO patients, improving the specificity of US to diagnose patients with subclinical PsA-related enthesitis.

Background: Intra-articular glucocorticoid injection (IAGI) is widely used for treatment of knee osteoarthritis (OA) flares. Response rates are generally around 70%. Several studies have tried to identify predictors of good response, but response to ultrasound (US)-guided injection has not yet been investigated. This study aimed to identify the predictors of response to IAGI performed under US guidance in patients with primary knee OA.

Materials and methods: A total of 116 patients (116 knees) presenting with unilateral or bilateral primary knee OA were enrolled for this prospective single-center study. All were aged >40 years and met the American College of Rheumatology (ACR) criteria for knee OA. Demographic, clinical, laboratory, and imaging data were collected, injection was performed using US guidance, and tolerance was assessed. The primary efficacy endpoint was ≥40% reduction in total WOMAC score (WOMAC40). Univariate and multivariate logistic regression analyses were conducted to identify the predictors of response.

Results: The mean age of the patients was 64.2 ± 9.4 years and mean BMI was 29.9 ± 3.8 kg/m2. Total WOMAC40 response rate was 61.2%. In multivariate analysis, the independent predictors of response were BMI.

Anti-HMGCR myopathy is an increasingly recognized immune-mediated necrotizing myopathy. However, there are currently no evidence-based treatments available, so case reports and clinical experience are used to guide current management. We report a case of a 49-year-old man, treated with atorvastatin, who presented to the emergency department with progressive proximal muscle weakness. Anti-HMGCR antibodies were detected, and muscle biopsy revealed necrotizing myopathy. Initially, therapy with high-dose glucocorticoids and methotrexate was started, but 12 weeks later, the patient developed clinical deterioration with dysphagia. Then, he was successfully treated with one cycle of rituximab along with physical therapy. The use of rituximab in immune-mediated necrotizing myopathy has been heterogeneously described in the literature but mostly in case reports. The European Neuromuscular Centre working group recommends the use of rituximab in refractory cases. However, some studies highlight the importance of early and aggressive treatment for this disease. Clinical prospective studies are necessary to make proper evidence-based recommendations.

Objectives: We aimed to identify clinical and serological predictors of myositis in mixed connective tissue disease (MCTD).

Methods: We performed a nationwide, retrospective, multicentre study including adult-onset MCTD patients fulfilling at least one of the following diagnostic criteria: Sharp's, Kasukawa, Alarcón-Segovia, or Kahn's. Univariate analysis was performed using Chi-square, Fisher exact, Student's t or Mann-Whitney U tests, as appropriate. Multivariate analysis was performed using binary logistic regression.

Results: Ninety-eight patients were included. Myositis was observed in 43.9% of patients, of whom 60.5% had myositis at disease onset. Proximal muscle weakness was described in 30 patients with muscle involvement (70%). Gastrointestinal involvement was identified in 28% and respiratory involvement in 29% of myositis patients. In the same subgroup of patients, 41.7% had a myopathic pattern on electromyography, and 47.1% had histological myositis features in the muscle biopsy. Fever (OR=6.96, p=0.022) was an independent predictor of myositis, regardless of sex, age at diagnosis, ancestry, and respiratory involvement. African ancestry (OR=8.39, p=0.019), leukopenia at the disease onset (OR 6.24, p=0.021), and younger age at diagnosis (OR=1.07/year, p=0.035) were identified as independent predictors of myositis at disease onset, regardless of sex and scleroderma pattern in capillaroscopy.

Conclusions: Myositis is a common manifestation of MCTD, even at the disease onset. African ancestry, leukopenia at the disease onset, younger age at diagnosis, and fever should prompt a thorough evaluation for myositis.

Introduction: Interstitial lung disease (ILD) is the most common pulmonary manifestation of rheumatoid arthritis (RA) and is associated with an increased mortality. Clinical trials have shown that antifibrotics (nintedanib and pirfenidone) can slow the progression of connective tissue disease-associated ILD. This study aims to evaluate the effectiveness and tolerability of antifibrotics in a national, real-world cohort of patients with RA-ILD.

Material and methods: We conducted an observational multicenter study of RA-ILD patients treated with antifibrotics, who were prospectively followed in Reuma.pt. Demographic and clinical data, pulmonary function tests (PFTs) results and adverse events (AEs) were collected. A linear mixed model with random intercept was used to compare PFT results within 12 (±6) months before to 12 (±6) months after antifibrotic initiation. Drug persistence was evaluated using Kaplan-Meier curves.

Results: We included 40 RA-ILD patients, 27 (67.5%) initially treated with nintedanib and 13 (32.5%) with pirfenidone. Most of the patients were female (55%), and current or past smokers (52.5%). At antifibrotic initiation, mean age was 70.9 ± 7.1 years and median ILD duration 5.0 [IQR 2.3-7.5] years. A total of 20 patients were included in effectiveness analysis, with the use of antifibrotics interrupting the decline of forced vital capacity (FVC; decline 300 ± 500 mL in the year before antifibrotic initiation vs. improvement of 200 ± 400 mL in the year following antifibrotic initiation, p=0.336) and total lung capacity (TLC; decline 800 ± 300 mL in the year before antifibrotic initiation vs. improvement of 600 ± 900 mL in the year following antifibrotic initiation, p=0.147). However, diffusion capacity for carbon monoxide remained in decline (3% decline in the year before antifibrotic initiation vs. 2.9% decline in the year following antifibrotic initiation, p=0.75). AEs were reported in 16 (40%) patients and led to drug discontinuation in 12 (30%). Median duration of drug persistence was 150.3 weeks (95 %CI 11.0-289.6), with no difference between nintedanib and pirfenidone (p = 0.976).

Conclusion: This study with real-world data corroborates the usefulness of antifibrotics in stabilizing lung function, based on FVC and TLC. However, AEs were frequently reported and were the main cause for drug discontinuation.