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Devoted to international progress in the research, diagnosis and treatment of rheumatic and musculoskeletal diseases.
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Predictors of myositis in mixed connective tissue disease: A multicentre retrospective study
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.
Read onlineAntifibrotics in rheumatoid arthritis-associated interstitial lung disease – real-world data from a nationwide cohort
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.
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Clinical characterisation of a multicentre nationwide cohort of patients with antisynthetase syndrome
Background: Antisynthetase syndrome (ASyS) is characterised by the association of inflammatory myopathy, interstitial lung disease (ILD), arthritis, Raynaud's phenomenon (RP) or mechanic's hands (MH), with the presence of anti-aminoacyl-tRNA-synthetase antibodies (anti-ARS). It has been suggested that...
Effectiveness and safety of original and biosimilar etanercept (Enbrel® vs Benepali®) in bDMARD-naïve patients in a real-world cohort of Portugal
Abstract
Objective: To compare the effectiveness and safety of original (Enbrel®) and biosimilar (Benepali®) etanercept in Biologic Disease-modifying Antirheumatic Drug (bDMARD)-naïve patients, measured by persistence rates over 36 months of follow-up.
Methods: A retrospective multicentre observational...
Methotrexate: Implications of pharmacogenetics in the treatment of patients with Rheumatoid Arthritis
Background: Methotrexate (MTX) is an anti-folate drug with anti-proliferative and anti-inflammatory effects. MTX proved to be the most highly effective, fast-acting disease modifying anti-rheumatic drug (DMARD), being widely used for the treatment of rheumatoid arthritis (RA). This review aims to describe...
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Complications associated with surgical treatment for pediatric spine deformities: A single center 10-years experience
Introduction: The surgical treatment for pediatric spine deformity is complex and often associated with complications, which generates important clinical and economic impact. Herein the authors analyze the prevalence of complications in surgery for pediatric spine deformity, the correlation between complications...
Methotrexate: Implications of pharmacogenetics in the treatment of patients with Rheumatoid Arthritis
Background: Methotrexate (MTX) is an anti-folate drug with anti-proliferative and anti-inflammatory effects. MTX proved to be the most highly effective, fast-acting disease modifying anti-rheumatic drug (DMARD), being widely used for the treatment of rheumatoid arthritis (RA). This review aims to describe...
Pseudotumoral calcinosis of the spine in systemic sclerosis - an atypical location
Pseudotumoral calcinosis, particularly around the spine, is a rare complication of systemic sclerosis (SSc). The authors report a case of a 60-year-old woman with a limited cutaneous SSc observed for a 4-month history of back pain. Physical examination revealed a left paravertebral mass measuring around...
Online First
Kikuchi-Fujimoto - an enigmatic and rare disease: a report of 3 cases and brief review of the literature
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...
The emerging role of Rituximab in the treatment of large granular lymphocytic leukemia associated with rheumatoid arthritis: a single center experience
Large Granular Lymphocytic (LGL) leukemia is a rare lymphoproliferative disorder with a peculiar association with Rheumatoid Arthritis (RA). The most common feature is neutropenia and patients can have splenomegaly, resembling Felty’s Syndrome. These diseases have similar clinical and laboratory abnormalities...