Mediterranean Journal of Rheumatology最新文献

Objectives: To describe the characteristics of primary Sjögren's syndrome (pSS) patients with interstitial lung disease (ILD) and to assess treatment response.

Methods: All patients of pSS from 2010 to 2019 were retrospectively identified. Lung function tests, high resolution computed tomography (HRCT) findings, and treatment outcomes were analysed.

Results: Out of 550 patients with pSS, ILD was detected in 33 patients (frequency of 6 %). The mean(±SD) age at the diagnosis of pSS was 50 (± 9.3) years. 28/33(84.8%) were females. ILD onset preceded pSS diagnosis in 2 (6%) patients, simultaneously diagnosed in 21 (63.6%) patients and developed after pSS onset in 10 (30.3%) patients. 5 patients (15.15 %) were asymptomatic for ILD. Non-specific interstitial pneumonia (NSIP) accounted for the most frequent ILD subtype, in 15 patients (45.5%). Mycophenolate mofetil (MMF) was the most frequently used steroid sparing agent, in 25 patients (75.7%). 7 patients were lost to follow up. Response was seen in 22 patients, whereas 3 patients were non responders. There was one mortality due to lower respiratory tract infection-related sepsis. Presence of sicca symptoms [91.5% vs 8.7% (p<0.001)], NSIP pattern of ILD [90% vs 10% (p = 0.002)], and absence of Raynaud's phenomenon [91.7% vs 8.3% (p<0.001)] were significantly associated with responder status when compared to non-responders.

Conclusion: ILD in primary Sjögren's syndrome is not an uncommon entity, and immunosuppression with steroids along with steroid-sparing agents led to good clinical outcomes of ILD in a majority of the patients in our cohort.

Introduction: Fatigue is a common, disabling, and poorly understood aspect of Rheumatoid arthritis (RA) treatment. Better understanding of fatigue is required for holistic treatment of RA. The present study was conducted to evaluate factors (disease activity, pain, sleep quality, and vitamin D) contributing to fatigue in RA.

Method: A cross-sectional study was conducted on 204 patients of RA. Fatigue was measured using CFQ-11 scale, pain and sleep impairment were assessed on visual analogue scale, disease activity by DAS 28 ESR, and vitamin D levels by enzyme chemiluminescence immunoassay. Univariate and multivariate binary logistic regression analyses were done to study association.

Results: Mean age of study subjects was 51±11.63 years with majority (89.7%) being females and mean duration of RA was 8.54 years. Prevalence of fatigue was 66.2% (CFQ-11 score >4/11). Deficiency of vitamin D was found in 12.3% subjects. Mean sleep impairment and pain score on VAS were 32.60±21.53 and 26.37±21.65 respectively. Univariate analysis revealed that CFQ-11 fatigue score was independently associated with disease activity, pain, sleep, and vitamin D deficiency. Further Multivariate binary logistic regression analysis showed strongest association of vitamin D deficiency with fatigue (OR of 6.38 with 95% confidence interval of 1.58, 25.71). Disease activity (OR - 1.714, 95% CI- 1.14, 2.55) and sleep impairment (OR - 1.038, 95% CI- 1.005, 1.071) have also been found to be significantly associated with fatigue.

Conclusion: Fatigue in RA is multifactorial, and it is mediated by disease-related factors (disease activity, sleep impairment) and non-disease-related factors (vitamin D deficiency).

Background: Rheumatoid arthritis (RA) is a multidimensional disease. In addition to quantitative factors, qualitative factors play an important role in the progress and outcome of the diseases. One of the most effective methods of collecting qualitative information is questionnaires reported by patients. The data obtained from the questionnaires are as important as the clinical criteria. Multidimensional health assessment questionnaire (MDHAQ) is one of the latest questionnaires that provide useful information in a short time.

Objectives: To investigate the reliability and validity of the Persian form of MDAHAQ for the use of Iranian patients.

Method: Two groups of participants were selected for this study. The validity test group included 110 patients, and the reliability test group included 140 patients. Translation and adaption of MDHAQ were performed by using Guillemin guidelines. The reliability was tested by using test-retest and Cronbach's alpha for internal consistency. Persian version of the health assessment questionnaire (HAQ) was used for assessing the criterion validity. The correlation between the MDHAQ score and Disease Activity Score-28 (DAS28), Clinical Disease Activity Index (CDAI), and the Persian version of the health assessment questionnaire (HAQ) was evaluated using the Spearman coefficient. Discriminant validity was tested in groups of patients based on two varied disease activities based on CDAI and DAS28.

Results: Test-retest with intra-class correlation coefficient (ICC) gave a coefficient of 0.865(95% CI: 0.809, 0.904) for physical function and 0.786(95% CI: 0.698, 0.848) for psychological items. Cronbach's alpha was 0.885 and 0.705 for physical function and psychological dimensions respectively. The Persian version of the MDHAQ had a good to strong correlation with the Persian version of the HAQ (ranging from 0.604 to 0.962) and also with CDAI (ranging from 0.616 to 0.838) and a moderate correlation with DAS28 (ranging from 0.415 to 0.439).

Conclusion: The Persian form of MDHAQ is a reliable and valid instrument for the routine evaluation of RA patients in rheumatology clinics in Iranian RA patients.

Background: Psoriatic arthritis (PsA) is a heterogenous chronic inflammatory disease affecting skin, joints, entheses, and spine with various extra-musculoskeletal manifestations and comorbidities. The reported patient, disease and treatment characteristics in the modern therapeutic era are limited.

Methods: In this cross-sectional, multi-centre, nationwide study, we recorded the demographic, clinical, and therapeutic characteristics as well as the comorbidities of patients with PsA seen for 1 year (1/1/2022-31/12/2022).

Results: 923 patients (55% females) with a median (IQR) age of 57 (48-65) years and a mean disease duration of 9.5 years were enrolled. Family history of psoriasis and PsA was noted in 28.3% and 6.3%, respectively. Most patients had limited psoriasis (BSA<3: 83%) while enthesitis, dactylitis, nail and axial involvement reported in 48.3%, 33.2%, 43% and 25.9% of patients, respectively. Regarding comorbidities, approximately half of patients had dyslipidaemia (42%) or hypertension (45.4%), 36.8% were obese and 17% had diabetes while 22.7% had a depressive disorder. Overall, 60.1% received biologics and among them more patients treated with anti-IL-17 or -12/23 agents were on monotherapy (64.2%) compared to those on TNFi monotherapy (49.4%, p=0.0001). The median PsA activity as assessed by the DAPSA score was 6 (IQR: 2.3 - 13.1) with 46% of patients reaching minimal disease activity status (MDA).

Conclusion: In this large, real life, modern cohort of patients with PsA with frequent comorbidities who were treated mainly with biologics, almost half achieved minimal disease activity. These results show the value of existing therapeutic approaches while at the same time highlight the existing unmet needs.

Rheumatoid arthritis (RA) is a prevalent chronic inflammatory arthritis worldwide, significantly impacting patients and population health. The disease affects women primarily, with a female-to-male ratio of three to one. Its pathogenesis is multifactorial, including genetic and environmental risk factors. Epidemiological studies highlight the link between the environment and genetic susceptibility to RA. The so-called shared epitope is the most significant risk factor that seems to act synergetic with other environmental factors in the disease occurrence. In addition, recent findings suggest a potential role of new substantial environmental factors, such as the observed pollution of the planet's natural resources, on the susceptibility and progression of the disease. This review summarises the most decisive evidence on epidemiology and genetic, environmental, and lifestyle risk factors for RA. It shows that studying genetic and environmental factors in correlation could lead to prevention strategies that may impact the natural history of the disease.

Introduction: Eosinophilic Fasciitis (EF) is a rare disease, originally proposed as "diffuse fasciitis with eosinophilia" by Shulman in 1974. Symptoms of EF include peripheral eosinophilia accompanied by symmetrical inflammation of the subcutaneous fascia and muscle, usually locating in the upper arms or thighs. There is no approved standard of care treatment.

Methods: Taking into account that eosinophils may be pathogenetically involved in EF, we performed a review on Medline focusing on anti-Interleukin-5 (IL-5) therapies in EF.

Results: Only one case of a patient with EF has been reported who was successfully treated with reslizumab, an anti-IL-5 therapy. The patient had EF refractory to the commonly used immunosuppressive treatment but when reslizumab was added, the patient experienced remission of her symptoms.

Discussion: The exact aetiology of EF is still unclear, and many therapeutic approaches have been tested. Commonly used immunosuppressive agents, such as corticosteroids are not always effective and associate with significant side effects. Eosinophils seem to have a role in the pathogenesis of the disease; anti-eosinophilic therapies targeting IL-5/IL-5 Receptor could be an attractive alternative for the treatment of the disease.

Objectives: To compare the presentation, angiographic features, evolution, and prognosis of prepulseless Takayasu arteritis (TAK) with TAK with pulse loss.

Methods: Pre-pulseless TAK (defined as without pulse loss in the upper limbs, lower limb, carotid, or subclavian arteries) were identified from a cohort of TAK. Demographic characteristics, clinical features, angiographic involvement, baseline and longitudinal patterns of disease activity, medication use, and mortality rates were compared between pre-pulseless TAK and TAK with pulse loss. Adjusted odds ratios (aOR, with 95%CI) for categorical variables between pre-pulseless TAK and TAK with pulse loss were computed using multivariable-adjusted logistic regression models. Time-to-event data was compared using hazard ratios (HR) with 95%CI.

Results: Compared with TAK with pulse loss, pre-pulseless TAK (91/238, 38.24%) more frequently had deranged renal function (aOR 4.43, 95%CI 1.58-12.37) and Hata's type IV disease (aOR 8.02, 95%CI 2.61-24.65), and less often had pulse or blood pressure asymmetry (aOR 0.34, 95%CI 0.18-0.63), limb claudication (aOR for upper limb 0.38, 95%CI 0.18-0.82, for lower limb 0.28, 95%CI 0.12-0.68), right subclavian (aOR 0.45, 95%CI 0.23-0.90) or left carotid artery involvement (aOR 0.42, 95%CI 0.21-0.84). Only two patients with pre-pulseless TAK developed pulse loss on follow-up. Despite fewer pre-pulseless TAK having active disease at presentation, similar proportions of patients in both groups had active disease on follow-up. Survival was similar in both groups (HR for mortality 0.41, 95%CI 0.09-1.90).

Conclusion: Pulse loss on follow-up is uncommon in those with prepulseless TAK. Pre-pulseless TAK is associated with similar long-term outcomes to TAK with pulse loss.