Mediterranean Journal of Rheumatology最新文献

Primary Sjögren's syndrome (pSS) is a systemic autoimmune disease and can rarely present with multiple extraglandular manifestations. Here we report a case of pSS with concomitant IgA nephropathy and autoimmune hepatitis as the initial manifestations. She presented with polyarthralgia, sicca symptoms and persistent fatigue but was asymptomatic for renal and liver involvement. Autoimmune diseases can have overlapping clinical features and occasionally, manifest nonspecific symptoms leading to delay in diagnosis. It is therefore imperative to thoroughly evaluate any patient of pSS for early recognition of the diverse extraglandular features and initiate prompt treatment to improve outcome.

Introduction: Neuro-Behçet's disease (NBD) is an uncommon presentation in Behçet's disease (BD) with severe course and worse prognosis. Both vascular and NBD presentation without the classical triad of BD in a single patient is rarely reported.

Case presentation: Here a 48-year-old male had an extensive aortic aneurysm eroding vertebra for which he was diagnosed as vascular BD. Two years later, he was presented with a severe headache and cerebrovascular accident, his brain imaging showed hyperintensity in the right thalamus, basal ganglia, temporal lobe, and internal capsule, suggesting the 'cascade sign' of NBD. Surprisingly, he never had oral or genital ulcers or skin and eye involvement. He had a good response to infliximab.

Conclusion: Clustering of BD phenotype is an emerging area of interest. It is hypothesised that severe phenotype of vascular and parenchymal NBD can happen in the same patient owing to similar underlying pathology. This case is unique due to its severe phenotype with no features of the typical triad of BD.

Background: Vascular purpura can be the clinical expression of infectious, inflammatory, drug-related, neoplastic, and endocrine pathologies. To date, there is no consensus codifying the investigation of vascular purpura, especially when it is isolated.

Patients and methods: We proposed to study through a retrospective study of 73 cases of vascular purpura, occurring during the period 2004-2019 in our internal medicine department, the contribution of various clinical and paraclinical data to the aetiological diagnosis of vascular purpura. Data were considered to be contributory only when they constituted a solid argument in favour of the aetiological diagnosis of vascular purpura.

Results: Our series involved 73 patients including 41 women and 32 men (Gender ratio: 0.78). Mean age was 49 ± 17 years [16-80]. Vascular purpura was isolated in 3% of cases. For the remaining patients, it was associated with functional (91%) or physical (48%) manifestations. It was associated with other skin lesions in 45% of cases. The accepted aetiologies were primary vasculitis (26%), drug-related (15%), infectious (11%) and secondary to connectivitis (10%). No cause was found in a third of cases. Clinical data alone made it possible to suggest the aetiology in more than half of cases. Special investigations were contributory in 46% of cases. The course was contributory in 18% of patients for drug-related and paraneoplastic causes.

Conclusion: vascular purpura's diverse clinical presentation presents diagnostic challenges. Aetiologies include vasculitis, drug reactions, infections, and connective tissue disorders. Comprehensive clinical assessment is essential.

Background: A sonographic scoring system, termed Halo count and Halo score, of temporal and axillary arteries (TAXA) in suspected giant cell arteritis (GCA) has been proposed for outcome prognostication.

Method: We conducted a retrospective review into the relationship of Halo count and Halo score and clinical-laboratory parameters amongst patients diagnosed with GCA via our rapid-access pathway to determine whether these measures should form part of our local routine clinical practice.

Result: This review of TAXA ultrasound (US) images in patients with diagnosed GCA did not identify any correlation between Halo count/score and ocular symptoms, jaw claudication, 6-month relapse risk or inflammatory markers.

Conclusion: This suggests that further prospective evaluation of Halo count and -score is required before adopting these measures into routine US scanning of TAXA for suspected GCA.

Aim: Extra-articular manifestations especially haematological abnormalities are common in Rheumatoid arthritis (RA). The aim is to study the haematological parameters and its correlation with disease severity and gender.

Methods: A cross-sectional study was conducted at a tertiary centre among 50 RA patients who attended the Rheumatology or General Medicine OPD from January 2020 to June 2021. The number of swollen joints, tender joints, the patient's global assessment, rheumatoid factor, and complete blood counts were recorded. Disease Activity Score (DAS-28) was calculated from these variables and data was analysed using SPSS version 29 with p<0.05 considered as statistically significant.

Results: Of the study subjects, forty (80%) were females; thirty (60%) belonged to the age group 41-50 years. Median age was 42.4(18, 50) years. 79 % (19 out of 24) of subjects with normal haemoglobin had moderate disease activity on DAS 28 score and 50% (13/26) of participants with low haemoglobin levels had high disease activity. Simple linear regression analysis showed low haemoglobin is inversely associated with DAS 28 score (R2 = 0.407, F= 32.888, p < 0.001). Compared to females, males had a higher haemoglobin level which was statistically significant. Female patients had statistically significant higher DAS 28 scores than males (p = 0.016).

Conclusion: Low haemoglobin levels are associated with high disease activity in RA patients and males had less severe disease than females.

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.