| 1. |
- Eberhard, Anna, et al.
(författare)
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Joint tenderness at 3 months follow-up better predicts long-term pain than baseline characteristics in early rheumatoid arthritis patients
- 2024
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Ingår i: Rheumatology. - 1462-0324. ; 63:3, s. 734-741
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Tidskriftsartikel (refereegranskat)abstract
- Objective To investigate pain course over time and to identify baseline and 3-month predictors of unacceptable pain with or without low inflammation in early RA. Methods A cohort of 275 patients with early RA, recruited in 2012-2016, was investigated and followed for 2 years. Pain was assessed using a visual analogue scale (VAS; 0-100 mm). Unacceptable pain was defined as VAS pain >40, and low inflammation as CRP Results After 2 years, 32% of patients reported unacceptable pain. Among those, 81% had low inflammation. Unacceptable pain, and unacceptable pain with low inflammation, at 1 and 2 years was significantly associated with several factors at 3 months, but not at baseline. Three-month predictors of these pain states at 1 and 2 years were higher scores for pain, patient global assessment, and the health assessment questionnaire, and more extensive joint tenderness compared with the number of swollen joints. No significant associations were found for objective inflammatory measures. Conclusion A substantial proportion of patients had unacceptable pain with low inflammation after 2 years. Three months after diagnosis seems to be a good time-point for assessing the risk of long-term pain. The associations between patient reported outcomes and pain, and the lack of association with objective inflammatory measures, supports the uncoupling between pain and inflammation in RA. Having many tender joints, but more limited synovitis, may be predictive of long-term pain despite low inflammation in early RA.
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| 2. |
- Hernandez-Molina, Gabriela, et al.
(författare)
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Characterization and outcomes of 414 patients with primary SS who developed haematological malignancies
- 2022
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Ingår i: Rheumatology. - : Oxford University Press. - 1462-0324 .- 1462-0332. ; 62:1, s. 243-255
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Tidskriftsartikel (refereegranskat)abstract
- ObjectiveTo characterize 414 patients with primary SS who developed haematological malignancies and to analyse how the main SS- and lymphoma-related features can modify the presentation patterns and outcomes.MethodsBy January 2021, the Big Data Sjögren Project Consortium database included 11 966 patients fulfilling the 2002/2016 classification criteria. Haematological malignancies diagnosed according to the World Health Organization (WHO) classification were retrospectively identified.ResultsThere were 414 patients (355 women, mean age 57 years) with haematological malignancies (in 43, malignancy preceded at least one year the SS diagnosis). A total of 376 (91%) patients had mature B-cell malignancy, nearly half had extranodal marginal zone lymphoma (MZL) of mucosa-associated lymphoid tissue (MALT lymphoma) (n = 197), followed by diffuse large B-cell lymphoma (DLBCL) (n = 67), nodal MZL lymphoma (n = 29), chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) (n = 19) and follicular lymphoma (FL) (n = 17). Rates of complete response, relapses and death were 80%, 34% and 13%, respectively, with a 5-year survival rate of 86.5% after a mean follow-up of 8 years. There were significant differences in age at diagnosis (younger in MALT, older in CLL/SLL), predominant clinical presentation (glandular enlargement in MALT lymphoma, peripheral lymphadenopathy in nodal MZL and FL, constitutional symptoms in DLBCL, incidental diagnosis in CLL/SLL), therapeutic response (higher in MALT lymphoma, lower in DLBCL) and survival (better in MALT, nodal MZL and FL, worse in DLBCL).ConclusionIn the largest reported study of haematological malignancies complicating primary SS, we confirm the overwhelming predominance of B-cell lymphomas, especially MALT, with the salivary glands being the primary site of involvement. This highly-specific histopathological scenario is linked with the overall good prognosis with a 5-year survival rate of nearly 90%.
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| 3. |
- Huijser, Erika, et al.
(författare)
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Serum IFNα2 measured by single-molecule array associates with systemic disease manifestations in Sjögren's syndrome
- 2022
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Ingår i: Rheumatology (Oxford, England). - : Oxford University Press (OUP). - 1462-0332 .- 1462-0324. ; 61:5, s. 2156-2166
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Tidskriftsartikel (refereegranskat)abstract
- OBJECTIVES: Type I IFN (IFN-I) activation is a prominent feature of primary SS (pSS), SLE, and SSc. Ultrasensitive single-molecule array (Simoa) technology has facilitated the measurement of subfemtomolar concentrations of IFNs. Here, we aimed to measure IFNα2 in serum from pSS, SLE, and SSc using a Simoa immunoassay and correlate these levels to blood IFN-stimulated gene (ISG) expression and disease activity.METHODS: Serum IFNα2 was measured in patients with pSS (n = 85; n = 110), SLE (n = 24), and SSc (n = 23), and healthy controls (HC; n = 68) using an IFNα Simoa assay on a HD-X analyser. IFN-I pathway activation was additionally determined from serum by an IFN-I reporter assay and paired samples of whole blood ISG expression of IFI44, IFI44L, IFIT1, IFIT3, and MxA by RT-PCR or MxA-ELISA.RESULTS: Serum IFNα2 levels were elevated in pSS (median=61.3 fg/mL) compared to HC (median ≤5 fg/mL; p < 0.001) and SSc (median=11.6 fg/mL; p = 0.043), lower compared to SLE (median=313.5 fg/mL; p = 0.068), and positively correlated with blood ISG expression (r = 0.66-0.94; p < 0.001). Comparable to MxA-ELISA (AUC=0.93), IFNα2 measurement using Simoa identified pSS with high ISG expression (AUC=0.90) with 80-93% specificity and 71-84% sensitivity. Blinded validation in an independent pSS cohort yielded a comparable accuracy. Multiple regression indicated independent associations of autoantibodies, IgG, HCQ treatment, cutaneous disease and history of extraglandular manifestations with serum IFNα2 concentrations in pSS.CONCLUSION: Thus, Simoa serum IFNα2 reflects blood ISG expression in pSS, SLE, and SSc. In light of IFN-targeting treatments, Simoa could potentially be applied for patient stratification or retrospective analysis of historical cohorts.
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| 4. |
- Jousse-Joulin, Sandrine, et al.
(författare)
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Is salivary gland ultrasonography a useful tool in Sjögren's syndrome? A systematic review
- 2016
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Ingår i: Rheumatology. - : Oxford University Press (OUP). - 1462-0324 .- 1462-0332. ; 55:5, s. 789-800
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Tidskriftsartikel (refereegranskat)abstract
- Objective. Ultrasonography (US) is a sensitive tool in the diagnosis of major salivary gland abnormalities in primary Sjögren's syndrome (pSS). The aim of this systematic review was to assess the metric properties of this technique. Methods. PUBMED and EMBASE databases were searched. All publications between January 1988 and January 2013 were considered. Data were extracted from the articles meeting the inclusion criteria according to US definition of salivary gland scoring system and metric properties studied. The type and number of glands tested, study design and metric properties according to OMERACT filter (truth, discrimination, feasibility) were assessed. Results. Of 167 publications identified initially with PUBMED and EMBASE, 31 met the inclusion criteria. The number of pSS patients varied among the studies from 16 to 140. The diagnosis of pSS was in line in most of the cases with the American-European Consensus Group (AECG) classification criteria for Sjögren's syndrome. The US examination was performed in suspected pSS only in studies in which the sensitivity ranged from 45.8 to 91.6% and specificity from 73 to 98.1%. There was heterogeneity in regard to the definition of US in B-mode and few studies used US in colour Doppler. Few studies reported reliability of US and sensitivity to change in pSS. Conclusion. US is a valuable tool for detecting salivary gland abnormalities in pSS. Its reliability has been poorly investigated and there is considerable variation in the definition of US abnormalities. Further studies are required to validate and standardize the US definition of salivary gland in pSS.
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| 5. |
- Mandl, Thomas, et al.
(författare)
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Autonomic nervous dysfunction development in patients with primary Sjogren's syndrome: a follow-up study.
- 2010
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Ingår i: Rheumatology (Oxford, England). - : Oxford University Press (OUP). - 1462-0332 .- 1462-0324. ; Apr 7, s. 1101-1106
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Tidskriftsartikel (refereegranskat)abstract
- Objectives. To investigate autonomic dysfunction (AD) development in patients with primary SS (pSS) and the associations between AD and clinical, inflammatory and serological features of pSS. Methods. Twenty-seven patients with pSS, who had previously been evaluated for AD, were included in the study. The patients were studied at baseline and at follow-up by objective autonomic reflex tests (ARTs) and by the autonomic symptom profile (ASP) questionnaire, evaluating AD symptoms. The median follow-up time was 5 years for the ART and 4 years for the ASP variables. The results were compared with previously investigated healthy ART controls and population-based ASP controls. Fatigue, anxiety and depression were assessed by the profile of fatigue and by the Hospital Anxiety and Depression scale. Results. Three of five ART variables as well as the ASP total score were significantly abnormal both at baseline and at follow-up in pSS patients in comparison with controls. When comparing ART and ASP results in pSS patients between baseline and follow-up, only the lowest diastolic blood pressure (lDBP) ratio significantly deteriorated during the follow-up period. The ART and ASP variables were not significantly correlated. However, the ASP total score significantly correlated with measurements of fatigue, anxiety and depression. Conclusions. Both objective signs and subjective symptoms of parasympathetic and sympathetic dysfunction were seen in pSS patients, both at baseline and at follow-up. During follow-up, only the lDBP ratio was found to significantly deteriorate. AD symptoms were significantly associated with fatigue, anxiety and depression.
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| 6. |
- Mandl, Thomas, et al.
(författare)
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Autonomic nervous symptoms in primary Sjogren's; syndrome.
- 2008
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Ingår i: Rheumatology. - : Oxford University Press (OUP). - 1462-0332 .- 1462-0324. ; 47:6, s. 914-919
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Tidskriftsartikel (refereegranskat)abstract
- Objectives. Objective signs of autonomic dysfunction (AD) have been reported in patients with primary SS (pSS) while the presence of associated symptoms has not been systematically studied. Therefore, the aims of this study were (i) to assess the presence and severity of various AD symptoms in pSS patients and (ii) to relate AD symptoms to other clinical features of pSS. Methods. Thirty-eight pSS patients and 200 population-based controls were studied for presence and severity of AD symptoms using the Autonomic Symptom Profile (ASP), a validated self-completed questionnaire evaluating various AD symptoms. In addition, patients were investigated by three different objective autonomic nervous function tests. Results. pSS patients scored significantly higher in the parasympathetic [secretomotor disorder, urinary disorder, gastroparesis (females only) and pupillomotor disorder] as well as sympathetic (orthostatic intolerance and vasomotor disorder) ASP domains compared with controls. Consequently, the standardized ASP total score was significantly increased in pSS patients [1.77 (0.57, 3.15) vs - 0.21 (-0.82, 0.72); P = 0.00] and 45% of pSS patients had an ASP total score >/=2 s.d. Furthermore, the autonomic nervous function tests showed signs of objective parasympathetic and sympathetic dysfunction as well. However, the ASP domain and total scores showed limited associations with the objective autonomic nervous function test parameters as well as clinical and serological factors of pSS. Conclusions. pSS patients showed subjective and objective signs of both a parasympathetic and a sympathetic dysfunction. However, AD symptoms showed limited associations with objective autonomic nervous function as well as other clinical features of the disease.
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| 7. |
- Mandl, Thomas, et al.
(författare)
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Frequent development of chronic obstructive pulmonary disease in primary SS--results of a longitudinal follow-up.
- 2012
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Ingår i: Rheumatology. - : Oxford University Press (OUP). - 1462-0332 .- 1462-0324. ; 51:5, s. 941-946
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Tidskriftsartikel (refereegranskat)abstract
- Objectives. To study the longitudinal development of pulmonary function in patients with primary SS (pSS) and its association with respiratory symptoms, pulmonary radiographic findings and clinical features of pSS.Methods. Forty-one pSS patients, previously evaluated by pulmonary function tests (PFTs), were included in the study. The patients were studied at baseline and follow-up by PFT and at follow-up also by high-resolution CT scan of the lungs, the St George's Respiratory Questionnaire and by inflammatory and serological tests. The PFT results were compared with previously studied population-based controls, standardizing results with regard to gender, age, height, weight and tobacco consumption.Results. The mean follow-up time was 11 years. The pSS patients displayed signs of both obstructive and restrictive lung disease at baseline and at follow-up, and deteriorated in forced expiratory volume in 1 s (FEV(1)), ratio of FEV(1) to vital capacity and in diffusing capacity for carbon monoxide during follow-up. Chronic obstructive pulmonary disease (COPD) was diagnosed in 37% of the pSS patients at follow-up. In pSS patients, respiratory symptoms and radiographic abnormalities were common, although with a poor association with PFT variables.Conclusion. The pSS patients showed signs of both obstructive and restrictive pulmonary disease and COPD commonly developed during follow-up. Respiratory symptoms and radiographic abnormalities were common but poorly associated with PFT in pSS patients.
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| 8. |
- Mohammad, Aladdin, et al.
(författare)
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Incidence, prevalence and clinical characteristics of Behcets disease in southern Sweden
- 2013
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Ingår i: Rheumatology. - : Oxford University Press (OUP): Policy B. - 1462-0324 .- 1462-0332. ; 52:2, s. 304-310
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Tidskriftsartikel (refereegranskat)abstract
- Objective. To study the incidence, prevalence and clinical characteristics of Behc, ets disease (BD) in a defined population in southern Sweden. less thanbrgreater than less thanbrgreater thanMethods. The study area consists of three health-care districts with an adult population (andgt;= 15 years) of 809 317 on 1 January 2011 (25% of non-Swedish ancestry), situated in Skane, the southernmost county in Sweden. Patients were identified using clinical registries in all the five hospitals within the study area. Only patients fulfilling the International Study Group criteria for diagnosis of BD were included. less thanbrgreater than less thanbrgreater thanResults. Forty patients (13 women) fulfilling the diagnosis criteria for BD (70% of non-Swedish ancestry) were identified. The point prevalence of BD on 1 January 2011 was 4.9/100 000 adults (95% CI 3.4, 6.5) and was higher among the population of non-Swedish ancestry (13.6 vs 2.0/100 000, Pandlt;0.001), and higher among men (6.8 vs 3.2/100 000, P=0.019). There were 20 incident cases (diagnosed in Sweden between 1997 and 2010). The annual incidence rate was 0.2/100 000 adults (95% CI 0.1, 0.3) and was higher among the population of non-Swedish ancestry (0.6 vs 0.1/100 000, Pandlt;0.001). The incidence was 0.3/100 000 adults in men and 0.1/100 000 in women, P = 0.143. During the course of the disease, 100% of the patients developed oral ulceration, 80% genital ulcers, 88% skin lesions, 53% eye disease, 40% arthritis/arthralgia and 20% venous thrombosis. less thanbrgreater than less thanbrgreater thanConclusion. The prevalence of BD is higher in Sweden than previously reported, mainly due to immigration. The incidence of BD remains elevated for immigrants from high-prevalence regions even long after settling in Sweden.
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| 9. |
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| 10. |
- Ramos-Casals, Manuel, et al.
(författare)
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Childhood-onset of primary Sjögren's syndrome : phenotypic characterization at diagnosis of 158 children
- 2021
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Ingår i: Rheumatology. - : Oxford University Press. - 1462-0324 .- 1462-0332. ; 60:10, s. 4558-4567
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Tidskriftsartikel (refereegranskat)abstract
- Objectives: To characterize the phenotypic presentation at diagnosis of childhood-onset primary SS.Methods: The Big Data Sjögren Project Consortium is an international, multicentre registry using worldwide data-sharing cooperative merging of pre-existing clinical SS databases from the five continents. For this study, we selected those patients in whom the disease was diagnosed below the age of 19 years according to the fulfilment of the 2002/2016 classification criteria.Results: Among the 12 083 patients included in the Sjögren Big Data Registry, 158 (1.3%) patients had a childhood-onset diagnosis (136 girls, mean age of 14.2 years): 126 (80%) reported dry mouth, 111 (70%) dry eyes, 52 (33%) parotid enlargement, 118/122 (97%) positive minor salivary gland biopsy and 60/64 (94%) abnormal salivary US study, 140/155 (90%) positive ANA, 138/156 (89%) anti-Ro/La antibodies and 86/142 (68%) positive RF. The systemic EULAR Sjögren's syndrome disease activity index (ESSDAI) domains containing the highest frequencies of active patients included the glandular (47%), articular (26%) and lymphadenopathy (25%) domains. Patients with childhood-onset primary SS showed the highest mean ESSDAI score and the highest frequencies of systemic disease in 5 (constitutional, lymphadenopathy, glandular, cutaneous and haematological) of the 12 ESSDAI domains, and the lowest frequencies in 4 (articular, pulmonary, peripheral nerve and CNS) in comparison with patients with adult-onset disease.Conclusions: Childhood-onset primary SS involves around 1% of patients with primary SS, with a clinical phenotype dominated by sicca features, parotid enlargement and systemic disease. Age at diagnosis plays a key role in modulating the phenotypic expression of the disease.
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