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ID PMID Title PublicationDate abstract
33868298 Serum Heparin-Binding Protein as a Potential Biomarker to Distinguish Adult-Onset Still's 2021 Adult-onset Still's disease (AOSD) is a systemic, multifactorial, autoinflammatory disease for which the etiopathogenesis is not well understood. Given the similarities in clinical and laboratory features between this disease and sepsis, and the differences in treatment strategies for these two diseases, specific diagnostic markers are crucial for the correct diagnosis and management of AOSD. Previous studies have shown plasma heparin-binding protein (HBP) is a promising potential biomarker for AOSD; thus, this study aimed to detect serum HBP levels in patients with AOSD or sepsis to assess its potential as a biomarker for differential diagnosis. We found that serum HBP levels were significantly higher in patients with active AOSD than that in those with inactive AOSD. Patients with sepsis had higher serum HBP levels compared with those who had active or inactive AOSD. We calculated the area under the receiver operating characteristic (ROC) curve to assess whether HBP could be used to differentiate active from inactive AOSD; this was 0.811 with sensitivity 0.650, specificity 0.811, and cutoff HBP value of 35.59 ng/ml. The area under the ROC curve for HBP as a biomarker to differentiate AOSD from sepsis was 0.653, with sensitivity 0.759, and specificity 0.552, and cutoff HBP value of 65.1 ng/ml. Taken together, the results of our study suggest that serum HBP could be a useful diagnostic biomarker to evaluate disease activity in patients with AOSD, and to differentiate AOSD from sepsis.
33108647 Granulomatous Cutaneous Drug Eruptions: A Systematic Review. 2021 Jan BACKGROUND: Granulomatous drug eruptions are rare entities, where granuloma formation occurs as an attempt to contain an exogenous or endogenous inciting agent. Granulomatous drug eruptions may be localized to the skin or may include major systemic involvement, and their characteristics depend both on the properties of the causative irritant and host factors. Because of the overlapping features amongst noninfectious granulomatous diseases, granulomatous drug eruptions are challenging to diagnose and distinguish both histologically and clinically. OBJECTIVE: The objective of this article is to provide a review and summary of the current literature on the five major types of cutaneous granulomatous drug eruptions: interstitial granulomatous drug reaction, drug-induced accelerated rheumatoid nodulosis, drug-induced granuloma annulare, drug-induced sarcoidosis, and miscellaneous presentations. METHODS: A systematic review was conducted through PubMed using the search terms "granulomatous drug eruption" and "cutaneous" or "skin". English full-text studies that included human subjects experiencing a cutaneous reaction comprising granulomatous inflammation as the direct result of a drug were included. Of 205 studies identified, 48 articles were selected after a full-text review. Evidence was evaluated using the Tool for evaluating the methodological quality of case reports and case series. RESULTS: Polypharmacy and a prolonged lag period from drug ingestion to rash onset may create diagnostic challenges. Ruling out tuberculosis is imperative in the endemic setting, particularly where anti-tumor necrosis factor therapy is the presumed cause. Interstitial granulomatous drug reactions and granuloma annulare are often localized to the skin whereas accelerated rheumatoid nodulosis and sarcoidosis may sometimes be associated with systemic features as well. Granulomatous drug eruptions typically resolve on discontinuing the offending medication; however, the decision for drug cessation is dependent on a risk-benefit assessment. In some situations, supplementation of an additional agent to suppress the reaction may resolve symptoms. In some cases, granulomatous drug eruptions may be pivotal in the successful outcome of the drug, as in cases of melanoma treatment. In all situations, the decision to continue or withdraw the drug should be carefully based on the severity of the eruption, necessity of continuing the drug, and availability of a suitable alternative. CONCLUSIONS: Granulomatous drug eruptions should always be considered in the differential diagnosis of noninfectious granulomatous diseases of the skin. Further research examining dose-response relationships and the recurrence of granulomatous drug eruptions on the rechallenge of offending agents is required. Increased awareness of granulomatous drug eruption types is important, especially with continuous development of new anti-cancer agents that may induce these reactions. CLINICAL TRIAL REGISTRATION: PROSPERO registration number CRD42020157009.
32413167 Comparison of Clinical Characteristics and Magnetic Resonance Imaging of Salivary Glands W 2021 Jan OBJECTIVES/HYPOTHESIS: To compare the results of magnetic resonance imaging with magnetic resonance sialography (MRSIAL) and the clinical and laboratory characteristics in a well-characterized cohort of patients with primary or secondary Sjögren's syndrome (SS) meeting the American-European Consensus Group criteria. STUDY DESIGN: Retrospective, observational, monocentric study. METHODS: Thirty-six patients (81% female, mean age = 48 ± 35 years) with primary or secondary SS who underwent MRSIAL were included in the study. RESULTS: MRSIAL revealed characteristic radiological signs in the parotid, sublingual, and submandibular salivary glands in 35/36 patients (97%). Patients presenting with anti-Sjögren's syndrome-related antigen A (SSA) autoantibodies showed more often fatty infiltration, a "pepper-and-salt" appearance, ductal stenosis, and/or ductal dilation of the parotid gland (88%, 88%, and 72% respectively) than patients negative for anti-SSA (12%, 4%, and 28% respectively). MRSIAL demonstrated signs characteristic of SS in all 11 patients with negative minor salivary gland biopsy. For 15 patients undergoing ultrasound examination only, 11 (73%) had SS findings, but all 15 had SS findings on MRSIAL. Two cases of parotid lymphoma were detected by MRSIAL (6%). CONCLUSIONS: MRSIAL is a reliable technique to detect glandular anomalies in patients with SS, and seems to provide a valuable aid in the diagnosis of SS. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:E83-E89, 2021.
34837998 Acute visual loss as the first ocular symptom in a Sjögren's syndrome patient with bilate 2021 Nov 27 BACKGROUND: Sjögren's syndrome may be a risk factor for carotid artery stenosis. Bilateral common carotid artery occlusion (BCCAO) in a patient with Sjögren's syndrome was not reported before. In this report, we describe a female with Sjögren's syndrome who had acute visual loss due to ocular ischemic syndrome (OIS) with BCCAO. CASE PRESENTATION: A 50-year-old female with Sjögren's syndrome visited our clinic with acute visual loss in the left eye. The best corrected visual acuity (BCVA) was 2/100 in the left eye, and the intraocular pressure (IOP) was normal in both eyes. Ocular ischemic change was observed during the ophthalmic examination. Aortography and computed tomography angiography (CTA) showed nearly total occlusion of the bilateral CCA. Thus, OIS with BCCAO was diagnosed. The vision in the left eye improved to 30/100 after carotid artery stenting for the left common carotid artery. CONCLUSIONS: BCCAO may be present in patients with Sjögren's syndrome. Large vessel abnormalities should be considered when acute visual loss is found in a patient with Sjögren's syndrome.
34596022 Research progress on the pathogenesis and quality of life of patients with primary Sjögre 2022 Mar In the past decade, an increasing number of studies have found a relationship between the occurrence and development of depression and autoimmune diseases, and the high prevalence of depression in patients with connective tissue diseases has also been confirmed. Primary Sjögren's syndrome (pSS) is a chronic autoimmune exocrinopathy characterised by lymphocytic infiltration and exocrine gland destruction. Depression in pSS patients is common, and the factors contributing to this condition are complicated. pSS patients with depression generally have a lower quality of life than pSS patients without depression. Several pathophysiological mechanisms involved in the condition have been proposed in recent years. Thus, in this review, we summarised recent progress on the impact of depression on pSS patients' quality of life, the possible pathogenesis underlying the development of depression in pSS patients and the management of such patients.
33765165 Systematic evaluation of laryngeal impairment in Sjögren's syndrome. 2021 Jul INTRODUCTION: Sjögren's syndrome (SjS) causes malfunction of the salivary and lacrimal glands. Consequently, patients suffer from xerostomia and keratoconjunctivitis sicca. This can further affect the voice and swallowing function resulting in an impaired quality of life. Aim of this study is the systematic evaluation of the impact on voice and swallowing-related quality of life in patients with SjS. MATERIAL AND METHODS: SjS patients were classified according to the American-European Consensus Group (AECG) criteria; antibodies to Ro (SS-A) or La (SS-B) antigens were detected, ESSPRI was completed. We used the following quality of life questionnaires: EORTC QLQ H&N 35, Anderson Dysphagia Inventory (ADI) and Voice Handicap Index (VHI). Patients additionally received a detailed phoniatric examination (auditory perception, videostroboscopy, acoustic analysis, Dysphonia Severity Index (DSI), aerodynamics measurements). RESULTS: Almost all the 54 patients (96.3%) had a limited quality of life due to their swallowing problems and 48% due to their voice problems. Both values correlated significantly with the degree of xerostomia. In the phoniatric examination, 77.8% had an increased DSI and two-thirds had abnormalities in videostroboscopy. CONCLUSIONS: A reasonable impairment of quality of life in patients with SjS due to the limitations in voice and swallowing function was observed. As SjS does not limitate life expectancy, preservation of quality of life is important. Detection of voice and swallowing problems as potential reasons for quality of life impairment should be detected and, if diagnosed, treated accordingly.
32934136 National Sjögren's Foundation Survey: Burden of Oral and Systemic Involvement on Quality 2021 Jul OBJECTIVE: To define the association between oral and systemic manifestations of Sjögren syndrome (SS) and quality of life (QOL). METHODS: We analyzed a cross-sectional survey conducted by the Sjögren's Foundation in 2016, with 2961 eligible responses. We defined oral symptom and sign exposures as parotid gland swelling, dry mouth, mouth ulcers/sores, oral candidiasis, trouble speaking, choking or dysphagia, sialolithiasis or gland infection, and dental caries. Systemic exposures included interstitial lung disease, purpura/petechiae/cryoglobulinemia, vasculitis, neuropathy, leukopenia, interstitial nephritis, renal tubular acidosis, autoimmune hepatitis, primary biliary cholangitis, or lymphoma. Outcomes included SS-specific QOL questions generated by SS experts and patients. RESULTS: Using multivariable regression models adjusted for age, sex, race, and employment, we observed that mouth ulcers or sores, trouble speaking, and dysphagia were associated with poor quality of life. The following oral aspects had the greatest effect on the following QOL areas: (1) mouth ulcers/sores on the challenge and burden of living with SS (OR 4.26, 95% CI 2.89-6.28); (2) trouble speaking on memory and concentration (OR 4.24, 95% CI 3.28-5.48); and (3) dysphagia on functional interference (OR 4.25, 95% CI 3.13-5.79). In contrast, systemic manifestations were associated with QOL to a lesser extent or not at all. CONCLUSION: Oral manifestations of SS, particularly mouth ulcers or sores, trouble speaking, and dysphagia, were strongly associated with worse QOL. Further study and targeted treatment of these oral manifestations provides the opportunity to improve quality of life in patients with SS.
32452353 The treatment of adult-onset Still's disease with anakinra, a recombinant human IL-1 recep 2021 Jan Adult-onset Still's disease (AOSD) is a rare, inflammatory disease of unknown aetiology, generally affecting young adults and requiring immunosuppressive treatment. In the last few years, bio- logic disease-modifying anti-rheumatic drugs (bDMARDs) have been successfully used in refractory cases, based on the pathogenic role of inflammatory cytokines in AOSD. Amongst bDMARDs, several observations confirmed the clinical usefulness of anakinra, a recombinant human non-glycosylated IL-1 receptor antagonist, in AOSD. At present, the treatment is still largely empirical and due to the possible fallacious aspects of clinical judgement, in this work, we performed a systematic review of literature (SRL) to summarise the evidence regarding the treatment with anakinra in AOSD, analysing rate of complete remission, corticosteroids (CCSs)-sparing effect, long-term retention rate, and safety. After screening titles, abstracts and analysis of full text, 15 manuscripts were analysed: 1 open randomised multicentre trial with two parallel groups and 14 observational single-arm retrospective studies. Collectively, results of the present SRL suggest the effectiveness of anakinra in the treatment of patients with AOSD. Furthermore, patients with AOSD are likely to achieve a good clinical response with anakinra and these outcomes are associated with a largely favourable safety profile. Furthermore, the majority of patients treated with anakinra may achieve a complete remission, also in monotherapy. Finally, the treatment with anakinra is associated with an important CCSs-sparing effect, and, a large percentage of these patients may stop CCSs, thus reducing predictable long-term CCSs side effects without the occurrence of new flares.
34396729 [ETIOLOGIES OF EXTREME HYPERFERRITINEMIA - ANALYSIS OF A LARGE DATABASE]. 2021 Aug INTRODUCTION: Besides its role in iron homeostasis and storage, ferritin is also regarded as an acute-phase reactant. Extreme Hyperferritinemia is seen in severe inflammatory conditions, severe infections, iron storage diseases and malignancies. A direct linkage between high ferritin levels and poor prognosis has been observed. OBJECTIVES: To characterize patients with extreme high ferritin levels in the serum for possible etiologies and assessment of the correlation between ferritin levels, prognosis and mortality. METHODS: We conducted a retrospective cohort study between the years 2002-2016 using the large database of Clalit Health Services. Patients older than 18 years with ferritin levels above 10,000 ng/ml that were taken during hospitalization and ambulatory visits were included in the study. After examining the medical files of each patient, we evaluated the demographic characteristics, etiologies, clinical presentation and relevant laboratory parameters. We calculated the proportion of this data and compared it to the general population by using chi square test. RESULTS: The incidence of extreme hyperferritinemia was statistically significant in patients with autoimmune and rheumatologic diseases in particular adult onset Still's disease compared to the general population. Among hospitalized patients, bacterial and viral infections were the leading cause in 62% of cases. In ambulatory patients, hyperferritinemia was mainly secondary to chronic blood transfusions in patients with hemoglobinopathies and poor compliance to iron chelators. Among 21 biopsies from involved organs including lymph nodes, bone marrow and liver, hemophagocytosis was only observed in 5 cases (6.8%). CONCLUSIONS: Extreme hyperferritinemia with values higher than 10,000 ng/ml can be attributed to many inflammatory autoimmune conditions.
33661446 Lubricating properties of chewing stimulated whole saliva from patients suffering from xer 2021 Jul OBJECTIVES: The study aimed to quantify the lubricating properties of chewing stimulated whole saliva from healthy controls (n = 22), from patients suffering from primary Sjögren's syndrome (n = 37) and from patients undergoing head-and-neck radiotherapy (n = 34). MATERIALS AND METHODS: All participants had to complete the Xerostomia Inventory questionnaire to score dry mouth sensation. Lubrication was measured using an ex vivo tongue-enamel friction system in terms of Relief and Relief period. MUC5b and total protein concentrations of the saliva samples were measured by an enzyme-linked immunosorbent assay and a bicinchoninic acid assay, respectively. RESULTS: Relief of Sjögren's patients' saliva and post-irradiation patients' saliva was similar compared with healthy controls, but saliva from post-irradiation patients lubricated significantly better than saliva from Sjögren's patients. The Relief period was similar between the three groups. The Relief and Relief period were higher for saliva samples post-irradiation compared to pre-irradiation. MUC5b and total protein concentrations were comparable in all groups. MUC5b and total protein output were significantly lower in patients subjected to radiotherapy compared to saliva from healthy controls and pre-irradiation patients. MUC5b concentrations positively correlated with lubricating properties of post-irradiation patient saliva. CONCLUSIONS: The lubricating properties of patient saliva were not any worse than healthy controls. Lower flow rate leads to lower availability of saliva in the oral cavity and decreases the overall output of protein and MUC5b, which might result in an insufficient replenishing of the mucosal salivary film. CLINICAL RELEVANCE: An insufficient replenishing might underlie the sensation of a dry mouth and loss of oral function.
33476812 Pathophysiologic role of Interleukin-33/ST2 in Sjögren's syndrome. 2021 Mar Interleukin-33 (IL-33) is a member of the IL-1 family and has dual functions as a nuclear factor as well as a cytokine. The pivotal role of IL-33 as an active player contributing to aberrant local and systemic damage has been highlighted in several inflammatory and autoimmune diseases. Primary Sjögren's syndrome (pSS) is an autoimmune disease characterized by dry eyes and mouth syndrome due to local dysfunctions of exocrine glands, but also accompanied with systemic manifestations. The pathophysiology of pSS has been advocated as a conjecture of activated B and T cells as well as the production of inflammatory cytokines and autoantibodies, driving epithelial tissue damage and disease progression. In pSS, IL-33 is released in the extracellular space from damaged salivary cells upon pro-inflammatory stimuli and/or dysfunction of epithelial barrier. Counter-regulatory mechanisms are initiated to limit the pro-inflammatory actions of IL-33 as portrayed by an increase in the decoy receptor for IL-33, the soluble form of ST2 (sST2). In pSS and associated diseases, the levels of IL-33 are significantly elevated in the serum or tears of patients. Mechanistically, IL-33 acts in synergy with IL-12 and IL-23 on NK and NKT cells to boost the production of IFN-γ contributing to inflammation. TNF-α, IL-1β and IFN-γ in turn further increase the activation of IL-33/ST2 pathway, thereby constituting a vicious inflammatory loop leading to disease exacerbation. IL-33/ST2 axis is involved in Sjögren's syndrome and opens new perspectives as therapeutic target of one of the culprits in the inflammatory perpetuation.
34718160 Cancer incidence in primary Sjögren's syndrome: Data from the French hospitalization data 2021 Dec The relationship between cancer and primary Sjögren's syndrome (pSS) is uncertain. While the increased risk of hematological malignancies is well-known, data on the comparative incidence of solid neoplasms is conflicting. This study aimed to explore the associations between cancer and pSS. This nationwide population-based retrospective study from the French health insurance database (PMSI) evaluated patients hospitalized with new-onset pSS from 2011 to 2018 against age- and sex-matched hospitalized controls (1:10). The incidence of hematological malignancies and solid neoplasms was compared between the two groups. Mortality and multiple cancer incidence were also evaluated. Adjusted Hazard Ratios (aHR) calculations included confounding factors, such as low socioeconomic status. Among 25,661 hospitalized patients with pSS versus 252,543 matched patients (median follow-up of 3.96 years), we observed a higher incidence rate of lymphomas (aHR, 1.97 [95% CI, 1.59-2.43]), Waldenström macroglobulinemia (aHR, 10.8 [6.5-18.0]), and leukemia (aHR, 1.61 [1.1-2.4]). Thyroid cancer incidence was higher (aHR, 1.7 [1.1-2.8]), whereas bladder and breast cancer incidences were lower (aHR, 0.58 [0.37-0.89] and 0.60 [0.49-0.74], respectively). pSS patients with breast cancer exhibited a lower mortality rate. A limitation was that the database only encompasses hospitalized patients, and immunological and histological details are not listed. We confirmed the increased risk of hematological malignancies and thyroid cancers among patients with pSS. The lower risk of breast cancer suggests a role of hormonal factors and raises questions of the concept of immune surveillance within breast tissue. Epidemiological and translational studies are required to elucidate the relationships between pSS and cancer.
33934073 Correlation Between Subjective and Objective Severity of Oral and Ocular Dryness in Primar 2021 Aug OBJECTIVE: Sjögren syndrome (SS) is a common autoimmune disease primarily affecting the eyes and mouth. With no single gold standard test for its diagnosis, accurate identification of patients with SS continues to be challenging. We aimed to assess the correlation of ocular and oral symptoms of dryness with objective measures in order to evaluate reliability in the screening of primary SS (pSS) in clinical practice. METHODS: We conducted a cross-sectional analysis of pre-screened pSS and sicca control patients assessed in the Multidisciplinary Sjögren's Clinic at the University Health Network in Toronto. The signs, symptoms, and objective measure of oral and ocular dryness and damage of each patient were prospectively recorded using a standardized protocol. RESULTS: Subjective measures of severity for xerophthalmia and xerostomia correlated in general with objective severity. Oral symptoms tend to have a stronger correlation with objective findings than ocular symptoms. Many patients with few or insignificant eye symptoms had profound ocular dryness and damage. Similarly, some patients with few or no symptoms of oral dryness had profound objective salivary hypofunction. The absence of symptoms does not rule out profound eye and mouth dryness or damage. CONCLUSION: Although objective measures of xerostomia may not be practical for general population screening, it is crucial that practicing specialists perform objective testing of all patients suspected of pSS, instead of relying on symptoms. Without objective testing, the physician cannot ensure the diagnosis of pSS and that the existence of significant damage is not overlooked and left untreated.
33493333 Childhood-onset of primary Sjögren's syndrome: phenotypic characterization at diagnosis o 2021 Oct 2 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.
33369675 Adult-onset Still's disease with neurological involvement: a single-centre report. 2021 Sep 1 OBJECTIVES: Adult-onset Still's disease (AOSD) is a multifactorial systemic autoinflammatory disease. Neurological damage has been rarely reported in AOSD. We aimed to characterize the clinical features of AOSD patients with neurological involvement. METHODS: A total of 187 AOSD patients were admitted to Peking Union Medical College Hospital from January 2015 to August 2019. The complete medical records were reviewed in this retrospective study. Clinical features of 14 AOSD patients with neurological involvement were collected and compared with those without. RESULTS: The prevalence of neurological involvement in AOSD inpatients was 7.5%. The median disease duration was 4.5 months, with a range of 1-15 months. The frequent symptoms were fever [14 (100%)], rash [13 (92.9%)], liver dysfunction [11 (78.6%)], arthralgia/arthritis [10 (71.4%)] and lymphadenopathy [10 (71.4%)]. Four (28.6%) patients had macrophage activation syndrome (MAS). Aseptic meningitis was the most common presentation (64.3%) when the nervous system was involved. Other rare manifestations included cranial nerve palsy, encephalitis and cerebral infarction. The rate of MAS, serum levels of lactate dehydrogenase and ferritin were significantly higher in AOSD patients with neurological involvement than in those without. All patients received high-dose corticosteroid therapy and immunosuppressive agents and two were given tocilizumab. Clinical remission was achieved in all 14 AOSD patients with neurological involvement. CONCLUSION: Neurological involvement, particularly aseptic meningitis, is not a rare complication of AOSD. It is frequently complicated by MAS. There may be a potential relationship between the neurological damage of AOSD and MAS.
32224257 Complementary therapy with Traditional Chinese Medicine for a patient with Sjögren's synd 2021 May OBJECTIVE: . A case study was used to discuss the effects of Traditional Chinese Medicine (TCM) treatments on Sjögren's syndrome. CLINICAL FEATURES AND OUTCOMES: . A 45-year-old woman suffered from dry eyes, dry mouth, and fatigue for six months and was diagnosed with Sjögren's syndrome. She had received regular treatment with hydroxychloroquine (HCQ) and artificial tears as well as artificial saliva for nearly one year, but the results were unsatisfactory. Therefore, she sought CHM for further intervention. After 7 months of Yi-Guan-Jian with Huai-Xiao-Mai (Triticum aestivum Linn.) and Tian-Hua-Fen (Trichosanthis Radix), on the seventh treatment with TCM, she reported no fatigue or sleep dysfunction and relief of dry eyes and mouth. Neither complications nor side effects were noted during the CHM treatment. CONCLUSIONS: . From this case, we concluded that CHM may be an effective and safe alternative therapy for the treatment of Sjögren's syndrome.
34366044 A 29-Year-Old Woman With Cough, Dry Eyes, Pulmonary Cysts, and Nodules. 2021 Aug A 29-year-old woman who is a never smoker and has a medical history of systemic hypertension presented with a 3-week history of generalized fatigue and dry cough. She endorsed sicca symptoms of dry eyes and dry mouth. She denied breathlessness, fever, chills, night sweats, or weight loss. She had no heartburn, postnasal drip, joint pain, swelling, or skin lesions. She had no known lung disease or history of pneumothorax. Her family history was unremarkable.
33188390 Baseline disease activity influences subsequent achievement of patient acceptable symptom 2021 Jun 18 OBJECTIVES: To investigate longitudinal changes of the EULAR SS Patient-Reported Index (ESSPRI) and EULAR SS Disease Activity Index (ESSDAI), and identify factors associated with patient acceptable symptom state (PASS) in patients with primary SS (pSS). METHODS: We assessed ESSPRI, ESSDAI, clinical ESSDAI (ClinESSDAI), EULAR Sicca Score, EuroQoL 5-dimension (EQ-5D), Fatigue Severity Score, Beck Depression Inventory, and patient global assessment (PGA) for pSS, and visual analogue scale (VAS) scores for glandular and extra-glandular symptoms at baseline and follow-up. The responses to the currently available standards of care were evaluated by the PASS, the minimal clinically important improvement (MCII) of ESSPRI and ESSDAI, and a modified SS Responder Index-30 (mSSRI-30) response. RESULTS: Among 115 patients enrolled, 102 (88.7%) completed a median 3-year follow-up. The ESSPRI, ClinESSDAI and EQ-5D levels remained stable, although the PGA and ESSDAI significantly improved (both P <0.05). Of the 102 patients, 52 (51.0%) patients achieved the PASS at the follow-up and tended to attain the ESSPRI-MCII and mSSRI-30 (both P < 0.001) more frequently than the non-PASS group. Multivariate analysis revealed that the PASS was significantly associated with baseline ESSPRI negatively [odds ratio (OR) 0.609] and ESSDAI positively (OR 1.224). When categorized using baseline ESSPRI and ESSDAI, a subgroup of low ESSPRI and high ESSDAI reached a PASS achievement rate of 79.3%. CONCLUSION: Although longitudinal changes in ESSPRI and ClinESSDAI are stable in pSS, baseline ESSPRI and ESSDAI could provide prognostic information on the subsequent achievement of PASS, using currently available treatments. A categorization model using ESSPRI and ESSDAI may have clinical implications.
34864891 Pregnancy outcomes in relation to disease activity and anti-rheumatic treatment strategies 2021 Dec 3 OBJECTIVES: To explore the association of maternal rheumatoid arthritis (RA) to pregnancy outcomes, especially preterm birth (PTB) and small for gestational age (SGA), in relation to disease activity and anti-rheumatic treatment before and during pregnancy. METHODS: By linking prospective clinical rheumatology registers (CRR) in Sweden (SRQ) and Denmark (DANBIO) with medical birth registers, we identified 1,739 RA-pregnancies and 17 390 control-pregnancies (matched 1:10 on maternal age, birth year, parity) with delivery 2006-2018. Disease activity (DAS28, CRP, HAQ-score) and anti-rheumatic treatment nine months before and during pregnancy were identified through CRR and prescribed drug registers. Using logistic regression, we estimated adjusted odds ratios (aOR) with 95% confidence intervals (CI) for PTB and SGA overall and stratified by disease activity and anti-rheumatic treatment before and during pregnancy, adjusting for maternal characteristics. RESULTS: We found increased aOR of PTB (1.92, 1.56-2.35) and SGA (1.93, 1.45-2.57) in RA-pregnancies vs control-pregnancies. For RA-pregnancies with DAS28-CRP ≥ 4.1 vs < 3.2 during pregnancy, aOR was 3.38 (1.52-7.55) for PTB and 3.90 (1.46-10.4) for SGA. Use of oral corticosteroids (yes/no) during pregnancy resulted in an aOR of 2.11 (0.94-4.74) for PTB. Corresponding figure for biologics was 1.38 (0.66-2.89). Combination therapy, including biologics before pregnancy, was a marker of increased risk of both PTB and SGA. CONCLUSION: During pregnancy, disease activity rather than treatment seems to be the most important risk factor for PTB and SGA in RA. Women with RA should be carefully monitored during pregnancy, especially if they have moderate to high disease activity or/and are treated with extensive anti-rheumatic treatment.
33613035 Assessment of Adipokines, CXCL16 Chemokine Levels in Patients With Rheumatoid Arthritis Co 2021 OBJECTIVE: Rheumatoid arthritis (RA), which is a chronic systemic inflammatory disease, is associated with accelerated atherosclerosis and an increased risk of cardiovascular disease (CVD), but the causal factors have yet to be completely elucidated. The studies show that the prevalence of metabolic syndrome (MtS) was significantly higher in RA patients compared to the population. In RA and MetS inflammation and atherosclerosis are closely linked. The level of chemokines and adipokines, which may play a role in the development of atherogenesis in RA with MetS patients is currently unknown. In this study, we investigated the level of chemokine C-X-C motif chemokine ligand 16 (CXCL16) and adipokine in RA with MetS patients and assessed the association of biomarkers with clinical and biochemical activity scores of RA and components of MetS. METHODS: Blood serum of 298 people (48-patients with RA and MetS, 82-with RA without MetS, 105-with MetS, 63-control group without both RA and MetS) was tested for (CXCL16), Resistin, Leptin and Fibroblast Growth Factor 21 (FGF21) levels by fluorescent antibody technique. Statistical analysis was performed using SPSS version 18.0. RESULTS: The biomarker study showed the highest level in the RA with MetS patient group; but as compared with the RA group the differences were insignificant. CXCL16 (Me = 426.2 pg/ml (Q(25-75) 250.5-527.6), resistin (Me = 8685.4 pg/ml (Q(25-75) 6480.8-13 629.1), and FGF21 (Me = 443.6 pg/ml (Q(25-75) 772.9-916.3) proved to be significantly augmented in RA with MetS patients group, and in RA without MetS patients group (Me = 312.7 (Q(25-75) 199.4-517.7) pg/ml; Me = 8265.3 (Q(25-75) 5779.7-13 340.5) pg/ml; Me = 412.4 (Q(25-75) 300.4-497.4) pg/ml, respectively) as compared with MetS patients group (Me = 189.4 (Q(25-75) 130.3-280.6) pg/ml; Me = 5364.8 (Q(25-75) 2368.9-10 160.9) pg/ml; Me = 133.2 (Q(25-75) 76.2-268.6) pg/ml, respectively; P = <.001). Leptin level in all groups was higher than in the control group, but there were no differences between groups. The correlation analysis found a positive relationship between the leptin level and the waist circumference (rs = 0.39; P = .007) in the RA with MetS patients, the association of biomarkers with DAS28 score and ESR did not have any statistical significance. Conclusions: The augmented chemokine, resistin and FGF21 in the RA with MetS patients proves the systemic inflammation which is the basis of RA; the augmented leptin is linked to the abdominal obesity. These data are somewhat of an explanation of the increased risk of the CVD development in RA with MetS people. A differentiated specification can be useful to assess the cardiovascular risk of patients and justify prompt personalized treatment.