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BACKGROUND: Head-to-head studies are important to select the optimal treatment in terms of efficacy and side effect profiles when several drugs are available. AIM OF THE WORK: This article describes all studies comparing the use of disease-modifying antirheumatic drugs (DMARD) in rheumatoid arthritis (RA) in head-to-head studies or in which a DMARD was at least included in an active comparison arm. RESULTS: A total of 23 studies comparing DMARDs were identified. These included comparisons of Janus kinase (JAK) inhibitors with methotrexate and with adalimumab as well as the oral surveillance study. DISCUSSION: There are already an exceptionally large number of head-to-head studies in RA, both for comparisons of efficacy and safety of DMARDs. Nevertheless, more such comparative studies are needed, for example to clarify whether adverse events of tofacitinib observed in the oral surveillance study are specific to the JAK 1/JAK 3 inhibitor or are a class effect of all JAK inhibitors.
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Sjögren's syndrome (SjS) is the most common connective tissue disease with a prevalence of 1:200. Predominantly affecting women, SjS is associated with destruction of the exocrine glands, leading to xerophthalmia and xerostomia. In over 50% of patients, there are also extraglandular manifestations, leading to multiple organ manifestations including polyneuropathies and interstitial lung disease as well as symptoms such as fatigue and arthralgia. Diagnostic procedures include biomarkers, in particular anti-SS-A/Ro antibodies, histology of salivary glands, and salivary gland sonography. There are currently no licensed immunosuppressive drugs for SjS, so current treatment is often based on off-label use of drugs. The European League Against Rheumatism (EULAR) has recently published treatment recommendations based on the prevailing organ manifestations. Several promising controlled trials with novel compounds and concepts are currently in progress.
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Enfermedades Reumáticas , Síndrome de Sjögren , Humanos , Femenino , Síndrome de Sjögren/diagnóstico , Glándulas Salivales/patología , Inmunosupresores/uso terapéutico , Enfermedades Reumáticas/tratamiento farmacológico , BiomarcadoresRESUMEN
BACKGROUND AND OBJECTIVE: The work situation is an important dimension of professional life and wellbeing, and a policy lever to strengthen recruitment and retention. This study aims to explore the work situation of physicians and residents in internal medical rheumatology, considering the impact of the coronavirus pandemic COVID-19. METHODS: A questionnaire-based online survey was conducted in early 2021 at the Hannover Medical School, supported by the German Society of Rheumatology. Target groups were all rheumatology physicians and residents in Germany. The main areas of investigation included work hours, task delegation, and collaboration; workload and mental health issues; discrimination and sexual harassment experiences; and the impact of COVID-19. Descriptive statistical analysis was performed for the standardized items and qualitative content analysis for the free-text information. RESULTS: The respondents (nâ¯= 101) expressed positive attitudes towards cooperation and task delegation to medical assistants, especially those specialized in rheumatology, while attitudes towards cooperation with GPs pointed to blockades. There was a strong mismatch between actual and desired work hours both in the group of women and in the group of men. 81% rated their workload as high or very high; every sixth rheumatologist has suffered from stress or burnout syndromes at least once in the past. Experiences of gender discrimination and sexual harassment/violence were frequently reported, mostly by women. COVID-19 was an amplifier of stress, with major stressors being digitalization and increased demand for communication and patient education. CONCLUSION: There is an urgent need to improve the work situation of rheumatologists and reduce stress and mental health risks.
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COVID-19 , Reumatología , Masculino , Humanos , Femenino , Reumatólogos/psicología , Alemania/epidemiología , Encuestas y CuestionariosRESUMEN
BACKGROUND AND OBJECTIVE: Health workforce shortage in German rheumatology has been identified as a healthcare service and delivery problem. Health policy has increased staffing targets, yet effective intervention strategies are lacking. This research aimed to systematically map the rheumatology workforce to improve the evidence for interventions and explore possibilities for more effective health workforce management. METHODS: The WHO National Health Workforce Accounts provided a conceptual framework for the mapping exercise. Four major sets of indicators were selected, comprising staffing levels, health labor market flows, composition and education/training. A comparison of age groups and time series was applied to explore trends. Public statistics and other secondary sources served our analysis using descriptive methodology. RESULTS: In Germany there are 1076 physicians specialized in internal medical rheumatology. Absolute numbers have nearly doubled (91%) since 2000 but with a strong demographic bias. Between 2000 and 2019 numbers markedly increased in the group aged 50 years and older but only by 9% in the younger group under 50 years; since 2010 the group aged 40-50 years even faces a decrease. In 2019, the absolute numbers of rheumatologists in retirement age exceeded those aged 40 years and under. Since 2015 an expanding workforce trend has overall flattened but this was strongest in the hospital sector; the numbers in resident training did not show any relevant growth. CONCLUSION: Health workforce trends reveal that an available number of rheumatologists cannot meet new health policy planning targets. There is a need for effective health workforce management, focusing on innovation in resident training, improved task delegation and gender equality.
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Reumatología , Humanos , Persona de Mediana Edad , Anciano , Fuerza Laboral en Salud , Reumatólogos , Recursos Humanos , Política de SaludRESUMEN
BACKGROUND: Telemedicine was implemented in outpatient care during the lockdown between March and May 2020. The aim of the study was to assess patients from a private practice and the university outpatient department with respect to patient satisfaction with telemedicine, COVID-19 worries and vaccination behavior and to compare the teleconsultation by a medical assistant for rheumatology (RFA) and a physician. METHODS: Patients with rheumatoid arthritis, psoriatric arthropathy or spondylarthritis without treatment modifications since the previous presentation were offered a telemedical replacement appointment within the framework of this study in the case of appointment cancellation by the treating center. Participants were randomized to a telemedicine appointment by a physician or an RFA (RFA university only). The patient history was carried out by telephone and standardized using a questionnaire. The disease activity was determined using the modified clinical disease activity score (CDAI) and the BASDAI. Subsequently, all patients received a pseudonymized evaluation questionnaire. RESULTS: In total 112/116 (96%) patients participated. Of these 88/112 (79%) returned the questionnaire. The RFAs conducted 19/112 (17%) of the telephone calls. The treatment was modified in 19/112 (17%) patients. Concerns about contracting COVID-19 correlated with high disease activity (pâ¯= 0.031) including the presence of painful joints (pâ¯= 0.001) and high pain levels (VAS ≥7, pâ¯= 0.009). These patients would have also cancelled their appointment themselves (pâ¯= 0.015). Patient satisfaction with the consultation was good (mean 4.3/5.0 modified FAPI) independent of the institution, the duration of the consultation and the consultation partner. Patients with a high pain intensity were the least satisfied (pâ¯= 0.036). Only 42/100 (38.2%) of the patients had been vaccinated against pneumococci and 59/100 (53.6%) against influenza. CONCLUSION: Telemedical care within the framework of a telephone consultation is well-suited for selected patients. With respect to patient satisfaction the delegation of a telemedical consultation to an RFA is possible. There is a need for improvement with respect to the vaccination behavior.
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COVID-19 , Consulta Remota , Reumatología , Telemedicina , COVID-19/epidemiología , COVID-19/prevención & control , Control de Enfermedades Transmisibles , Humanos , Satisfacción del Paciente , SARS-CoV-2 , Telemedicina/métodos , Teléfono , VacunaciónRESUMEN
Genetic studies have led to identification of an increasing number of monogenic primary immunodeficiency disorders. Monoallelic pathogenic gain-of-function (GOF) variants in NFKBIA, the gene encoding IκBα, result in an immunodeficiency disorder, typically accompanied by anhidrotic ectodermal dysplasia (EDA). So far, 14 patients with immunodeficiency due to NFKBIA GOF mutations have been reported. In this study we report three patients from the same family with immunodeficiency, presenting with recurrent respiratory tract infections, bronchiectasis and viral skin conditions due to a novel pathogenic NFKBIA variant (c.106â¯Tâ¯>â¯G, p.Ser36Ala), which results in reduced IκBα degradation. Immunological investigations revealed inadequate antibody responses against vaccine antigens, despite hypergammaglobulinemia. Interestingly, none of the studied patients displayed features of EDA. Therefore, missense NFKBIA variants substituting serine 36 of IκBα, differ from the rest of pathogenic GOF NFKBIA variants in that they cause combined immunodeficiency, even in the absence of EDA.
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Mutación con Ganancia de Función/genética , Síndromes de Inmunodeficiencia/diagnóstico , Leucocitos Mononucleares/inmunología , Meningitis Bacterianas/diagnóstico , Inhibidor NF-kappaB alfa/genética , Neisseria meningitidis/fisiología , Papillomaviridae/fisiología , Infecciones por Pseudomonas/diagnóstico , Pseudomonas aeruginosa/fisiología , Virosis/inmunología , Adulto , Artritis Juvenil , Azitromicina/uso terapéutico , Bronquiectasia , Proliferación Celular , Células Cultivadas , Niño , Displasia Ectodérmica , Gentamicinas/uso terapéutico , Humanos , Síndromes de Inmunodeficiencia/genética , Masculino , Meningitis Bacterianas/tratamiento farmacológico , Linaje , Infecciones por Pseudomonas/tratamiento farmacológico , Virosis/diagnóstico , Verrugas , Adulto JovenRESUMEN
There have been concerns about high rates of thus far undiagnosed SARS-CoV-2 infections in the health-care system. The COVID-19 Contact (CoCo) Study follows 217 frontline health-care professionals at a university hospital with weekly SARS-CoV-2-specific serology (IgA/IgG). Study participants estimated their personal likelihood of having had a SARS-CoV-2 infection with a mean of 21% [median 15%, interquartile range (IQR) 5-30%]. In contrast, anti-SARS-CoV-2 IgG prevalence was about 1-2% at baseline. Regular anti-SARS-CoV-2 IgG testing of health-care professionals may aid in directing resources for protective measures and care of COVID-19 patients in the long run.
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Anticuerpos Antivirales/sangre , Infecciones por Coronavirus/inmunología , Personal de Salud , Neumonía Viral/inmunología , Adolescente , Adulto , Anciano , Betacoronavirus , COVID-19 , Infecciones por Coronavirus/diagnóstico , Femenino , Alemania , Hospitales Universitarios , Humanos , Inmunoglobulina A/sangre , Inmunoglobulina G/sangre , Masculino , Persona de Mediana Edad , Pandemias , Neumonía Viral/diagnóstico , Prevalencia , SARS-CoV-2 , Estudios Seroepidemiológicos , Pruebas Serológicas , Adulto JovenRESUMEN
In the diagnosis of Sjögren's syndrome the Salivary gland sonography (SGUS) has become established and can lead to a higher specificity of the applicable classification criteria. The OMERACT score is used to objectify the SGUS findings. In laboratory diagnostics, the subspecification of anti-SSA/Ro antibodies, but possibly also new biomarkers, are becoming increasingly important regarding diagnostic safety and the expected manifestations. When it comes to prevention, it has been shown that not only psychological stress, but also cardiovascular risk and the risk of lymphoma allow high-risk patients to be identified more precisely in the future. Using cluster analyses, various phenotype groups could be identified to which clinical parameters could be assigned. In 2020, therapy recommendations were published that are based on the clinical manifestations of SjS and recommend medications that are also used in the treatment of systemic lupus erythematosus (SLE) or rheumatoid arthritis. A particularly large number of therapeutic approaches are dedicated to the B cell: Rituximab and Belimumab have been included in the EULAR recommendations for serious manifestations and Ianalumab has a promising effect. Another focus of current research is the inhibition of co-stimulation between immune cells. After recent disappointing results for Abatacept, clinical trials show promising effects on Iscalimab and Dazodalibep.
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Síndrome de Sjögren , Síndrome de Sjögren/terapia , Síndrome de Sjögren/diagnóstico , Humanos , Glándulas Salivales/patología , Anticuerpos Monoclonales Humanizados/uso terapéutico , Rituximab/uso terapéutico , Ultrasonografía , Biomarcadores/sangreRESUMEN
BACKGROUND: MicroRNAs (miRNAs) can regulate gene expression, controlling numerous cellular processes. Dysregulation of miRNA function is linked to various diseases, making them attractive diagnostic and therapeutic targets. Examples include hsa-miR-92a-3p, hsa-miR-126-3p, hsa-miR-143-3p, hsa-miR-145-5p and hsa-miR-204-5p, which are associated with endothelial function. Their prevalence in Sjögren's disease (SjD) is unknown. We assessed the prevalence of these miRNAs in serum of patients with SjD, correlating levels with cardiovascular risk factors and carotid intima-media thickness (cIMT) to evaluate their utility in risk stratification. METHODS: 199 patients with SjD and 100 age and sex-matched healthy controls (HC) were included in the study. Five different miRNAs (hsa-miR-92a-3p; hsa-miR-126-3p; hsa-miR143-3p; hsa-miR-145-5p; hsa-miR-204-5p) were analysed by quantitative real-time PCR. The miRNA results were compared with known clinical and disease-related parameters. RESULTS: Four miRNAs showed significantly different expressions compared with HC. MiR-92a-3p was upregulated (p=0.025) and miR-126-3p (p=0.044), miR-143-3p (p=0.006) and miR-204-5p (p=0.009) downregulated in SjD compared with HC. The comparison between HC and SjD with/without organ involvement revealed descriptively increased miR-92a-3p levels in patients with SjD with organ involvement (p=0.087). Furthermore, miR-92a-3p levels correlated positively with cIMT as an expression of subclinical atherosclerosis (r=0.148, p=0.04). CONCLUSION: In conclusion, patients with SjD demonstrated differences in their expression of miRNAs linked to regulation of endothelial function. Reduction of specific miRNAs was associated with increased cardiovascular risk, suggesting a potentially protective role for these miRNAs. Furthermore, miR-92a-3p could be helpful for molecular detection of early-stage atherosclerosis and increased cardiovascular risk in SjD.
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Aterosclerosis , Biomarcadores , Grosor Intima-Media Carotídeo , MicroARNs , Síndrome de Sjögren , Humanos , MicroARNs/genética , MicroARNs/sangre , Femenino , Persona de Mediana Edad , Masculino , Aterosclerosis/etiología , Aterosclerosis/diagnóstico , Aterosclerosis/genética , Síndrome de Sjögren/complicaciones , Síndrome de Sjögren/diagnóstico , Síndrome de Sjögren/genética , Síndrome de Sjögren/sangre , Estudios de Casos y Controles , Adulto , Perfilación de la Expresión Génica , Anciano , Regulación de la Expresión GénicaRESUMEN
OBJECTIVES: Cardiovascular comorbidities are common in patients with autoimmune diseases. This study investigates the extent of subclinical atherosclerosis in patients with primary Sjögren's syndrome (pSS). Correlations with clinical factors such as organ involvement (OI) or disease activity were analysed and oxLDL antibodies (oxLDL ab) were measured as potential biomarkers of vascular damage. METHODS: Patients with pSS were consecutively included from the rheumatology outpatient clinic. Age- and sex-matched controls were recruited (2:1 ratio). Data collection was performed by a standardised questionnaire and Doppler ultrasound to evaluate the plaque extent and carotid intima-media thickness (cIMT). Propensity score matching included all cardiovascular risk (CVR) factors and corresponding laboratory markers. RESULTS: Data were available for 299 participants (199 pSS/100 controls), aged 59.4 years (50.6-65.0), 19.1% male. After matching, the pSS cohort had greater cIMT (p<0.001) and plaque extent (OR=1.82; 95% CI 1.14 to 2.95). Subgroup analyses of patients with pSS revealed that OI was associated with increased cIMT (p=0.025) and increased plaque occurrence compared with patients without OI (OR=1.74; 95% CI 1.02 to 3.01). OxLDL ab tended to be lower in patients with plaque (p=0.052). Correlations of higher Oxidized Low Density Lipoprotein (oxLDL) ab with EULAR Sjögren's Syndrome Disease Activity Index (p<0.001) and anti-Sjögren's-syndrome-related antigen A autoantibodies (SSA/Ro antibodies) (p=0.026) were observed. CONCLUSIONS: Subclinical atherosclerosis occurs earlier and more severely in patients with pSS. The difference in cIMT between pSS and controls seems mainly driven by patients with OI, suggesting that this subgroup is particularly at risk. OxLDL ab might protect against atherosclerotic progression in patients with pSS. CVR stratification and preventive medications such as Hydroxymethylglutaryl-CoA (HMG-CoA) reductase inhibitors should be discussed and further longitudinal studies are needed.
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Aterosclerosis , Biomarcadores , Grosor Intima-Media Carotídeo , Lipoproteínas LDL , Síndrome de Sjögren , Humanos , Síndrome de Sjögren/complicaciones , Síndrome de Sjögren/epidemiología , Síndrome de Sjögren/inmunología , Síndrome de Sjögren/diagnóstico , Masculino , Persona de Mediana Edad , Femenino , Aterosclerosis/etiología , Aterosclerosis/epidemiología , Aterosclerosis/diagnóstico , Lipoproteínas LDL/sangre , Anciano , Estudios de Casos y Controles , Autoanticuerpos/sangre , Autoanticuerpos/inmunología , Factores de Riesgo , Placa Aterosclerótica/epidemiologíaRESUMEN
Sjögren's syndrome is a potentially treatable cause of Small Fiber Neuropathy (SFN)-a condition that severely affects patients' quality of life. We therefore aimed to characterize patients with SFN and Sjögren's syndrome to raise awareness of this disease and facilitate its early recognition as an essential step for appropriate treatment. In 97 SFN patients (median age 48 years, 77% female), we studied the clinical features associated with Sjögren's syndrome compared to the idiopathic SFN subtype. According to the current ACR/EULAR classification criteria (Shiboski et al., Ann Rheum Dis 76:9-16, 2017), 24/97 individuals (25%, median age 48.5 years, 75% female) were diagnosed with Sjögren's syndrome. We did not observe any differences in SFN-defining sensory plus symptoms. Furthermore, intraepidermal nerve fiber densities (IENFD) were significantly lower in patients with SFN and Sjögren's syndrome (mean 2.6 ± 1.2/mm) compared to patients with idiopathic SFN (mean 3.2 ± 1.5/mm; p = 0.048). There were no significant group differences when analyzing cerebrospinal fluid (CSF) parameters. We conclude that Sjögren's syndrome-associated SFN is difficult to distinguish from idiopathic forms based on initial clinical symptoms and CSF results. However, lower IENFD values in patients with Sjögren's syndrome-associated SFN might indicate a distinct different pathomechanism in this entity compared to idiopathic SFN.
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Síndrome de Sjögren , Neuropatía de Fibras Pequeñas , Humanos , Femenino , Persona de Mediana Edad , Masculino , Neuropatía de Fibras Pequeñas/complicaciones , Neuropatía de Fibras Pequeñas/diagnóstico , Síndrome de Sjögren/complicaciones , Síndrome de Sjögren/diagnóstico , Calidad de Vida , Biopsia/efectos adversosRESUMEN
OBJECTIVE: Neurological manifestations of Sjögren's syndrome can be severe but also treatment-responsive. We aimed to systematically evaluate neurological manifestations of primary Sjögren's syndrome and find clinical features allowing sufficient identification of affected patients (pSSN) among those with Sjögren's syndrome without neurological involvement (pSS). METHODS: Para-/clinical features of patients with primary Sjögren's syndrome (2016 ACR/EULAR classification criteria) were compared between pSSN and pSS. At our university-based center, patients with suggestive neurological symptoms undergo screening for Sjögren's syndrome, and newly diagnosed pSS patients are thoroughly evaluated for neurologic involvement. pSSN disease activity was rated by the Neurological Involvement of Sjögren's Syndrome Disease Activity Score (NISSDAI). RESULTS: 512 patients treated for pSS/pSSN at our site between 04/2018 and 07/2022 were included (238 pSSN patients [46%] vs. 274 pSS patients [54%], cross-sectional design). Independent predictors of neurological involvement in Sjögren's syndrome were male sex [p < 0.001], older age at disease onset [p < 0.0001], hospitalization at first presentation [p < 0.001], lower IgG levels [p = 0.04] and higher eosinophil values (treatment-naïve) [p = 0.02]. Univariate regression additionally showed older age at diagnosis [p < 0.001], lower prevalence of rheumatoid factor [p = 0.001], SSA(Ro)/SSB(La) antibodies [p = 0.03; p < 0.001], higher white blood cell count [p = 0.02] and CK levels [p = 0.02] (treatment-naïve) in pSSN. INTERPRETATION: Patients with pSSN had different clinical characteristics than patients with pSS and represented a large proportion of the cohort. Our data suggest that neurological involvement in Sjögren's syndrome has been underestimated. Intensified screening for neurologic involvement should be included in the diagnostic algorithm for Sjögren's syndrome, especially in males of older age and with severe disease course requiring hospitalization.
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Síndrome de Sjögren , Humanos , Masculino , Femenino , Síndrome de Sjögren/complicaciones , Síndrome de Sjögren/diagnóstico , Estudios Transversales , Prevalencia , Progresión de la EnfermedadRESUMEN
OBJECTIVE: The clinical spectrum of primary antibody deficiencies (PADs) and especially common variable immunodeficiency (CVID) includes various autoimmune disorders. We studied the prevalence and the features of articular rheumatic disease in a cohort of patient with PADs. METHODS: In this retrospective cohort study, complete clinical data of 268 patients with PADs, mainly consisting of patients with CVID, visiting the immunology outpatient clinic of a German tertiary hospital between 2018 and 2021 were collected. Those included case history, physical examination, laboratory as well as radiological findings. RESULTS: Inflammatory arthritis was diagnosed in 16.4% of studied patients and was significantly more common among patients with PAD-associated enteropathy (OR 13.39, p=0.0001), splenomegaly (OR 6.09, p=0.0001) or atopic diseases (OR 3.31, p=0.021). Given HLA-B27 status, the involvement of the axial skeleton and the presence of features, such as anterior uveitis, inflammatory bowel disease, psoriasis and/or dactylitis, 75% of studied patients fulfilled the Assessment of Spondyloarthritis International Society classification criteria. CONCLUSION: PAD-associated arthritis frequently shares features with spondyloarthritis (SpA) and enteropathic arthritis. The latter may suggest the interconnected pathomechanisms of inflammatory arthritis in SpA and PADs.
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Osteoartritis , Enfermedades de Inmunodeficiencia Primaria , Psoriasis , Espondiloartritis , Uveítis Anterior , Humanos , Estudios Retrospectivos , Espondiloartritis/complicaciones , Espondiloartritis/diagnóstico , Espondiloartritis/epidemiología , Uveítis Anterior/complicaciones , Psoriasis/complicaciones , Osteoartritis/complicaciones , Enfermedades de Inmunodeficiencia Primaria/complicacionesRESUMEN
Background: Sjögren's syndrome (SS) is an autoimmune disease characterized by sicca symptoms and various extra-glandular manifestations. The diagnosis of SS requires sicca symptoms, anti-SSA(Ro)-antibody positivity, and/or pathological focus scores on a minor salivary gland biopsy. Previous studies have investigated different biomarkers in order to avoid invasive diagnostic procedures. It was found that kappa and lambda free light chains (KFLC and LFLC) in saliva are specific for SS. Methods: FLC concentrations in saliva and serum were determined in 130 patients-50 with SS and neurological involvement (Neuro-Sjögren) and 80 neurological controls. The EULAR SS disease activity index and patient reported index (ESSPRI) were determined in patients with SS. Results: Patients with SS revealed increased pain and decreased saliva production according to the ESSPRI and Saxon test, respectively, with increasing FLC concentrations in the saliva. No significant differences in serum and salivary protein concentrations were observed between patients with SS and controls. Conclusion: KFLC and LFLC concentrations in saliva are not suitable to distinguish patients with Neuro-Sjögren and neurological control subjects, thus a diagnostic biopsy is still required. The association of salivary KFLC and LFLC concentrations with saliva production and ESSPRI pain score suggests a complex relationship between dryness and pain in patients with SS.
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Introduction: Primary Sjögren's syndrome (pSS) is associated with an increased prevalence of traditional risk factors and cardiovascular diseases (CVDs). The study aimed to identify specific risk factors for CVD in pSS patients. Methods: PSS patients with and without CVD were compared. All patients fulfilled the EULAR/ACR classification criteria. Patients with CVD presented at least one of the following manifestations: myocardial infarction, transient ischemic attacks, ischemic or hemorrhagic stroke, peripheral artery disease, coronary artery disease, and carotid plaques. Data were collected by a standardized protocol and review of medical records. Results: 61/312 (19.6%) pSS patients presented with CVD. Traditional risk factors such as hypertension, hypercholesterinemia and diabetes (p < 0.05), pSS manifestations, in particular vasculitis (p = 0.033) and Raynaud's phenomenon (p = 0.018) were associated with CVD. Among patients with ischemic events (28/312, 9%), particularly cerebrovascular disease (n = 12/28, 42.9%), correlations with increased EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI) (p = 0.039) and EULAR Sjögren's Syndrome Patient Reported Index (ESSPRI) (p = 0.048) were observed. Age at first cerebrovascular event was 55.2 [48.9-69.6] years. Multivariate analysis confirmed hypertension [odds ratio (OR) 3.7, 95% confidence interval (CI) 1.87-7.18, p < 0.001], hypercholesterinemia (OR 3.1, 95% CI 1.63-5.72, p < 0.001), male gender (OR 0.4, 95% CI 0.17-0.78, p = 0.009), Raynaud's phenomenon (OR 2.5, 95% CI 1.28-4.82, p = 0.007), and CNS involvement (OR 2.7, 95% CI 1.00-7.15, p = 0.048) as independent CVD predictors. Conclusion: Raynaud's phenomen as well as vasculitis and high ESSDAI have shown a significant association to CVD. PSS patients with cerebrovascular events were younger than expected. Knowledge about risk factors may help clinicians to identify pSS patients at risk for CVD. After diagnosis of pSS, patients should be screened for risk factors such as hypertension and receive appropriate therapy to prevent or at least reduce sequelae such as infarction. However, further investigations are necessary in order to achieve a reliable risk stratification for these patients.
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BACKGROUND: Patients with Sjögren's syndrome and polyneuropathy more frequently develop cranial nerve affection when compared to patients with chronic inflammatory demyelinating polyneuropathy (CIDP). We therefore aimed to analyze trigeminal corneal nerve fibre characteristics in both patient groups. METHODS: A total of 26 patients with Sjögren's syndrome associated neuropathy and 29 patients with CIDP were recruited at our university hospital and compared to 6 healthy controls. Dry eye symptoms and signs were assessed via clinical examination and the Ocular Disease Surface Index questionnaire. Trigeminal corneal nerve fibres were analyzed via corneal confocal microscopy (CCM) as a non-invasive in vivo microscopy. RESULTS: CCM revealed significantly reduced corneal nerve fibre density and corneal nerve fibre main branch density in the Neuro-Sjögren group when compared with healthy controls. There were no significant group differences between the Neuro-Sjögren and the CIDP group for any of the microscopic parameters. Dry eye assessment showed similarly reduced scores for both patient groups, while healthy controls showed better results for objective dry eye signs. There was no correlation between microscopic parameters of the corneal confocal microscopy and parameters of dry eye assessment. CONCLUSIONS: Our data revealed trigeminal corneal nerve affection in patients with neuropathy associated with Sjögren's syndrome and patients with CIDP detected by CCM. No difference was found between both neuropathy groups indicating that CCM is not able to distinguish between both entities.
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Background: Sicca syndrome represents a heterogeneous group of conditions, such as Sjögren syndrome, causing xerophthalmiaand xerostomia. This study characterizes in depth patients with Sicca syndrome and evaluates salivary gland ultrasound (SGUS). Methods: Principal component analysis and hierarchical clustering of clinical parameters, such as ESSPRI, ESSDAI and laboratory data, were performed on all referrals for assessment of Sicca symptoms between October 2018 and March 2021. SGUS and labial gland biopsies were compared across groups. Results: A total of 583 patients were assessed. Objective dryness was confirmed in 73% of the patients. Cluster analysis identified 3 groups with post-hoc analysis confirming distinct phenotypes: Somatic Group (283/583; 49%) with more frequent symptoms but limited objective dryness; Dry Without Autoimmune Features (DAFneg, 206/584; 35%), and Dry With Autoimmune Features (DAFpos, 94/584;16%). DAFpos patients had highest autoantibody titers (anti-SSA(Ro) 240 vs. 3.6 vs. 3.8; p < 0.001), most extra-glandular manifestations (p < 0.001), and highest median SGUS Score (DAFpos: 8 [IQR 4-10] vs. SG: 2 [1-4] vs. DAFneg 4 [2-5]; p < 0.001). No tangible correlation with primary Sjögren syndrome criteria was observed. Discussion: SGUS score correlated with a subset of patients with Sjögren syndrome, identified in the DAFpos cluster. This study highlights heterogeneity within sicca and, indeed, Sjögren syndrome, highlighting the need for further studies.
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BACKGROUND AND PURPOSE: The phenotype of Sjögren's syndrome-associated neuropathy has been better characterized in recent years. However, Sjögren's syndrome-associated neuropathy remains an underdiagnosed entity with only few insights considering the pathomechanisms of nerve damage. Nerve ultrasound has proven to be a useful and efficient tool in detecting nerve damage of autoimmune origin. We, therefore, aimed to evaluate this method for Sjögren's syndrome-associated neuropathy. METHODS: Patients with Sjögren's syndrome and clinical signs of neuropathy underwent sonographic examination of both median and ulnar nerves. Nerve thickening was classified for cross-sectional areas of >12 mm² at the median nerve and for >10 mm² at the ulnar nerve. Fascicle thickening was documented for cross-sectional areas ≥5 mm² at the median and ≥3 mm² at the ulnar nerve. RESULTS: Forty-three patients were included in the analysis (median age 60 years [interquartile range 53-73 years], female rate 60%). 31/43 patients (72%) showed abnormalities on nerve ultrasound, while nerve thickening was found more frequently than fascicle thickening (90% vs. 52% of patients with sonographic abnormalities, respectively). Abnormal findings were observed more frequently at the median nerve and in proximal localization. Abnormal findings on nerve conduction studies were evident in 36/43 patients (84%). Nerve conduction studies revealed a tendency of demyelinating nerve damage patterns being associated with abnormal findings on nerve ultrasound. CONCLUSIONS: In addition to nerve conduction studies, nerve ultrasound may have a supporting role in the diagnosis of Sjögren's syndrome-associated neuropathy. Also, our data support an immune-mediated inflammatory demyelinating pathogenesis of Sjögren's syndrome-associated neuropathy.
Asunto(s)
Enfermedades del Sistema Nervioso Periférico , Síndrome de Sjögren , Femenino , Humanos , Examen Neurológico/efectos adversos , Enfermedades del Sistema Nervioso Periférico/diagnóstico por imagen , Enfermedades del Sistema Nervioso Periférico/etiología , Síndrome de Sjögren/complicaciones , Síndrome de Sjögren/diagnóstico por imagen , Ultrasonografía/efectos adversosRESUMEN
BACKGROUND: This study addresses the challenging characterisation and differentiation of CIDP versus CIDP in association with Sjögren's syndrome to facilitate the process in clinical routine. METHODS: Patients with both CIDP and Sjögren's syndrome and CIDP without Sjögren's syndrome were compared concerning relevant differences in clinical, laboratory and electrophysiological findings. 154 patients who fulfilled the diagnostic EFNS/PNS criteria for CIDP were included in the analysis. 54 of these patients additionally fulfilled the ACR/EULAR classification criteria for Sjögren's syndrome. RESULTS: The frequency of female patients was higher in patients with CIDP and Sjögren's syndrome (52%) versus CIDP patients without Sjögren's syndrome (28%). Furthermore, the occurrence of cranial nerve impairment was significantly higher in patients with Sjögren's syndrome (39% versus 14%). There were no significant group differences in the evaluation of initial symptoms, severity of disability judged by INCAT disability scale score, presence or distribution of sensory deficits, limb weakness and the presence of ataxia, pain or dysautonomia, CSF laboratory or electrophysiological findings. CONCLUSIONS: In conclusion, our data indicate that cranial nerve impairment and female gender might represent red flags for an additional Sjögren's syndrome in patients with CIDP. The patterns of clinical disabilities and electrophysiological findings due to peripheral nerve damage are similar in both CIDP entities.