This study aims to investigate ultrasound (US) findings on salivary glands (SG) in patients with Sjögren syndrome (SS) vs. other connective tissue diseases (CTDs) and to assess the relationship of ...SGUS abnormalities with autoantibody profile in both groups. We enrolled 81 patients, 45 diagnosed with SS (39 with primary SS, 6 with secondary SS) and 36 diagnosed with other CTDs. All patients underwent a prospective evaluation of sicca symptoms, a Schirmer’s test, and a B-mode US assessment of the parotid and submandibular glands, all blinded to the diagnosis. Each SG was semi-quantitatively scored 0–3; a grade ≥ 2 was considered pathological. SGUS involvement was classified as normal or pathological at the patient level and for each pair at the gland level. In addition, a total SGUS score of 0–12 and a parotid/submandibular score of 0–6 were calculated for each patient. Autoimmunity laboratory data were also obtained. All SGUS scores were higher in SS patients than in those with CTD (
p
< 0.001) and significantly more SS patients showed a pathological global (
p
< 0.001), parotid (
p
< 0.001), or submandibular (
p
= 0.001) US score compared with CTD patients. In SS patients, the presence of autoantibodies was significantly associated with pathological SGUS and higher scores, particularly at the parotid level, while in CTD patients, xerostomia and a pathological Schirmer’s test were associated with pathological US and higher scores at the submandibular level (
p
< 0.05). SGUS showed a different grade of abnormality, site involvement, and associated autoantibody profile in SS patients as compared with other CTD.
Key Points
•
Patients with SS and other CTDs showed different grades of SGUS abnormality
.
•
Patients with SS and other CTDs showed different gland involvement and associated autoantibody profiles
.
•
Anti-Ro60 and anti-Ro52 Ro60 positivity were associated with the severity of parotid involvement in SS patients.
To formulate SER recommendations for the use of biological agents in primary Sjögren's syndrome (pSS).
Relevant clinical research questions were identified on the use of biological agents in pSS. The ...clinical questions were reformulated into 4PICO questions. A search strategy was designed and a review of the scientific evidence of studies published until May 2017 was carried out. The scientific evidence available was systematically reviewed. The overall level of scientific evidence was assessed using the SIGN evidence levels. After that, specific recommendations were made.
Rituximab is recommended as the biological agent of choice for extraglandular manifestations refractory to conventional treatment. The use of anti-TNF agents is discouraged. The scientific evidence with belimumab and abatacept is scarce, so they should be considered only in cases refractory to rituximab.
Rituximab is the biological agent of choice in severe extraglandular manifestations of pSS. Belimumab or abatacept may be useful in selected cases.
The objective of the present study is to describe the characteristics of interstitial pneumonia with autoimmune features (IPAF) patients, to assess the incidence rate of functional respiratory ...impairment over time and to evaluate the influence of therapeutic alternatives on the prognosis of these patients.
A longitudinal observational multicenter study was performed (NEREA registry). It was carried out by a multidisciplinary team in seven Hospitals of Madrid. Patients were included from IPAF diagnosis.
poor prognosis as functional respiratory impairment (relative decline in FVC % defined as ≥ 5% every 6 months). Covariates: therapy, sociodemographic, clinical, radiological patterns, laboratory and functional tests.
Survival techniques were used to estimate IR per 100 patients-semester with their 95% confidence interval CI. The influence of covariates in prognosis were analyzed through cox multivariate regression models (hazard ratio (HR) and CI).
79 IPAF were included, with a mean and a maximum follow-up of 3.17 and 12 years respectively. Along the study, 77.2% received treatment (52 glucocorticoids, 25 mycophenolate, 21 azathioprine, 15 rituximab and 11 antifibrotics). IR was 23.9 19.9-28.8, and 50% of IPAF developed functional respiratory impairment after 16 months from its diagnosis. Multivariate analysis: usual interstitial pneumonia (UIP) had poorer prognosis compared to non-specific interstitial pneumonia (NSIP) (p = 0.001). In NSIP, positive ANA, increased the risk of poor prognosis. In UIP, glucocorticoids (HR: 0.53 0.34-0.83), age (HR: 1.04 1.01-1.07), and Ro-antibodies (HR: 0.36 0.19-0.65) influenced the prognosis.
IPAF have functional impairment during the first years of disease. Factors predicting deterioration differ between radiographic patterns. Our real-life study suggests the potential benefit of particular therapies in IPAF.
Abstract
Background
To assess mortality rate (MR) and standardized mortality rate (SMR) of rheumatoid arthritis-related interstitial lung disease (RA-ILD) patients and to evaluate the role of ...radiographic patterns in mortality.
Methods
A longitudinal multicentric study was conducted in RA-ILD patients from 2005 to 2015 and followed-up until October 2018 in Madrid. Patients were included in the Neumologia-Reumatología y Enfermedades Autoinmunes Registry, from diagnosis of ILD. The main outcome was all-cause mortality. The radiographic pattern at baseline usual interstitial pneumonia (UIP), nonspecific interstitial pneumonia (NSIP), or others was the independent variable. Covariables included sociodemographic and clinical data. Survival techniques were used to estimate MR, expressed per 1000 persons-year with their 95% confidence intervals CI. Cox multiple regression model was run to examine the influence of radiographic patterns on survival. SMR CI was calculated comparing MR obtained with MR expected in the general population of Madrid by indirect age-gender standardization.
Results
47 patients were included with a follow-up 242 patients-year. There were 16 (34%) deaths, and most frequent causes were acute ILD exacerbation and pneumonia. MR was 64.3 39.4–104.9, and 50% of the patients died at 8.3 years from ILD diagnosis. After adjusting for confounders, (UIP compared to NSIP was associated with higher mortality risk. The overall SMR was 2.57 1.4–4.17. Women of 60–75 years of age were the group with the highest SMR.
Conclusions
RA-ILD is associated with an excess of mortality compared to general population. Our results support that UIP increases the risk of mortality in RA-ILD, regardless other factors.
This study aims to determine similarities and differences in clinical characteristics between the patients from two waves of severe acute respiratory syndrome coronavirus-2 infection at the time of ...hospital admission, as well as to identify risk biomarkers of coronavirus disease 2019 severity.
Retrospective observational study.
A single tertiary-care center in Madrid.
Coronavirus disease 2019 adult patients admitted to hospital from March 4, 2020, to March 25, 2020 (first infection wave), and during July 18, 2020, and August 20, 2020 (second infection wave).
Treatment with a hospital-approved drug cocktail during hospitalization.
Demographic, clinical, and laboratory data were compared between the patients with moderate and critical/fatal illness across both infection waves. The median age of patients with critical/fatal coronavirus disease 2019 was 67.5 years (interquartile range, 56.75-78.25 yr; 64.5% male) in the first wave and 59.0 years (interquartile range, 48.25-80.50 yr; 70.8% male) in the second wave. Hypertension and dyslipidemia were major comorbidities in both waves. Body mass index over 25 and presence of bilateral pneumonia were common findings. Univariate logistic regression analyses revealed an association of a number of blood parameters with the subsequent illness progression and severity in both waves. However, some remarkable differences were detected between both waves that prevented an accurate extrapolation of prediction models from the first wave into the second wave. Interleukin-6 and d-dimer concentrations at the time of hospital admission were remarkably higher in patients who developed a critical/fatal condition only during the first wave (
< 0.001), although both parameters significantly increased with disease worsening in follow-up studies from both waves. Multivariate analyses from wave 1 rendered a predictive signature for critical/fatal illness upon hospital admission that comprised six blood biomarkers: neutrophil-to-lymphocyte ratio (≥ 5; odds ratio, 2.684 95% CI, 1.143-6.308), C-reactive protein (≥ 15.2 mg/dL; odds ratio, 2.412 95% CI, 1.006-5.786), lactate dehydrogenase (≥ 411.96 U/L; odds ratio, 2.875 95% CI, 1.229-6.726), interleukin-6 (≥ 78.8 pg/mL; odds ratio, 5.737 95% CI, 2.432-13.535), urea (≥ 40 mg/dL; odds ratio, 1.701 95% CI, 0.737-3.928), and d-dimer (≥ 713 ng/mL; odds ratio, 1.903 95% CI, 0.832-4.356). The predictive accuracy of the signature was 84% and the area under the receiver operating characteristic curve was 0.886. When the signature was validated with data from wave 2, the accuracy was 81% and the area under the receiver operating characteristic curve value was 0.874, albeit most biomarkers lost their independent significance. Follow-up studies reassured the importance of monitoring the biomarkers included in the signature, since dramatic increases in the levels of such biomarkers occurred in critical/fatal patients over disease progression.
Most parameters analyzed behaved similarly in the two waves of coronavirus disease 2019. However, univariate logistic regression conducted in both waves revealed differences in some parameters associated with poor prognosis in wave 1 that were not found in wave 2, which may reflect a different disease stage of patients on arrival to hospital. The six-biomarker predictive signature reported here constitutes a helpful tool to classify patient's prognosis on arrival to hospital.
Purpose: To report 2 cases of refractory idiopathic scleritis treated with rituximab.
Design: Interventional case series.
Methods and Results: Case 1: A 54-year-old woman, presented with anterior ...idiopathic scleritis in her right eye. Treatment with systemic steroids was not effective. Intravenous cyclophosphamide was discontinued after an adverse event. Case 2: A 43-year-old woman presented with idiopathic posterior scleritis in her left eye. Initial treatment with steroids was ineffective. In both cases, long-lasting remission without further relapses was achieved after 4 weekly doses (375 mg/m2) of rituximab.
Conclusion: Rituximab was found to be an effective treatment for refractory idiopathic anterior and posterior scleritis.
To formulate SER recommendations for the use of biological agents in primary Sjögren's syndrome (pSS).
Relevant clinical research questions were identified on the use of biological agents in pSS. The ...clinical questions were reformulated into 4 PICO questions. A search strategy was designed and a review of the scientific evidence of studies published until May 2017 was carried out. The scientific evidence available was systematically reviewed. The overall level of scientific evidence was assessed using the SIGN evidence levels. After that, specific recommendations were made.
Rituximab is recommended as the biological agent of choice for extraglandular manifestations refractory to conventional treatment. The use of anti-TNF agents is discouraged. The scientific evidence with belimumab and abatacept is scarce, so they should be considered only in cases refractory to rituximab.
Rituximab is the biological agent of choice in severe extraglandular manifestations of pSS. Belimumab or abatacept may be useful in selected cases.
Elaborar recomendaciones SER sobre el uso de agentes biológicos en el síndrome de Sjögren primario (SSp).
Se identificaron preguntas clínicas de investigación relevantes sobre el uso de agentes biológicos en el SSp. Las preguntas clínicas se reformularon en 4preguntas PICO. Se diseñó una estrategia de búsqueda y se realizó una revisión de la evidencia científica de estudios publicados hasta mayo de 2017. Se revisó sistemáticamente la evidencia científica disponible. Se evaluó el nivel global de la evidencia científica utilizando los niveles de evidencia del SIGN. Tras ello, se formularon recomendaciones específicas.
Se recomienda rituximab como el fármaco biológico de elección para las manifestaciones extraglandulares refractarias al tratamiento convencional. Se desaconseja el uso de agentes anti-TNF. La evidencia científica es escasa con belimumab y abatacept, por lo que deberían considerarse solamente en los casos resistentes a rituximab.
El rituximab es el fármaco biológico de elección en las manifestaciones graves extraglandulares del SSp. Belimumab o abatacept podrían ser de utilidad en casos seleccionados.
To assess performance of interstitial pneumonia (IP) with autoimmune features (IPAF) criteria in clinical practice and describe the utility of additional workup in identifying patients with ...underlying connective tissue diseases (CTD).
We set a retrospective study of our patients with autoimmune IP, who were allocated to CTD-IP, IPAF or undifferentiated autoimmune IP (uAIP) subgroups according to the updated classification criteria. Presence of the process-related variables comprising IPAF defining domains was scrutinized in all patients, and, when available, the results of nailfold videocapillaroscopy (NVC) were recorded.
Thirty nine out of 118 patients, accounting for 71% of former undifferentiated cases, fulfilled IPAF criteria. Arthritis and Raynaud's phenomenon were prevalent in this subgroup. While systemic sclerosis-specific autoantibodies were restricted to CTD-IP patients, anti-tRNA synthetase antibodies were also present in IPAF. In contrast, rheumatoid factor, anti-Ro antibodies and ANA nucleolar patterns could be found in all subgroups. Usual interstitial pneumonia (UIP) / possible UIP were the most frequently observed radiographic patterns Therefore, the presence of thoracic multicompartimental findings as also performance of open lung biopsies were useful in characterizing as IPAF those UIP cases lacking a clinical domain. Interestingly, we could observe NVC abnormalities in 54% of IPAF and 36% of uAIP tested patients, even though many of them did not report Raynaud's phenomenon.
Besides application of IPAF criteria, distribution of IPAF defining variables along with NVC exams help identify more homogeneous phenotypic subgroups of autoimmune IP of potential relevance beyond clinical diagnosis.
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