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2.
Healthcare (Basel) ; 12(5)2024 Feb 24.
Artigo em Inglês | MEDLINE | ID: mdl-38470651

RESUMO

PURPOSE: To assess vision-related quality of life (VRQoL) in patients with systemic lupus erythematosus (SLE) under treatment with hydroxychloroquine (HCQ), and to find the influencing factors. METHODS: Cross-sectional study enrolling SLE patients for less than ten years (Group 1), SLE patients for more than ten years (Group 2), and healthy controls (Group 3). SLE patients should be under treatment with HCQ but without ophthalmological affection. Schirmer test, best-corrected visual acuity (BCVA), axial length (AL) with optical biometry, and swept-source optical coherence tomography-angiography (OCTA) Triton (Topcon) were performed. All participants fulfilled the Impact of Visual Impairment questionnaire, and SLE patients answered the Lupus Impact Tracker (LIT) questionnaire. Additional data were obtained from clinical records. RESULTS: A totals of 41 eyes (41 patients), 31 eyes (31 patients) and 45 eyes (45 volunteers) were enrolled in the study groups. The mean ages were 41.09 ± 9.56, 45.06 ± 8.47 and 40.25 ± 10.83 years, respectively (p = 0.10). The LIT outcomes were 33.49 ± 20.74 and 35.98 ± 22.66 (p = 0.63), respectively. Group 3 referred to a better VRQoL than Group 2 in all categories and than Group 1 in some of them. A linear regression analysis showed that serum ferritin, SLE activity scales, body-mass index (BMI), age, and BCVA influenced VRQoL. The LIT questionnaire was correlated to two categories of the Impact of Visual Impairment questionnaire (IVI). CONCLUSIONS: Despite no ophthalmological affection, SLE patients refer to poorer VRQoL because of disease activity and a low health-related quality of life, which has a negative influence on VRQoL. This masks the effect of other ophthalmological conditions such as dry eyes. Other variables influencing VRQoL are age and BMI, and BCVA, to a lesser extent.

3.
Int Ophthalmol ; 44(1): 40, 2024 Feb 09.
Artigo em Inglês | MEDLINE | ID: mdl-38334832

RESUMO

PURPOSE: To evaluate the influence of hydroxychloroquine (HCQ) in choroidal thickness (CT) in patients with systemic lupus erythematous (SLE), considering the possible impact of disease activity on the choroid. METHODS: Cross-sectional study comparing three groups: two groups of SLE patients treated with HCQ without HCQ-retinopathy (32 eyes/32 patients with < 5 years of HCQ (group 1) and 44 eyes/44 patients with > 5 years of HCQ (group 2)), and an age-matched healthy control group of 46 eyes/46 patients (group 3). A complete ophthalmic examination was performed, including swept-source optical coherence tomography (SS-OCT) Triton (Topcon). Data were correlated to systemic disease activity parameters. RESULTS: CT was thicker in group 1 compared to group 3 in central, nasal, and superior sectors, and to group 2 in inner superior and outer inferior sectors (p < 0.05). In the correlation analysis, disease activity and CT were inversely correlated in most sectors (p < 0.05). In the regression analysis, HCQ was related to thinner CT in temporal and inferior sectors and disease activity with variations in nasal sectors (p < 0.05). CONCLUSIONS: In SLE patients, HCQ is correlated to decreased CT, especially in the inferior and temporal areas. The choroid shows different responses to SLE activity and HCQ, and some sectors may be more sensitive than others.


Assuntos
Antirreumáticos , Lúpus Eritematoso Sistêmico , Doenças Retinianas , Humanos , Hidroxicloroquina/uso terapêutico , Estudos Transversais , Doenças Retinianas/diagnóstico , Corioide , Tomografia de Coerência Óptica/métodos , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Antirreumáticos/uso terapêutico
5.
Clin Exp Rheumatol ; 41(8): 1695-1703, 2023 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-37534953

RESUMO

OBJECTIVES: To assess the associations and prognostic value of scleroderma patterns by nailfold videocapillaroscopy (NVC) in patients with systemic sclerosis (SSc) and cutaneous subsets. METHODS: At baseline, 1356 SSc patients from the RESCLE registry were compared according to the scleroderma pattern as Late pattern and non-Late pattern, which included Early and Active patterns. Patient characteristics, disease features, survival time and causes of death were analysed. RESULTS: Late pattern was identified in 540 (39.8%), and non-Late pattern in 816 (60.2%) patients (88% women; 987 lcSSc/251 dcSSc). Late pattern was associated to dcSSc (OR=1.96; p<0.001), interstitial lung disease (ILD) (OR=1.29; p=0.031), and scleroderma renal crisis (OR=3.46; p<0.001). Once the cutaneous subset was disregarded in an alternative analysis, both digital ulcers (DU) (OR=1.29; p<0.037) and anti-topoisomerase I antibodies (OR=1.39; p< 0.036) emerged associated with the Late pattern. By cutaneous subsets, associations with Late pattern were: (1) in dcSSc, acro-osteolysis (OR=2.13; p=0.022), and systolic pulmonary artery pressure >40 mmHg by Doppler echocardiogram (OR=2.24; p<0.001); and (2) in lcSSc, ILD (OR=1.38; p=0.028). Survival was reduced in dcSSc with Late pattern compared to non-Late pattern (p=0.049). Risk factors for SSc mortality in multivariate regression Cox analysis were age at diagnosis (HR=1.03; p<0.001), dcSSc (HR=2.48; p<0.001), DU (HR=1.38; p=0.046), ILD (HR=2.81; p<0.001), and pulmonary arterial hypertension (HR=1.99; p<0.001). CONCLUSIONS: SSc patients with Late pattern more frequently present dcSSc and develop more fibrotic and vascular manifestations. Advanced microangiopathy by NVC identifies dcSSc patients at risk of reduced survival due to SSc-related causes.


Assuntos
Doenças Pulmonares Intersticiais , Escleroderma Sistêmico , Humanos , Feminino , Masculino , Prognóstico , Angioscopia Microscópica , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/diagnóstico , Doenças Pulmonares Intersticiais/diagnóstico
7.
Clin Exp Rheumatol ; 41(8): 1605-1611, 2023 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-37140670

RESUMO

OBJECTIVES: Automated systems to analyse nailfold videocapillaroscopy (NVC) images are needed to promptly and comprehensively characterise patients with systemic sclerosis (SSc) or Raynaud's phenomenon (RP). We previously developed, and validated in-house, a deep convolutional neural network-based algorithm to classify NVC-captured images according to the presence/absence of structural abnormalities and/or microhaemorrhages. We present its external clinical validation. METHODS: A total of 1,164 NVC images of RP patients were annotated by 5 trained capillaroscopists according to the following categories: normal capillary; dilation; giant capillary; abnormal shape; tortuosity; microhaemorrhage. The images were also presented to the algorithm. Matches and discrepancies between algorithm predictions and those annotations obtained by consensus of ≥3 or ≥4 interobservers were analysed. RESULTS: Consensus among ≥3 capillaroscopists was achieved in 86.9% of images, 75.8% of which were correctly predicted by the algorithm. Consensus among ≥4 experts occurred in 52.0% of cases, in which 87.1% of the algorithm's results matched with those of the expert panel. The algorithm's positive predictive value was >80% for microhaemorrhages and unaltered, giant or abnormal capillaries. Sensitivity was >75% for dilations and tortuosities. Negative predictive value and specificity were >89% for all categories. CONCLUSIONS: This external clinical validation suggests that this algorithm is useful to assist in the diagnosis and follow-up of SSc or RP patients in a timely manner. It may also be helpful in the management of patients with any pathology presenting with microvascular changes, as the algorithm has been designed to also be useful for research aiming at extending the usage of nailfold capillaroscopy to more conditions.


Assuntos
Doença de Raynaud , Escleroderma Sistêmico , Humanos , Angioscopia Microscópica/métodos , Unhas/irrigação sanguínea , Escleroderma Sistêmico/diagnóstico por imagem , Escleroderma Sistêmico/patologia , Doença de Raynaud/diagnóstico por imagem , Software , Capilares/diagnóstico por imagem , Capilares/patologia
8.
Placenta ; 136: 29-34, 2023 05.
Artigo em Inglês | MEDLINE | ID: mdl-37028222

RESUMO

INTRODUCTION: Obstetric antiphospholipid syndrome (OAPS) is an autoimmune disease related to antiphospholipid antibodies (aPL) with primaryinflammatory injury followed by clot cascade activation and thrombus formation. Complement system activation and their participation in aPL-related thrombosis is unclosed. METHODS: We haveanalysed adverse pregnancy outcomes (APO) related to low complement (LC) levels in a cohort of 1048 women fulfilling classification criteria for OAPS. RESULTS: Overall, 223 (21.3%) women presented LC values, during pregnancy. The length of pregnancy was shorter in OAPS women with LC compared to those with normal complement (NC) (median: 33 weeks, interquartile range: [24-38] vs. 35 weeks [27-38]; p = 0.022). Life new-born incidence was higher in patients with NC levels than in those with LC levels (74.4% vs. 67.7%; p = 0.045). Foetal losses were more related to women with triple or double aPL positivity carrying LC than NC values (16.3% vs. 8.0% NC; p = 0.027). Finally, some placental vasculopathies were affected in OAPS patients with LC as late Foetal Growth Restriction (FGR >34 weeks) rise to 7.2% in women with LC vs. 3.2% with NC (p = 0.007). DISCUSSION: Data from our registry indicate that incidence of APO was higher in OAPS women with LC levels and some could be reverted by the correct treatment.


Assuntos
Síndrome Antifosfolipídica , Complicações na Gravidez , Feminino , Gravidez , Humanos , Masculino , Síndrome Antifosfolipídica/complicações , Síndrome Antifosfolipídica/epidemiologia , Placenta , Anticorpos Antifosfolipídeos , Sistema de Registros
9.
Autoimmun Rev ; 21(10): 103167, 2022 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-35931315

RESUMO

AIM: Patients with systemic sclerosis (SSc) are at increased risk of cancer, a growing cause of non-SSc-related death among these patients. We analyzed the increased cancer risk among Spanish patients with SSc using standardized incidence ratios (SIRs) and identified independent cancer risk factors in this population. MATERIAL AND METHODS: Spanish Scleroderma Registry data were analyzed to determine the demographic characteristics of patients with SSc, and logistic regression was used to identify cancer risk factors. SIRs with 95% confidence intervals (CIs) relative to the general Spanish population were calculated. RESULTS: Of 1930 patients with SSc, 206 had cancer, most commonly breast, lung, hematological, and colorectal cancers. Patients with SSc had increased risks of overall cancer (SIR 1.48, 95% CI 1.36-1.60; P < 0.001), and of lung (SIR 2.22, 95% CI 1.77-2.73; P < 0.001), breast (SIR 1.31, 95% CI 1.10-1.54; P = 0.003), and hematological (SIR 2.03, 95% CI 1.52-2.62; P < 0.001) cancers. Cancer was associated with older age at SSc onset (odds ratio [OR] 1.22, 95% CI 1.01-1.03; P < 0.001), the presence of primary biliary cholangitis (OR 2.35, 95% CI 1.18-4.68; P = 0.015) and forced vital capacity <70% (OR 1.8, 95% CI 1.24-2.70; P = 0.002). The presence of anticentromere antibodies lowered the risk of cancer (OR 0.66, 95% CI 0.45-0.97; P = 0.036). CONCLUSIONS: Spanish patients with SSc had an increased cancer risk compared with the general population. Some characteristics, including specific autoantibodies, may be related to this increased risk.


Assuntos
Neoplasias , Esclerodermia Localizada , Escleroderma Sistêmico , Autoanticorpos , Humanos , Incidência , Neoplasias/complicações , Neoplasias/epidemiologia , Sistema de Registros , Fatores de Risco , Esclerodermia Localizada/complicações , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/epidemiologia
10.
Semin Arthritis Rheum ; 55: 152033, 2022 08.
Artigo em Inglês | MEDLINE | ID: mdl-35691226

RESUMO

OBJECTIVES: Left ventricular diastolic dysfunction (LVDD) remains poorly studied in Systemic Sclerosis (SSc). To determine the prevalence and to define factors associated with LVDD and survival in a large cohort of patients with SSc. METHODS: An observational study was conducted with data from the multicentre Spanish Scleroderma Registry (RESCLE) to identify factors associated with LVDD and estimate survival. RESULTS: Out of 1517 patients, 319 (21.0%) had LVDD. The subset of sine scleroderma SSc was associated to LVDD (14.7% vs. 10.6%, p =0.048), whilst diffuse cutaneous SSc was more prevalent in non-LVDD (16.0 % vs. 21.2%, p =0.041). Multivariable analysis identified that LVDD was associated with older age at diagnosis of SSc (OR 1.05; 95% CI 1.04 to 1.06), longer time from diagnosis (OR 1.04; 95% CI 1.03 to 1.06), presence of telangiectasia (OR 1.42; 95% CI 1.08 to 1.88), treatment with calcium channel blockers (CCB) (OR 1.51; 95% CI 1.16 to 1.96), and inversely related to angiotensin-converting-enzyme inhibitors (ACEi) use (OR 0.59; 95% CI 0.44 to 0.80). SSc patients with LVDD had increased mortality (23.8 vs. 17.4%, p =0.010) and shortened survival from the first SSc symptom (p =0.040), even though it was not found to be an independent risk factor for death. CONCLUSIONS: LVDD is relatively common in SSc patients, and it is associated with worst prognosis, older age, longer time from diagnosis of SSc, presence of telangiectasia and vasodilator treatment.


Assuntos
Esclerodermia Difusa , Escleroderma Sistêmico , Telangiectasia , Disfunção Ventricular Esquerda , Estudos de Coortes , Humanos , Sistema de Registros , Disfunção Ventricular Esquerda/complicações , Disfunção Ventricular Esquerda/diagnóstico
11.
Clin Exp Rheumatol ; 40(10): 1926-1932, 2022 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-34936544

RESUMO

OBJECTIVES: Although classification systems and scores for capillaroscopy interpretation have been published, there is a lack of homogenization for the procedure, especially in the way and place the images are taken, the counting of the capillaries and the measuring of their size. Our objective is to provide a deep learning-based software to obtain objective and exhaustive data for the whole nailfold without increasing the time or effort needed to do the examination, or requiring expensive equipment. METHODS: An automated software to count nailfold capillaries has been designed, through an exploratory image dataset of 2,713 images with 18,000 measurements of 3 different types. Subsequently, application rules have been created to detect the morphology of nailfold videocapillaroscopy images, through a training set of images. The software reliability has been evaluated with standard metrics used in the machine learning field for object detection tasks, comparing automatic and manual counting on the same NVC images. RESULTS: A mean average precision (mAP) of 0.473 is achieved for detecting and classifying capillaries and haemorrhages by their shape, and a mAP of 0.515 is achieved for detecting and classifying capillaries by their size. A precision of 83.84% and a recall of 92.44% in the identification of capillaries was estimated. CONCLUSIONS: Deep learning is a useful tool in nailfold videocapillaroscopy that allows to analyse objectively and homogeneously images taken with multiple devices. It should make the assessment of the capillary morphology in nailfold video capillaroscopy easier, quicker, more complete and accessible to everyone.


Assuntos
Angioscopia Microscópica , Unhas , Humanos , Angioscopia Microscópica/métodos , Reprodutibilidade dos Testes , Unhas/diagnóstico por imagem , Unhas/irrigação sanguínea , Capilares/diagnóstico por imagem , Software
13.
Eur J Anaesthesiol ; 38(9): 916-922, 2021 09 01.
Artigo em Inglês | MEDLINE | ID: mdl-34397526

RESUMO

BACKGROUND: The combination of low-dose aspirin (LDA) and low-molecular-weight heparin (LMWH) until the end of gestation are the currently the accepted standard of care for the treatment of antiphospholipid-related obstetric disorders. In refractory cases, hydroxychloroquine (HCQ) can be added to this standard of care. OBJECTIVE: To evaluate the haemostatic safety of LDA and LMWH (medium to high prophylactic doses) during pregnancy and the puerperium in women with both full-blown obstetric antiphospholipid syndrome (OAPS) (Sydney criteria) and noncriteria - incomplete - OAPS. STUDY DESIGN: Retrospective/prospective multicentre observational study. Obstetric background, laboratory categories, delivery mode, antithrombotic regimens and bleeding complications were compared. SETTING: A total of 30 tertiary European hospitals. PATIENTS: Mainly, Caucasian/Arian pregnant women were included. Other ethnicities were minimally present. Women were controlled throughout pregnancy and puerperium. MAIN OUTCOME MEASURES: The primary end-point was to evaluate the number of major and minor haemorrhagic complications in this cohort of women. Neuraxial anaesthetic bleeding complications were particularly assessed. Secondly, we aimed to compare local/general bleeding events between groups. RESULTS: We studied 1650 women, of whom 1000 fulfilled the Sydney criteria of the OAPS and 650 did not (noncriteria OAPS). Data on antithrombotic-related complications were available in 1075 cases (65.15%). Overall, 53 (4.93%) women had bleeding complications, with 34 being considered minor (3.16%) and 19 major (1.76%). Neither obstetric complications nor laboratory categories were bleeding-related. Assisted vaginal delivery and caesarean section were related to local haemorrhage. Heparin doses and platelet count were not associated with major bleeding. CONCLUSIONS: LDA and medium to high prophylactic LMWH during pregnancy in women with full-blown OAPS/noncriteria OAPS are safe. A slight increase in bleeding risk was noted in instrumental deliveries. No women who underwent spinal or epidural anaesthesia suffered bleeding complications. No haemorrhage was observed in cases where HCQ was added to standard therapy.


Assuntos
Fibrinolíticos , Complicações na Gravidez , Cesárea , Feminino , Fibrinolíticos/efeitos adversos , Hemorragia/induzido quimicamente , Hemorragia/diagnóstico , Hemorragia/epidemiologia , Heparina de Baixo Peso Molecular/efeitos adversos , Humanos , Gravidez , Estudos Prospectivos , Sistema de Registros , Estudos Retrospectivos
14.
Reumatol. clín. (Barc.) ; 17(4): 183-186, Abr. 2021. tab
Artigo em Espanhol | IBECS | ID: ibc-211861

RESUMO

Objetivo: Las Sociedades Españolas de Ginecología y Obstetricia, de Medicina Interna y de Reumatología han constituido un grupo de trabajo paritario para la elaboración de tres documentos de consenso sobre el control del embarazo en mujeres con lupus eritematoso sistémico y síndrome antifosfolípido. Métodos: Cada una de las sociedades científicas implicadas propuso cinco representantes en base a su experiencia en el área del control del embarazo en pacientes con enfermedades autoinmunes. Las recomendaciones se elaboraron siguiendo la metodología Delphi.Resultados: En este tercer documento se incluyen las recomendaciones que abordan el manejo del parto, puerperio y lactancia, incluyendo el manejo de los diferentes fármacos en estos periodos. Además se incluye una sección sobre los cuidados iniciales del recién nacido y sobre anticoncepción. Conclusiones: Estas recomendaciones multidisciplinares se consideran herramientas en la toma de decisiones para los clínicos involucrados en la asistencia a pacientes con lupus eritematoso sistémico/síndrome antifosfolípido durante el embarazo.(AU)


Objective: In order to agree on the fundamental aspects related to the management of pregnancy in patients with systemic lupus erythematosus and antiphospholipid syndrome, the Spanish Societies of Gynaecology and Obstetrics, Internal Medicine and Rheumatology have set up a working group for the preparation of three consensus documents. Methods: Each of the Scientific Societies involved proposed five representatives based on their experience in the field of pregnancy control in patients with autoimmune diseases. The recommendations were developed following the Delphi methodology. Results: This third document contains the recommendations regarding the management of delivery, puerperium and lactation, including medication use during these periods and the care of the newborn. In addition, a section on contraception is included. Conclusions: These multidisciplinary recommendations are considered decision-making tools for clinicians involved in the care of patients with systemic lupus erythematosus/antiphospholipid syndrome during pregnancy.(AU)


Assuntos
Humanos , Feminino , Gravidez , Recém-Nascido , Lactente , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/terapia , Período Pós-Parto , Complicações na Gravidez , Parto , Síndrome Antifosfolipídica , Anticoncepção , Reumatologia , Doenças Reumáticas
16.
Reumatol Clin (Engl Ed) ; 17(3): 125-131, 2021 Mar.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-31780250

RESUMO

OBJECTIVE: In order to agree on the fundamental aspects related to the management of pregnancy in patients with systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS), the Spanish Societies of Gynaecology and Obstetrics, Internal Medicine and Rheumatology set up a working group for the preparation of three consensus documents. METHODS: Each of the Scientific Societies involved proposed five representatives based on their experience in the field of pregnancy control in patients with autoimmune diseases. The recommendations were developed following the Delphi methodology. RESULTS: This second document contains the recommendations regarding the management of pregnancy in women with SLE and APS, including complications such as lupus activity, congenital heart block, thrombotic and obstetric manifestations of APS and placental vascular disease. CONCLUSIONS: These multidisciplinary recommendations are considered decision-making tools for clinicians involved in the care of patients with SLE/APS during pregnancy.

17.
Reumatol Clin (Engl Ed) ; 17(2): 61-66, 2021 Feb.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-31784396

RESUMO

OBJECTIVE: Pregnancy and puerperium are considered a risk situation in women with systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS). Therefore, specialized assessment is essential both preconception and during pregnancy and the puerperium. Likewise, it is very important that different specialists in autoimmune diseases and high-risk pregnancies participate in the follow-up of these patients in a coordinated manner. The Spanish Society of Gynaecology and Obstetrics, the Spanish Society of Internal Medicine, and the Spanish Society of Rheumatology have set up a working group for the preparation of three consensus documents. METHODS: The stages of the work were: distribution of work in three groups corresponding to the three periods related to pregnancy (preconception, during pregnancy and childbirth and puerperium), identification of key areas, exhaustive review of the literature and formulation of recommendations. RESULTS: This first document includes the 48 recommendations that address aspects related to infertility, the need for and treatments for gonadal preservation and preconception assessment. CONCLUSIONS: These multidisciplinary recommendations are considered decision-making tools for clinicians involved in the care of patients with SLE/APS during pregnancy.

18.
Reumatol Clin (Engl Ed) ; 17(4): 183-186, 2021 Apr.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-31831386

RESUMO

OBJECTIVE: In order to agree on the fundamental aspects related to the management of pregnancy in patients with systemic lupus erythematosus and antiphospholipid syndrome, the Spanish Societies of Gynaecology and Obstetrics, Internal Medicine and Rheumatology have set up a working group for the preparation of three consensus documents. METHODS: Each of the Scientific Societies involved proposed five representatives based on their experience in the field of pregnancy control in patients with autoimmune diseases. The recommendations were developed following the Delphi methodology. RESULTS: This third document contains the recommendations regarding the management of delivery, puerperium and lactation, including medication use during these periods and the care of the newborn. In addition, a section on contraception is included. CONCLUSIONS: These multidisciplinary recommendations are considered decision-making tools for clinicians involved in the care of patients with systemic lupus erythematosus/antiphospholipid syndrome during pregnancy.

19.
Rheumatology (Oxford) ; 60(2): 872-880, 2021 02 01.
Artigo em Inglês | MEDLINE | ID: mdl-32844220

RESUMO

INTRODUCTION: Endothelin antagonist receptors (ERAs) and phosphodiesterase-5 inhibitors (PDE5i) are beneficial in pulmonary arterial hypertension (PAH) and digital ulcers (DU) and prevent from DU recurrences. Our study aimed to determine the difference in the incidence rate of PAH and scleroderma renal crisis (SRC) in patients with SSc and DU (SSc-DU) under ERAs/PDE5i or without treatment. METHODS: We conducted a retrospective cohort study including SSc-DU patients from the Spanish Scleroderma Registry (RESCLE). The primary outcome was the incidence rate of PAH and SRC in patients under ERAs/PDE5i or not. RESULTS: Some 544 patients out of 1817 (29.9%) in the RESCLE database had DU, 221 (40.6%) under ERAs/PDE5i and 323 (59.4%) not. The incidence rate (95% CI) difference between patients under treatment or not under did not reach statistical significance in PAH [-0.1 (-4.8, 4.69), P = 0.988] or in SRC [0.7 (-2.2, 3.7), P = 0.620]. However, the time from the first DU to the diagnosis of SRC was delayed in treated patients [mean (s.d.) 7.6 (5.8) years vs 2.9 (5.3); P = 0.021]. The dcSSc subset was more prevalent in the treatment group (36 vs 26%; P = 0.018), along with anti-topoisomerase I antibodies (34 vs 18%; P < 0.001) and tendon friction rubs (12 vs 6%; P = 0.038), whereas the lcSSc subset was more prevalent in the no-treatment group (57 vs 66%; P = 0.031) along with ACA (37 vs 46%; P = 0.031). CONCLUSION: There was no difference in the incidence rate of PAH and SRC between groups. However, treatment with ERAs and/or PDE5i appeared to delay the occurrence of SRC.


Assuntos
Injúria Renal Aguda , Antagonistas dos Receptores de Endotelina/uso terapêutico , Inibidores da Fosfodiesterase 5/uso terapêutico , Hipertensão Arterial Pulmonar , Escleroderma Sistêmico , Úlcera Cutânea , Injúria Renal Aguda/epidemiologia , Injúria Renal Aguda/etiologia , Injúria Renal Aguda/prevenção & controle , Vasos Sanguíneos/efeitos dos fármacos , Feminino , Dedos , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Hipertensão Arterial Pulmonar/diagnóstico , Hipertensão Arterial Pulmonar/epidemiologia , Hipertensão Arterial Pulmonar/etiologia , Hipertensão Arterial Pulmonar/prevenção & controle , Sistema de Registros/estatística & dados numéricos , Escleroderma Sistêmico/tratamento farmacológico , Escleroderma Sistêmico/epidemiologia , Escleroderma Sistêmico/fisiopatologia , Úlcera Cutânea/diagnóstico , Úlcera Cutânea/epidemiologia , Úlcera Cutânea/etiologia , Úlcera Cutânea/prevenção & controle , Espanha/epidemiologia , Resultado do Tratamento
20.
Rheumatology (Oxford) ; 60(1): 217-223, 2021 01 05.
Artigo em Inglês | MEDLINE | ID: mdl-32658963

RESUMO

OBJECTIVES: Several IgG4-related disease (IgG4-RD) phenotypes have been proposed and the first set of classification criteria have been recently created. Our objectives were to assess the phenotype distribution and the performance of the classification criteria in Spanish patients as genetic and geographical differences may exist. METHODS: We performed a cross-sectional multicentre study (Registro Español de Enfermedad Relacionada con la IgG4, REERIGG4) with nine participating centres from Spain. Patients were recruited from November 2013 to December 2018. The 2019 American College of Rheumatology/European League Against Rheumatism classification criteria (AECC) were used. RESULTS: We included 105 patients; 88% had Caucasian ethnicity. On diagnosis, 86% met the international pathology consensus while 92% met the Japanese comprehensive criteria. The phenotype distribution was head and neck 25%, Mikulicz and systemic (MS) 20%, pancreato-hepato-biliary (PHB) 13%, retroperitoneal and aorta (RA) 26%. Sixteen per cent had an undefined phenotype. Seventy-seven per cent of the cases met the AECC. From the 24 patients not meeting the AECC, 33% met exclusion criteria, and 67% did not get a score ≥20 points. Incomplete pathology reports were associated to failure to meet the AECC. CONCLUSIONS: The PHB phenotype was rare among Spanish IgG4-RD patients. The MS phenotype was less frequent and the RA phenotype was more prevalent than in other, Asian patient series. An undefined phenotype should be considered as some patients do not fall into any of the categories. Three quarters of the cases met the 2019 AECC. Incomplete pathology reports were the leading causes of failure to meet the criteria.


Assuntos
Doença Relacionada a Imunoglobulina G4/classificação , Imunoglobulina G , Fenótipo , Sistema de Registros , Fatores Etários , Estudos Transversais , Bases de Dados Factuais , Feminino , Humanos , Imunoglobulina G/sangue , Doença Relacionada a Imunoglobulina G4/diagnóstico , Doença Relacionada a Imunoglobulina G4/etnologia , Doença Relacionada a Imunoglobulina G4/patologia , Masculino , Pessoa de Meia-Idade , Proibitinas , Fatores Sexuais , Espanha , População Branca/estatística & dados numéricos
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