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1.
Rheumatol Int ; 2024 Sep 23.
Article de Anglais | MEDLINE | ID: mdl-39311913

RÉSUMÉ

To describe the characteristics of systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still's disease (AOSD), compare their presentation and evolution, and analyse possible complication predictors. Multicenter study. Data were retrieved from a hospital-based study of patients with a diagnosis or suspected diagnosis of sJIA or AOSD according to the responsible physician and followed-up for at least one year. Descriptive variables (classification criteria, clinical manifestations, complications, family, and personal history) were collected at disease onset and during follow-up. We present the clinical characteristics of 326 patients, 67% of whom had a diagnosis of sJIA and 33% of AOSD. Clinical manifestation frequencies were similar between the two groups, except for odynophagia, which was significantly more frequent in AOSD than in sJIA (78.4% vs. 25.5%; p < 0.0001). Among the complications, macrophage activation syndrome (MAS) was significantly more common in sJIA than in AOSD (24.4% vs. 9.5%; p = 0.002), to the extent that an sJIA diagnosis significantly increased the risk of MAS, together with serositis presence, and the need for biological therapy. Patients with sJIA and AOSD showed similar characteristics, supporting the idea that they are both part of Still's disease, but are expressed at different ages. Differences in manifestations and complications might be due to different management between diseases and immune response maturity.

2.
Lupus Sci Med ; 11(2)2024 Aug 03.
Article de Anglais | MEDLINE | ID: mdl-39097409

RÉSUMÉ

OBJECTIVE: To assess organ damage, with emphasis on the cardiovascular system, over the different stages of the disease in a large SLE cohort. METHODS: Multicentre, longitudinal study of a cohort of 4219 patients with SLE enrolled in the Spanish Society of Rheumatology Lupus Registry. Organ damage was ascertained using the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI). We longitudinally analysed SDI (globally and for each domain) over time only in the 1274 patients whose dates of damage events had been recorded. RESULTS: During the first year after diagnosis of SLE, 20% of the 1274 patients presented with new damage manifestations. At years 2 and 3, new damage was recorded in 11% and 9% of patients. The annual percentage of patients with new damage after year 5 decreased to 5%. In the first year with the disease, most damage was accumulated in the musculoskeletal, neuropsychiatric and renal systems; in later stages, most damage was in the musculoskeletal, ocular and cardiovascular systems. Considering 'cerebrovascular accident' and 'claudication for 6 months' as cardiovascular items, the cardiovascular system was the second most affected system during the early stages of SLE, with 19% of the patients who presented with damage affected at first year after diagnosis. During the late stages, 20-25% of the patients presenting with new damage did so in this modified cardiovascular domain of the SDI. CONCLUSIONS: New damage occurs mainly during the first year following diagnosis of SLE. Cardiovascular damage is relevant in both the early and the late stages of the disease. Strategies to prevent cardiovascular damage should be implemented early after diagnosis of SLE.


Sujet(s)
Système cardiovasculaire , Lupus érythémateux disséminé , Enregistrements , Humains , Lupus érythémateux disséminé/complications , Lupus érythémateux disséminé/épidémiologie , Études longitudinales , Mâle , Femelle , Adulte , Espagne/épidémiologie , Adulte d'âge moyen , Système cardiovasculaire/physiopathologie , Maladies cardiovasculaires/épidémiologie , Indice de gravité de la maladie , Évolution de la maladie , Rhumatologie
3.
Lupus ; 33(5): 481-489, 2024 Apr.
Article de Anglais | MEDLINE | ID: mdl-38446533

RÉSUMÉ

BACKGROUND: In 2017, belimumab (BEL) was approved in subcutaneous (SQ) administration. The effectiveness after switching from intravenous (IV) to SQ and patient satisfaction in daily clinical practice has not been studied. During the pandemic, patient follow-up and treatment were significantly affected, and some patients need a change from IV to SQ. Our aim was to evaluate daily clinical practice satisfaction to SQ BEL therapy in patients previously treated IV BEL. We hypothesized that SQ BEL in SLE patients previously treated with IV BEL was similar in effectiveness and conferred higher satisfaction. METHODS: Observational, multicenter study, conducted in 7 reference centers in Catalonia. We included stable SLE patients (EULAR/ACR 2019) on treatment with SQ BEL and previous use of IV BEL (at least 3 months on IV BEL before switching). Since there are no well-validated tools for SQ BEL treatment satisfaction, we used RASQ-SQ, validated in patients with lymphoma who switched from IV Rituximab to SQ treatment, and modified for BEL treatment. RESULTS: Twenty-seven patients were included. The more prevalent clinical manifestations observed were related to the skin and joints and the patients had a mean baseline SLEDAI of 2.96 (SD 2.4) and SLICC score of 0.67 (SD 0.88). The median time from treatment with IV BEL before switching to SQ was 21 months (range). 84% of patients reported confidence in SQ BEL. 85.2% felt that treatment with SQ BEL was convenient or very convenient. 85% felt they had gained time with the change. 89% would recommend the SQ injection to other patients. Disease activity (mean SLEDAI) and remission rates remain stable after switching. No major new adverse effects were reported. CONCLUSIONS: Overall satisfaction, satisfaction with via of administration, and satisfaction with the time taken to receive BEL were higher for SQ BEL treatment. A switching SQ strategy is a reasonable alternative for BEL patients.


Sujet(s)
Anticorps monoclonaux humanisés , Immunosuppresseurs , Lupus érythémateux disséminé , Humains , Immunosuppresseurs/usage thérapeutique , Résultat thérapeutique , Lupus érythémateux disséminé/traitement médicamenteux , Satisfaction personnelle
4.
Semin Arthritis Rheum ; 58: 152121, 2023 02.
Article de Anglais | MEDLINE | ID: mdl-36375359

RÉSUMÉ

OBJECTIVES: To analyze the prevalence, incidence, survival and contribution on mortality of major central nervous system (CNS) involvement in systemic lupus erythematosus (SLE). METHODS: Patients fulfilling the SLE 1997 ACR classification criteria from the multicentre, retrospective RELESSER-TRANS (Spanish Society of Rheumatology Lupus Register) were included. Prevalence, incidence and survival rates of major CNS neuropsychiatric (NP)-SLE as a group and the individual NP manifestations cerebrovascular disease (CVD), seizure, psychosis, organic brain syndrome and transverse myelitis were calculated. Furthermore, the contribution of these manifestations on mortality was analysed in Cox regression models adjusted for confounders. RESULTS: A total of 3591 SLE patients were included. Of them, 412 (11.5%) developed a total of 522 major CNS NP-SLE manifestations. 61 patients (12%) with major CNS NP-SLE died. The annual mortality rate for patients with and without ever major CNS NP-SLE was 10.8% vs 3.8%, respectively. Individually, CVD (14%) and organic brain syndrome (15.5%) showed the highest mortality rates. The 10% mortality rate for patients with and without ever major CNS NP-SLE was reached after 12.3 vs 22.8 years, respectively. CVD (9.8 years) and organic brain syndrome (7.1 years) reached the 10% mortality rate earlier than other major CNS NP-SLE manifestations. Major CNS NP-SLE (HR 1.85, 1.29-2.67) and more specifically CVD (HR 2.17, 1.41-3.33) and organic brain syndrome (HR 2.11, 1.19-3.74) accounted as independent prognostic factors for poor survival. CONCLUSION: The presentation of major CNS NP-SLE during the disease course contributes to a higher mortality, which may differ depending on the individual NP manifestation. CVD and organic brain syndrome are associated with the highest mortality rates.


Sujet(s)
Lupus érythémateux disséminé , Vascularite lupique du système nerveux central , Rhumatologie , Humains , Études rétrospectives , Lupus érythémateux disséminé/épidémiologie , Vascularite lupique du système nerveux central/complications , Vascularite lupique du système nerveux central/épidémiologie , Vascularite lupique du système nerveux central/psychologie , Système nerveux central
5.
Clin Exp Rheumatol ; 40(7): 1361-1367, 2022 Jul.
Article de Anglais | MEDLINE | ID: mdl-34596027

RÉSUMÉ

OBJECTIVES: In a cohort of early rheumatoid arthritis (RA) patients, we aimed to determine and characterise fatigue trajectories over 10 years of follow-up and identify predictors of trajectory membership. METHODS: We selected patients fulfilling the 2010 ACR/EULAR criteria for RA included in the ESPOIR cohort. We used a cluster analysis to obtain fatigue (assessed by fatigue visual analogue scale) trajectories over the course of 10 years from enrolment. Chi-square tests or ANOVA were performed to evaluate differences of baseline variables between fatigue trajectories. Using a multinomial logistic regression we were able to identify predictors of trajectory membership. RESULTS: We analysed 598 patients with mean disease duration at enrolment of 26.2±40.9 days. Cluster analysis revealed 3 trajectories: high (18%), moderate (52%) and low fatigue (30%). Compared to patients with moderate or low fatigue trajectory, patients with high fatigue trajectory were predominantly women and reported significantly higher duration and intensity of morning stiffness, HAQ score, tender joints count, levels of pain, number of awakenings due to arthritis, frequency of fibromyalgic RA, levels of physician and patient global assessment, more frequent sleep problems, and increased psychological distress. Female patients with pain, psychological distress and presence of sicca symptoms had a higher risk of being in the high trajectory group. CONCLUSIONS: These findings suggest that levels of fatigue are rather stable over time in each trajectory. Baseline clinical measures and baseline patient-reported measures of functional status better distinguished the three fatigue trajectories. We did not find any differences between trajectories in baseline laboratory measures. Inflammatory activity was not a predictor of being in the high trajectory fatigue group.


Sujet(s)
Polyarthrite rhumatoïde , Polyarthrite rhumatoïde/complications , Polyarthrite rhumatoïde/diagnostic , Études de cohortes , Fatigue/diagnostic , Fatigue/étiologie , Femelle , Humains , Mâle , Douleur/diagnostic , Douleur/étiologie , Mesure de la douleur
7.
Lupus ; 30(10): 1596-1602, 2021 Sep.
Article de Anglais | MEDLINE | ID: mdl-34192953

RÉSUMÉ

OBJECTIVES: Recent data suggest that some adult patients with autoimmune rheumatic diseases may develop cardiac conduction and repolarization abnormalities mediated by anti-Ro/SSA antibodies. We aim to investigate the utility of a cardiac screening in patients with systemic lupus erythematous (SLE) and anti-Ro/SSA positivity. METHODS: SLE patients who consecutively attended a Rheumatology clinic during 1 year where evaluated for the presence and levels of anti-Ro/SSA antibodies, and clinical and biological markers of organ damage and disease activity. All participants underwent a cardiovascular anamnesis and physical examination, ECG, echocardiography, and 24-hour Holter. RESULTS: Of the 145 recruited patients, 49 (32%) had anti-Ro/SSA positivity. None had any degree of atrioventricular block in the ECG or Holter monitoring. No significant differences were observed between anti-Ro/SSA-positive vs. negative patients in terms of PR, QRS or QTc intervals. No clinically significant arrhythmias were recorded during Holter monitoring and no differences in average heart rate, heart rate variability, or atrial or ventricular ectopy burden were observed. Finally, no differences were found in echocardiographic measurements. CONCLUSIONS: In this study of SLE patients, anti-Ro/SSA positivity was not associated with significant alterations in ECG, echocardiography, or 24-hour Holter. These findings do not support ordinary cardiac evaluation in these patients. (Clinicaltrials.gov registration number: NCT02162992).


Sujet(s)
Bloc atrioventriculaire , Lupus érythémateux disséminé , Adulte , Anticorps antinucléaires/sang , Anticorps antinucléaires/immunologie , Troubles du rythme cardiaque/diagnostic , Troubles du rythme cardiaque/étiologie , Électrocardiographie ambulatoire , Rythme cardiaque/physiologie , Humains , Lupus érythémateux disséminé/complications , Lupus érythémateux disséminé/diagnostic
10.
Rheumatology (Oxford) ; 60(4): 1793-1803, 2021 04 06.
Article de Anglais | MEDLINE | ID: mdl-33106841

RÉSUMÉ

OBJECTIVE: The aim of the present study was to describe the demographic, clinical and immunological characteristics of patients with late-onset (≥50 years) SLE vs patients with early-onset SLE (<50 years). METHODS: We performed a cross-sectional retrospective study of 3619 patients from the RELESSER database (National Register of Patients with Systemic Lupus Erythematosus of the Spanish Society of Rheumatology). RESULTS: A total of 565 patients (15.6%) were classified as late-onset SLE and 3054 (84.4%) as early-onset SLE. The male-to-female ratio was 5:1. Mean (s.d.) age at diagnosis in the late-onset group was 57.4 (10.4) years. At diagnosis, patients with late-onset SLE had more comorbid conditions than patients with early-onset SLE; the most frequent was cardiovascular disease (P <0.005). Furthermore, diagnostic delay was longer in patients with late-onset SLE [45.3 (3.1) vs 28.1 (1.0); P <0.001]. Almost all patients with late-onset SLE (98.7%) were Caucasian. Compared with early-onset SLE and after adjustment for time since diagnosis, patients with late-onset SLE more frequently had serositis, major depression, thrombotic events, cardiac involvement and positive lupus anticoagulant values. They were also less frequently prescribed immunosuppressive agents. Mortality was greater in late-onset SLE (14.3% vs 4.7%; P <0.001). CONCLUSION: Late-onset SLE is insidious, with unusual clinical manifestations that can lead to diagnostic errors. Clinical course is generally indolent. Compared with early-onset disease, activity is generally reduced and immunosuppressants are less commonly used. Long-term prospective studies are necessary to determine whether the causes of death are associated with clinical course or with age-associated comorbidities in this population.


Sujet(s)
Âge de début , Lupus érythémateux disséminé/diagnostic , Lupus érythémateux disséminé/épidémiologie , Adulte , Maladies cardiovasculaires/épidémiologie , Comorbidité , Études transversales , Retard de diagnostic , Dépression/épidémiologie , Femelle , Humains , Immunosuppresseurs/usage thérapeutique , Inhibiteur lupique de la coagulation/sang , Lupus érythémateux disséminé/traitement médicamenteux , Mâle , Adulte d'âge moyen , , Enregistrements , Études rétrospectives , Sérite/épidémiologie , Répartition par sexe , Espagne/épidémiologie , Thrombose/épidémiologie
12.
Arch Bronconeumol (Engl Ed) ; 57(5): 351-358, 2021 May.
Article de Anglais, Espagnol | MEDLINE | ID: mdl-33069461

RÉSUMÉ

INTRODUCTION: Serum autoantibodies support the diagnosis of interstitial lung disease (ILD) related to systemic autoimmune diseases (SAD-ILD). Nevertheless, their presence in the bronchoalveolar lavage (BAL) has not been explored. OBJECTIVES: To demonstrate the presence of autoantibodies in the BAL of ILD patients at onset of clinical evaluation, its relation with serum autoantibodies and to analyze clinical features of patients with autoantibodies in BAL. METHODS: Autoantibodies against extractable nuclear antigens (ENAs) were analyzed by immunoblot in the BAL of 155 patient with suspected diagnosis of ILD and 10 controls. RESULTS: Seven ENAs were detected in the BAL of 19 patients (Anti-Ro52, Anti-Ro60, CENP-B, Anti-La, Jo-1, Sm/RNP and Anti-SL70). The most frequent ENA was anti-Ro52 (13 patients; 68,4% of positives ones). Seven patients presented more than one ENAs. Fourteen were diagnosed of SAD-ILD, 3 of interstitial pneumonia with autoimmune features, one of non-specific idiopathic pneumonia and other of silicosis. In 10 cases (52%) IgA autoantibodies were also detected. The autoantibodies observed in BAL were also detected in the serum of 17 patients (90%). There were no significant clinical differences with the patients with SAD-ILD or interstitial pneumonia with autoimmune features with patients with negative BAL. CONCLUSION: The study of ENAs in BAL is feasible and can be a useful tool in the ILD initial algorithm, specifically sustaining the suspected diagnosis of SAD-ILD.


Sujet(s)
Autoanticorps , Pneumopathies interstitielles , Lavage bronchoalvéolaire , Humains , Pneumopathies interstitielles/diagnostic
13.
Clin Exp Rheumatol ; 38 Suppl 127(5): 69-75, 2020.
Article de Anglais | MEDLINE | ID: mdl-33331268

RÉSUMÉ

OBJECTIVES: The objective of the present study was to assess the efficacy of apremilast (APR) in the management of refractory oral and/or genital ulcers in patients with Behçet's disease (BD). METHODS: National multicentre open-label observational study on BD patients with recurrent oral and/or genital ulcers. In all cases orogenital ulcers were refractory to conventional therapy. APR was given and maintained at standard dose of 30 mg twice daily. The main outcome was the achievement of oral and/or genital ulcers remission. Efficacy of APR for other clinical manifestations was also evaluated. RESULTS: We included 51 patients (35 women/16 men; mean age 44.7±13.2 years). Before APR, all patients had received several systemic conventional and/or biologic drugs. APR was initiated because of refractory oral (n=19) or genital (n=2) aphthous ulcers or both (n=30). Other manifestations found at APR onset were arthralgia/arthritis (n=16), folliculitis/pseudofolliculitis (n=14), erythema nodosum (n=3), furunculosis (n=2), paradoxical psoriasis induced by TNF-α-inhibitors (n=2), ileitis (n=2), deep venous thrombosis (n=2), leg ulcers (n=1), erythematosus and scaly skin lesions (n=1), fever (n=1), unilateral anterior uveitis (n=1) and neuro Behçet (n=1). After a mean follow-up of 8.5±6.9 months, most patients had experienced improvement of orogenital ulcers and prednisone dose had been successfully reduced or discontinued. APR also yielded improvement of some non-aphthous manifestations such as the cutaneous follicular and intestinal manifestations. However, the effect on musculoskeletal manifestations was variable. CONCLUSIONS: APR yielded a rapid and maintained improvement of refractory mucocutaneous ulcers of BD, even in patients refractory to several systemic drugs including biologic therapy.


Sujet(s)
Maladie de Behçet , Stomatite aphteuse , Adulte , Maladie de Behçet/complications , Maladie de Behçet/diagnostic , Maladie de Behçet/traitement médicamenteux , Femelle , Humains , Mâle , Adulte d'âge moyen , Thalidomide/effets indésirables , Thalidomide/analogues et dérivés , Ulcère
15.
Rheumatology (Oxford) ; 59(12): 3906-3916, 2020 12 01.
Article de Anglais | MEDLINE | ID: mdl-33068439

RÉSUMÉ

OBJECTIVE: To assess the efficacy of abatacept (ABA) in RA patients with interstitial lung disease (ILD) (RA-ILD). METHODS: This was an observational, multicentre study of RA-ILD patients treated with at least one dose of ABA. ILD was diagnosed by high-resolution CT (HRCT). We analysed the following variables at baseline (ABA initiation), 12 months and at the end of the follow-up: Modified Medical Research Council (MMRC) scale (1-point change), forced vital capacity (FVC) or diffusion lung capacity for carbon monoxide (DLCO) (improvement or worsening ≥10%), HRCT, DAS on 28 joints evaluated using the ESR (DAS28ESR) and CS-sparing effect. RESULTS: We studied 263 RA-ILD patients [150 women/113 men; mean (s.d.) age 64.6 (10) years]. At baseline, they had a median duration of ILD of 1 (interquartile range 0.25-3.44) years, moderate or severe degree of dyspnoea (MMRC grade 2, 3 or 4) (40.3%), FVC (% of the predicted) mean (s.d.) 85.9 (21.8)%, DLCO (% of the predicted) 65.7 (18.3) and DAS28ESR 4.5 (1.5). The ILD patterns were: usual interstitial pneumonia (UIP) (40.3%), non-specific interstitial pneumonia (NSIP) (31.9%) and others (27.8%). ABA was prescribed at standard dose, i.v. (25.5%) or s.c. (74.5%). After a median follow-up of 12 (6-36) months the following variables did not show worsening: dyspnoea (MMRC) (91.9%); FVC (87.7%); DLCO (90.6%); and chest HRCT (76.6%). A significant improvement of DAS28ESR from 4.5 (1.5) to 3.1 (1.3) at the end of follow-up (P < 0.001) and a CS-sparing effect from a median 7.5 (5-10) to 5 (2.5-7.5) mg/day at the end of follow-up (P < 0.001) was also observed. ABA was withdrawn in 62 (23.6%) patients due to adverse events (n = 30), articular inefficacy (n = 27), ILD worsening (n = 3) and other causes (n = 2). CONCLUSION: ABA may be an effective and safe treatment for patients with RA-ILD.


Sujet(s)
Abatacept/usage thérapeutique , Antirhumatismaux/usage thérapeutique , Polyarthrite rhumatoïde/complications , Pneumopathies interstitielles/traitement médicamenteux , Abatacept/effets indésirables , Antirhumatismaux/effets indésirables , Femelle , Humains , Pneumopathies interstitielles/imagerie diagnostique , Pneumopathies interstitielles/étiologie , Mâle , Tomodensitométrie , Résultat thérapeutique
16.
Clin Exp Rheumatol ; 38 Suppl 126(4): 110-115, 2020.
Article de Anglais | MEDLINE | ID: mdl-33025900

RÉSUMÉ

OBJECTIVES: Digestive involvement (DI) has been reported in 10-30% of primary Sjögren's syndrome (pSS) patients, and few studies have systematically analysed the prevalence of DI in pSS patients. The aim of this study was to describe DI prevalence in pSS patients from the Sjögrenser Study, and to analyse its clinical associations. METHODS: All patients included in the Sjögrenser study, a Spanish multicentre randomised cohort, containing demographic, clinical and histologic data, have been analysed retrospectively. Patients were classified according to the presence of DI (oesophageal, gastric, intestinal, hepatic and pancreatic), and we have performed DI clinical associations, descriptive statistics, Student t or χ2 test, and uni and multivariate logistic regression. RESULTS: From 437 included patients, 95% were women, with a median age of 58 years, 71 (16.2%) presented DI: 21 (29.5%) chronic atrophic gastritis, 12 (16.9%) oesophageal motility dysfunction, 3 (4.2%) lymphocytic colitis, 18 (25.3%) primary biliary cholangitis, 15 (21.1%) autoimmune hepatitis, 7 (9.8%) pancreatic involvement and 5 (7%) coeliac disease. Half of them developed DI at the same time or after pSS diagnosis. Patients with DI were significantly older at pSS diagnosis (p=0.032), more frequently women (p=0.009), presented more autoimmune hypothyroidism and C3 hypocomplementaemia (p=0.040), and were treated more frequently with glucocorticoids, immunosuppressant and biologic therapies. Patients with pancreatic involvement presented more central nervous system and renal involvement, Raynaud's phenomenon, lymphoma and C3/C4 hypocomplementaemia. CONCLUSIONS: DI is frequent in Sjögrenser patients, mainly in the form of autoimmune disorders, and seem to be associated with a more severe phenotype. Our results suggest that DI should be evaluated in pSS patients, especially those with more severe disease.


Sujet(s)
Hépatite auto-immune , Syndrome de Gougerot-Sjögren , Études de cohortes , Femelle , Humains , Mâle , Adulte d'âge moyen , Essais contrôlés randomisés comme sujet , Enregistrements , Études rétrospectives , Syndrome de Gougerot-Sjögren/complications , Syndrome de Gougerot-Sjögren/diagnostic , Syndrome de Gougerot-Sjögren/épidémiologie
17.
J Dtsch Dermatol Ges ; 18(11): 1250-1259, 2020 Nov.
Article de Anglais | MEDLINE | ID: mdl-33015940

RÉSUMÉ

BACKGROUND AND OBJECTIVES: Cutaneous polyarteritis nodosa (CPAN) is a comparatively rare form of vasculitis that affects small arteries and arterioles in the panniculus and dermo-subcutaneous junction. Limited information is available regarding its course in the European population. The aim of this study is to characterize the manifestations and prognostic markers of recurrence in CPAN. PATIENTS AND METHODS: We report a retrospective study of patients with clinical and histopathologic evidence of CPAN, which was treated at two tertiary referral centers in Spain between 1989 and 2019. RESULTS: 31 patients were included. The most frequent manifestation was subcutaneous nodules (90.3 %); ulcers were frequent at diagnosis (35.5 %). Two thirds of the patients had at least one extracutaneous manifestation. Seventeen patients (54.8 %) experienced relapse. The strongest predictor of recurrence was ulceration in the initial episode (OR 18.6; 95 % CI 2.73-38; p < 0.01). The pre-treatment results of laboratory parameters associated with inflammation (such as C-reactive protein and neutrophil-to-lymphocyte ratio) were significantly higher in the relapsing group. There were no disease-related deaths and none of the patients developed systemic PAN. CONCLUSIONS: Although CPAN is a vasculitis limited to the skin, symptoms may involve adjacent skeletal muscle or peripheral nerves. While the condition is not life-threatening, the presence of ulceration and elevation of certain laboratory parameters predicts a worse prognosis.


Sujet(s)
Polyartérite noueuse , Humains , Récidive tumorale locale , Pronostic , Études rétrospectives , Espagne
18.
Clin Exp Rheumatol ; 38 Suppl 126(4): 116-124, 2020.
Article de Anglais | MEDLINE | ID: mdl-33095138

RÉSUMÉ

OBJECTIVES: To investigate the prevalence, associated factors, and effects of primary overt renal disease on morbidity in patients with primary Sjögren's syndrome (pSS). METHODS: All patients in the Sjögrenser (registry of adult pSS patients of the Spanish Society of Rheumatology) cohort were retrospectively investigated for the presence of clinically significant renal involvement directly related to pSS activity. RESULTS: Of the 437 patients investigated, 39 (9%) presented overt renal involvement during follow-up. Severe renal disease necessitating kidney biopsy was relatively rare (2%). Renal involvement may complicate pSS at any time during the disease course and is associated with severe disease (indicated by higher scores of involvement, activity, and damage), systemic multiorgan involvement, and a higher frequency of lymphoma. Multivariate analysis showed that older age (odds ratio [OR] 1.03, 95% confidence interval [CI] 1.00-1.07), higher European League Against Rheumatism Sjögren's Syndrome Disease Activity Index scores (OR 1.1, CI 1.03-1.18), serum anti-La/SSB positivity (OR 6.65, CI 1.41-31.372), and non-vasculitic cutaneous involvement (OR 5.47, 1.03-29.02) were independently associated with this complication. Chronic renal failure developed in 23 of 39 patients (59%); only 1 of them progressed to end-stage renal disease necessitating renal replacement therapy. Patients with overt renal disease showed higher Sjögren's syndrome disease damage index scores, higher rates of hospitalisation due to disease activity and higher rates of clinically relevant comorbidities. CONCLUSIONS: Overt renal involvement in pSS is not uncommon. Although it usually shows a favourable prognosis, is associated with significant morbidity.


Sujet(s)
Maladies du rein , Syndrome de Gougerot-Sjögren , Adulte , Sujet âgé , Études de cohortes , Humains , Rein , Maladies du rein/diagnostic , Maladies du rein/épidémiologie , Études rétrospectives , Syndrome de Gougerot-Sjögren/diagnostic , Syndrome de Gougerot-Sjögren/épidémiologie
19.
Med. clín (Ed. impr.) ; 155(3): 126-129, ago. 2020. tab
Article de Espagnol | IBECS | ID: ibc-195754

RÉSUMÉ

OBJETIVO: Analizar las características clínicas, tratamiento y evolución de la vasculitis reumatoide. MÉTODOS: Estudio retrospectivo (1975-2017). Pacientes con vasculitis reumatoide diagnosticados en 2 servicios de reumatología. RESULTADOS: Se incluyó a 41 pacientes: 17 (41,5%) varones y 24 (58,5%) mujeres; con una edad media al diagnóstico de 67 ± 9 años y una duración de la artritis reumatoide de 10 ± 8,3 años. La artritis fue erosiva en 33 (80%) pacientes. Tanto el factor reumatoide como los anticuerpos antipéptido citrulinados fueron positivos en todos los casos. Los síntomas constitucionales se presentaron en 30 pacientes (73%) y las manifestaciones extrarticulares en 17 (41%). Las manifestaciones clínicas más frecuentes fueron: cutáneas 28 (68%) y neuropatía periférica 26 (63%). Todos los pacientes fueron tratados con glucocorticoides. En 24 pacientes (58%) se asoció a un inmunosupresor y 5 (12%) pacientes fueron tratados con fármacos biológicos. La mortalidad a los 2 años de seguimiento fue del 33%. Las principales causas de muerte fueron: la infección y la progresión de la vasculitis reumatoide. La frecuencia de la vasculitis reumatoide disminuyó en la última década. CONCLUSIONES: Las manifestaciones clínicas de la vasculitis reumatoide en España son similares a las descritas. La frecuencia disminuye; sin embargo, el cuadro clínico y la gravedad se mantienen invariables


AIM: To describe the clinical manifestations, evolution and treatment of patients with rheumatoid vasculitis. METHODS: Retrospective study (1975-2017) of all patients diagnosed with rheumatoid vasculitis in 2 Rheumatology Services. RESULTS: A total of 41 patients were included, 17 (41.5%) males and 24 (58.5%) females; mean age at diagnosis: 67 ± 9 years; duration of rheumatoid arthritis: 10 ± 8.3 years. Most patients had erosive disease, 33 (80%). Rheumatoid factor and anticitrullinated antibodies were positive in all patients. Constitutional symptoms were present in 30 (73%) patients and extra-articular manifestations in 17 (41%) patients. The clinical manifestations of rheumatoid vasculitis were mainly: cutaneous 28 (68%), and polyneuritis 26 (63%). All patients were treated with glucocorticoids. An immunosuppressant was associated in 24 (58.5%) patients. Five (12%) patients were treated with the association of glucocorticoids and a biologic treatment. The mortality after 2years of follow-up was 33%, the most common causes being infection and progression of the vasculitis. The frequency of rheumatoid vasculitis has decreased over the last decade. CONCLUSION: The clinical manifestations of rheumatoid vasculitis were similar to previous studies. The frequency of rheumatoid vasculitis seems to decrease. However, the clinical picture and severity remains invariable


Sujet(s)
Humains , Mâle , Femelle , Adulte d'âge moyen , Sujet âgé , Vascularite rhumatoïde/diagnostic , Vascularite rhumatoïde/thérapie , Évolution de la maladie , Études rétrospectives , Facteur rhumatoïde/analyse , Facteur rhumatoïde/effets des médicaments et des substances chimiques , Anticorps anti-protéines citrullinées/analyse , Glucocorticoïdes/usage thérapeutique , Biothérapie , Anticorps anti-cytoplasme des polynucléaires neutrophiles/analyse , Immunosuppresseurs
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