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.
Objective The objective of this study was to describe the demographic, clinical, and immunological manifestations of systemic lupus erythematosus (SLE) in male patients. Methods A cross-sectional, multicenter study was carried out of 3651 patients (353 men, 9.7%, and 3298 women, 90.2%) diagnosed with SLE, included in the Spanish Rheumatology Society SLE Registry (RELESSER). Results Mean ages (18-92 years) of symptom onset were 37 (SD 17) years (men) and 32 (SD 14) years (women). Male/female ratio was 1/9. Age of onset of symptoms and age at diagnosis were higher in men than in women ( p < 0.001). Males were diagnosed earlier than females (p = 0.04) and had more cardiovascular comorbidities ( p < 0.001). Two hundred and thirty-six males (68%) with SLE required hospitalization in comparison with 1713 females (53%) ( p < 0.001). During follow-up, 208 patients died: 30 men (9.3%) and 178 women (5.9%) ( p = 0.02). As regards clinical manifestations, loss of weight ( p = 0.01), lymphadenopathies ( p = 0.02), and splenomegaly ( p = 0.02) were more common in male patients. Female patients were more likely to have inflammatory rash, alopecia, and arthritis ( p < 0.05). As for lung involvement, men with SLE had more pleural fibrosis ( p < 0.001) and pulmonary embolism ( p = 0.01). However, Raynaud's phenomenon was more common in women (35%) than in men (23.7%) ( p < 0.001); lupus nephritis was more common in men, being present in 155 (44.8%) of males versus 933 (29%) of females ( p < 0.001). Multivariate analysis showed that SLE patients with a high Charlson index (more than 3 points) and age > 50 years had a higher mortality (odds ratios 3.6 and 2.1, respectively). Furthermore, SLE patients who developed pulmonary hemorrhage, pulmonary hypertension, psychiatric involvement, complement deficiency, and hemophagocytic syndrome also had higher mortality, regardless of gender. Conclusion Patients with SLE over the age of 50 years have an increased risk of mortality. In Caucasians, age at diagnosis and symptom onset is higher in men than in women. The diagnostic delay is shorter in men. Male SLE patients present more cardiovascular comorbidities, and also more serositis, adenopathies, splenomegaly, renal involvement, convulsion, thrombosis, and lupus anticoagulant positivity than women.
ObjectiveThe main study objective was to determine how giant cell arteritis (GCA) is diagnosed in our clinical practice and whether the EULAR recommendations have influenced the diagnostic procedures used.MethodsARTEritis of the Rheumatology Spanish Society -Sociedad Española de Reumatología (ARTESER) is a multicentre observational retrospective study conducted in 26 hospitals with support from the Spanish Society of Rheumatology. All patients diagnosed with GCA between 1 June 2013 and 29 March 2019 were included. The gold standard for the diagnosis of GCA was the judgement of the physician in charge, according to clinical criteria, supported by data available from laboratory tests, imaging studies (ultrasound, positron emission tomography (PET) and MRI/CT angiography) and temporal artery biopsy (TAB) when available.ResultsWe included 1675 patients with GCA (mean age±SD (76.9±8.1) years, 1178 women (70.3%)). Of these, 776 patients had a positive TAB (46.3%), 503 (30.0%) positive ultrasound, 245 positive PET (14.6%) and 64 positive MRI/CT angiography (3.8%). These percentages changed substantially over the study. From 2013 to 2019, the use of ultrasound in diagnosis grew from 25.8% to 52.9% and PET from 12.3% to 19.6%, while use of TAB decreased from 50.3% to 33.3%.ConclusionsBiopsy was the most widely used diagnostic test for confirming GCA, but use of imaging as a diagnostic tool has grown in recent years. Following publication of the 2018 EULAR recommendations, ultrasound has displaced biopsy as the first-line diagnostic test; TAB was performed in a third and PET in a fifth of cases.
BackgroundBehçet's disease is a recurrent systemic vasculitis which may have neurological manifestations: Neuro-Behçet (NB). The estimated frequency is 1-59%. It is more common in males. Central neurological system (CNS) involvement, presenting as pseudotumoral masses are very rareObjectivesTo describe the clinical characteristics, incidence, treatment, and outcome of patients diagnosed of pseudotumoral neuro-Behçet disease. We performed a systematic review of cases reported in the literature.MethodsDesing: retrospective (1984-2014). Location: Two University hospitals. A hundred and fifty five patients records were reviewed with a diagnosis of Behçet's disease from the Rheumatology service data base. Inclusion criteria: patients with pseudotumoral neuro-Behçet. A systematic search of all published cases with this diagnosis through PubMed was also performed. (1987-2014, keywords: pseudotumoral, neuro-Behçet)ResultsThree new cases of pseudotumoral Neuro-Behçet were identified. After a systematic review, other 27 cases were found with this diagnosis. The whole series were composed of 30 patients: a predominance of male (73.3%) was observed, with a mean age: 39.2±12.1 (range: 23-63 years). The mean duration of Behçet's disease prior to the diagnosis of NB, was 9.3±7.8 years, in 50% of patients.The diagnosis was performed simultaneously or later in the remaining patients. The location of the lesion was: capsular thalamus region 15 (50%), peduncle and cerebellar 4 (13.3%), fronto parietal region 2 (6.7%), fronto temporal and temporo -occipital region in 1 (3.3%). Four (13.3%) patients presented more than one lesion. In 14 (46.7%) patients biopsy of the brain mass was performed in order to confirm the diagnostic. All reported patients were initially treated with high-dose of glucocorticosteroids (oral or intravenous), other immunosuppressive agents such as azathioprine, cyclophosphamide, methotrexate or chlorambucil were added. Three (10%) patients (2 of own serie and 1 described in literature) were treated with biological agents (anti TNF alpha, rituximab and tocilizumab). Only in one case plasmapheresis was performed because of refractory disease. Most patients showed progressive improvement, and three (10%) patients died.ConclusionsThe presence of pseudotumoral lesions is a very rare presentation of Neuro-Behçet. It is more common in males and occurs years after diagnosis; however it can also occurs simultaneously. A differential diagnosis with other CNS diseases and malignancy is mandatory. The response to glucocorticoides plus immunosuppressive drugs is good. Biological agents may be useful for refractory cases.Disclosure of InterestNone declared
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