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Artigo em Inglês | MEDLINE | ID: mdl-31033198


OBJECTIVE: to compare the accuracy of Birmingham Vasculitis score (BVAS) v.3, and Five Factors Score (FFS) v.1996 and v.2009, to assess survival in ANCA-associated Vasculitis (AAV). METHODS: 550 patients with AAV (41.1% GPA, 37.3% MPA, 21.6% EGPA) diagnosed between 1990-2016 were analyzed. ROC curves and multivariable Cox analysis were used to assess the relationships between the outcome and the different scores. RESULTS: Overall mortality was 33.1%. The mean BVAS at diagnosis was 17.96±7.82, and was significantly higher in non-survivors than in survivors (20.0±8.14 vs. 16.95±7.47, p<0.001). The mean 1996FFS and 2009FFS were 0.81±0.94 and 1.47±1.16, respectively, and were significantly higher in non-survivors than in survivors (1.17±1.07 vs. 0.63±0.81, p<0.001; 2.13±1.09 vs. 1.15±1.05, p<0.001). Mortality rates increased accordingly to the different 1996FFS and 2009FFS categories. In multivariate analysis BVAS, 1996FFS and 2009FFS were significantly related to death (p=0.007, p=0.020, p<0.001), but the stronger predictor was the 2009FFS (HR 2.9, 2.4-3.6). When the accuracy of BVAS, 1996FFS and 2009FFS to predict survival was compared in the global cohort, ROC analysis yielded AUC values of 0.60, 0.65 and 0.74, respectively, indicating that 2009FFS had the best performance. Similar results were obtained when comparing these scores in patients diagnosed before and after 2001, and assessing the 1-year, 5-years and long-term mortality. Correlation among BVAS and 1996FFS was modest (r=0.49, p<0.001), but higher than between BVAS and 2009FFS (r=0.28, p<0.001). CONCLUSION: BVAS and FFS are useful to predict survival in AAV, but 2009FFS has the best prognostic accuracy at any point of the disease course. SIGNIFICANCE AND INNOVATION: This is the first study comparing the BVAS, 1996FFS and 2009FFS accuracy to assess survival in patients with AAV, and the first to validate 2009FFS in these patients. This article is protected by copyright. All rights reserved.

Clin Exp Rheumatol ; 36 Suppl 112(3): 121-129, 2018 May-Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30156546


OBJECTIVES: To analyse the clinical features and outcomes of patients presenting with life-threatening systemic disease in a large cohort of Spanish patients with primary Sjögren's syndrome (SS). METHODS: The GEAS-SS multicentre registry was formed in 2005 with the aim of collecting a large series of Spanish patients with primary SS, and included more than 20 Spanish reference centres with substantial experience in the management of SS patients. By January 2018, the database included 1580 consecutive patients fulfilling the 2002 classification criteria for primary SS. Severe, life-threatening systemic disease was defined as an activity level scored as "high" in at least one ESSDAI domain. RESULTS: Among 1580 patients, 208 (13%) were classified as presenting a severe, potentially life-threatening systemic disease: 193 presented one ESSDAI domain classified as high, 14 presented two high scored domains and only one presented three high activity domains. The ESSDAI domains involved consisted of lymphadenopathy in 78 (37%) cases, CNS in 28 (13%), PNS in 25 (12%), pulmonary in 25 (12%), renal in 21 (10%), cutaneous in 19 (9%), articular in 18 (9%), haematological in 7 (3%) and muscular in 4 (2%). Patients with severe systemic disease were more frequently men (p=0.001) and had a higher frequency of anaemia (p<0.001), lymphopenia (p<0.001), rheumatoid factor (p=0.021), low C3 levels (p=0.015), low C4 levels (p<0.001) and cryoglobulins (p<0.001). From a therapeutic point of view, systemic patients received more frequently glucocorticoids (p<0.001), immunosuppressants (p<0.001), intravenous immunoglobulins (p=0.008) and rituximab (p<0.001). We found an overall mortality rate of 20% in severe systemic patients, a rate that reached to 33% in patients presenting two or more high systemic involvements; these patients had a higher frequency of low C4 levels (p=0.012) and cryoglobulins (p=0.001) in comparison with those with a single severe organ involved. CONCLUSIONS: 13% of patients with primary SS develop a potentially life-threatening systemic disease (mainly lymphoma, but also severe internal organ involvements including nervous system, the lungs and the kidneys). This subset of patients requires intensive therapeutic management with a mortality rate of nearly 20% of cases.

Síndrome de Sjogren/epidemiologia , Adulto , Idoso , Técnicas de Apoio para a Decisão , Progressão da Doença , Feminino , Glucocorticoides/uso terapêutico , Humanos , Imunossupressores/uso terapêutico , Masculino , Pessoa de Meia-Idade , Fenótipo , Valor Preditivo dos Testes , Sistema de Registros , Medição de Risco , Fatores de Risco , Índice de Gravidade de Doença , Síndrome de Sjogren/diagnóstico , Síndrome de Sjogren/mortalidade , Síndrome de Sjogren/terapia , Espanha/epidemiologia , Resultado do Tratamento
Rev Esp Enferm Dig ; 110(6): 411-412, 2018 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-29739228


Listeria monocytogenes can cause invasive disease in immunocompromised patients. We have retrospectively reviewed the clinical characteristics, treatment and evolution of the cases of listeriosis registered at our center during a long period of 14 years. Listeria monocytogenes infection is potentially serious with high mortality. Therefore, a high index of suspicion and early directed antibiotic treatment are needed, especially for immunocompromised patients.

Listeriose/epidemiologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Listeriose/diagnóstico , Listeriose/terapia , Masculino , Pessoa de Meia-Idade , Prognóstico , Espanha/epidemiologia
Joint Bone Spine ; 85(6): 721-726, 2018 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-29452298


BACKGROUND: IgG4-related disease (IgG4-RD) is an autoimmune disease triggering an inflammatory cascade that leads to fibrosis. Outcome measures are limited and treatment options remain underexplored. OBJECTIVES: To assess the variation of the IgG4 responder index (IgG4-RI) in a cohort of IgG4-RD patients and to explore their treatments and outcomes. METHODS: We studied the clinical phenotype, severity of the disease and response to treatment in an ambispective multicenter cohort study including 14 different hospitals in Spain. All patients met the 2012 international consensus on pathology criteria for diagnosis. RESULTS: Sixty-eight patients were included, with a mean age of 53.4 years and predominance of male sex. The most commonly involved tissues were: retroperitoneum (33%), orbital pseudotumor (28%) and maxillary and paranasal sinuses (24%). IgG4-RI values were higher in patients with multiorgan disease and before treatment. After being treated, IgG4-RI values were lower, in accordance with the high rates of treatment response. Most patients received: glucocorticoids (GC), surgery, azathioprine (AZA), mofetil mycophenolate or rituximab. GC alone, GC plus surgery and GC plus AZA were given in the most of the IgG4-RD disease activity episodes. All treatments had high response rates but relapses and flares were common. CONCLUSIONS: IgG4-RI is a promising outcome measure in IgG4-RD, but still in development. Treatment algorithms are ill defined. GC and rituximab are the drugs with more evidence available. Disease modifying anti-rheumatic drugs may have a role in IgG4-RD and warrant more prospective studies.

Arch Esp Urol ; 68(10): 755-7, 2015 Dec.
Artigo em Espanhol | MEDLINE | ID: mdl-26634577


OBJECTIVE: To report two new cases of IgG4-related retroperitoneal fibrosis, a recently described pathology. METHODS: We analyze two cases diagnosed in our center and performed a literature review. RESULT: IgG4 related disease is a recently described entity that includes previously not related pathologies. The clinical manifestations are highly variable and its presentation is usually subacute. The treatment of choice is glucocorticoids. In our first case the outcome was favorable with corticosteroids and azathioprine. However, the second case required surgery on 2 occasions with radical nephrectomy. The diagnosis of the latter was made nine years after the onset of symptoms when the biopsy was reviewed; at that moment immunosuppressive therapy was not started. CONCLUSIONS: It is very important to know and diagnose this disease because of the good response to treatment that prevents complications.

Imunoglobulina G , Fibrose Retroperitoneal/imunologia , Adulto , Idoso , Feminino , Humanos , Masculino , Fibrose Retroperitoneal/diagnóstico , Fibrose Retroperitoneal/terapia
Medicine (Baltimore) ; 94(32): e1275, 2015 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-26266361


IgG4-related disease (IgG4-RD) is a rare entity consisting of inflammation and fibrosis that has been described in multiple organs. Concrete diagnostic criteria have been established recently and there is a lack of large series of patients.To describe the clinical presentation, histopathological characteristics, treatment and evolution of a series of IgG4-RD Spanish patients.A retrospective multicenter study was performed. Twelve hospitals across Spain included patients meeting the current 2012 consensus criteria on IgG4-RD diagnosis.Fifty-five patients were included in the study, 38 of whom (69.1%) were male. Median age at diagnosis was 53 years. Thirty (54.5%) patients were included in the Histologically Highly Suggestive IgG4-RD group and 25 (45.5%) in the probable IgG4-RD group. Twenty-six (47.3%) patients had more than 1 organ affected at presentation. The most frequently affected organs were: retroperitoneum, orbital pseudotumor, pancreas, salivary and lachrymal glands, and maxillary sinuses.Corticosteroids were the mainstay of treatment (46 patients, 83.6%). Eighteen patients (32.7%) required additional immunosuppressive agents. Twenty-four (43.6%) patients achieved a complete response and 26 (43.7%) presented a partial response (<50% of regression) after 22 months of follow-up. No deaths were attributed directly to IgG4-RD and malignancy was infrequent.This is the largest IgG4-RD series reported in Europe. Patients were middle-aged males, with histologically probable IgG4-RD. The systemic form of the disease was frequent, involving mainly sites of the head and abdomen. Corticosteroids were an effective first line treatment, sometimes combined with immunosuppressive agents. Neither fatalities nor malignancies were attributed to IgG4-RD.

Doenças Autoimunes/diagnóstico , Imunoglobulina G , Doenças Autoimunes/tratamento farmacológico , Doenças Autoimunes/epidemiologia , Humanos , Imunossupressores/uso terapêutico , Paraproteinemias/tratamento farmacológico , Paraproteinemias/epidemiologia , Paraproteinemias/patologia , Sistema de Registros , Espanha/epidemiologia