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1.
Semin Arthritis Rheum ; 47(6): 849-857, 2018 06.
Artigo em Inglês | MEDLINE | ID: mdl-29246416

RESUMO

OBJECTIVE: To assess the prevalence and causes of hepatobiliary involvement (HBI) in systemic sclerosis (SSc), to investigate the clinical characteristics and prognosis of SSc patients with HBI (SSc-HBI) and without HBI (SSc-non-HBI), and to compare both groups according to the cutaneous SSc subsets. METHODS: In all, 1572 SSc patients were collected in the RESCLE registry up to January 2015, and all hepatobiliary disturbances were recorded. We investigated the HBI-related characteristics and survival from the entire SSc cohort and according to the following cutaneous subsets: diffuse cutaneous SSc (dcSSc), limited cutaneous SSc (lcSSc), and SSc sine scleroderma (ssSSc). RESULTS: Out of 1572, 118 (7.5%) patients had HBI. Primary biliary cholangitis (PBC) was largely the main cause (n = 67, 4.3%), followed by autoimmune hepatitis (n = 19, 1.2%), and anti-mitochondrial negative PBC (n = 6, 0.4%). Other causes of HBI were as follows: secondary liver diseases (n = 11, 0.7%), SSc-related HBI (n = 7, 0.4%), nodular regenerative hyperplasia (n = 3, 0.2%), liver cirrhosis (n = 3, 0.2%), and HBI of unknown origin (n = 2, 0.1%). In multivariate analysis, HBI was independently associated to lesser risk of dcSSc (5.1% vs. 24.4%), and higher frequency of calcinosis (26% vs. 18%), left ventricular diastolic dysfunction (46% vs. 27%), sicca syndrome (51% vs. 29%), and anti-centromere antibodies (ACA, 73% vs. 44%). According to the cutaneous subsets, HBI was associated (1) in lcSSc, to longer time from SSc onset to diagnosis (10.8 ± 12.5 vs. 7.2 ± 9.3 years), sicca syndrome (54% vs. 33%), and ACA (80% vs. 56%); (2) in ssSSc, to sicca syndrome (44% vs. 19%), and (3) in dcSSc, no associations were found. HBI was the cause of death in 2.3% patients but the cumulative survival according to the presence or absence of HBI showed no differences. CONCLUSIONS: HBI prevalence in SSc is 7.5% and dcSSc is the least involved subset. PBC is the main cause of HBI. Patients with SSc-HBI exhibited specific clinical and immunologic profile. Survival is similar for SSc patients with HBI.


Assuntos
Colangite/etiologia , Hepatite Autoimune/etiologia , Escleroderma Sistêmico/complicações , Adulto , Idoso , Colangite/mortalidade , Feminino , Hepatite Autoimune/mortalidade , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Sistema de Registros , Escleroderma Sistêmico/mortalidade , Síndrome de Sjogren/complicações , Síndrome de Sjogren/mortalidade , Espanha , Taxa de Sobrevida
2.
Clin Exp Rheumatol ; 35 Suppl 106(4): 98-105, 2017 Sep-Oct.
Artigo em Inglês | MEDLINE | ID: mdl-28980912

RESUMO

OBJECTIVES: To assess the clinical manifestations and prognosis of Spanish patients with systemic sclerosis (SSc) according to their immunological profile. METHODS: From the Spanish Scleroderma Study Group or RESCLE (Registro de ESCLErodermia as Spanish nomenclature) Registry we selected those patients in which anti-centromere (ACA), anti-topoisomerase I (ATA), and anti-RNA polymerase III (ARA) antibodies had been determined, and a single positivity for each SSc specific antibody was detected. Demographic, clinical, laboratory, and survival data were compared according to the serologic status of these antibodies. RESULTS: Overall, 209 SSc patients were included. In 128 (61%) patients ACA was the only positive antibody, 46 (22%) were only positive for ATA, and 35 (17%) for ARA. Of note, the three groups were mutually exclusive. In univariate analysis, patients with ACA presented more frequently limited cutaneous SSc (lcSSc) (p<0.001), whereas diffuse cutaneous SSc (dcSSc) was the most frequent subtype in patients with ATA (54%) and ARA (62%) (both p<0.001). Positive patients for ARA showed the highest prevalence of joint involvement (p<0.001) and those from ATA group had a higher prevalence of interstitial lung disease (ILD) (p<0.001). Scleroderma renal crisis was more frequent in the ARA group (p<0.001). In multivariate analysis, ACA were associated with female gender and were protective for dcSSc and ILD. ATA were found to be protective for lcSSc and they were independently associated with interstitial reticular pattern. ARA positivity was independently associated with dcSSc. We did not find differences in mortality between the three groups. CONCLUSIONS: In Spanish SSc patients, the presence of SSc specific antibodies conferred a distinctive clinical profile.


Assuntos
Autoanticorpos/análise , Escleroderma Sistêmico/imunologia , Adulto , Idoso , Centrômero/imunologia , Estudos de Coortes , DNA Topoisomerases Tipo I/imunologia , Feminino , Humanos , Doenças Pulmonares Intersticiais/epidemiologia , Masculino , Pessoa de Meia-Idade , Prognóstico , RNA Polimerase III/imunologia , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/mortalidade
3.
Clin Exp Rheumatol ; 35 Suppl 106(4): 40-47, 2017 Sep-Oct.
Artigo em Inglês | MEDLINE | ID: mdl-28229826

RESUMO

OBJECTIVES: To determine the changes in the pattern of death of patients with systemic sclerosis (SSc) throughout 20 years. METHODS: Data were collected from the Spanish Scleroderma Registry (RESCLE), retrospective multicentre database from 1990 to 2009. SSc-related and SSc-non related causes of death were assessed. RESULTS: 987 patients were recruited. Overall standardised mortality ratio (SMR) was 2.34 (2.24-2.44). SSc-related causes of death were responsible of 72% of all deaths of those patients diagnosed within 1990-99 vs. 48% within 2000-09 (p=0.006). Relative pulmonary death rate was stable over time (68.1% within 1990-99 vs. 63.9% within 2000-09, p=0.815). Relative renal death rate was decreasing over time (17% within 1990-99 vs. 5.5% within 2000-09, p=0.175). Heart distribution tripled its ratio (12.8% within 1990-99 vs. 30.6% within 2000-09, p=0.058). CONCLUSIONS: SSc-related causes of death were decreasing over time and, among them, pulmonary involvement was the leading cause of death in both decades. The ratio of renal causes decreased since 1990 at the time that the ratio of cardiac causes increased.


Assuntos
Sistema de Registros , Escleroderma Sistêmico/mortalidade , Adulto , Idoso , Causas de Morte , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Espanha/epidemiologia , Fatores de Tempo
4.
Semin Arthritis Rheum ; 46(2): 200-8, 2016 10.
Artigo em Inglês | MEDLINE | ID: mdl-27312381

RESUMO

OBJECTIVE: Digital ulcers (DU) are the most common vascular complication of systemic sclerosis (SSc). We compared the characteristics between patients with prior or current DU with those never affected and evaluated whether a history of DU may be a predictor of vascular, organ involvement, and/or death in patients with SSc. METHODS: Data from SSc patients with or without prior or current DU were collected by 19 referral centers in an ongoing registry of Spanish SSc patients, named Registro de ESCLErodermia (RESCLE). Demographics, organ involvement, autoimmunity features, nailfold capillary pattern, survival time, and causes of death were analyzed to identify DU related characteristics and survival of the entire series and according to the following cutaneous subsets-diffuse cutaneous SSc (dcSSc), limited cutaneous SSc (lcSSc), and SSc sine scleroderma (ssSSc). RESULTS: Out of 1326, 552 patients enrolled in the RESCLE registry had prior or current DU, 88% were women, the mean age was 50 ± 16 years, and the mean disease duration from first SSc symptom was 7.6 ± 9.6 years. Many significant differences were observed in the univariate analysis between patients with and without prior/current DU. Multivariate analysis identified that history of prior/current DU in patients with SSc was independently associated to younger age at SSc diagnosis, diffuse cutaneous SSc, peripheral vascular manifestations such Raynaud's phenomenon, telangiectasia, and acro-osteolysis but no other vascular features such as pulmonary arterial hypertension or scleroderma renal crisis. DU was also associated to calcinosis cutis, interstitial lung disease, as well as worse survival. Multivariate analysis performed in the cutaneous subsets showed that prior/current DU were independently associated: (1) in dcSSc, to younger age at SSc diagnosis, presence of telangiectasia and calcinosis and rarely a non-SSc pattern on nailfold capillaroscopy; (2) in lcSSc, to younger age at SSc diagnosis, presence of Raynaud's phenomenon as well as calcinosis cutis, interstitial lung disease, and higher incidence of death from all causes; and (3) in ssSSc, to younger age at first SSc symptom and greater incidence of death from all causes. CONCLUSIONS: Digital ulcers develop in patients with SSc younger at diagnosis, mainly in patients with dcSSc and lcSSc, and they are associated to other peripheral vascular manifestations such as Raynaud's phenomenon, telangiectasia, and acro-osteolysis but also to calcinosis, and interstitial lung disease. History of DU in SSc leads to worse survival, also noticeable for lcSSc and ssSSc subsets but not for dcSSc patients.


Assuntos
Escleroderma Sistêmico/complicações , Úlcera Cutânea/etiologia , Adulto , Idoso , Estudos Transversais , Feminino , Humanos , Masculino , Angioscopia Microscópica , Pessoa de Meia-Idade , Sistema de Registros , Escleroderma Sistêmico/mortalidade , Escleroderma Sistêmico/fisiopatologia , Úlcera Cutânea/mortalidade , Úlcera Cutânea/fisiopatologia , Espanha/epidemiologia , Taxa de Sobrevida
5.
Med. clín (Ed. impr.) ; 146(1): 8-15, ene. 2016. ilus, tab
Artigo em Espanhol | IBECS | ID: ibc-147353

RESUMO

Introducción y objetivos: La cirrosis biliar primaria (CBP) se asocia a algunas enfermedades autoinmunes sistémicas (EAS), en particular a la esclerosis sistémica (ES. Determinar la prevalencia de EAS en una cohorte de pacientes con CBP, específicamente la ES y sus diferentes subtipos clínicos, y establecer el perfil clínico-biológico propio de estos pacientes. Métodos: Estudio observacional de 62 pacientes con CBP, con un protocolo que incluía una anamnesis y exploración física dirigidas a detectar una EAS, la realización de una capilaroscopia ungueal microscópica y un amplio estudio de autoinmunidad, incluido el perfil de anticuerpos específicos de ES. Se realizó un análisis comparativo entre el grupo de pacientes con CBP aislada y los pacientes con CBP y una EAS asociada. Resultados: Se asoció una EAS en 22 pacientes (35,4%), y la ES fue la entidad más frecuente (21%), del subtipo cutáneo limitado (11%). Cinco pacientes (8%) sin EAS previa cumplían criterios de preesclerodermia, según los criterios de LeRoy y Medsger. Los anticuerpos anticentrómero (54,5 vs. 5%, p < 0,001) fueron el único parámetro inmunológico identificado con mayor frecuencia en pacientes con EAS. El patrón capilar sugestivo de ES se visualizó en 11 pacientes (20,4%). No se identificaron factores asociados a mayor morbimortalidad en ningún grupo. Conclusiones: Existe un subgrupo de pacientes con CBP con características clínico-biológicas que sugieren la asociación con una EAS, con elevada probabilidad, y que recomiendan el estudio protocolizado de estos pacientes con CBP para detectar de forma precoz EAS (AU)


Background and objectives: Primary biliary cirrhosis (PBC) is associated to any systemic autoimmune disease (SAD), in particular systemic sclerosis (SSc). To investigate the prevalence of SAD in a cohort of patients with PBC, specifically the prevalence of SSc and its clinical subtypes, and determining the clinical and biological profile of patients with associated PBC and SSc. Methods: Observational study of 62 patients with PBC following a protocol that included an anamnesis and physical examination to detect the presence of SAD as well as a nailfold capillaroscopy and an immunological study with specific SSc autoantibodies. A comparative analysis was conducted between patients with isolated PBC and patients with PBC and an associated SAD. Results: SAD was associated to PBC in 22 patients (35,4%), and SSc was the most frequent illness, identified in 13 cases (21%). Five patients (8%) without previous diagnosis of SAD fulfilled pre-scleroderma criteria, according to LeRoy and Medsger criteria. The presence of anticentromere antibodies (54,5% vs. 5%,P < .001) was the unique immunological determination identified more frequently in patients with PBC-SAD. The SSc suggestive capillary pattern was visualized in 11 patients (20,4%), mainly the slow pattern. No factors associated with greater morbi-mortality were identified in the PBC-SAD group. Conclusions: It does exist a subgroup of patients with PBC and clinical-biological features suggestive of an SAD, which advise a protocolized study to detect early the association to an SAD (AU)


Assuntos
Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Cirrose Hepática Biliar/complicações , Cirrose Hepática Biliar/epidemiologia , Doenças Autoimunes/epidemiologia , Cirrose Hepática Biliar/imunologia , Prognóstico , Ductos Biliares Extra-Hepáticos/imunologia , Ductos Biliares Extra-Hepáticos/patologia , Autoimunidade/imunologia , Síndrome de Sjogren/complicações , Artrite Reumatoide/complicações , Doenças Autoimunes/complicações , Anamnese/métodos , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/epidemiologia , Protocolos Clínicos , Dedos/microbiologia , Dedos/patologia , Dedos , Autoanticorpos , Técnica Indireta de Fluorescência para Anticorpo/métodos
6.
Med Clin (Barc) ; 146(1): 8-15, 2016 Jan 01.
Artigo em Espanhol | MEDLINE | ID: mdl-25978920

RESUMO

BACKGROUND AND OBJECTIVES: Primary biliary cirrhosis (PBC) is associated to any systemic autoimmune disease (SAD), in particular systemic sclerosis (SSc). To investigate the prevalence of SAD in a cohort of patients with PBC, specifically the prevalence of SSc and its clinical subtypes, and determining the clinical and biological profile of patients with associated PBC and SSc. METHODS: Observational study of 62 patients with PBC following a protocol that included an anamnesis and physical examination to detect the presence of SAD as well as a nailfold capillaroscopy and an immunological study with specific SSc autoantibodies. A comparative analysis was conducted between patients with isolated PBC and patients with PBC and an associated SAD. RESULTS: SAD was associated to PBC in 22 patients (35,4%), and SSc was the most frequent illness, identified in 13 cases (21%). Five patients (8%) without previous diagnosis of SAD fulfilled pre-scleroderma criteria, according to LeRoy and Medsger criteria. The presence of anticentromere antibodies (54,5% vs. 5%, P<.001) was the unique immunological determination identified more frequently in patients with PBC-SAD. The SSc suggestive capillary pattern was visualized in 11 patients (20,4%), mainly the slow pattern. No factors associated with greater morbi-mortality were identified in the PBC-SAD group. CONCLUSIONS: It does exist a subgroup of patients with PBC and clinical-biological features suggestive of an SAD, which advise a protocolized study to detect early the association to an SAD.


Assuntos
Doenças Autoimunes/diagnóstico , Cirrose Hepática Biliar/etiologia , Angioscopia Microscópica , Escleroderma Sistêmico/diagnóstico , Adulto , Idoso , Idoso de 80 Anos ou mais , Doenças Autoimunes/complicações , Doenças Autoimunes/epidemiologia , Feminino , Humanos , Cirrose Hepática Biliar/diagnóstico , Cirrose Hepática Biliar/fisiopatologia , Masculino , Pessoa de Meia-Idade , Prevalência , Prognóstico , Estudos Retrospectivos , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/epidemiologia
7.
Clin Exp Rheumatol ; 32(3 Suppl 82): S30-3, 2014 May-Jun.
Artigo em Inglês | MEDLINE | ID: mdl-24709033

RESUMO

OBJECTIVES: The Xq28 region, containing IRAK and MECP2, represent a common susceptibility locus for a high number of autoimmune diseases. Our aim in the present study was to evaluate the influence of the IRAK1 and MECP2 autoimmunity-associated genetic variants in the giant cell arteritis (GCA) susceptibility and its clinical subphenotypes. METHODS: We analysed a total of 627 female biopsy-proven GCA patients and 1,520 female healthy controls of Spanish Caucasian origin. Two polymorphisms, rs1059702 and rs17345, located at IRAK1 and MECP2, respectively, were genotyped using TaqMan® allelic discrimination assays. RESULTS: No association with any of the analysed polymorphisms was evident when genotype and allele frequencies were compared between GCA patients and controls (rs1059702: allelic p-value=0.699, OR=0.96, CI 95% 0.80-1.17; rs17435: allelic p-value=0.994, OR=1.00, CI 95% 0.84-1.19). Likewise, the subphenotype analysis yield similar negative results. CONCLUSIONS: We have assessed for the first time the possible role of IRAK1 and MECP2 autoimmune disease-associated polymorphisms in GCA. Our data suggest that IRAK1 rs1059702 and MECP2 rs17435 genetic variants do not play a significant role in GCA susceptibility or severity.


Assuntos
Artérias/patologia , Autoimunidade/genética , Arterite de Células Gigantes , Quinases Associadas a Receptores de Interleucina-1/genética , Proteína 2 de Ligação a Metil-CpG/genética , Idoso , Biópsia , Grupo com Ancestrais do Continente Europeu/genética , Feminino , Frequência do Gene , Predisposição Genética para Doença , Arterite de Células Gigantes/epidemiologia , Arterite de Células Gigantes/genética , Arterite de Células Gigantes/patologia , Humanos , Polimorfismo de Nucleotídeo Único , Espanha/epidemiologia
9.
Clin Exp Rheumatol ; 30(1 Suppl 70): S29-33, 2012 Jan-Feb.
Artigo em Inglês | MEDLINE | ID: mdl-22512842

RESUMO

OBJECTIVES: CD226 genetic variants have been associated with a number of autoimmune diseases. The aim of this study was to investigate the potential implication of the CD226 loci in the susceptibility to and main clinical manifestations of giant cell arteritis (GCA). METHODS: A Spanish Caucasian cohort of 455 patients diagnosed with biopsy-proven GCA and 1414 healthy controls were included in the study. Three CD226 polymorphisms, rs727088, rs34794968 and rs763361, were genotyped using the TaqMan® allelic discrimination technology. PLINK software was used for the statistical analyses. RESULTS: No significant association between the CD226 polymorphisms and susceptibility to GCA was found (rs727088: p=0.92, OR=1.01, CI 95% 0.86-1.18; rs34794968: p=0.61, OR=1.04, CI 95% 0.89-1.22; rs763361: p=0.88, OR=0.99, CI 95% 0.84-1.16). Similarly, when patients were stratified according to the specific clinical features of GCA such as polymyalgia rheumatica, visual ischaemic manifestations or irreversible occlusive disease, no association was observed either between the case subgroups and the control set or between GCA patients with and without the specific features of the disease. Furthermore, the haplotype analysis revealed no significant association with the clinical manifestations of the disease. CONCLUSIONS: Our results show that the three CD226 polymorphisms analysed do not play a relevant role in the susceptibility to GCA and clinical manifestations of this vasculitis.


Assuntos
Antígenos de Diferenciação de Linfócitos T/genética , Autoimunidade/genética , Arterite de Células Gigantes/genética , Polimorfismo de Nucleotídeo Único , Idoso , Biópsia , Estudos de Casos e Controles , Distribuição de Qui-Quadrado , Feminino , Frequência do Gene , Testes Genéticos , Arterite de Células Gigantes/epidemiologia , Arterite de Células Gigantes/imunologia , Arterite de Células Gigantes/patologia , Haplótipos , Humanos , Masculino , Razão de Chances , Fenótipo , Reação em Cadeia da Polimerase em Tempo Real , Medição de Risco , Fatores de Risco , Espanha/epidemiologia
10.
J Rheumatol ; 38(12): 2598-601, 2011 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-21965647

RESUMO

OBJECTIVE: To investigate whether a functional integrin alpha M (ITGAM) variant is involved in susceptibility to and clinical manifestations of giant cell arteritis (GCA). METHODS: A Spanish cohort of 437 white patients with biopsy-proven GCA and 1388 healthy controls were genotyped using the TaqMan allele discrimination technology. RESULTS: No association was observed between ITGAM rs1143679 and GCA (p = 0.80, OR 0.97). Similarly, subphenotype analyses did not yield significant differences between the case subgroups and the control set or between GCA patients with or without the main specific features of GCA. CONCLUSION: Our results suggest that the ITGAM rs1143679 variant does not play an important role in the pathophysiology of GCA.


Assuntos
Antígeno CD11b/genética , Predisposição Genética para Doença , Arterite de Células Gigantes/genética , Arterite de Células Gigantes/patologia , Polimorfismo Genético , Idoso , Biópsia , Feminino , Genótipo , Humanos , Masculino , Pessoa de Meia-Idade , Espanha
12.
Artigo em Inglês | MEDLINE | ID: mdl-11817522

RESUMO

The novel antipsychotic olanzapine has structural and pharmacological properties similar to clozapine. Until recently, no haematological toxicity has been reported with olanzapine use and it was expected to be a safer alternative. The authors report a case of agranulocytosis induced by olanzapine and reviewed the 11 cases reported in the literature.


Assuntos
Agranulocitose/induzido quimicamente , Antipsicóticos/efeitos adversos , Pirenzepina/efeitos adversos , Agranulocitose/sangue , Agranulocitose/fisiopatologia , Benzodiazepinas , Humanos , Masculino , Pessoa de Meia-Idade , Olanzapina , Pirenzepina/análogos & derivados
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