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11. El proceso de divulgación de la donación y el trasplante del siglo XXI: experiencia en redes sociales de «Coordinación Sectorial de Trasplantes de Sevilla-Huelva»

Author

Egea-Guerrero, Juan José, Martín-Villén, Luis, Ruiz de Azúa-López, Zaida, Maroto Monserrat, F., Sánchez-Román, J. A., Cabeza-Cabeza, F., Egea-Guerrero, Juan José, Martín-Villén, Luis, Ruiz de Azúa-López, Zaida, Maroto Monserrat, F., Sánchez-Román, J. A., and Cabeza-Cabeza, F.

Published
2020

15. Registry of the Spanish Network for Systemic Sclerosis

Author

Simeón-Aznar, C.P., Fonollosa-Plá, V., Tolosa-Vilella, Carles, Espinosa-Garriga, G., Campillo-Grau, M., Ramos-Casals, M., García-Hernández, F.J., Castillo-Palma, M.J., Sánchez-Román, J., Callejas-Rubio, J.L., Ortego-Centeno, N., Egurbide-Arberas, M.V., Trapiellla-Martínez, L., Caminal-Montero, L., Sáez-Comet, L., Velilla-Marco, J., Camps-García, M.T., de Ramón-Garrido, E., Esteban-Marcos, E.M., Pallarés-Ferreres, L., Navarrete-Navarrete, N., Vargas-Hitos, J.A., de la Torre, R. Gómez, Salvador-Cervello, G., Rios-Blanco, J.J., and Vilardell-Tarrés, M.

Subjects
Adult, Male, Scleroderma, Systemic, Observational Study, Middle Aged, Risk Factors, Spain, Cause of Death, Humans, Female, Registries, Research Article, Aged, Retrospective Studies
Abstract

Systemic sclerosis (SSc) is a rare, multisystem disease showing a large individual variability in disease progression and prognosis. In the present study, we assess survival, causes of death, and risk factors of mortality in a large series of Spanish SSc patients. Consecutive SSc patients fulfilling criteria of the classification by LeRoy were recruited in the survey. Kaplan–Meier and Cox proportional-hazards models were used to analyze survival and to identify predictors of mortality. Among 879 consecutive patients, 138 (15.7%) deaths were registered. Seventy-six out of 138 (55%) deceased patients were due to causes attributed to SSc, and pulmonary hypertension (PH) was the leading cause in 23 (16.6%) patients. Survival rates were 96%, 93%, 83%, and 73% at 5, 10, 20, and 30 years after the first symptom, respectively. Survival rates for diffuse cutaneous SSc (dcSSc) and limited cutaneous SSc were 91%, 86%, 64%, and 39%; and 97%, 95%, 85%, and 81% at 5, 10, 20, and 30 years, respectively (log-rank: 67.63, P

Published
2015

16. Registry of the Spanish network for systemic sclerosis: Survival, prognostic factors, and causes of death

Author

Simeón Aznar, Carmen Pilar, Fonollosa Pla, Vicent, Tolosa Vilella, Carles, Espinosa Garriga, Gerard, Campillo Grau, M., Ramos Casals, Manuel, García Hernández, F.J., Castillo Palma, María Jesús, Sánchez Román, J., Callejas Rubio, José Luis, Ortego Centeno, Norberto, Egurbide Arberas, María Victoria, Trapiella Martínez, Luis, Caminal Montero, L., Sáez Comet, Luis, Velilla Marco, J., Camps García, María Teresa, Ramón Garrido, E . de, Esteban Marcos, E.M., Pallarés Ferreres, Lucio, Navarrete Navarrete, N., Vargas Hitos, José Antonio, Gómez de la Torre, Ricardo, Salvador Cervelló, Gonzalo, Ríos Blanco, Juan José, Vilardell Tarrés, M., Spanish Scleroderma Study Group (SSSG), Autoimmune Diseases Study Group (GEAS), Spanish Society of Internal Medicine (SEMI), Systemic Autoimmune Diseases Group (GEAS), Spanish Scleroderma Study Group (SSSG), Spanish Society of Internal Medicine, Spain, [Simeón-Aznar,CP, Fonollosa-Plá,V, Vilardell-Tarrés,M] Department of Internal Medicine, Hospital Valld’Hebron. [Tolosa-Vilella,C] Department of Internal Medicine, Hospital Parc Taulí, Sabadell. [Espinosa-Garriga,G, Campillo-Grau,M] Department of Autoimmune Diseases, Hospital Clinic. [Campillo-Grau,M] Laboratori of Computacional Medicine, Bioestatistics Unit, Universitat Autònoma de Barcelona, Bellaterra, Barcelona. [García-Hernández,FJ, Castillo-Palma,MJ, Sánchez-Román,J] Unit of Connective Tissue Diseases, Department of Internal Medicine, Hospital Virgen del Rocio, Sevilla. [Callejas-Rubio,JL, Ortego-Centeno,N] Unit of Autoimmune Systemic Diseases, Department of Internal Medicine, Hospital Clínico San Cecilio, Granada. [Egurbide-Arberas,MV] Department of Internal Medicine, Hospital de Cruces, Galdakano, Bilbao. [Trapiellla-Martínez,L] Department of Internal Medicine, Hospital de Cabueñes, Gijón. [Caminal-Montero,L] Department of Internal Medicine, Hospital Universitario Central de Asturias, Oviedo. [Sáez-Comet,L, Velilla-Marco,J] Department of Internal Medicine, Hospital Miguel Servet, Zaragoza. [Camps-García,MT, de Ramón-Garrido,E] Department of Internal Medicine, Hospital Carlos Haya, Málaga. [Esteban-Marcos,EM, Pallarés-Ferreres,L]Department of Internal Medicine, Hospital Son Espases, Palma de Mallorca. [Navarrete-Navarrete,N, Vargas-Hitos,JA] Department of Internal Medicine, Hospital Virgen de las Nieves, Granada. [Gómez de la Torre,R] Department of Internal Medicine, Hospital San Agustín, Avilés. [Salvador-Cervello,G] Department of Internal Medicine, Hospital La Fe, Valencia. [Rios-Blanco,JJ] Department of Internal Medicine, Hospital La Paz, Madrid, and Universitat de Barcelona

Subjects
Male, Multivariate analysis, Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Statistics as Topic::Models, Statistical::Proportional Hazards Models [Medical Subject Headings], sistema de registros, Diseases::Respiratory Tract Diseases::Lung Diseases::Hypertension, Pulmonary [Medical Subject Headings], Autoimmune diseases, humanos, Scleroderma Renal Crisis, España, Diseases::Pathological Conditions, Signs and Symptoms::Pathologic Processes::Ulcer [Medical Subject Headings], Scleroderma, Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Statistics as Topic::Probability::Risk::Risk Factors [Medical Subject Headings], Diseases::Respiratory Tract Diseases::Lung Diseases::Lung Diseases, Interstitial [Medical Subject Headings], Diseases::Skin and Connective Tissue Diseases::Connective Tissue Diseases::Scleroderma, Systemic [Medical Subject Headings], Organisms::Eukaryota::Animals::Chordata::Vertebrates::Mammals::Primates::Haplorhini::Catarrhini::Hominidae::Humans [Medical Subject Headings], Úlcera, Risk Factors, Cause of Death, Registries, Masculino, Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Data Collection::Vital Statistics::Morbidity::Prevalence [Medical Subject Headings], Diseases::Skin and Connective Tissue Diseases::Connective Tissue Diseases::Scleroderma, Systemic::Scleroderma, Limited [Medical Subject Headings], mediana edad, Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Data Collection::Vital Statistics::Mortality::Cause of Death [Medical Subject Headings], Cause of death, Esclerodermia limitada, anciano, Esclerodermia difusa, Malalties autoimmunitàries, Interstitial lung disease, Pronóstico, Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Data Collection::Questionnaires [Medical Subject Headings], General Medicine, adulto, Middle Aged, Modelos de riesgos proporcionales, Diseases::Skin and Connective Tissue Diseases::Connective Tissue Diseases::Scleroderma, Systemic::Scleroderma, Limited::CREST Syndrome [Medical Subject Headings], Humanos, Encuestas y cuestionarios, Female, Factores de riesgo, Adult, medicine.medical_specialty, Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Statistics as Topic::Analysis of Variance::Multivariate Analysis [Medical Subject Headings], Check Tags::Male [Medical Subject Headings], causas de muerte, Internal medicine, Progresión de la enfermedad, Análisis multivariante, medicine, factores de riesgo, Tasa de supervivencia, Humans, Espanya, Survival rate, Aged, Retrospective Studies, Geographical Locations::Geographic Locations::Europe::Spain [Medical Subject Headings], Diseases::Skin and Connective Tissue Diseases::Connective Tissue Diseases::Scleroderma, Systemic::Scleroderma, Diffuse [Medical Subject Headings], Scleroderma, Systemic, Diseases::Pathological Conditions, Signs and Symptoms::Pathologic Processes::Disease Attributes::Disease Progression [Medical Subject Headings], Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Data Collection::Vital Statistics::Mortality::Survival Rate [Medical Subject Headings], business.industry, estudios retrospectivos, Retrospective cohort study, medicine.disease, Pulmonary hypertension, Surgery, Causas de muerte, Scleroderma (Disease), Enfermedades pulmonares Intersticiales, Spain, Analytical, Diagnostic and Therapeutic Techniques and Equipment::Diagnosis::Prognosis [Medical Subject Headings], Esclerodèrmia, business, Síndrome CREST, Prevalencia, Hipertensión pulmonar
Abstract

Systemic sclerosis (SSc) is a rare, multisystem disease showing a large individual variability in disease progression and prognosis. In the present study, we assess survival, causes of death, and risk factors of mortality in a large series of Spanish SSc patients. Consecutive SSc patients fulfilling criteria of the classification by LeRoy were recruited in the survey. Kaplan-Meier and Cox proportional-hazards models were used to analyze survival and to identify predictors of mortality. Among 879 consecutive patients, 138 (15.7%) deaths were registered. Seventy-six out of 138 (55%) deceased patients were due to causes attributed to SSc, and pulmonary hypertension (PH) was the leading cause in 23 (16.6%) patients. Survival rates were 96%, 93%, 83%, and 73% at 5, 10, 20, and 30 years after the first symptom, respectively. Survival rates for diffuse cutaneous SSc (dcSSc) and limited cutaneous SSc were 91%, 86%, 64%, and 39%; and 97%, 95%, 85%, and 81% at 5, 10, 20, and 30 years, respectively (log-rank: 67.63, P

Published
2015

19. Ocular toxocariasis: A case report

Author

Gómez, L., Rueda, T., Pulido, C., and Sánchez-Román, J.

Subjects
anthelmintics, antihelmínticos, Toxocariasis, genetic structures, vitrectomy, vitrectomía, corticoides, mácula, eye diseases, corticosteroids, macula, sense organs, granuloma, Toxocara
Abstract

Caso clínico: Paciente de 7 años diagnosticado de toxocariasis ocular. El fondo de ojo mostraba una vitritis que no permitía ver la retina. Se instauró tratamiento con corticoides, evolucionando favorablemente, pero existía un granuloma papilar y periférico que levantaba la mácula por lo que se realizó una vitrectomía consiguiéndose la estabilización del proceso. Discusión: La Toxocariasis ocular es rara en los países desarrollados. El diagnóstico está basado en el aspecto oftalmoscópico, la serología y la Ig G + en vítreo. En cuanto al tratamiento sigue siendo controvertido el uso de antihelmínticos y aceptado el uso de corticoides y la vitrectomía. Case report: We present the case of a seven-year-old male with ocular toxocariasis. The fundus of the eye showed a vitritis, as a result of which the retina could not be seen. Following treatment with systemic corticosteroids the condition evolved favorably. However, due to a papillary and peripheral granuloma that raised the macula, a vitrectomy was performed which stabilized the process. Discussion: Ocular toxocariasis is not common in developed countries. The diagnosis is based on funduscopic aspects, serology and IgG positivity of the vitreous. In relation to treatment, as the use of anthelminthics therapy is controversial, the use of corticosteroids and vitrectomy is recommended.

Published
2008

20. Toxocariasis ocular: A propósito de un caso

Author

Sánchez-Román J, Gómez L, Rueda T, and Pulido C

Subjects
Ocular toxocariasis, medicine.medical_specialty, Pathology, antihelmínticos, genetic structures, business.industry, medicine.medical_treatment, Vitrectomy, vitrectomía, Eye infection, corticoides, medicine.disease, mácula, eye diseases, Ophthalmology, Granuloma, medicine, Toxocariasis, sense organs, business, granuloma, Toxocara
Abstract

espanolCaso clinico: Paciente de 7 anos diagnosticado de toxocariasis ocular. El fondo de ojo mostraba una vitritis que no permitia ver la retina. Se instauro tratamiento con corticoides, evolucionando favorablemente, pero existia un granuloma papilar y periferico que levantaba la macula por lo que se realizo una vitrectomia consiguiendose la estabilizacion del proceso. Discusion: La Toxocariasis ocular es rara en los paises desarrollados. El diagnostico esta basado en el aspecto oftalmoscopico, la serologia y la Ig G + en vitreo. En cuanto al tratamiento sigue siendo controvertido el uso de antihelminticos y aceptado el uso de corticoides y la vitrectomia. EnglishCase report: We present the case of a seven-year-old male with ocular toxocariasis. The fundus of the eye showed a vitritis, as a result of which the retina could not be seen. Following treatment with systemic corticosteroids the condition evolved favorably. However, due to a papillary and peripheral granuloma that raised the macula, a vitrectomy was performed which stabilized the process. Discussion: Ocular toxocariasis is not common in developed countries. The diagnosis is based on funduscopic aspects, serology and IgG positivity of the vitreous. In relation to treatment, as the use of anthelminthics therapy is controversial, the use of corticosteroids and vitrectomy is recommended.

Published
2008

21. [Iliopsoas abscess and systemic lupus erythematosus]

Author

Fj, García Hernández, Sánchez Román J, Ocaña Medina C, Mateos Romero L, Molinillo López J, and Wichmann I

Subjects
Adult, Staphylococcus aureus, Antitubercular Agents, Mycobacterium tuberculosis, Opportunistic Infections, Staphylococcal Infections, Treatment Outcome, Drainage, Humans, Lupus Erythematosus, Systemic, Psoas Abscess, Tuberculosis, Female, Tomography, X-Ray Computed
Abstract

Iliopsoas muscle abscess (IPA) is an uncommon condition, and it is usually associated with immunosuppression. Three out of a cohort of 552 patients diagnosed of systemic lupus erythematosus (SLE) developing an IPA, are reported herein. Patients showed fever and other symptoms related to SLE. They improved only partially under SLE therapy, and showed pain suggestive of IPA. It was confirmed by CT in all cases. S. aureus was isolated in one patient (primary IPA), and M. tuberculosis in the others. Specific antimicrobial therapy and surgical drainage were required. In summary, SLE might be considered as a risk condition for the development of IPA, due to the immunosuppression inherent in the disease and its treatment.

Published
2003

22. Iliopsoas abscess and systemic lupus erythematosus

Author

García Hernández, F. J., Sánchez Román, J., Ocaña Medina, C., Mateos Romero, L., Molinillo López, J., and Wichmann, I.

Subjects
Immunosupression, Systemic lupus erythematosus, Lupus eritematoso sistémico, Iliopsoas muscle abscess, Absceso del músculo iliopsoas, skin and connective tissue diseases, Imunosupresión
Abstract

El absceso del músculo iliopsoas (AIP) es una patología infrecuente, que se asocia a situaciones de inmunodeficiencia. Se presentan 3 casos de lupus eritematoso sistémico (LES), de una serie de 552 pacientes, que desarrollaron un AIP. El cuadro clínico se caracterizó por fiebre y otras manifestaciones relacionadas con su enfermedad de base, que cursaron de forma tórpida hasta que apareció dolor sugestivo de la existencia de un AIP. El agente etiológico fue S. aureus en un paciente (AIP primario), y M. tuberculosis en los otros 2 (AIP secundario). Para su tratamiento se requirió, además de antibioterapia específica, drenaje quirúrgico de la colección (fracaso previo del drenaje percutáneo en 2 casos). En la opinión de los autores, el LES, por la inmunodepresión subyacente relacionada tanto con la enfermedad en sí como con la medicación utilizada en el tratamiento, debe incluirse entre las circunstancias de riesgo para el desarrollo de AIP. Iliopsoas muscle abscess (IPA) is an uncommon condition, and it is usually associated with immunosupression. Three out of a cohort of 552 patients diagnosed of systemic lupus erythematosus (SLE) developing an IPA, are reported herein. Patients showed fever and other symptons related to SLE. They improved only partially under SLE therapy, and showed pain suggestive of IPA. It was confirmed by CT in all cases. S. aureus was isolated in one patient (primary IPA), and M. tuberculosis in the others. Specific antimicrobial therapy and surgical drainage were required. In summary, SLE might be considered as a risk condition for the development of IPA, due to the immunosupression inherent in the disease and its treatment.

Published
2003

23. Preferential Binding to Elk-1 by SLE-Associated IL10 Risk Allele Upregulates IL10 Expression

Author

Sakurai, D., Zhao, J., Deng, Y., Kelly, J. A., Brown, E. E., Harley, J. B., Bae, S. C., Alarcón-Riquelme, M. E., Edberg, J. C., Kimberly, R. P., Ramsey-Goldman, R., Caeiro, F., Soriano, E. R., Bertoli, A., Prigione, C., Ramos, F. A., Romero, E. J., Tsao, B. P., Chen, W., Truedsson, L., Yu, C. Y., Migliarese, S., García, M. A., Marcos, J. C., Eimon, A., Sánchez-Román, J., Battagliotti, C. G., Kaufman, K. M., Vyse, T. J., Jacob, C. O., Gaffney, P. M., Sebastiani, G. D., Ramón, Enrique de, Hahn, B. H., Song, Y. W., Grossman, J. M., Sivils, K. M., James, J. A., Kamen, D. L., Gilkeson, G. S., Niewold, T. B., D'Alfonso, Sandra, Merrill, J. T., Martín, J., Scofield, R. H., Chang, D. M., Criswell, L. A., Langefeld, C. D., Stevens, A. M., Cantor, R. M., Frostegård, Johan, Boackle, S. A., Kim, J. H., Choi, J., Pons-Estel, B. A., Freedman, Barry I., Sabio, José Mario, Anaya, J. M., Ortego-Centeno, N., Callejas-Rubio, J. L., González-Escribano, María Francisca, Buchanan, G., Graf, C. E., Paira, S., Galeazzi, M., Witte, Torsten, Lauwerys, B. R., Endreffy, E., Kovács, L., Vasconcelos, C., Silva, B. M. da, Scherbarth, H. R., Catoggio, L. J., Manni, J., Caprarulo, C., Guillerón, C., Marino, P. C., Motta, E. L., Gamron, S., Drenkard, C., Menso, E., Allievi, A., Roverano, S., Tate, G. A., Bertero, E., Presas, J. L., Navarro, S. M., Parque, S., Grimaudo, S., Palatnik, S. A., Abdala, M., Acevedo, E., Bearzotti, M., Santos, C. D., Alvarellos, A., Berbotto, G. A., Jorfen, M., Marcos, A. I., Perandones, C. E., Cucho, M., Torre, I. G. de la, Ríos, M. C., Moctezuma, J. F., Ceceña, M. M., Petri, M. A., Vilá, Luis M., Reveille, J. D., Alarcón, G. S., Sakurai, D., Zhao, J., Deng, Y., Kelly, J. A., Brown, E. E., Harley, J. B., Bae, S. C., Alarcón-Riquelme, M. E., Edberg, J. C., Kimberly, R. P., Ramsey-Goldman, R., Caeiro, F., Soriano, E. R., Bertoli, A., Prigione, C., Ramos, F. A., Romero, E. J., Tsao, B. P., Chen, W., Truedsson, L., Yu, C. Y., Migliarese, S., García, M. A., Marcos, J. C., Eimon, A., Sánchez-Román, J., Battagliotti, C. G., Kaufman, K. M., Vyse, T. J., Jacob, C. O., Gaffney, P. M., Sebastiani, G. D., Ramón, Enrique de, Hahn, B. H., Song, Y. W., Grossman, J. M., Sivils, K. M., James, J. A., Kamen, D. L., Gilkeson, G. S., Niewold, T. B., D'Alfonso, Sandra, Merrill, J. T., Martín, J., Scofield, R. H., Chang, D. M., Criswell, L. A., Langefeld, C. D., Stevens, A. M., Cantor, R. M., Frostegård, Johan, Boackle, S. A., Kim, J. H., Choi, J., Pons-Estel, B. A., Freedman, Barry I., Sabio, José Mario, Anaya, J. M., Ortego-Centeno, N., Callejas-Rubio, J. L., González-Escribano, María Francisca, Buchanan, G., Graf, C. E., Paira, S., Galeazzi, M., Witte, Torsten, Lauwerys, B. R., Endreffy, E., Kovács, L., Vasconcelos, C., Silva, B. M. da, Scherbarth, H. R., Catoggio, L. J., Manni, J., Caprarulo, C., Guillerón, C., Marino, P. C., Motta, E. L., Gamron, S., Drenkard, C., Menso, E., Allievi, A., Roverano, S., Tate, G. A., Bertero, E., Presas, J. L., Navarro, S. M., Parque, S., Grimaudo, S., Palatnik, S. A., Abdala, M., Acevedo, E., Bearzotti, M., Santos, C. D., Alvarellos, A., Berbotto, G. A., Jorfen, M., Marcos, A. I., Perandones, C. E., Cucho, M., Torre, I. G. de la, Ríos, M. C., Moctezuma, J. F., Ceceña, M. M., Petri, M. A., Vilá, Luis M., Reveille, J. D., and Alarcón, G. S.

Abstract

Immunoregulatory cytokine interleukin-10 (IL-10) is elevated in sera from patients with systemic lupus erythematosus (SLE) correlating with disease activity. The established association of IL10 with SLE and other autoimmune diseases led us to fine map causal variant(s) and to explore underlying mechanisms. We assessed 19 tag SNPs, covering the IL10 gene cluster including IL19, IL20 and IL24, for association with SLE in 15,533 case and control subjects from four ancestries. The previously reported IL10 variant, rs3024505 located at 1 kb downstream of IL10, exhibited the strongest association signal and was confirmed for association with SLE in European American (EA) (P = 2.7×10-8, OR = 1.30), but not in non-EA ancestries. SNP imputation conducted in EA dataset identified three additional SLE-associated SNPs tagged by rs3024505 (rs3122605, rs3024493 and rs3024495 located at 9.2 kb upstream, intron 3 and 4 of IL10, respectively), and SLE-risk alleles of these SNPs were dose-dependently associated with elevated levels of IL10 mRNA in PBMCs and circulating IL-10 protein in SLE patients and controls. Using nuclear extracts of peripheral blood cells from SLE patients for electrophoretic mobility shift assays, we identified specific binding of transcription factor Elk-1 to oligodeoxynucleotides containing the risk (G) allele of rs3122605, suggesting rs3122605 as the most likely causal variant regulating IL10 expression. Elk-1 is known to be activated by phosphorylation and nuclear localization to induce transcription. Of interest, phosphorylated Elk-1 (p-Elk-1) detected only in nuclear extracts of SLE PBMCs appeared to increase with disease activity. Co-expression levels of p-Elk-1 and IL-10 were elevated in SLE T, B cells and monocytes, associated with increased disease activity in SLE B cells, and were best downregulated by ERK inhibitor. Taken together, our data suggest that preferential binding of activated Elk-1 to the IL10 rs3122605-G allele upregulates IL10 expressio

Published
2013

24. Analysis of autosomal genes reveals gene-sex interactions and higher total genetic risk in men with systemic lupus erythematosus

Author

Hughes, T., Adler, A. J., Merrill, J. T., Kelly, J. A., Kaufman, K. M., Williams, A. H., Langefeld, C. D., Gilkeson, G. S., Sánchez, Elena, Martín, J., Boackle, S. A., Stevens, A. M., Alarcón, G. S., Niewold, T. B., Brown, E. E., Kimberly, R. P., Edberg, J. C., Ramsey-Goldman, R., Petri, M., Reveille, J. D., Criswell, L. A., Vilá, Luis M., Jacob, C. O., Gaffney, P. M., Moser, K. L., Vyse, T. J., Alarcón-Riquelme, M. E., James, J. A., Tsao, B. P., Scofield, R. H., Harley, J. B., Richardson, B. C., Sawalha, A. H., Frostegård, Johan, Truedsson, L., Ramón, Enrique de, Sabio, José Mario, González-Escribano, María Francisca, Ortego-Centeno, N., Callejas-Rubio, J. L., Sánchez-Román, J., D'Alfonso, Sandra, Migliarese, S., Sebastiani, G. D., Galeazzi, M., Witte, Torsten, Lauwerys, B. R., Endreffy, E., Kovács, L., Vasconcelos, C., Silva, B. M. da, Hughes, T., Adler, A. J., Merrill, J. T., Kelly, J. A., Kaufman, K. M., Williams, A. H., Langefeld, C. D., Gilkeson, G. S., Sánchez, Elena, Martín, J., Boackle, S. A., Stevens, A. M., Alarcón, G. S., Niewold, T. B., Brown, E. E., Kimberly, R. P., Edberg, J. C., Ramsey-Goldman, R., Petri, M., Reveille, J. D., Criswell, L. A., Vilá, Luis M., Jacob, C. O., Gaffney, P. M., Moser, K. L., Vyse, T. J., Alarcón-Riquelme, M. E., James, J. A., Tsao, B. P., Scofield, R. H., Harley, J. B., Richardson, B. C., Sawalha, A. H., Frostegård, Johan, Truedsson, L., Ramón, Enrique de, Sabio, José Mario, González-Escribano, María Francisca, Ortego-Centeno, N., Callejas-Rubio, J. L., Sánchez-Román, J., D'Alfonso, Sandra, Migliarese, S., Sebastiani, G. D., Galeazzi, M., Witte, Torsten, Lauwerys, B. R., Endreffy, E., Kovács, L., Vasconcelos, C., and Silva, B. M. da

Abstract

Objectives: Systemic lupus erythematosus (SLE) is a sexually dimorphic autoimmune disease which is more common in women, but affected men often experience a more severe disease. The genetic basis of sexual dimorphism in SLE is not clearly defined. A study was undertaken to examine sex-specific genetic effects among SLE susceptibility loci. Methods: A total of 18 autosomal genetic susceptibility loci for SLE were genotyped in a large set of patients with SLE and controls of European descent, consisting of 5932 female and 1495 male samples. Sex-specific genetic association analyses were performed. The sex-gene interaction was further validated using parametric and non-parametric methods. Aggregate differences in sex-specific genetic risk were examined by calculating a cumulative genetic risk score for SLE in each individual and comparing the average genetic risk between male and female patients. Results: A significantly higher cumulative genetic risk for SLE was observed in men than in women. (P=4.52x10 -8) A significant sex-gene interaction was seen primarily in the human leucocyte antigen (HLA) region but also in IRF5, whereby men with SLE possess a significantly higher frequency of risk alleles than women. The genetic effect observed in KIAA1542 is specific to women with SLE and does not seem to have a role in men. Conclusions: The data indicate that men require a higher cumulative genetic load than women to develop SLE. These observations suggest that sex bias in autoimmunity could be influenced by autosomal genetic susceptibility loci.

Published
2012

25. Novel association of acid phosphatase locus 1*C allele with systemic lupus erythematosus

Author

Teruel, María, Martín, J. E., Ortego-Centeno, N., Jiménez-Alonso, Juan, Sánchez-Román, J., Ramón, Enrique de, González-Escribano, María Francisca, Pons-Estel, B. A., D'Alfonso, Sandra, Sebastiani, G. D., Witte, Torsten, Bottini, N., González-Gay, M. A., Alarcón-Riquelme, M. E., Martín, J., Teruel, María, Martín, J. E., Ortego-Centeno, N., Jiménez-Alonso, Juan, Sánchez-Román, J., Ramón, Enrique de, González-Escribano, María Francisca, Pons-Estel, B. A., D'Alfonso, Sandra, Sebastiani, G. D., Witte, Torsten, Bottini, N., González-Gay, M. A., Alarcón-Riquelme, M. E., and Martín, J.

Abstract

The red cell acid phosphatase (ACP1) gene, which encodes a low-molecular-weight phosphotyrosine phosphatase, has been suggested as a common genetic factor of autoimmunity. In the present study, we aim to investigate the possible association of ACP1 with the susceptibility of systemic lupus erythematosus (SLE). A total of 1,546 SLE patients and 1,947 healthy individuals from 4 Caucasians populations were included in the present study. Four single-nucleotide polymorphisms (SNPs) were genotyped in this study: rs10167992, rs11553742, rs7576247, and rs3828329. ACP1*A, ACP1*B, and ACP1*C codominant ACP1 alleles were determined using 2 of the SNPs and analyzed. After the meta-analysis test was performed, a significant association of rs11553742*T was observed (ppooled = 0.005, odds ratios = 1.37 [1.10-1.70]), retaining significance after multiple testing was applied (pFDR = 0.019). Our data indicate for first time the association of rs11553742*T with increased susceptibility in SLE patients. © 2012 American Society for Histocompatibility and Immunogenetics.

Published
2012

26. [Soft tissue visualization with 99m Tc-HDP in a case of dermatomyositis]

Author

Jv, Ruiz Franco-Baux, Fj, García Hernández, Correa García M, Mj, Castillo Palma, Sánchez Román J, and Jr, Rodríguez Rodríguez

Subjects
Adult, Radiography, Calcinosis, Humans, Female, Radiopharmaceuticals, Technetium Tc 99m Medronate, Pelvic Bones, Radionuclide Imaging, Whole-Body Counting, Bone and Bones, Dermatomyositis
Published
1999

27. Kallikrein genes are associated with lupus and glomerular basement membrane-specific antibody-induced nephritis in mice and humans

Author

Liu, Kui, Li, Quan-Zhen, Delgado-Vega, Angelica M., Abelson, Anna-Karin, Sánchez, Elena, Kelly, Jennifer A., Li, Li, Liu, Yang, Zhou, Jinchun, Yan, Mei, Ye, Qiu, Liu, Shenxi, Xie, Chun, Zhou, Xin J., Chung, Sharon A., Pons-Estel, Bernardo, Witte, Torsten, de Ramón, Enrique, Bae, Sang-Cheol, Barizzone, Nadia, Sebastiani, Gian Domenico, Merrill, Joan T., Gregersen, Peter K., Gilkeson, Gary G., Kimberly, Robert P., Vyse, Timothy J., Kim, Il, D'Alfonso, Sandra, Martin, Javier, Harley, John B., Criswell, Lindsey A., Wakeland, Edward K., Alarcón-Riquelme, Marta E., Mohan, Chandra, Danieli, M.G., Galeazzi, M., Querini, P.R., Migliaresi, S., Scherbarth, H.R., Lopez, J.A., Motta, E.L., Gamron, S., Drenkard, C., Menso, E., Allievi, A., Tate, G.A., Presas, J.L., Palatnik, S.A., Abdala, M., Bearzotti, M., Alvarellos, A., Caeiro, F., Bertoli, A., Paira, S., Roverano, S., Graf, C.E., Bertero, E., Caprarulo, C., Buchanan, G., Guillerón, C., Grimaudo, S., Manni, J., Catoggio, L.J., Soriano, E.R., Santos, C.D., Prigione, C., Ramos, F.A., Navarro, S.M., Berbotto, G.A., Jorfen, M., Romero, E.J., Garcia, M.A., Marcos, J.C., Marcos, A.I., Perandones, C.E., Eimon, A., Battagliotti, C.G., Armadi-Simab, K., Gross, W.L., Gromica-Ihle, E., Peter, H.H., Manger, K., Schnarr, S., Zeidler, H., Schmidt, R.E., Ortego, N., Callejas, J.L., Jiménez-Alonso, J., Sabio, M., Sánchez-Román, J., Garcia-Hernandez, F.J., Camps, M., López-Nevot, M.A., González-Escribano, M.F., Harley, J.H., Riquelme, M.A., Kimberly, R., Criswell, L., Langefeld, C., Tsao, B., Jacob, C., Liu, Kui, Li, Quan-Zhen, Delgado-Vega, Angelica M., Abelson, Anna-Karin, Sánchez, Elena, Kelly, Jennifer A., Li, Li, Liu, Yang, Zhou, Jinchun, Yan, Mei, Ye, Qiu, Liu, Shenxi, Xie, Chun, Zhou, Xin J., Chung, Sharon A., Pons-Estel, Bernardo, Witte, Torsten, de Ramón, Enrique, Bae, Sang-Cheol, Barizzone, Nadia, Sebastiani, Gian Domenico, Merrill, Joan T., Gregersen, Peter K., Gilkeson, Gary G., Kimberly, Robert P., Vyse, Timothy J., Kim, Il, D'Alfonso, Sandra, Martin, Javier, Harley, John B., Criswell, Lindsey A., Wakeland, Edward K., Alarcón-Riquelme, Marta E., Mohan, Chandra, Danieli, M.G., Galeazzi, M., Querini, P.R., Migliaresi, S., Scherbarth, H.R., Lopez, J.A., Motta, E.L., Gamron, S., Drenkard, C., Menso, E., Allievi, A., Tate, G.A., Presas, J.L., Palatnik, S.A., Abdala, M., Bearzotti, M., Alvarellos, A., Caeiro, F., Bertoli, A., Paira, S., Roverano, S., Graf, C.E., Bertero, E., Caprarulo, C., Buchanan, G., Guillerón, C., Grimaudo, S., Manni, J., Catoggio, L.J., Soriano, E.R., Santos, C.D., Prigione, C., Ramos, F.A., Navarro, S.M., Berbotto, G.A., Jorfen, M., Romero, E.J., Garcia, M.A., Marcos, J.C., Marcos, A.I., Perandones, C.E., Eimon, A., Battagliotti, C.G., Armadi-Simab, K., Gross, W.L., Gromica-Ihle, E., Peter, H.H., Manger, K., Schnarr, S., Zeidler, H., Schmidt, R.E., Ortego, N., Callejas, J.L., Jiménez-Alonso, J., Sabio, M., Sánchez-Román, J., Garcia-Hernandez, F.J., Camps, M., López-Nevot, M.A., González-Escribano, M.F., Harley, J.H., Riquelme, M.A., Kimberly, R., Criswell, L., Langefeld, C., Tsao, B., and Jacob, C.

Abstract

Immune-mediated nephritis contributes to disease in systemic lupus erythematosus, Goodpasture syndrome (caused by antibodies specific for glomerular basement membrane [anti-GBM antibodies]), and spontaneous lupus nephritis. Inbred mouse strains differ in susceptibility to anti-GBM antibody-induced and spontaneous lupus nephritis. This study sought to clarify the genetic and molecular factors that maybe responsible for enhanced immune-mediated renal disease in these models. When the kidneys of 3 mouse strains sensitive to anti-GBM antibody-induced nephritis were compared with those of 2 control strains using microarray analysis, one-fifth of the underexpressed genes belonged to the kallikrein gene family,which encodes serine esterases. Mouse strains that upregulated renal and urinary kallikreins exhibited less evidence of disease. Antagonizing the kallikrein pathway augmented disease, while agonists dampened the severity of anti-GBM antibody-induced nephritis. In addition, nephritis-sensitive mouse strains had kallikrein haplotypes that were distinct from those of control strains, including several regulatory polymorphisms,some of which were associated with functional consequences. Indeed, increased susceptibility to anti-GBM antibody-induced nephritis and spontaneous lupus nephritis was achieved by breeding mice with a genetic interval harboring the kallikrein genes onto a disease-resistant background. Finally, both human SLE and spontaneous lupus nephritis were found to be associated with kallikrein genes, particularly KLK1 and the KLK3 promoter, when DNA SNPs from independent cohorts of SLE patients and controls were compared. Collectively, these studies suggest that kallikreins are protective disease-associated genes in anti-GBM antibody-induced nephritis and lupus.

Published
2009
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30. No evidence for genetic association of interferon regulatory factor 3 in systemic lupus erythematosus

Author

Sánchez, E, primary, González-Gay, MA, additional, Callejas-Rubio, JL, additional, Ortego-Centeno, N, additional, Sabio, JM, additional, Jiménez-Alonso, J, additional, Micó, L, additional, Suarez, A, additional, Gutierrez, C, additional, de Ramón, E, additional, Camps, M, additional, Garcia-Portales, R, additional, Tolosa, C, additional, López-Nevot, MA, additional, Sánchez-Román, J, additional, Hernández, FJ, additional, González-Escribano, MF, additional, and Martín, J, additional

Published
2009
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32. Study of a functional polymorphism in thep53 gene in systemic lupus erythematosus: lack of replication in a Spanish population

Author

Sánchez, E, primary, Sabio, J M, additional, Callejas, J L, additional, de Ramón, E, additional, de Haro, M, additional, Jiménez-Alonso, J, additional, Ortego-Centeno, N, additional, Sánchez-Román, J, additional, González-Gay, M A, additional, López-Nevot, M A, additional, González-Escribano, M F, additional, and Martín, J, additional

Published
2006
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34. Reply to Bogdanos et al.

Author

Wichmann, I., primary, Montes‐Cano, M.A, additional, Respaldiza, N., additional, Alvarez, A., additional, Franco, E., additional, Sánchez‐Román, J., additional, and Nuñez‐Roldán, A., additional

Published
2004
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38. Study of a functional polymorphism in the p53 gene in systemic lupus erythematosus: lack of replication in a Spanish population.

Author

Sánchez, E., Sabio, J. M., Callejas, J. L., de Ramón, E., de Haro, M., Jiménez-Alonso, J., Ortego-Centeno, N., Sánchez-Román, J., González-Gay, M. A., López-Nevot, M. A., González-Escribano, M. F., and Martín, Javier

Subjects
P53 antioncogene, SYSTEMIC lupus erythematosus, AUTOIMMUNE diseases, GENETIC polymorphisms, DISEASE susceptibility, SENSITIVITY (Personality trait)
Abstract

The aim of this study was to assess the possible association between the p53 suppressor gene codon 72 polymorphism and systemic lupus erythematosus (SLE). Our study population consisted of 513 SLE patients and 567 healthy controls. All the individuals were of Spanish Caucasian origin. Genotyping of the p53 codon 72 polymorphism was performed by allele-specific PCR. No statistically significant differences were observed between SLE patients and healthy controls when p53 codon 72 genotype and allele frequencies were compared. In addition, no evidence for association with clinical subfeatures of SLE was found. In conclusion, the p53 codon 72 polymorphism associated with SLE in a Korean population does not appear to play a major role in the susceptibility or severity of SLE in the Spanish population. [ABSTRACT FROM AUTHOR]

Published
2006
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41. Confirmation of CCR6 as a risk factor for anti-topoisomerase I antibodies in systemic sclerosis

Author

Ochoa, E., Martin, J. -E, Assassi, S., Beretta, L., Carreira, P., Guillén, A., Simeón, C. P., Koumakis, E., Dieude, P., Allanore, Y., García-Hernández, F. J., Gerard Espinosa, Castellví, I., Trapiella, J. L., Rodriguez, L., González-Gay, M., Egurbide, M. V., Sáez, L., Callejas-Rubio, J. L., Vargas-Hitos, J. A., Hunzelmann, N., Riemekasten, G., Witte, T., Distler, J. H. W., Kreuter, A., Lunardi, C., Santaniello, A., Tan, F. K., Shiels, P. G., Herrick, A., Worthington, J., Vonk, M. C., Koeleman, B. P., Radstake, T. R. D. J., Mayes, M. D., Martin, J., Ortego-Centeno, N., Ríos, R., Granada, C., Camps, M. T., Fernández-Nebro, A., Sánchez-Román, J., Castillo, M. J., Aguirre, M., Gómez-Gracia, I., Fernández-Gutiérrez, B., Vicente, E., Andreu, J. L., Castro, M. F., La Peña, P. G., López-Longo, F. J., Martínez, L., Fonollosa, V., Tolosa, C., Pros, A., Carballeira, M. R., Narváez, F. J., Rivas, M. R., Santamaría, V. O., Madroñero, A. B., Díaz, B., Freire, M., Sousa, A., Mateo, P. F., Díaz, F., Hernández, V., Beltrán, E., Grau, E., Román-Ivorra, J. A., Alegre Sancho, J. J., Blanco García, F. J., Oreiro, N., and Spanish Scleroderma Group

42. LILRA3 deficiency is not involved in the giant cell arteritis and systemic sclerosis predisposition

Author

Márquez, A., Fernández-Aranguren, T., Witte, T., González-Gay, M. A., Martín, J., Carreira, P. E., Espinosa, G., Castellví, I., Egurbide, M. V., Vicente, E., Pros, A., Centeno, N. O., Ríos, R., Callejas, J. L., Hitos, J. A. V., Portales, R. G., Camps, M. T., Fernández-Nebro, A., González-Escribano, M. F., Sánchez-Román, J., García-Hernández, F. J., Castillo, M. J., MA Aguirre, Gómez-Gracia, I., Fernández-Gutiérrez, B., Andreu, J. L., Castro, M. F., La Peña, P. G., López-Longo, F. J., Martínez, L., Simeón, C. P., Fonollosa, V., Guillén, A., Tolosa, C., Carballeira, M. R., Rivas, M. R., Santamaría, V. O., Madroñero, A. B., Díaz, B., Trapiella, L., Freire, M., Sousa, A., Mateo, P. F., Díaz, F., Hernández, V., Beltrán, E., Grau, E., Román-Ivorra, J. A., Alegre Sancho, J. J., Blanco García, F. J., Oreiro, N., and Sáez-Comet, L.

43. Investigation of TLR5 and TLR7 as candidate genes for susceptibility to systemic lupus erythematosus

Author

Elena Sanchez, Jl, Callejas-Rubio, Jm, Sabio, Ma, Gónzalez-Gay, Jimenez-Alonso J, Micó L, de Ramón E, Camps M, Suarez A, Gutierrez C, Garcia-Portales R, Tolosa C, Ortego-Centeno N, Sánchez-Román J, Fj, Garcia-Hernández, Mf, Gónzalez-Escribano, and Martin J

Subjects
Toll-Like Receptor 5, Toll-Like Receptor 7, Case-Control Studies, Odds Ratio, Humans, Lupus Erythematosus, Systemic, Genetic Predisposition to Disease, Polymorphism, Single Nucleotide, White People
Abstract

The aim of this study was to evaluate the relevance of genetic variants of TLR5 (rs5744168) and TLR7 (rs179008) gene in systemic lupus erythematosus (SLE) in a Spanish population.Our study population consisted of 752 SLE patients and 1107 healthy controls. All individual were of Spanish Caucasian origin. The TLR5 and TLR7 polymorphisms were genotyped using a PCR system with pre-developed TaqMan allelic discrimination assay.No statistically significant differences were observed when the allele and genotype distribution of TLR5 rs5744168 and TLR7 rs179008 polymorphisms was compared between SLE patients and healthy controls. A significant increase frequency in the CC genotype of the TLR5 rs5744168 polymorphism among SLE patients without nephritis was found (93% vs. 87% in SLE patients with nephritis, p=0.03, OR=2.11 95%CI 0.93-3.51). However, this difference did not reach statistical significance in the allele frequencies (p=0.08).These results suggest that the tested variations of TLR5 and TLR7 genes do not confer a relevant role in the susceptibility or severity to SLE in the Spanish population.

48. Use of noninvasive measurement of the indocyanine green plasma disappearance rate in patients with septic shock.

Author

Gutiérrez-Morales I, Loza-Vázquez A, Sánchez-Román JA, Grilo-Reina A, and Navarro-Puerto MA

Subjects
Humans, Male, Middle Aged, Female, Prospective Studies, Aged, Coloring Agents, APACHE, Prognosis, Shock, Septic mortality, Shock, Septic blood, Indocyanine Green pharmacokinetics, Hospital Mortality, Intensive Care Units
Abstract

Objective: Our aim was to analyse the relation between serial values of the indocyanine green plasma disappearance rate (ICG-PDR) with hospital mortality in the first 48 hours of ICU admission in patients with septic shock., Methods: A prospective observational study was carried out over 12 months of patients admitted to the ICU with septic shock. Each patient underwent noninvasive determination of ICG-PDR at 24 and 48 hours with the LiMON® module. Follow-up was performed until hospital discharge or exitus., Results: 63 patients. Age 61.1±12.3 years. 60.3% men. SOFA score on admission 8.7±3.3, APACHE II score was 27.9±10.7 points. A total of 44.4% of patients died. The ICG-PDR values in the first 24 hours of ICU admission were lower in nonsurvivors: 10.5 (5.7-13.0)%/min vs. 15.9 (11.4-28.0)%/min, p <0.001. Furthermore, in nonsurvivors, there was no improvement in ICG-PDR between 24 h and 48 h, while in survivors, there was an increase of 25%: 15.9 (11.4-28.0)%/min and 20.9 (18.0-27.0)%/min, p=0.020. The silhouette measure of ICG-PDR cohesion and separation for the clusters analysed (nonsurvivors and survivors) was satisfactory (0.6). ICG-PDR<11.7%/min was related to in-hospital mortality, ICG-PDR> 18%/min to survival, and the interval between 11.7% and 18%/min covered a range of uncertainty. In the two-stage cluster, ICG-PDR, SOFA and APACHE II present satisfactory predictive scores 24 hours after patient admission., Conclusions: ICG-PDR in our setting is a useful clinical prognostic tool and could optimise the decision tree in patients with septic shock., (©The Author 2024. Published by Sociedad Española de Quimioterapia. This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 International (CC BY-NC 4.0)(https://creativecommons.org/licenses/by-nc/4.0/).)

Published
2024
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49. Routine invasive strategy in acute coronary syndrome patients with renal dysfunction. Results of the ARIAM-SEMICYUC registry.

Author

Latour-Pérez J, Gómez-Tello V, de-Miguel-Balsa E, Llamas-Álvarez A, Carrillo-López A, Sánchez-Román JA, Ruíz-Ruíz J, Martín-Rodríguez MC, Fernández-González CJ, Fernández-Lozano JA, and Picazos Vicente C

Subjects
Acute Coronary Syndrome complications, Acute Coronary Syndrome diagnostic imaging, Aged, Aged, 80 and over, Female, Hospital Mortality, Humans, Intensive Care Units statistics & numerical data, Male, Middle Aged, Non-ST Elevated Myocardial Infarction complications, Non-ST Elevated Myocardial Infarction diagnostic imaging, Propensity Score, Recurrence, Registries, Retrospective Studies, Risk, Severity of Illness Index, Spain epidemiology, Treatment Outcome, Acute Coronary Syndrome therapy, Coronary Angiography, Kidney Diseases complications, Myocardial Revascularization, Non-ST Elevated Myocardial Infarction therapy
Abstract

Objective: To evaluate the use and effectiveness of a routine invasive strategy (RIS) in patients with acute coronary syndrome without persistent ST-segment elevation with renal dysfunction in the real world scenario., Methods: A retrospective cohort study based on the ARIAM-SEMICYUC Registry (2011-2014) was carried out. Renal dysfunction was defined as GFR (Cockroft-Gault)<60ml/min (moderate dysfunction) or<30ml/min (severe dysfunction). Patients in which early angiography (<72h) was performed due to cardiogenic shock or recurrent myocardial ischemia were excluded. The primary endpoint was hospital mortality. Confounding factors were controlled using propensity score analysis., Results: A total of 4,279 patients were analyzed, of which 26% had moderate renal dysfunction and 5% severe dysfunction. Patients with renal dysfunction had greater severity and comorbidity, higher hospital mortality (8.6 vs. 1.8%), and lesser use of the RIS (40 vs. 52%). The adjusted OR for mortality in patients without/with renal dysfunction were 0.38 (95% confidence interval [95%CI] 0.17 to 0.81) and 0.52 (95%CI 0.32 to 0.87), respectively (interaction P-value=.4779). The impact (adjusted risk difference) of RIS was higher in the group with renal dysfunction (-5.1%, 95%CI -8.1 to -2.1 vs. -1.6%, 95%CI -2.6 to -0.6; interaction P-value=.0335). No significant interaction was detected for the other endpoints considered (ICU mortality, 30-day mortality, myocardial infarction, acute renal failure or moderate/severe bleeding)., Conclusions: The results suggest that the effectiveness of IRS is similar in patients with normal or abnormal renal function, and alert to the under-utilization of this strategy in such patients., (Copyright © 2015 Elsevier España, S.L.U. y SEMICYUC. All rights reserved.)

Published
2016
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50. [Screening of pulmonary hypertension in a Spanish cohort of patients with systemic sclerosis].

Author

García Hernández FJ, Castillo Palma MJ, Montero Mateos E, González León R, López Haldón JE, and Sánchez Román J

Subjects
Adult, Aged, Female, Humans, Hypertension, Pulmonary epidemiology, Hypertension, Pulmonary etiology, Male, Middle Aged, Prevalence, Spain, Echocardiography, Doppler, Hypertension, Pulmonary diagnostic imaging, Mass Screening, Scleroderma, Systemic complications
Abstract

Background and Objective: Pulmonary arterial hypertension (PAH) is an important cause of morbimortality in systemic sclerosis (SSc). Evolution is worse than that of subjects with idiopathic PAH, but prognosis improves when PAH is diagnosed early. The aim of this research is to describe results of a screening program for diagnosis of pulmonary hypertension (PH) carried out in a cohort of Spanish patients with SSc., Patients and Method: PH screening was performed by transthoracic doppler echocardiography (TTDE) in 184 patients with SSc. Patients with systolic pulmonary arterial pressure estimated by TTDE>35 mmHg were evaluated per protocol to confirm diagnosis and type of PH., Results: PAH was diagnosed in 25 patients (13.6%). Patients with diffuse and limited SSc developed PAH in a similar degree, 9/60 (15%) vs. 16/100 (16%), with no cases among patients with SSc "sine scleroderma" or "pre-scleroderma" (P<.001). The only clinical or epidemiological data characterizing patients with PAH were older age (mean age 67 years for patients with PAH vs. 56 years for those without PAH, P=.007), limited SSc, a trend toward shorter evolution of the underlying disease (median 8 years for patients with PAH vs. 10 years for those without PAH, P=.73), and a higher frequency of positive anticentromere antibodies (16 patients [64%] with PAH vs. 70 (48,3%) without PAH, P=.19)., Conclusions: Prevalence of PAH in SSc was high and supports the implementation of a regular screening program., (Copyright © 2015 Elsevier España, S.L.U. All rights reserved.)

Published
2016
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