Comparative study between two European inception cohorts of patients with early systemic lupus erythematosus.

OBJECTIVES: To compare the main characteristics of two inception cohorts (Italian [ITC] and Spanish [SPC]) cohorts of patients with systemic lupus erythematosus (SLE) at the time of diagnosis and at one year of follow-up. METHODS: Demographic, clinical and immunological characteristics, and treatments at SLE diagnosis and at 12 months of follow-up of ITC and SPC were compared. RESULTS: One hundred and sixty-four patients in the ITC and 231 patients in the SPC were compared. the patients from ITC were younger at SLE diagnosis (41.1±15.0 years vs. 46.4±15.6 years; p<0.001) and had a higher prevalence of arthritis (62.8% vs. 45.5%; p=0.001), serositis (25.6% vs. 16.0%; p=0.026), neurological involvement (7.9% vs. 1.7%; p=0.006), and immunological abnormalities (anti-dsDNA, anti-Sm, antiphospholipid antibodies) (93.9% vs. 77.8%; p<0.001). Conversely, photosensitivity (29.5% in ITC vs. 45.9% in SPC; p=0.001) and oral ulcers (12.4% vs. 30.3%; p<0.001) were more frequent at onset of SLE in the Spanish patients. At the first 12 months of follow-up, these differences were maintained. At SLE onset, more Italian patients received glucocorticoids (85.4% vs. 50.2%; p<0.001) and immunosuppressive agents. At 12 months of follow-up, more Spanish patients were treated with antimalarials (75.6% in ITC vs. 90.0% in SPC; p<0.001). Conversely, the use of glucocorticoids was lower in SPC (89.0% in ITC vs. 57.1% in SPC; p<0.001). CONCLUSIONS: These cohorts presented different profiles in terms of pattern of organ/system involvement and disease treatment, possibly as a consequence of patient selection or different disease management approaches between Italy and Spain.

PTPN22 is not associated with Behçet's disease. Study spanning the complete gene region in the Spanish population and meta-analysis of the functional variant R620W.

OBJECTIVES: The functional variant R620W of the protein tyrosine phosphatase non receptor-22 (PTPN22) gene plays an important role in susceptibility to several immuno-mediated pathologies. Behçet's disease (BD) is a complex disease related to the immune system with a demonstrated genetic base. The HLA class I genes are the most important genetic factors in BD although other genes are also involved in the susceptibility to this disease. The PTPN22 has been proposed as a candidate gene in BD but this association has not been clearly demonstrated yet. The aim of this study was to assess the association of PTPN22 with BD. METHODS: A cohort composed of 404 Spanish BD patients and 1517 unrelated healthy individuals ethnically matched was genotyped in rs2476601 (R620W). Five tag SNPs: rs1217412, rs2476599, rs3789607, rs3765598 and rs1217419 (spanning a 57 Kb region between 3'UTR and 5'UTR) and rs2488457 (located at the promoter region) were also studied in order to perform a screening of the complete gene. Genotyping was performed using TaqMan® assays. The rs2476601 data were included in a meta-analysis together with those published till the date. The rest of SNPs were used in a case-control study. RESULTS: No evidence of the association of rs2476601 with BD in the meta-analysis (P = 0.504 in the model of alleles) was found. In the case-control study, no statistically significant differences were observed when comparing the distribution of variants in patients and controls. CONCLUSIONS: Our results do not support a major role of the PTPN22 gene in BD.

Patterns of drug therapy in newly diagnosed Spanish patients with systemic lupus erythematosus.

OBJECTIVES: This is the first Spanish multicentric inception lupus cohort, formed by SLE patients attending Spanish Internal Medicine Services since January 2009. We aimed to analyse drug therapy during the first year of follow-up according to disease severity. METHODS: 223 patients who had at least one year of follow-up were enrolled upon diagnosis of SLE. Therapy with prednisone, pulse methyl-prednisolone, hydroxychloroquine, immunosuppressives and calcium/vitamin D was analysed. RESULTS: Prednisone was given to 65% patients, at a mean (SD) daily dose of 11 (10) mg/d. 38% patients received average doses >7.5 mg/d during the first year. Patients with nephritis and with a SLEDAI ≥6 were treated with higher doses of prednisone. 81% of patients were treated with hydroxychloroquine, with higher frequency among those with a SLEDAI ≥6 (88% vs. 68%, p<0.001). The use of immunosuppressive drugs and methyl-prednisolone pulses was higher in patients with a baseline SLEDAI ≥6, however, differences were no longer significant when patients with lupus nephritis were excluded. The use of calcium/vitamin D increased with the dose of prednisone, however, 43% of patients on medium-high doses of prednisone did not take any calcium or vitamin D. CONCLUSIONS: This study gives a real-world view of the current therapeutic approach to early lupus in Spain. The generalised use of hydroxychloroquine is well consolidated. There is still a tendency to use prednisone at medium to high doses. Pulse methyl-prednisolone and immunosuppressive drugs were used in more severe cases, but not as steroid sparing agents. Vitamin D use was suboptimal.

Cribado de hipertensión pulmonar en una cohorte española de pacientes con esclerosis sistémica / Screening of pulmonary hypertension in a Spanish cohort of patients with systemic sclerosis

Fundamento y objetivo: La hipertensión arterial pulmonar (HAP) es causa importante de morbimortalidad en la esclerosis sistémica (ES). Su evolución es peor que en la HAP idiopática, pero el pronóstico mejora si se diagnostica precozmente. El objetivo de este trabajo es describir el resultado de un programa de cribado para el diagnóstico de hipertensión pulmonar (HP) desarrollado en una cohorte de pacientes españoles con ES. Pacientes y método: Se realizó cribado de HP mediante ecocardiografía-doppler transtorácica (EDTT) en 184 pacientes con ES. Los pacientes con valor de presión arterial pulmonar sistólica estimada por EDTT > 35 mmHg se evaluaron de forma protocolizada para establecer o no el diagnóstico de certeza de HP y su tipo. Resultados: Se diagnosticó HAP en 25 pacientes (13,6%). Los pacientes con ES difusa y limitada desarrollaron HAP en proporciones semejantes: 9 de 60 (15%) frente a 16 de 100 (16%). No se registraron casos entre pacientes con ES «sine esclerodermia» o «preesclerodermia» (p < 0,001). Los únicos datos clinicoepidemiológicos que caracterizaron a los pacientes con HAP fueron una edad más avanzada (edad media de 67 años para pacientes con HAP frente a 56 años sin HAP, p = 0,007), especialmente relacionada con la ES limitada, y una tendencia hacia un menor tiempo de evolución de la enfermedad de base (mediana de 8 años para pacientes con HAP frente a 10 años sin HAP, p = 0,73) y una mayor frecuencia de positividad para anticuerpos anticentrómero: 16 (64%) pacientes con HAP frente a 70 (48,3%) sin HAP (p = 0,19). Conclusiones: La prevalencia de HAP en ES resultó elevada y apoya la implantación de programas de cribado sistemático (AU)

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 (AU)

[Screening of pulmonary hypertension in a Spanish cohort of patients with systemic sclerosis]. / Cribado de hipertensión pulmonar en una cohorte española de pacientes con esclerosis sistémica.

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.

Epistatic interaction of ERAP1 and HLA-B in Behçet disease: a replication study in the Spanish population.

Behçet's disease (BD) is a multifactorial disorder associated with the HLA region. Recently, the ERAP1 gene has been proposed as a susceptibility locus with a recessive model and with epistatic interaction with HLA-B51. ERAP1 trims peptides in the endoplasmic reticulum to optimize their length for MHC-I binding. Polymorphisms in this gene have been related with the susceptibility to other immune-mediated diseases associated to HLA class I. Our aim was, the replication in the Spanish population of the association described in the Turkish population between ERAP1 (rs17482078) and BD. Additionally, in order to improve the understanding of this association we analyzed four additional SNPs (rs27044, rs10050860, rs30187 and rs2287987) associated with other diseases related to HLA class I and the haplotype blocks in this gene region. According to our results, frequencies of the homozygous genotypes for the minor alleles of all the SNPs were increased among patients and the OR values were higher in the subgroup of patients with the HLA-B risk factors, although differences were not statistically significant. Moreover, the presence of the same mutation in both chromosomes increased the OR values from 4.51 to 10.72 in individuals carrying the HLA-B risk factors. Therefore, although they were not statistically significant, our data were consistent with an association between ERAP1 and BD as well as with an epistatic interaction between ERAP1 and HLA-B in the Spanish population.

IL2/IL21 region polymorphism influences response to rituximab in systemic lupus erythematosus patients.

To determine whether the IL2/IL21 region, a general autoimmunity locus, contributes to the observed variation in response to rituximab in patients with systemic lupus erythematosus as well as to analyze its influence in a cohort including other autoimmune diseases. rs6822844 G/T polymorphism at the IL2-IL21 region was analyzed by TaqMan assay in 84 systemic lupus erythematosus (SLE) and 60 different systemic autoimmune diseases Spanish patients receiving rituximab. Six months after the first infusion patients were classified, according to the EULAR criteria, as good responders, partial responders and non-responders. A statistically significant difference was observed in GG genotype frequency between responder (total and partial response) (83.56%) and non-responder (45.45%) SLE patients (p=0.010, odds ratio (OR)=6.10 [1.28-29.06]). No association with the response was evident in the group of patients with autoimmune diseases other than lupus. Furthermore, when both groups of patients were pooled in a meta-analysis, a reduced statistical significance of the association was observed (p=0.024, OR=3.53 [1.06-11.64]). Our results show for a first time that IL2-IL21 region seems to play a role in the response to rituximab in SLE patients but not in other autoimmune diseases.

Anticuerpos antiprotrombina positivos en una paciente con síndrome antifosfolípido secundario y clínica hemorrágica / Antiprothrombin antibodies in a patient with secondary antiphospholipid syndrome and bleeding

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Marcadores biológicos. Utilidad para el control del paciente con hipertensión pulmonar / Biological markers. Utility in the management of patients with pulmonary hypertension

Se entiende como marcador biológico cualquier característica que puede ser objetivamente medida y evaluada como indicadora de un proceso biológico normal, un proceso patogénico o una respuesta farmacológica a una intervención terapéutica. En el terreno de la hipertensión arterial pulmonar (HAP), además de los marcadores habituales (hemodinámicos y funcionales), se cuenta con un número creciente de biomarcadores que permiten un acercamiento cada vez más completo al conocimiento de la susceptibilidad y al establecimiento del diagnóstico, pronóstico y respuesta al tratamiento. Estos marcadores pueden ser tanto constitutivos (genéticos) como reactivos a la enfermedad (relacionados con el fallo ventricular derecho, como BMP/NT-proBNP, con la disfunción endotelial, como la endotelina-1, o con la inflamación, como determinadas citocinas y quimiocinas). Los nuevos descubrimientos en genómica y proteómica permiten augurar avances fundamentales en este campo (AU)

A biological marker can be defined as any substance that can be objectively measured and evaluated as an indicator of a normal biological process, a pathogenic process or pharmacological responses to a therapeutic intervention. In pulmonary hypertension (PH), in addition to routine markers (hemodynamic and functional), there are a growing number of biomarkers that allow an increasingly comprehensive approach to knowledge of susceptibility to this disease and to diagnosis, prognosis and treatment response. These markers can be both constitutive (genetic) and disease-related (related to right ventricular failure, such as BMP/NT-proBNP, endothelial dysfunction, such as endothelin-1, or inflammation, such as certain cytokines and chemokines). Novel insights in genomics and proteomics may allow major advances in this field (AU)

[Biological markers. Utility in the management of patients with pulmonary hypertension]. / Marcadores biológicos. Utilidad para el control del paciente con hipertensión pulmonar.

A biological marker can be defined as any substance that can be objectively measured and evaluated as an indicator of a normal biological process, a pathogenic process or pharmacological responses to a therapeutic intervention. In pulmonary hypertension (PH), in addition to routine markers (hemodynamic and functional), there are a growing number of biomarkers that allow an increasingly comprehensive approach to knowledge of susceptibility to this disease and to diagnosis, prognosis and treatment response. These markers can be both constitutive (genetic) and disease-related (related to right ventricular failure, such as BMP/NT-proBNP, endothelial dysfunction, such as endothelin-1, or inflammation, such as certain cytokines and chemokines). Novel insights in genomics and proteomics may allow major advances in this field.

Tuberculosis en una cohorte de pacientes con lupus eritematoso sistémico / Tuberculosis in a cohort of patients with systemic lupus erythematosus

Objetivos. Analizar los casos de tuberculosis (TB) en una cohorte de pacientes con lupus eritematoso sistémico (LES) y comparar la frecuencia y características de la TB en nuestra serie con las de otras series publicadas; identificar características diferenciales entre los pacientes que presentaron TB y los que no la presentaron, y evaluar si las formas más graves se relacionaron con dosis más altas de glucocorticoides (GC) u otros inmunosupresores. Material y método. Análisis descriptivo de 13 pacientes con TB de una serie de 789 pacientes con LES. Revisión de las historias clínicas de los casos. Búsqueda bibliográfica en MEDLINE-PubMed de las series LES/TB publicadas, utilizando los términos «infection», «tuberculosis», «lupus erythematosus». Estudio comparativo de casos (LES/TB+) y controles (LES/TB–) en cuanto a las características clínicas, de laboratorio y el tratamiento realizado, mediante test X2 y test exacto de Fisher. Resultados. Trece pacientes estuvieron afectados por TB (10 mujeres, con edad media de 36 años; DE de 11,2, y prevalencia del 1,6%). Se diagnosticaron 9 primoinfecciones (69,2%) y 4 reactivaciones (30,8%). El diagnóstico se confirmó mediante aislamiento microbiológico (baciloscopia y/o cultivo) en 11 casos (84,6%). La afectación pulmonar fue la más frecuente (69,2%). Ocho pacientes (61,5%) presentaron formas extrapulmonares, de las que 6 (46%) fueron diseminadas. En el momento del diagnóstico, 9 pacientes (69,2%) recibían tratamiento con GC. Fallecieron 4 pacientes (30,8%). La afectación muscular fue más frecuente en el grupo casos (p < 0,05). Conclusiones. La TB en nuestra serie supuso una alta mortalidad (30,8%) en los enfermos con LES. Las formas extrapulmonares representaron el doble con respecto a la observada en la población general. Los pacientes que recibieron dosis mayores de GC fueron los que presentaron formas más graves de TB. Los datos son similares a los publicados en la mayoría de las series nacionales y extranjeras (AU)

Objectives. 1) To study tuberculosis (TB) infection in a cohort of patients with systemic lupus erythematosus (SLE) and to compare its frequency and characteristics with that of others series. 2) To look for differential characteristic among SLE patients with and without TB. 3) To investigate if there was any relationship between TB's most severe forms and higher doses of glucocorticoids (GC) or other immunosuppressants. Patients and Method Retrospective review of medical records of 789 SLE patients and description of the clinical characteristics of 13 cases of active TB infection among them. Bibliographical search in MEDLINE-PubMed of the SLE/TB series published, using the terms: infection, tuberculosis, systemic lupus erythematosus. Comparative study of clinical, biological and therapeutic differences between cases (SLE/TB+) and controls (SLE/TB) using X2 and Fisher exact test. Results. Thirteen patients with active tuberculosis were detected (10 women, average age 36 years/SD 11,2/prevalence 1,6%). Nine (69,2%) of them were primary infections and 4 (30,8%) reactivations. Microbiological diagnosis (smear examination for acid-fast bacilli and/or culture on Lowestein-Jensen medium) was established in 11 patients (84,6%). TB Pulmonary manifestations was present in 9 patients (69,2%) and extra-pulmonary manifestations were found in 8 [(61,5%); 6 of them (46%) were disseminated forms]. Nine (69,2%) patients were on GC therapy at the moment TB was diagnosed. Four of the TB patients died (30,8%). Myositis was more frequent in TB cases (p < 0,05). This data is similar to that reported in the literature. Conclusions. In our series, TB mortality was high (30,8%) in a patients with SLE. Frequency of extrapulmonary forms was double than that described in the Spanish population. Patients with higher GC dose had more severe forms of TB (AU)

Infecciones graves en una cohorte de pacientes con lupus eritematoso sistémico / Severe infections in a cohort of patients with systemic lupus erythematosus

Fundamento y objetivos: Analizar los episodios infecciosos graves en una cohorte de pacientes con lupus eritematoso sistémico. Pacientes y método: Análisis retrospectivo de 705 pacientes seguidos desde enero de 1980 hasta enero de 2008. Los datos se expresan en valores absolutos y porcentajes. Resultados: La frecuencia de complicaciones infecciosas fue del 38,6%. La etiología fue bacteriana en un 54,4%, vírica en un 30,4% y oportunista en un 5,2%. La afectación visceral lúpica fue pulmonar en un 38,2%, renal en un 48,9% y del sistema nervioso central en un 43%. El 43,75% de los pacientes recibió bolos de ciclofosfamida y el 88,6% glucocorticoides (el 39,7% en bolos). La mortalidad por infección fue del 27,7%. Conclusiones: La infección continúa siendo una causa importante de mortalidad en el lupus eritematoso sistémico, por lo que su diagnóstico precoz es de gran importancia (AU)

Background and objective: To study severe infectious complications in a cohort of patients with systemic lupus erythematosus (SLE). Patients and method: Retrospective study of 705 patients followed from January 1980 to January 2008. Data are expressed in percentages. Results: The frequency of severe infectious was 38,6%. The etiology was bacterial 54,4%, viric 30,4% and opportunist in 15,2% patients. Involved organs were: Lung 38,2%, kidney 48,9%, central nervous system 43%. 43,75% patients received pulsed ciclofosfamide therapy and 88,6% received glucocorticoids (39,7% pulsed). The mortality was 27,7%.Conclusions: At present, infection is an important cause of mortality in patients with SLE. Early diagnosis of infectious complications is very important in SLE (AU)

[Severe infections in a cohort of patients with systemic lupus erythematosus]. / Infecciones graves en una cohorte de pacientes con lupus eritematoso sistémico.

BACKGROUND AND OBJECTIVE: To study severe infectious complications in a cohort of patients with systemic lupus erythematosus (SLE). PATIENTS AND METHOD: Retrospective study of 705 patients followed from January 1980 to January 2008. Data are expressed in percentages. RESULTS: The frequency of severe infectious was 38.6%. The etiology was bacterial 54.4%, viric 30.4% and opportunist in 15.2% patients. Involved organs were: Lung 38.2%, kidney 48.9%, central nervous system 43%. 43.75% patients received pulsed ciclofosfamide therapy and 88.6% received glucocorticoids (39.7% pulsed). The mortality was 27.7%. CONCLUSIONS: At present, infection is an important cause of mortality in patients with SLE. Early diagnosis of infectious complications is very important in SLE.

[Tuberculosis in a cohort of patients with systemic lupus erythematosus]. / Tuberculosis en una cohorte de pacientes con lupus eritematoso sistémico.

OBJECTIVES: 1) To study tuberculosis (TB) infection in a cohort of patients with systemic lupus erythematosus (SLE) and to compare its frequency and characteristics with that of others series. 2) To look for differential characteristic among SLE patients with and without TB. 3) To investigate if there was any relationship between TB's most severe forms and higher doses of glucocorticoids (GC) or other immunosuppressants. PATIENTS AND METHOD: Retrospective review of medical records of 789 SLE patients and description of the clinical characteristics of 13 cases of active TB infection among them. Bibliographical search in MEDLINE-PubMed of the SLE/TB series published, using the terms: infection, tuberculosis, systemic lupus erythematosus. Comparative study of clinical, biological and therapeutic differences between cases (SLE/TB+) and controls (SLE/TB) using χ(2) and Fisher exact test. RESULTS: Thirteen patients with active tuberculosis were detected (10 women, average age 36 years/SD 11,2/prevalence 1,6%). Nine (69,2%) of them were primary infections and 4 (30,8%) reactivations. Microbiological diagnosis (smear examination for acid-fast bacilli and/or culture on Lowestein-Jensen medium) was established in 11 patients (84,6%). TB Pulmonary manifestations was present in 9 patients (69,2%) and extra-pulmonary manifestations were found in 8 [(61,5%); 6 of them (46%) were disseminated forms]. Nine (69,2%) patients were on GC therapy at the moment TB was diagnosed. Four of the TB patients died (30,8%). Myositis was more frequent in TB cases (p < 0,05). This data is similar to that reported in the literature. CONCLUSIONS: In our series, TB mortality was high (30,8%) in a patients with SLE. Frequency of extrapulmonary forms was double than that described in the Spanish population. Patients with higher GC dose had more severe forms of TB.

Disfunción tiroidea en pacientes con hipertensión arterial pulmonar. Estudio de una cohorte de 58 pacientes / Thyroid dysfunction in patients with pulmonary arterial hipertensión. A cohort study of 58 patients

Fundamento y objetivo: La patología tiroidea (PT) es más frecuente en pacientes con hipertensión arterial pulmonar (HAP) que en la población general. No están bien establecidas la frecuencia ni la causa de esta asociación. El objetivo de este trabajo es cuantificar y analizar la incidencia y las características de la PT en una cohorte de pacientes con HAP (idiopática o asociada preferentemente a enfermedades sistémicas) y realizar una revisión de la literatura científica. Pacientes y método: Se estudió a 58 pacientes con HAP prospectivamente según un protocolo preestablecido (incluido cateterismo cardíaco derecho) y se determinaron la tirotropina (TSH), la tiroxina libre (T4l) y los anticuerpos antitiroglobulínicos y anticuerpos antiperoxidasa. Se definió la PT como alteración de la TSH o elevación de cualquiera de los 2 anticuerpos antitiroideos (AcAT). Se compararon las variables clínicas, biológicas y hemodinámicas entre los grupos con y sin PT. Resultados: Había PT en 30 pacientes (51%): TSH elevada en 21 (36,21%), hipertiroidismo en 2 pacientes (3,45%) y AcAT elevados en 16 de los 54 pacientes (27,59%), 7 de los cuales eran eutiroideos. En el grupo con PT el tiempo de evolución de la HAP fue mayor (4,62 frente a 2,61 años; intervalo de confianza [IC] del 95%: 0,63 a 3,38; p=0,005), hubo más pacientes en clase funcional iv (13 frente a 5, el 43,3 frente al 15,8%; IC del 95%: 0,05 a 11,75; p=0,046), el gasto cardíaco fue menor (IC del 95%: 3,16 a 4,89; p=0,032) y fue más frecuente el tratamiento con epoprostenol (14 frente a 4, el 46 frente al 14,3%; IC del 95%: 1,46 a 18,85; odds ratio de 5,25; p=0,008) que en el grupo sin PT (AU)

Background and objective: Thyroid disease (TD) is more prevalent in patients with pulmonary arterial hypertension (PAH) than in the general population. The frequency and the cause of this association are not well established. We aimed to quantify and analyze the incidence and characteristics of TD in a cohort of PAH patients (idiopathic or preferentially associated with systemic diseases) and review the literature. Patients and method: Fifty eight PAH patients were prospectively studied, according to a previously established protocol (that included right heart catheterization); TSH, T4, and antithyroglobulin and antiperoxidase antibodies were determined. TD was defined as an abnormal TSH level and/or elevated antithyroid antibodies (TAbs). Clinical, biological and hemodynamic variables were compared between patients with and without TD. Results: TD was detected in 30 patients (51%): high TSH levels were observed in 21 (36,21%); hyperthyroidism in 2 (3,45%); and TAbs in 16 of 54 (27,59%), 7 of whom were euthyroid. In the TD group, PAH evolution time was longer (4,62 vs 2,61 years; P=.005, CI 95%, 0,63–3,38), more patients were in functional class IV (13;43,3% vs 5;15,8%, P=.046, CI 95% ,0,05–11,75), cardiac output was lower (P=.032, CI 95%, 3,16–4,89) and epoprostenol treatment was more frequently used (14;46,6% vs 4;14,3%, P=.008, CI 95%, 1,46–18,85; OR=5,25). Conclusions: The frequency of TD detected in this PAH cohort reaches 51% and it was associated with a longer evolution time of PAH and worse hemodynamic situation. Although epoprostenol was used more frequently in TD patients, a causal relationship with TD could not be established (AU)

[Thyroid dysfunction in patients with pulmonary arterial hypertension. A cohort study of 58 patients]. / Disfunción tiroidea en pacientes con hipertensión arterial pulmonar. Estudio de una cohorte de 58 pacientes.

BACKGROUND AND OBJECTIVE: Thyroid disease (TD) is more prevalent in patients with pulmonary arterial hypertension (PAH) than in the general population. The frequency and the cause of this association are not well established. We aimed to quantify and analyze the incidence and characteristics of TD in a cohort of PAH patients (idiopathic or preferentially associated with systemic diseases) and review the literature. PATIENTS AND METHOD: Fifty eight PAH patients were prospectively studied, according to a previously established protocol (that included right heart catheterization); TSH, T(4), and antithyroglobulin and antiperoxidase antibodies were determined. TD was defined as an abnormal TSH level and/or elevated antithyroid antibodies (TAbs). Clinical, biological and hemodynamic variables were compared between patients with and without TD. RESULTS: TD was detected in 30 patients (51%): high TSH levels were observed in 21 (36,21%); hyperthyroidism in 2 (3,45%); and TAbs in 16 of 54 (27,59%), 7 of whom were euthyroid. In the TD group, PAH evolution time was longer (4,62 vs 2,61 years; P=.005, CI 95%, 0,63-3,38), more patients were in functional class IV (13;43,3% vs 5;15,8%, P=.046, CI 95% ,0,05-11,75), cardiac output was lower (P=.032, CI 95%, 3,16-4,89) and epoprostenol treatment was more frequently used (14;46,6% vs 4;14,3%, P=.008, CI 95%, 1,46-18,85; OR=5,25). CONCLUSIONS: The frequency of TD detected in this PAH cohort reaches 51% and it was associated with a longer evolution time of PAH and worse hemodynamic situation. Although epoprostenol was used more frequently in TD patients, a causal relationship with TD could not be established.

Eficacia de rituximab combinado con ciclofosfamida en una paciente con lupus eritematoso sistémico y vasculitis peritoneal resistente a tratamiento inmunosupresor convencional / Efficacy of rituximab combined with cyclophosphamide in a patient with systemic lupus erythematosus and peritoneal vasculitis refractory to conventional inmunosupressive therapy

La vasculitis peritoneal es una manifestación clínica infrecuente y grave del lupus eritematoso sistémico. Se presenta el caso de una paciente con ascitis por vasculitis peritoneal y afección cutánea, articular, hemática y renal. El tratamiento con glucocorticoides e inmunosupresores resultó ineficaz para el control de la ascitis. Dada la resistencia al tratamiento convencional, se administró rituximab en cuatro infusiones semanales de 375mg/m2, potenciado con pulsos de ciclofosfamida las semanas 1 y 3. Con esta estrategia se consiguió una respuesta completa, que se repitió en brotes posteriores (el segundo y el tercero, multisistémicos y de nuevo con ascitis significativa, y el cuarto con nefritis) (AU)

Peritoneal vasculitis is a rare and severe clinical manifestation of systemic lupus erythematosus. We report a patient who presented with ascites due to peritoneal vasculitis and cutaneous, articular, hematological and renal inflammatory activity. Treatment with glucocorticoids and immunosuppressive drugs was ineffective. In view of the resistance to different therapies, 4 weekly infusions of 375mg/m2 of rituximab (RTX) were started, in association with cyclophosphamide pulses during the first and the third weeks. With this treatment strategy, the patient reached a complete response which was achieved in later flares of inflammatory activity (the second and third flares were multisystemic and with ascites again, and the fourth flare with nephritis) (AU)