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1.
Cells ; 9(3)2020 Mar 11.
Artigo em Inglês | MEDLINE | ID: mdl-32168865

RESUMO

The pathogenesis of Rheumatoid Arthritis (RA) is not fully understood, probably influenced by genetic and environmental factors. Interstitial Lung Disease (ILD) is an extra-articular manifestation of RA, which contributes significantly to morbidity and mortality. The identification of anti-HLA antibodies has been useful in the transplantation field; however, its contribution to autoimmune diseases as RA has not been fully studied. We aimed to determine the presence of anti-HLA antibodies in RA patients with and without ILD and its possible association with clinical and biochemical markers. One-hundred and forty-seven RA patients, of which 65 had ILD (RA-ILD group), were included. Sera samples for Anti-HLA Class II LABScreen panel-reactive antibodies (PRA) were analyzed. In both groups, women predominated, and lung function was worse in patients with ILD. The anti-CCP+ (UI/mL) was higher in the RA group in comparison to RA-ILD (p < 0.001). Expositional risk factors (tobacco smoking and biomass-burning smoke) were higher in RA-ILD patients. PRA+ was identified in ~25% RA-ILD patients, while ~29% in the RA group. The CRP levels have a positive correlation with the percentage of reactivity (%PRA, p = 0.02, r2 = 0.60) in the RA-ILD group. In conclusion, anti-HLA antibodies correlate with C-reactive protein levels in RA patients with ILD.

2.
J Rheumatol ; 47(3): 415-423, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31203227

RESUMO

OBJECTIVE: To describe the evolution of the pulmonary function in patients with interstitial lung disease (ILD) who are positive for at least 1 of the antisynthetase antibodies (ASAB) after medical treatment, and to compare whether the evolution of pulmonary function is associated with the type of ASAB. METHODS: Patients with ILD and positive for at least 1 of the ASAB (anti-Jo1, anti-PL7, anti-PL12, anti-EJ, or anti-OJ) were included. The clinical evolution, time until death or censoring, and improvement of lung disease were registered. RESULTS: The study included 118 patients. Most of the patients had a high extent of ground glass opacities in high-resolution computed tomography (HRCT) and low extent of fibrosis. In the final evaluation of pulmonary function (median 749.5 days of followup), 67% of the patients had lung disease improvement. The improvement occurred within the first 6 months after initiating medical treatment; thereafter, pulmonary function remained stable in most of the patients. A decrease of the extent of ground glass opacities was demonstrated in HRCT at followup in those patients with pulmonary improvement. No differences were observed in the percentage of patients who achieved improvement between the ASAB groups, or in survival. CONCLUSION: Improvement of pulmonary function was observed in 67% of the patients. Improvement was observed in all ASAB groups and occurred within 6 months after initiating medical treatment.

3.
Arch Bronconeumol ; 2019 Nov 26.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-31784348

RESUMO

BACKGROUND: Chronic hypersensitivity pneumonitis (cHP) represents a severe lung disease often evolving to fibrosis with the subsequent destruction of the lung parenchyma. There are no approved therapies with confirmed efficacy to deal with this disease. METHODS: We performed an open-label, proof of concept study, to evaluate the efficacy and safety of pirfenidone added to immunosuppressive drugs on the treatment of cHP. We included 22 patients assigned to two groups: Group 1, nine patients that received prednisone plus azathioprine and Group 2, thirteen patients, received prednisone plus azathioprine and pirfenidone (ClinicalTrials.gov identifier NCT02496182). There were no significant imbalances in clinically relevant baseline characteristics between two study groups. RESULTS: After 1 year of treatment, inclusion of pirfenidone was not associated with improved forced vital capacity (primary end-point). A not significant tendency to show higher improvement of diffusion capacity of the lung for carbon monoxide (DLCO) was observed in the group receiving pirfenidone (p=0.06). Likewise, a significant improvement in the total score on the SGRQ was found in the group 2 (p=0.02) without differences in other two questionnaires related to quality of life (ATAQ-IPF and EQ-5D-3L). HRCT showed a decrease of the ground glass attenuation without changes in the fibrotic lesions and without differences between both groups. CONCLUSIONS: These findings suggest that the addition of pirfenidone to the anti-inflammatory treatment in patients with chronic HP may improve the outcome with acceptable safety profile. However, prospective randomized double-blind, placebo-controlled trials in largest cohorts are needed to validate its efficacy.

4.
PLoS One ; 14(11): e0224772, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31703067

RESUMO

BACKGROUND: Interstitial lung disease (ILD) is a frequent manifestation of Sjögren's syndrome (SS), an autoimmune disease of salivary and lacrimal glands, and affects approximately 20% of patients. No clinical or serological features appear to be useful to predict its presence, severity or progression, and chest high-resolution computed tomography (CT) remains the gold standard for diagnosis. Semiquantitative CT (SQCT) based on visual assessment (Goh and Taouli scoring) can estimate ILD extent, although it is burdened by relevant intra- and interobserver variability. Quantitative chest CT (QCT) is a promising alternative modality to assess ILD severity. AIM: To determine whether QCT assessment can identify extensive or limited lung disease in patients with SS and ILD. METHODS: This multi-center, cross-sectional and retrospective study enrolled patients with SS and a chest CT scan. SQCT assessment was carried out in a blinded and centralized manner to calculate both Goh and Taouli scores. An operator-independent analysis of all CT scans with the open-source software platform Horos was used to evaluate the QCT indices. Patients were classified according to the extent of ILD and differences in QCT index distribution were investigated with non-parametric tests. RESULTS: From a total of 102 consecutive patients with SS, the prevalence of ILD was 35.3% (36/102). There was a statistically significant difference in QCT index distribution between the SS with ILD and SS without ILD groups (p<0.001). Moreover, SS-ILD patients with ILD >20% (by Goh score) had a QCT index statistically different from those with limited ILD extent (p<0.001). Finally, QCT indices showed a moderate-to-good correlation with the Goh and Taouli scores (from 0.44 to 0.65; p<0.001). CONCLUSIONS: QCT indices can identify patients with SS and ILD and discriminate those with lesser or greater lung disease.

5.
J Clin Med ; 8(11)2019 Nov 18.
Artigo em Inglês | MEDLINE | ID: mdl-31752231

RESUMO

Antisynthetase syndrome (ASSD) is a rare clinical condition that is characterized by the occurrence of a classic clinical triad, encompassing myositis, arthritis, and interstitial lung disease (ILD), along with specific autoantibodies that are addressed to different aminoacyl tRNA synthetases (ARS). Until now, it has been unknown whether the presence of a different ARS might affect the clinical presentation, evolution, and outcome of ASSD. In this study, we retrospectively recorded the time of onset, characteristics, clustering of triad findings, and survival of 828 ASSD patients (593 anti-Jo1, 95 anti-PL7, 84 anti-PL12, 38 anti-EJ, and 18 anti-OJ), referring to AENEAS (American and European NEtwork of Antisynthetase Syndrome) collaborative group's cohort. Comparisons were performed first between all ARS cases and then, in the case of significance, while using anti-Jo1 positive patients as the reference group. The characteristics of triad findings were similar and the onset mainly began with a single triad finding in all groups despite some differences in overall prevalence. The "ex-novo" occurrence of triad findings was only reduced in the anti-PL12-positive cohort, however, it occurred in a clinically relevant percentage of patients (30%). Moreover, survival was not influenced by the underlying anti-aminoacyl tRNA synthetase antibodies' positivity, which confirmed that antisynthetase syndrome is a heterogeneous condition and that antibody specificity only partially influences the clinical presentation and evolution of this condition.

6.
J Rheumatol ; 46(3): 279-284, 2019 03.
Artigo em Inglês | MEDLINE | ID: mdl-30442823

RESUMO

OBJECTIVE: To describe nailfold videocapillaroscopy (NVC) features of patients with antisynthetase syndrome (AS) and to investigate possible correlations with clinical and serological features of the disease. METHODS: We retrospectively analyzed NVC images of 190 patients with AS [females/males 3.63, mean age 49.7 ± 12.8 yrs, median disease duration 53.7 mos (interquartile range 82), 133 anti-Jo1 and 57 non-anti-Jo1-positive patients]. For each patient, we examined number of capillaries, giant capillaries, microhemorrhages, avascular areas, ramified capillaries, and the presence of systemic sclerosis (SSc)-like pattern. Finally, we correlated NVC features with clinical and serological findings of patients with AS. Concomitantly, a historical cohort of 75 patients with antinuclear antibody-negative primary Raynaud phenomenon (RP) and longterm followup was used as a control group (female/male ratio 4.13/1, mean age 53.9 ± 17.6 yrs) for NVC measures. RESULTS: NVC abnormalities were observed in 62.1% of AS patients compared with 29.3% of primary RP group (p < 0.001). An SSc-like pattern was detected in 67 patients (35.3%) and it was associated with anti-Jo1 antibodies (p = 0.002) and also with a longer disease duration (p = 0.004). Interestingly, there was no significant correlation between the presence of SSc-like pattern and RP, and only 47% of patients with SSc-like pattern had RP. CONCLUSION: NVC abnormalities are commonly observed in AS, independently from the occurrence of RP. The presence of an SSc-like pattern could allow identification of a more defined AS subtype, and prospective studies could confirm the association with clinical and serological features of AS.

7.
N Engl J Med ; 379(23): 2209-2219, 2018 12 06.
Artigo em Inglês | MEDLINE | ID: mdl-30345907

RESUMO

BACKGROUND: Given the phenotypic similarities between rheumatoid arthritis (RA)-associated interstitial lung disease (ILD) (hereafter, RA-ILD) and idiopathic pulmonary fibrosis, we hypothesized that the strongest risk factor for the development of idiopathic pulmonary fibrosis, the gain-of-function MUC5B promoter variant rs35705950, would also contribute to the risk of ILD among patients with RA. METHODS: Using a discovery population and multiple validation populations, we tested the association of the MUC5B promoter variant rs35705950 in 620 patients with RA-ILD, 614 patients with RA without ILD, and 5448 unaffected controls. RESULTS: Analysis of the discovery population revealed an association of the minor allele of the MUC5B promoter variant with RA-ILD when patients with RA-ILD were compared with unaffected controls (adjusted odds ratio, 3.8; 95% confidence interval [CI], 2.8 to 5.2; P=9.7×10-17). The MUC5B promoter variant was also significantly overrepresented among patients with RA-ILD, as compared with unaffected controls, in an analysis of the multiethnic case series (adjusted odds ratio, 5.5; 95% CI, 4.2 to 7.3; P=4.7×10-35) and in a combined analysis of the discovery population and the multiethnic case series (adjusted odds ratio, 4.7; 95% CI, 3.9 to 5.8; P=1.3×10-49). In addition, the MUC5B promoter variant was associated with an increased risk of ILD among patients with RA (adjusted odds ratio in combined analysis, 3.1; 95% CI, 1.8 to 5.4; P=7.4×10-5), particularly among those with evidence of usual interstitial pneumonia on high-resolution computed tomography (adjusted odds ratio in combined analysis, 6.1; 95% CI, 2.9 to 13.1; P=2.5×10-6). However, no significant association with the MUC5B promoter variant was observed for the diagnosis of RA alone. CONCLUSIONS: We found that the MUC5B promoter variant was associated with RA-ILD and more specifically associated with evidence of usual interstitial pneumonia on imaging. (Funded by Société Française de Rhumatologie and others.).


Assuntos
Artrite Reumatoide/genética , Mutação com Ganho de Função , Doenças Pulmonares Intersticiais/genética , Mucina-5B/genética , Idoso , Artrite Reumatoide/complicações , Feminino , Predisposição Genética para Doença , Genótipo , Humanos , Fibrose Pulmonar Idiopática/genética , Pulmão/química , Pulmão/patologia , Doenças Pulmonares Intersticiais/complicações , Masculino , Pessoa de Meia-Idade , Mucina-5B/análise , Razão de Chances , Regiões Promotoras Genéticas
8.
Reumatol. clín. (Barc.) ; 14(2): 75-80, mar.-abr. 2018. tab
Artigo em Espanhol | IBECS | ID: ibc-171557

RESUMO

Objetivos. Agrupar a los pacientes con enfermedad pulmonar intersticial (EPI) asociada a enfermedad indiferenciada del tejido conectivo (EITC) según la presencia o no de ciertas manifestaciones clínicas o inmunológicas, esperando encontrar diferentes expresiones tomográficas o funcionales. Métodos. Estudio de cohortes retrospectivas. Se incluyeron pacientes que cumplían criterios de Kinder para EITC. Se consideraron variables predictoras: manifestaciones «altamente específicas de enfermedad del tejido conectivo (ETC)» (Raynaud, xeroftalmia o artritis), títulos altos de anticuerpos antinucleares (ANA) (mayores a 1:320) y patrones específicos de ANA (centromérico, citoplásmico y nucleolar). El cambio en la capacidad vital forzada% (CVF%) en el tiempo y el patrón en TCAR fueron las variables de resultado estudiadas. Resultados. Se incluyeron 66 pacientes. Veintinueve presentaron al menos una manifestación «altamente específica de ETC» (43,94%), 16 ANA específico (28,57%) y 29 ANA alto título (43,94%). Aquellos con manifestaciones «altamente específicas de ETC» presentaron menor frecuencia de sexo masculino (10,34% vs 48,65%, p<0,001), menor edad en años (media 52 [DE14,58] vs 62,08 [9,46], p<0,001) y menor mediana de declinación de CVF% (1[RIC −1 a 10] vs −6 [RIC −16 a −4], p<0,006). En el análisis de regresión lineal múltiple la presencia de manifestaciones «altamente específicas de ETC» se asoció con mejoría en CVF% (coeficiente B de 13,25 [IC95% 2,41 a 24,09]). No encontramos asociaciones en cuanto al patrón en TACAR. Conclusiones. La presencia de manifestaciones «altamente específicas de ETC» se asoció con sexo femenino, menor edad al inicio y una evolución más favorable en cuanto a la CVF%, lo cual evidencia el impacto de las manifestaciones clínicas en la evolución de estos pacientes (AU)


Objectives. To identify clinical or immunological features in patients with undifferentiated connective tissue disease (UCTD) associated interstitial lung disease (ILD), in order to group them and recognize different functional and high resolution computed tomography (HRCT) behavior. Methods. Retrospective cohort study. Patients meeting Kinder criteria for UCTD were included. We defined the following predictive variables: ‘highly specific’ connective tissue disease (CTD) manifestations (Raynaud's phenomenon, dry eyes or arthritis), high antinuclear antibody (ANA) titer (above 1: 320), and ‘specific’ ANA staining patterns (centromere, cytoplasmic and nucleolar patterns). We evaluated the following outcomes: change in the percentage of the predicted forced vital capacity (FVC%) during the follow-up period, and HRCT pattern. Results. Sixty-six patients were included. Twenty-nine (43.94%) showed at least one ‘highly specific’ CTD manifestation, 16 (28.57%) had a ‘specific’ ANA staining pattern and 29 (43.94%) high ANA titer. Patients with ‘highly specific’ CTD manifestations were younger (mean [SD] 52 years [14.58] vs 62.08 years [9.46], P<.001), were more likely men (10.34% vs 48.65%, P<.001) and showed a smaller decline of the FVC% (median [interquartile range] 1% [−1 to 10] vs -6% [−16 to −4], P<.006). In the multivariate analysis, the presence of highly specific manifestations was associated with improvement in the FVC% (B coefficient of 13.25 [95% confidence interval, 2.41 to 24.09]). No association was observed in relation to the HRCT pattern. Conclusion. The presence of ‘highly specific’ CTD manifestations was associated with female sex, younger age and better functional behavior. These findings highlight the impact of the clinical features in the outcome of patients with UCTD ILD (AU)


Assuntos
Humanos , Doenças do Tecido Conjuntivo/complicações , Doenças Pulmonares Intersticiais/complicações , Padrões de Prática Médica , Estudos Retrospectivos , Doenças Autoimunes/epidemiologia , Anticorpos Antinucleares/isolamento & purificação , Fatores de Risco , Doença de Raynaud/epidemiologia
9.
Clin Exp Rheumatol ; 36(1): 44-49, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-28770709

RESUMO

OBJECTIVES: To evaluate if the timing of appearance with respect to disease onset may influence the arthritis presentation pattern in antisynthetase syndrome (ASSD). METHODS: The patients were selected from a retrospective large international cohort of ASSD patients regularly followed-up in centres referring to AENEAS collaborative group. Patients were eligible if they had an antisynthetase antibody testing positive in at least two determinations along with arthritis occurring either at ASSD onset (Group 1) or during the course of the disease (Group 2). RESULTS: 445 (70%; 334 females, 110 males, 1 transsexual) out of the 636 ASSD we collected had arthritis, in the majority of cases (367, 83%) from disease onset (Group 1). Patients belonging to Group 1 with respect to Group 2 had an arthritis more commonly polyarticular and symmetrical (p=0.015), IgM-Rheumatoid factor positive (p=0.035), erosions at hands and feet plain x-rays (p=0.036) and more commonly satisfying the 1987 revised classification criteria for rheumatoid arthritis (RA) (p=0.004). Features such as Raynaud's phenomenon, mechanic's hands and fever (e.g. accompanying findings) were more frequently reported in Group 2 (p=0.005). CONCLUSIONS: In ASSD, the timing of appearance with respect to disease onset influences arthritis characteristics. In particular, RA features are more common when arthritis occurs from ASSD onset, suggesting an overlap between RA and ASSD in these patients. When arthritis appears during the follow-up, it is very close to a connective tissue disease-related arthritis. Also, the different prevalence of accompanying features between these two groups is in line with this possibility.


Assuntos
Artrite/epidemiologia , Miosite/epidemiologia , Adulto , Artrite/diagnóstico , Artrite/imunologia , Autoanticorpos/sangue , Biomarcadores/sangue , Europa (Continente)/epidemiologia , Feminino , Humanos , Masculino , México/epidemiologia , Pessoa de Meia-Idade , Miosite/diagnóstico , Miosite/imunologia , Fenótipo , Prevalência , Prognóstico , Estudos Retrospectivos , Fatores de Risco , Fatores de Tempo
10.
Reumatol Clin ; 14(2): 75-80, 2018.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-27979657

RESUMO

OBJECTIVES: To identify clinical or immunological features in patients with undifferentiated connective tissue disease (UCTD) associated interstitial lung disease (ILD), in order to group them and recognize different functional and high resolution computed tomography (HRCT) behavior. METHODS: Retrospective cohort study. Patients meeting Kinder criteria for UCTD were included. We defined the following predictive variables: 'highly specific' connective tissue disease (CTD) manifestations (Raynaud's phenomenon, dry eyes or arthritis), high antinuclear antibody (ANA) titer (above 1: 320), and 'specific' ANA staining patterns (centromere, cytoplasmic and nucleolar patterns). We evaluated the following outcomes: change in the percentage of the predicted forced vital capacity (FVC%) during the follow-up period, and HRCT pattern. RESULTS: Sixty-six patients were included. Twenty-nine (43.94%) showed at least one 'highly specific' CTD manifestation, 16 (28.57%) had a 'specific' ANA staining pattern and 29 (43.94%) high ANA titer. Patients with 'highly specific' CTD manifestations were younger (mean [SD] 52 years [14.58] vs 62.08 years [9.46], P<.001), were more likely men (10.34% vs 48.65%, P<.001) and showed a smaller decline of the FVC% (median [interquartile range] 1% [-1 to 10] vs -6% [-16 to -4], P<.006). In the multivariate analysis, the presence of highly specific manifestations was associated with improvement in the FVC% (B coefficient of 13.25 [95% confidence interval, 2.41 to 24.09]). No association was observed in relation to the HRCT pattern. CONCLUSION: The presence of 'highly specific' CTD manifestations was associated with female sex, younger age and better functional behavior. These findings highlight the impact of the clinical features in the outcome of patients with UCTD ILD.


Assuntos
Doenças Pulmonares Intersticiais/etiologia , Doenças do Tecido Conjuntivo Indiferenciado/diagnóstico , Adulto , Idoso , Feminino , Seguimentos , Humanos , Modelos Lineares , Modelos Logísticos , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Doenças Pulmonares Intersticiais/imunologia , Masculino , Pessoa de Meia-Idade , Razão de Chances , Estudos Retrospectivos , Doenças do Tecido Conjuntivo Indiferenciado/complicações , Doenças do Tecido Conjuntivo Indiferenciado/imunologia
11.
Eur Arch Otorhinolaryngol ; 275(2): 425-429, 2018 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-29230559

RESUMO

PURPOSE: To present the results of an endoscopic and histopathologic evaluation of suspected nasal active granulomatosis with polyangiits (GPA) lesions, describe them as seen by the ENT specialist, and propose a guide for tissue sampling of the nasal cavity to improve the yield of confirmatory histology. METHODS: Randomly selected patients seen from December 1997-October 2007 had a thorough endoscopic nasal evaluation, preceded by careful cleansing of the nasal cavity. Endoscopic lesions were described; sensitivities, specificities, and predictive values of the composites of endoscopic and histological activity were determined. RESULTS: Six lesions, some not previously described in detail, were observed: white submucosal nodules, mucosal swelling, polypoid nodules, vascular submucosal dilatations, bloody submucosal patches, and ulcers. Of these, polypoid nodules (PPV 100%), persistent white submucosal nodules (PPV 81%), and bloody submucosal patches (PPV 93%) had the better diagnostic performance with confirmed histological diagnosis. CONCLUSIONS: Careful nasal cavity preparation, observation, and description of the nasal mucosa can guide tissue sampling documenting active GPA. This can lead to a better histological yield when definitive proof of the disease is needed.


Assuntos
Endoscopia/métodos , Granulomatose com Poliangiite/patologia , Cavidade Nasal/patologia , Mucosa Nasal/patologia , Adulto , Feminino , Granulomatose com Poliangiite/diagnóstico , Humanos , Masculino , Sensibilidade e Especificidade
12.
Clin Rheumatol ; 36(7): 1493-1500, 2017 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-28585060

RESUMO

Interstitial lung disease (ILD) is a severe rheumatoid arthritis (RA) manifestation. The worst survival has been associated with usual interstitial pneumonia (UIP) definitive pattern in high-resolution chest tomography (HRCT) scans. Moreover, the use of methotrexate in RA-ILD is controversial. Our aim was to evaluate prognostic factors including methotrexate in an RA-ILD cohort and their association with survival. RA-ILD patients referred for medical evaluation and treatment at a single center were included. At the baseline, pulmonary function tests were carried out and a HRCT was obtained. A radiologist evaluated the ILD tomographic pattern and the extent of lung disease. Patients were considered as receiving methotrexate therapy if this drug was specifically prescribed for the treatment of RA-ILD at the beginning of follow up. Seventy-eight patients were included. UIP definite pattern in HRCT was not associated to worse survival. Variables associated with mortality reflected the severity of lung disease. Treatment with methotrexate was associated with survival (HR 0.13, 95% CI 0.02-0.64); older patients had worse prognosis (HR 1.04, 95% CI 1.003-1.09). After adjusting for confounding variables, methotrexate was strongly associated with survival. Methotrexate treatment during follow up was associated with survival. The severity of lung disease and not the tomographic pattern is associated with mortality; older patients had worse prognosis.


Assuntos
Antirreumáticos/uso terapêutico , Artrite Reumatoide/complicações , Doenças Pulmonares Intersticiais/etiologia , Metotrexato/uso terapêutico , Adulto , Idoso , Artrite Reumatoide/diagnóstico por imagem , Artrite Reumatoide/tratamento farmacológico , Feminino , Humanos , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Doenças Pulmonares Intersticiais/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Prognóstico , Índice de Gravidade de Doença , Tomografia Computadorizada por Raios X , Resultado do Tratamento
13.
Respir Med ; 123: 79-86, 2017 02.
Artigo em Inglês | MEDLINE | ID: mdl-28137500

RESUMO

OBJECTIVE: to describe the clinical manifestations and survival of patients with ILD and myositis-specific and associated autoantibodies, and to evaluate the performance of the new ATS/ERS classification criteria for IPAF. PATIENTS AND METHODS: Patients with ILD and positive in at least one of the following autoantibodies: anti-Jo-1, anti-Ej, anti-PL7, anti-PL 12, anti-PM/SCL 75 and anti-PM/SCL100 were included. Patients were separated into three groups according to their autoantibody profile: 1. Jo-1 positive patients, 2. Non-Jo-1 antisynthetase autoantibody positive patients, and 3. PM/SCL positive patients. Relevant clinical characteristics were registered. Patients were evaluated had they fulfilled Bohan and Peter's criteria (BPC) for inflammatory myopathies. We evaluated the performance of the IPAF ATS/ERS proposal to classify as such the patients that did not fulfilled BPC, and evaluated whether IPAF patients had a worse survival that BPC patients. RESULTS: Sixty-eight patients were included. Jo-1 was the most frequent autoantibody (65%), followed by non Jo1 anti-synthetase autoantibodies (31%). Non-Jo1 patients had lower Creatin Kinase serum levels at the baseline and less frequency of arthritis. Only 50% of patients fulfilled BPC. All patients not complying with BPC did comply with IPAF criteria. There was no difference in survival between IPAF and BPC patients. Anti Jo-1 positive was associated to survival and the extent of lung inflammation was associated to mortality. CONCLUSIONS: Patients differ in clinical manifestations according to the autoantibody profile. All patients not complying with BPC did comply with the new IPAF criteria. There was no difference in survival between BPC and IPAF patients. Jo-1 patients had a better survival. Extent of lung inflammation was associate to mortality.


Assuntos
Autoanticorpos/sangue , Doenças Autoimunes/imunologia , Doenças Pulmonares Intersticiais/imunologia , Miosite/imunologia , Adulto , Anticorpos Antinucleares/sangue , Doenças Autoimunes/diagnóstico , Doenças Autoimunes/diagnóstico por imagem , Biomarcadores/sangue , Feminino , Humanos , Estimativa de Kaplan-Meier , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Masculino , Pessoa de Meia-Idade , Miosite/diagnóstico por imagem , Prognóstico , Índice de Gravidade de Doença , Tomografia Computadorizada por Raios X
14.
J Med Econ ; 19(12): 1187-1199, 2016 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-27376404

RESUMO

OBJECTIVES: To estimate economic impact resulting from increased biologics use for treatment of rheumatoid arthritis (RA) and Crohn's disease (CD) in Argentina, Brazil, Colombia, and Mexico. METHODS: The influence of increasing biologics use for treatment of RA during 2012-2022 and for treatment of CD during 2013-2023 was modeled from a societal perspective. The economic model incorporated current and projected medical, indirect, and drug costs and epidemiologic and economic factors. Costs associated with expanded biologics use for RA were compared with non-expanded use in Argentina, Brazil, Colombia, and Mexico. A similar analysis was conducted for CD in Brazil, Colombia, and Mexico. RESULTS: Accounting for additional costs of biologics and medical and indirect cost offsets, the model predicts that expanded use of biologics for patients with RA from 2012 to 2022 will result in cumulative net cost savings of ARS$2.351 billion in Argentina, R$9.004 billion in Brazil, COP$728.577 billion in Colombia, and MXN$18.02 billion in Mexico; expanded use of biologics for patients with CD from 2013 to 2023 will result in cumulative net cost savings for patients with CD of R$0.082 billion in Brazil, COP$502.74 billion in Colombia, and MXN$1.80 billion in Mexico. Indirect cost offsets associated with expanded biologics use were a key driver in reducing annual per-patient net costs for RA and CD. LIMITATIONS: Future economic projections are limited by the potential variance between projected and actual future values of biologic prices, wages, medical costs, and gross national product for each country. CONCLUSIONS: Increasing biologics use to treat RA and CD may limit cost growth over time by reducing medical and indirect costs. These findings may inform policy decisions regarding biologics use in Argentina, Brazil, Colombia, and Mexico.


Assuntos
Antirreumáticos/economia , Antirreumáticos/uso terapêutico , Artrite Reumatoide/tratamento farmacológico , Produtos Biológicos/economia , Terapia Biológica/economia , Terapia Biológica/estatística & dados numéricos , Doença de Crohn/tratamento farmacológico , Custos de Cuidados de Saúde/tendências , Análise Custo-Benefício , Custos de Medicamentos , Humanos , México , América do Sul
15.
J Clin Rheumatol ; 22(2): 51-6, 2016 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-26906295

RESUMO

BACKGROUND: Factors associated with survival in patients with idiopathic inflammatory myopathies are heterogeneous. OBJECTIVE: This study aimed to describe clinical and prognostic factors associated with survival in Mexican patients with idiopathic inflammatory myopathies. METHODS: Patients with dermatomyositis (DM) and polymyositis (PM) seen at a tertiary care center from 1985 to 2012 were included. Demographic and clinical characteristics, comorbidities, treatment, and the time to death were recorded. Patients with juvenile DM were excluded. Univariate and multivariate analyses were performed to identify factors associated with mortality. RESULTS: A total of 264 patients with DM and 69 patients with PM were studied. Patients with DM had lower levels of creatine phosphokinase, less cumulative dose of prednisone, higher frequency of dysphagia, and no difference in frequency of interstitial lung disease compared with patients with PM. Patients with DM had lower survival during the first 4 years of disease (80%; 95% confidence interval [CI], 0.74-0.85 vs 89%; 95% CI, 0.78-0.95; P = 0.03 log-rank). Respiratory failure due to pulmonary infection was the main cause of death in patients with DM; miscellaneous causes were responsible for death in patients with PM. Muscular strength (hazard ratio [HR], 0.48; 95% CI, 0.27-0.83; P = 0.01), platelet count (HR, 0.98; 95% CI, 0.98-0.99; P = 0.002), as well as ever use of methotrexate (HR, 0.21; 95% CI, 0.07-0.65; P = 0.007) and azathioprine (HR, 0.21; 95% CI, 0.06-0.68; P = 0.009) were independent factors associated with mortality in patients with DM; in those with PM, only cancer was associated (HR, 8.0; 95% CI, 1.4-43.9; P = 0.01). CONCLUSIONS: Patients with DM had lower survival during the first 4 years of disease than patients with PM. Factors associated with mortality differed in both groups.


Assuntos
Miosite/mortalidade , Adulto , Causas de Morte , Feminino , Humanos , Masculino , México , Pessoa de Meia-Idade , Miosite/complicações , Prognóstico , Estudos Retrospectivos , Fatores de Risco , Análise de Sobrevida , Taxa de Sobrevida
16.
Clin Rheumatol ; 34(9): 1563-9, 2015 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-26219488

RESUMO

The objectives of the present study were to compare the survival function of antisynthetase syndrome (ASS) Jo1-positive patients with ASS non-Jo1 patients, all with interstitial lung disease (ILD), and to evaluate other factors such as the extension of pulmonary disease and the time between the onset of symptoms and diagnosis and its association to survival in a cohort of ASS patients. Patients with ASS, all with ILD, were included. At the baseline, pulmonary function tests were realized and a high-resolution chest tomography was obtained; lung inflammation and fibrosis were measured with the Goh score and the Kazerooni index. The following autoantibodies were measured: Jo1, Ej, Oj, PL7, and PL12. Patients had to be positive for one of them in order to be included in the study. The survival function was estimated and compared with the log rank test, and the hazard ratio (HR) was estimated using Cox regression procedure. Forty-three patients were included, of which six patients died (14 %). Patients who died were different in comparison with survivors as regards the frequency of anti-Jo1 positivity: Survivors had anti-Jo1 autoantibodies more frequently (86 %) than patients who died (50 %). The univariate Cox regression analysis identified four variables associated with survival: Jo1 status, arthritis, extent of ground glass, and consolidation (inflammation) in high-resolution computed tomography (HRCT) and baseline forced vital capacity. The serological status of patients (Jo1-positive vs non-Jo1), the extent of lung inflammation in the HRCT scan, a low forced vital capacity, and arthritis are associated with survival in ASS patients.


Assuntos
Autoanticorpos/sangue , Doenças Pulmonares Intersticiais/sangue , Miosite/mortalidade , Capacidade Vital/fisiologia , Adulto , Artrite/diagnóstico por imagem , Estudos de Coortes , Feminino , Humanos , Masculino , México , Pessoa de Meia-Idade , Análise Multivariada , Prognóstico , Análise de Regressão , Taxa de Sobrevida , Centros de Atenção Terciária , Tomografia Computadorizada por Raios X
17.
Arch Med Res ; 46(3): 228-32, 2015 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-25892606

RESUMO

BACKGROUND AND AIMS: Despite increase in survival of HIV patients due to highly active antiretroviral therapy (HAART), non-infectious complications are still prevalent such as presentation of lung vasculopathy, even in asymptomatic patients. Endothelin-1 (ET-1) is a potent vasoconstrictor that causes pulmonary vasculopathy. Participation of this protein in the pulmonary circulation in HIV patients has not been elucidated. In this work we studied the presence and expression of ET-1 in pulmonary complex vascular lesions associated with human immunodeficiency virus (PCVL/HIV). METHODS: We used immunohistochemistry and immunochemiluminescence (imagej) to determine the different degrees of expression of ET-1 in PCVL/HIV in comparison with non-PCVL/HIV. Reagents used were anti-endothelin-1 and an automated system. All data are presented as mean and standard deviation (SD). Differences were analyzed with one-way ANOVA; p < 0.05 was accepted as statistically significant. RESULTS: Lung tissues from 56 patients who died from complications of HIV pulmonary infection and with PCVL were studied. Histological evidence of pulmonary vasculopathy was shown as different types (proliferative, obliterative and plexiform). A statistically significant increase in ET-1 expression was observed in all PCVL/HIV tissue samples and is associated directly with different grades of severity of endothelial dysfunction. CONCLUSIONS: ET-1 has a relevant role in the pathogenesis of pulmonary vasculopathy in acquired immunodeficiency syndrome (AIDS) patients. It is necessary to determine in the future the participation of ET-1 and other mechanisms involved in PCVL/HIV.


Assuntos
Síndrome de Imunodeficiência Adquirida/complicações , Endotelina-1/biossíntese , Pneumopatias/metabolismo , Doenças Vasculares/metabolismo , Adulto , Feminino , Humanos , Pulmão/irrigação sanguínea , Pulmão/metabolismo , Pulmão/patologia , Pneumopatias/etiologia , Pneumopatias/fisiopatologia , Masculino , Pessoa de Meia-Idade , Artéria Pulmonar/patologia , Circulação Pulmonar , Doenças Vasculares/etiologia , Doenças Vasculares/fisiopatologia , Adulto Jovem
18.
Reumatol. clín. (Barc.) ; 11(1): 12-16, ene.-feb. 2015. tab, ilus
Artigo em Inglês | IBECS | ID: ibc-132357

RESUMO

Objective. To describe the association between rheumatoid arthritis disease activity (RA) and interstitial lung damage (inflammation and fibrosis), in a group of patients with rheumatoid arthritis-associated interstitial lung disease (RA-ILD). Methods. A retrospective study of RA patients with interstitial lung disease (restrictive pattern in lung function tests and evidence of interstitial lung disease in high resolution computed tomography (HRCT)). Patients were evaluated to exclude other causes of pulmonary disease. RA disease activity was measured with the CDAI index. Interstitial lung inflammation and fibrosis were determined by Kazerooni scale. We compared Kazerooni ground-glass score with the nearest CDAI score to HRCT date scan of the first medical evaluation at our institution. In nine patients, we compared the first ground-glass score with a second one after treatment with DMARDs and corticosteroids. Spearman's rank correlation coefficient was used to evaluate association between RA disease activity and the Kazerooni ground-glass and fibrosis scores. Results. Thirty-four patients were included. A positive correlation between CDAI and ground-glass scores was found (rs = 0.3767, P < 0.028). Fibrosis and CDAI scores were not associated (rs = −0.0747, P < 0.6745). After treatment, a downward tendency in the ground-glass score was observed (median [IQR]): (2.33 [2,3] vs. 2 [1.33–2.16]), P < 0.056, along with a lesser CDAI score (27 [8–43] vs. 9 [5–12]), P < 0.063. Conclusion. There is a correlation between RA disease activity and ground-glass appearance in the HRCT of RA-ILD patients. These results suggest a positive association between RA disease activity and lung inflammation in RA-ILD (AU)


Objetivo. Describir la asociación entre la actividad de la artritis reumatoide (AR) y la afectación intersticial pulmonar (inflamación y fibrosis), en un grupo de pacientes con enfermedad pulmonar intersticial asociada a la artritis reumatoide. Métodos. Estudio retrospectivo de pacientes con AR y enfermedad pulmonar intersticial (patrón restrictivo en las pruebas de la mecánica pulmonar y evidencia de enfermedad pulmonar intersticial en la tomografía de alta resolución (TCAR)). Los pacientes fueron evaluados para excluir otras causas de enfermedad pulmonar. La actividad de la AR fue medida con el índice clínico de actividad de la enfermedad (CDAI). La inflamación y la fibrosis pulmonar fueron evaluadas con el método de Kazerooni. Se realizó una correlación entre las puntuaciones de vidrio deslustrado del método de Kazerooni con la puntuación más cercano del índice de CDAI. En 9 pacientes se comparó la primera puntuación del vidrio deslustrado con la segunda puntuación después del tratamiento con corticosteroides y fármacos modificadores de la enfermedad (FARME). El coeficiente de correlación de Spearman fue utilizado para evaluar la correlación del índice de CDAI y la puntuación de Kazerooni de vidrio despulido y fibrosis. Resultados. Se incluyeron 34 pacientes. Se encontró una correlación de rs= 0.3767 (P < 0.028) entre las puntuaciones del CDAI y las del vidrio deslustrado. El índice de CDAI y las puntuaciones de fibrosis no estaban asociadas (rs= -0.0747, p < 0.6745). En los 9 pacientes con una segunda TCAR, después del tratamiento, se encontró una tendencia a una menor puntuación de la escala de vidrio deslustrado (mediana [IIC]): (2.33 [2,3] vs. 2 [1.33-2.16]), p < 0.056, además de una tendencia a una menor puntuación del CDAI (mediana [IIC]): (2.33 [2,3] vs. 2 [1.33-2.16]), p < 0.056. Conclusiones. Existe una correlación entre la actividad de la AR y la presencia de vidrio despulido en la TCAR en los pacientes con EPI-AR. Este resultado sugiere una asociación positiva entre la actividad de la AR y el daño pulmonar (AU)


Assuntos
Humanos , Feminino , Pessoa de Meia-Idade , Doenças Pulmonares Intersticiais/complicações , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/cirurgia , Artrite Reumatoide/complicações , Artrite Reumatoide/tratamento farmacológico , Corticosteroides/uso terapêutico , Doenças Pulmonares Intersticiais/fisiopatologia , Doenças Pulmonares Intersticiais , Artrite Reumatoide , Tomografia Computadorizada de Emissão , Pneumonia/complicações , Pneumonia , Estudos Retrospectivos
19.
Reumatol Clin ; 11(1): 12-6, 2015.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-24913966

RESUMO

OBJECTIVE: To describe the association between rheumatoid arthritis disease activity (RA) and interstitial lung damage (inflammation and fibrosis), in a group of patients with rheumatoid arthritis-associated interstitial lung disease (RA-ILD). METHODS: A retrospective study of RA patients with interstitial lung disease (restrictive pattern in lung function tests and evidence of interstitial lung disease in high resolution computed tomography (HRCT)). Patients were evaluated to exclude other causes of pulmonary disease. RA disease activity was measured with the CDAI index. Interstitial lung inflammation and fibrosis were determined by Kazerooni scale. We compared Kazerooni ground-glass score with the nearest CDAI score to HRCT date scan of the first medical evaluation at our institution. In nine patients, we compared the first ground-glass score with a second one after treatment with DMARDs and corticosteroids. Spearman's rank correlation coefficient was used to evaluate association between RA disease activity and the Kazerooni ground-glass and fibrosis scores. RESULTS: Thirty-four patients were included. A positive correlation between CDAI and ground-glass scores was found (rs=0.3767, P<0.028). Fibrosis and CDAI scores were not associated (rs=-0.0747, P<0.6745). After treatment, a downward tendency in the ground-glass score was observed (median [IQR]): (2.33 [2,3] vs. 2 [1.33-2.16]), P<0.056, along with a lesser CDAI score (27 [8-43] vs. 9 [5-12]), P<0.063. CONCLUSION: There is a correlation between RA disease activity and ground-glass appearance in the HRCT of RA-ILD patients. These results suggest a positive association between RA disease activity and lung inflammation in RA-ILD.


Assuntos
Artrite Reumatoide/complicações , Doenças Pulmonares Intersticiais/etiologia , Adulto , Idoso , Artrite Reumatoide/diagnóstico , Feminino , Seguimentos , Humanos , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Índice de Gravidade de Doença , Tomografia Computadorizada por Raios X
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