Design and validation of LupusCol, an instrument for the evaluation of health-related quality of life in Colombian adult patients with systemic lupus erythematosus.

OBJECTIVE: The aim of this study was to design and validate LupusCol, an instrument for the evaluation of health-related quality of life (HRQoL) in Colombian adult patients with SLE. METHODS: Items and domains of the initial instrument were defined. Preliminary tests were made with the participation of patients. Validity and reliability tests of the administration method were conducted. Usability tests were applied to the version obtained in the previous phases to complete the validation process. RESULTS: Following preliminary tests, six items and one domain were excluded and two new items were added to the instrument, producing a form with 44 questions and 7 domains, which was submitted for validity and reliability tests. Factor analysis excluded three items, obtaining a Pearson's correlation (PC) for the criteria validity of -0, 48; a Cronbach's α coefficient for internal consistency of 0, 96; an intraclass correlation coefficient (ICC) for personal test-retest-telephone of 0.96 and an ICC personal test-retest-personal of 0.96. For interrater concordance a PC of 0.8, an ICC of 0.77 and a Lin's coefficient of 0.86 were found. Sensitivity to change was demonstrated through analysis of variance, obtaining significant indicators about the scale, demonstrating the instrument's ability to detect changes in HRQoL. CONCLUSION: The design and validation process was completed successfully. The scale has significant values for validity, reliability and sensitivity to change in the studied population.

Colonoscopy aspiration lavages for mucosal metataxonomic profiling of spondylarthritis-associated gastrointestinal tract alterations.

The study of the GI-tract microbiota of spondylarthritis (SpA) patients has focused on the analysis of feces samples, that picture mostly the luminal microbiota. The aim of this study was to determine the contribution of mucosal and luminal microbiome to the gut dysbiosis in SpA, using colonoscopy aspiration lavages (CAL), a recent alternative for regional studies of the GI-tract. We analyzed 59 CAL (from sigmoid colon and distal ileum), and 41 feces samples, from 32 SpA patients and 7 healthy individuals, using 16S rRNA gene-targeted metataxonomic profiling. It was found high prevalence of GI-tract manifestations among SpA patients (65.3%). Metataxonomic profiling, confirmed CAL samples from the lower GI tract (colon or ileum) presented a distinctive and undifferentiated bacteriome and separate from that found in feces' samples or in the beginning of the GI tract (oral cavity (OC)). Lower GI-tract samples and feces of SpA patients exhibited similar behavior to the microbiota of IBD group with reduced microbial richness and diversity, comparing to the healthy controls. Interestingly, it was found increase in proinflammatory taxa in SpA patients, such as Enterobacteriaceae family (mostly in the ileum), Succinivibrio spp. and Prevotella stercorea. Conversely, SpA patients presented significant decrease in the SCFA producers Coprococcus catus and Eubacterium biforme. Our data support the value of CAL samples for the regional study of GI-tract and contribute with information of potential "disruptor taxa" involved in the GI-tract associated disorders observed in SpA patients.

Burden of rheumatoid arthritis on patients' work productivity and quality of life.

BACKGROUND: To determine the burden of Rheumatoid Arthritis (RA) on patients' work productivity and health related quality of life (HRQoL), and examine the influence of several exposure variables; to analyze the progression of RA over 1 year and its impact on work productivity and HRQoL. METHODS: International multicenter prospective survey including patients in 18 centers in Argentina, Brazil, Colombia and Mexico with diagnosis of RA and aged between 21-55 years. The following standard questionnaires were completed at baseline and throughout a 1-year follow-up: WPAI:RA, WALS, WLQ-25, EQ-5D-3 L and SF-36. Clinical and demographic variables were also collected through interview. RESULTS: The study enrolled 290 patients on baseline visit. Overall mean scores at baseline visit were: WPAI:RA (presenteeism) = 29.5% (SD = 28.8%); WPAI:RA (absenteeism) = 9.0% (SD = 23.2%); WPAI:RA (absenteeism and presenteeism) = 8.6% (SD = 22.6%); WALS = 9.0 (SD = 6.1); WLQ-25 = 7.0% (SD = 5.1%); SF-36 Physical Scale = 39.1 (SD = 10.3) and Mental Scale = 45.4 (SD = 11.3); EQ-5D-3 L VAS = 69.8 (SD = 20.4) and EQ-5D-3 L index = 0.67 (SD = 0.23). Higher educational levels were associated with better results in WLQ-25, while previous orthopedic surgeries reduced absenteeism results of WPAI:RA and work limitations in WLQ-25. Higher disease duration was associated with decreased HRQoL. Intensification of disease activity was associated with decreased work productivity and HRQoL, except in WLQ-25. In the longitudinal analysis, worsening in disease activity was associated with a decrease in both work productivity and HRQoL. CONCLUSIONS: RA patients are dealing with workplace disabilities and limitations and loss in HRQoL, and multiple factors seems to be associated with this. Worsening of disease activity further decreased work productivity and HRQoL, stressing the importance of disease tight control.

Are obesity, ACPAs and periodontitis conditions that influence the risk of developing rheumatoid arthritis in first-degree relatives?

The aim of this study was to investigate the body mass index (BMI), anti-citrullinated protein antibodies (ACPAs) status and the presence of periodontitis and IgG-1/IgG-2 antibodies against Porphyromonas gingivalis (Pg) in the first-degree relatives (FDRs) of rheumatoid arthritis (RA) patients and compare these variables with a control group of healthy individuals from the general population. In total, 100 FDR individuals and 200 healthy controls matched by age and gender were included. Rheumatologic and periodontal assessment was performed, and the presence of ACPAs and anti-P. gingivalis antibodies was evaluated. Groupwise comparisons were analysed using the McNemar and Wilcoxon tests. A conditional logistic regression analysis was performed to establish the associations between BMI, ACPAs and periodontitis in both groups. In the FDR group, 70% of the subjects were female, with a mean age of 37.3 ± 13 years. Obesity was observed in 17 and 7% of the FDRs and controls, respectively. ACPAs were found in 7% of the FDRs vs. 2.5% of the controls. Periodontitis was diagnosed in 79 and 56% of the FDRs and controls, respectively. Among the FDRs, 15% had severe periodontitis. There were associations in the FDR group related to the presence of obesity (OR 2.93, 95% CI 1.03-8.28), ACPAs (OR 2.45, 95% CI 0.7-8.32) and periodontitis (OR 3.70 95% CI 1.89-7.29). Regarding anti-P. gingivalis antibodies and smoking history, no differences were found between the groups. Obesity, ACPAs and periodontitis (diagnosis and severity) can be considered as relevant conditions associated with the development of RA in FDRs.

Burden of rheumatoid arthritis on patients' work productivity and quality of life

Abstract Background: To determine the burden of Rheumatoid Arthritis (RA) on patients' work productivity and health related quality of life (HRQoL), and examine the influence of several exposure variables; to analyze the progression of RA over 1 year and its impact on work productivity and HRQoL. Methods: International multicenter prospective survey including patients in 18 centers in Argentina, Brazil, Colombia and Mexico with diagnosis of RA and aged between 21-55 years. The following standard questionnaires were completed at baseline and throughout a 1-year follow-up: WPAI:RA, WALS, WLQ-25, EQ-5D-3 L and SF-36. Clinical and demographic variables were also collected through interview. Results: The study enrolled 290 patients on baseline visit. Overall mean scores at baseline visit were: WPAI:RA (presenteeism) = 29.5% (SD = 28.8%); WPAI:RA (absenteeism) = 9.0% (SD = 23.2%); WPAI:RA (absenteeism and presenteeism) = 8.6% (SD = 22.6%); WALS = 9.0 (SD = 6.1); WLQ-25 = 7.0% (SD = 5.1%); SF-36 Physical Scale = 39.1 (SD = 10.3) and Mental Scale = 45.4 (SD = 11.3); EQ-5D-3 L VAS = 69.8 (SD = 20.4) and EQ-5D-3 L index = 0.67 (SD = 0.23). Higher educational levels were associated with better results in WLQ-25, while previous orthopedic surgeries reduced absenteeism results of WPAI:RA and work limitations in WLQ-25. Higher disease duration was associated with decreased HRQoL. Intensification of disease activity was associated with decreased work productivity and HRQoL, except in WLQ-25. In the longitudinal analysis, worsening in disease activity was associated with a decrease in both work productivity and HRQoL. Conclusions: RA patients are dealing with workplace disabilities and limitations and loss in HRQoL, and multiple factors seems to be associated with this. Worsening of disease activity further decreased work productivity and HRQoL, stressing the importance of disease tight control.

Single anti-P ribosomal antibodies are not associated with lupus nephritis in patients suffering from active systemic lupus erythematosus.

BACKGROUND: In clinical practice, it is sometimes difficult to diagnose a relapse in patients suffering from systemic lupus erythematosus (SLE) and lupus nephritis (LN) having potential complications, including renal failure and death. Some immunological markers can help to determine their association with LN and, therefore, diagnose the early onset of complications. OBJECTIVES: Evaluating the association between systemic and/or kidney activity and anti-P ribosomal and anti-dsDNA antibodies in patients suffering from active SLE. METHODS: 389 patients were evaluated, 140 of whom were subsequently included in the study. The patients were divided into two groups by means of case-control studies, including Colombian patients having American College of Rheumatology (ACR) classification criteria for SLE (1997). The SLE disease activity index (SLEDAI) was applied and all patients presenting an increase of 5 or more compared to their last evaluation, as well as presenting renal manifestations, were considered to be cases; all patients had an activity score. An ELISA kit and the indirect immunofluorescence method with Crithidia luciliae were used for determining the presence of anti-P ribosomal and anti-dsDNA antibodies, respectively. RESULTS: No association was found between anti-P ribosomal antibodies and LN (p=0.2971) but anti-P ribosomal antibodies showed association with a >5 SLEDAI score (OR=4.87; 1.32-17.98 95% CI; p=0.008). The coexistence of anti-P ribosomal and anti-dsDNA antibodies was associated with LN (OR=3.52; 1.07-13.42 95% CI; p=0.019) and anti-dsDNA was associated with LN (p=0.001). CONCLUSION: There was no association between anti-P ribosomal antibodies and LN but anti-P ribosomal antibodies coexisting with anti-dsDNA antibodies was associated with LN, thereby suggesting that the coexistence of two antibodies is nephritogenic to a greater extent. Additional studies are needed to evaluate the coexistence of kidney-specific antibodies in SLE to determine the biological nature of LN.

Polyautoimmunity and familial autoimmunity in systemic sclerosis.

Characterization of the extent to which particular combinations of autoimmune diseases occur in excess of that expected by chance may offer new insights into possible common pathophysiological mechanisms. The goal of this study was to investigate the spectrum of polyautoimmunity (i.e. autoimmune diseases co-occurring within patients) and familial autoimmunity (i.e. diverse autoimmune diseases co-occurring within families) in patients with systemic sclerosis (SSc). A cross-sectional study of two convenience samples of patients with SSc, one in Canada and the other in Colombia, was performed. History of other autoimmune diseases in the SSc patients as well as a family history of autoimmunity was obtained. Of 719 patients, 273 (38%) had at least one other autoimmune disease. A total of 366 autoimmune diseases were reported, of which the most frequent were autoimmune thyroid disease (AITD, 38%), rheumatoid arthritis (RA, 21%), Sjögren's syndrome (18%), and primary biliary cirrhosis (4%). There were 260 (36%) patients with first-degree relatives with at least one autoimmune disease, of which the most frequent were RA (18%) and AITD (9%). Having at least one first-degree relative with autoimmune disease was a significant predictor of polyautoimmunity in SSc patients. No significant differences in polyautoimmunity or familial autoimmunity were noted between diffuse and limited subsets of disease. Our results indicate that polyautoimmunity is frequent in patients with SSc and autoimmune diseases cluster within families of these patients. Clinically different autoimmune phenotypes might share common susceptibility variants, which acting in epistatic pleiotropy may represent risk factors for autoimmunity.

Risk factors associated with pulmonary arterial hypertension in Colombian patients with systemic sclerosis: review of the literature.

OBJECTIVE: Considering the significant morbidity and mortality of pulmonary arterial hypertension (PAH) in patients with systemic sclerosis (SSc) and the lack of precise information on disease in Latin America, we investigated the clinical and laboratory characteristics associated with PAH in Colombian patients with SSc and review the literature. METHODS: This multicenter study included patients followed at 5 rheumatology units that were systematically assessed using a pretested questionnaire on clinical and immunological variables, focusing on PAH. Conditional logistic regression was employed to assess association between PAH and specific clinical characteristics. A systematic review of the literature was performed through electronic databases. RESULTS: Of a total of 349 patients with SSc, 61 (17%) met the criteria for PAH. Pulmonary fibrosis [adjusted odds ratio (AOR) 7.37, 95% CI 3.67-14.81, p < 0.0001], microstomia (AOR 3.3, 95% CI 1.70-6.28, p < 0.0001), gastroesophageal reflux (AOR 2.41, 95% CI 1.31-4.43, p = 0.005), dysphagia (AOR 2.7, 95% CI 1.49-4.77, p = 0.001), hyperpigmentation (AOR 2.15, 95% CI 1.11-4.16, p = 0.02), and hypopigmentation (AOR 2.4, 95% CI 1.26-4.64, p = 0.008) were the most prevalent clinical characteristics associated with PAH, while anemia (AOR 5.4, 95% CI 1.98-14.93, p = 0.001) was observed as the unique laboratory risk factor. Association between subtypes of SSc and PAH was not observed. Significant differences in both clinical and laboratory data were observed among different series. CONCLUSION: PAH may be a frequent complication of SSc in the Colombian population regardless of disease subtype. The identified clinical and laboratory risk factors might assist earlier diagnosis and guide decisions on therapeutic interventions on this critical complication of SSc. The reasons underlying the reported divergences among patients from different ethnicities are not fully understood, but it is most likely that both genetic and environmental factors are responsible for them.

Síndrome de anticuerpos antifosfolípidos asociado a la presencia de inhibidor adquirido del factor IX en una paciente sin hemofilia / Antiphospholipid antibody syndrome associated with acquired factor IX inhibitor in a non-hemophilic patient

Se presenta el caso de una paciente de 23 años de edad, con diagnóstico de síndrome de anticuerpos antifosfolípidos, con un cuadro típico de trombosis venosa profunda recurrente y pérdidas fetales, en presencia de anticoagulante lúpico y anticuerpos anticardiolipina positivos. En el transcurso de su enfermedad presenta manifestaciones hemorrágicas, no explicadas por el uso de anticoagulantes orales. Los estudios de hematología permitieron documentar una disminución de los factores IX y VIII, con evidencia de inhibidor específico del factor IX. La deficiencia de factores de la coagulación mejoró gracias al uso de esteroides y ciclofosfamida. (Acta Med Colomb 2011; 36: 196-199).

We report the case of a 23-year-old female patient with an antiphospholipid syndrome characterized by recurrent deep venous thromboses and fetal losses, with positive lupus anticoagulant and anticardiolipin antibodies. During the course of the disease she had hemorrhagic manifestations not attributable to oral anticoagulants. Hematological analyses documented reduced levels of factors IX and VIII, with evidence of a specific inhibitor of factor IX. The deficiency of clotting factors improved with steroids and cyclophosphamide. (Acta Med Colomb 2011; 36: 196-199).

Relación de los anticuerpos anti-péptido citrulinado con manifestaciones osteo-articulares en una cohorte de pacientes con esclerodermia / Relationship between anti-cyclic citrullinated protein antibodies and osteoarticular manifestations in a cohort of systemic sclerosis patients

Introducción: la esclerodermia es una alteración autoinmune caracterizada por induración y engrosamiento de la piel. Durante el curso de la enfermedad los pacientes pueden padecer síntomas de afectación musculoesquelética que incluyen artralgias, artritis, pérdida de función articular y discapacidad consecuente. Objetivos: se estudió la relación de las manifestaciones osteo-articulares clínicas y radiológicas con los anticuerpos anti-péptido citrulinado en una cohorte de pacientes con esclerodermia. Materiales y métodos: se evaluó la historia clínica de pacientes con esclerodermia buscando datos con características de la enfermedad, afectación de órgano blanco y síntomas osteo-articulares. A todos los pacientes se les realizó anticuerpos anti-péptido citrulinado de tercera generación y se les tomó radiografía de manos y pies. Resultados: se incluyeron en forma consecutiva 123 pacientes y se analizaron los datos de 100 pacientes. Ochenta y cuatro pacientes con esclerodermia limitada y 16 con esclerodermia difusa. El promedio de edad fue de 55,5 años y la mediana del tiempo de diagnóstico correspondió a 6,9 años. Catorce pacientes presentaron anticuerpos anti-péptido citrulinado positivos (14%), 4 pacientes con esclerodermia difusa y 9 pacientes con esclerodermia limitada. Dieciocho pacientes (18%) presentaron el factor reumatoide IgM positivo y no se observó relación entre la afectación de órgano blanco con los anticuerpos. El 72% de los pacientes presentaron síntomas osteo-articulares, 17 de ellos presentaron artralgias (23,6%). La combinación de artralgias y artritis se observó en 25 (34,7%) de los pacientes. Artralgias, artritis y contracturas en 10 (13,9%) pacientes. Dos pacientes presentaron artritis (2,8%), 3 contracturas (4,2%) y 5 calcinosis (6,9%). Analizando la asociación entre los anticuerpos antiCCP y la afectación clínica articular, no se encontró asociación estadísticamente significativa (p= 0,095). En el análisis radiológico se observó: En las manos con mayor frecuencia resorción ósea u osteolisis (72%). También se observó calcinosis (54%), aumento de tejidos blandos (49%) y contracturas en flexión (31%). En los pies se observó con mayor frecuencia resorción falángica u osteolisis (13%). Además calcinosis (10%), disminución del espacio articular (6%). Se evidenció una asociación de calcinosis con los antiCCP positivos. Conclusión: los hallazgos articulares fueron frecuentes en esta cohorte de pacientes con esclerodermia. La prevalencia de los anticuerpos antiCCP y factor reumatoide fueron de alrededor del 15%. Se observó más alteraciones radiológicas en las manos que los pies probablemente por factores mecánicos. Se observó una asociación de calcinosis en las manos con los antiCCP.

Introduction: scleroderma is an autoimmune disease characterized by induration and thickening of skin. Patients may suffer from musculoskeletal symptoms which include arthralgias, arthritis, loss of articular function and disability. Objectives: we sought to assess any association between radiographic features, osteoarticular manifestations, and anti-CCP antibodies in a cohort of scleroderma patients. Materials and methods: we evaluated the clinical features of patients searching for demographic characteristics, organ system involvement and osteoarticular symptoms. The presence of anti-CCP IgG was evaluated in all patients. Standard radiographs of hands and feet were taken and analyzed. Results: one hundred twenty three consecutive patients were included and 100 patients were analyzed in the present study. Eighty four patients had limited scleroderma and 16 patients had diffuse scleroderma. The mean age was 55.5 years and the median time of diagnostic was 6.9 years. Fourteen patients were positive for presence of anti-CCP (14%), 4 patient for diffuse form and 9 patients for limited form. Eighteen patients were positive for IgM rheumatoid factor test (18%). It was not seen an association between organ involvement and antibodies. Seventy two percent of patients showed osteoarticular symptoms, 17 of them had arthralgias (23.6%), 25 had arthralgias and arthritis (34.7%), and 10 had arhtralgias, arthritis and contractures (13.9%). Two patients showed arthritis (2.8%), three contractures (4.2%) and 5 calcinosis (6.9%). There was no association between the presence of anti-CCP antibodies and osteoarticular manifestations (p= 0.095). Radiographic features seen on hands were osteolysis (72%), calcinosis (54%), increasing of soft tissues (49%) and flexion contractures (31%). On feet was seen osteolysis (13%), calcinosis (10%) and decreasing of articular space (6%). There was association between calcinosis and anti-CCP antibodies. Conclusion: the articular findings were frequently seen in this scleroderma cohort. Prevalence of anti-CCP antibodies and rheumatoid factor was around 15% positive. Hands showed more alterations than feet, probably for mechanistic factors. It was seen an association between hand calcinosis and anti-CCP antibodies.

Descripción de un grupo de pacientes con poliserositis como manifestación temprana de lupus eritematoso sistémico

Es frecuente encontrar que los pacientes con lupus eritematoso sistémico (LES) presenten, durante el transcurso de la enfermedad, afección de las membranas serosas, principalmente la pleura y el pericardio. La poliserositis es, sin embargo, bastante rara como modo de presentación del LES, más aun cuando hay efección del peritoneo. Presentamos un grupo de cinco pacientes jóvenes con derrame pleural, pericárdico y ascitis como manifestación de una poliserositis lúpica temprana. Todos tuvieron compromiso sistémico y afección renal, que requirieron un tratamiento agresivo. Dos pacientes fallecieron (uno por complicaciones infecciosas y otro por causas no esclarecidas). La poliserositis temprana en el LES quizás constituya un marcador clínico de actividad de la enfermedad

Tratamiento de la artritis reumatoide en Colombia: aplicacion practica de los conceptos teoricos por parte de los reumatologos colombianos / RA treatment in Colombia

En la última década han ocurrido cambios dramáticos en el tratamiento de la artritis reumatoide (AR). En general, la tendencia es a tratar de manera más agresiva y tempranamente a los pacientes con AR utilizando diferentes Medicamentos Modificadores del Curso de la Artritis Reumatoide (DMARD). El objetivo del presente estudio es conocer la forma como actualmente es tratada la AR en Colombia por parte de los médicos reumatólogos del país. En una reunión nacional a la que acudieron 37 reumatólogos de todo el país, se les entregó un cuestionario con 23 preguntas sobre el tratamiento de la AR y la AR temprana, el cual llenaron todos los asistentes. Es la segunda vez que se aplica, siendo la primera en 1998. Los DMARD que actualmente utilizan más en su consulta son el Metotrexate (MTX), la cloroquina e hidroxicloroquina. En un paciente nuevo el DMARD más utilizado es MTX, siendo seguido por antimaláricos y terapia combinada (ej. MTX y antimaláricos). En relación con las nuevas terapias la leflunamida tiene utilización similar a la SSZ (18.9 por ciento). La SSZ no es tan utilizada como en Europa. Los biológicos no se utilizan como primera elección, sin embargo, 16.2 por ciento de los reumatólogos refieren utilizarlos como segunda elección. Los corticoides se utilizan con frecuencia como terapia de puente, por corto tiempo y a dosis bajas

Artropatía rápidamente destructiva asociada a cristales de hidroxiapatita: presentación de un caso

La artritis inducida por microcristales constituye un grupo heterogéneo de entidades caracterizadas clínicamente por episodios de inflamación inducida por la presencia de cristales dentro de la articulación. Se presenta un caso de artropatía rápidamente destructiva ocasionada por el depósito de cristales de hidroxiapatita, entidad que no había sido descrita en la literatua colombiana

Historia de las manifestaciones gastrointestinales del lupus eritematoso sistemico / History of the gastrointestinal manifestations in systemic lupus erythematosus

En este articulo revisamos la historia de las manifestaciones gastrointestinales en el Lupus Eritematoso Sistemico desde el siglo XIX hasta nuestros dias, recorriendo cada uno de los organos involucrados en este sistema y haciendo especial mencion de la gastropatia, enteritis, ileitis, sindrome de malabsorcion, vasculitis y vasculopatia intestinal, trombosis mesenterica, pancreatitis, ascitis, peritonitis, hepatitis autoinmune, entre otros

Enfermedad de Kawasaki y fenómeno de Raynaud en un paciente de 15 años de edad

La enfermedad de Kawasaki es una síndrome febril agudo acompañado por inyección conjuntival, exantema polimorfo, eritema e induración en manos, afección de la mucosa oral y linfadenopatías cervicales. Durante la fase de convalecencia se presenta descamación en palmas y plantas. Aunque esta entidad se manifiesta principalmente en niños menores de cinco años, también ha sido descrita en otros grupos etáreos. Describimos el caso de un individuo de 15 años de edad que cumple con todos los criterios diagnósticos y que tiene además la particularidad de presentar un fenomeno de Raynaud como manifestación asociada. Fue manjeado con gammaglobulina endovenosa y aspirina. No se identificaron complicaciones cardiovasculares. Es el primer caso descrito en un adolescente colombiano y el tercero informado en nuestra literatura.

Síndrome antifosfolípido/cofactor

Los anticuerpos antifosfolípidos representan un grupo heterogéneo de anticuerpos, que se presentan en el contexto de una patología autoinmune, primaria o secundaria, y que se asocian con un síndrome clínico bien definido, caracterizado por la tríada: trombosis, trombocitopenia y pérdida fetal recurrente