Correlation and agreement between physical and ultrasound examination after a training session dedicated to the standardization of synovitis assessment in rheumatoid arthritis patients.

OBJECTIVES: Assessing disease activity in rheumatoid arthritis (RA) patients requires comprehensive quantification of tender and swollen joints. We aimed to evaluate the correlation and agreement between rheumatologists after a training session dedicated to the standardization of synovitis assessment and compare its performance with a reference imaging modality such as musculoskeletal ultrasonography (MSUS). METHODS: In this cross-sectional study, a total of 28 and 10 joints in RA patients were evaluated by physical examination and ultrasound (US), respectively. After participating in a training session, individual joint assessment for tenderness and swelling was performed by three rheumatologists. MSUS examination was performed separately by an experimented radiologist in a standardized manner, evaluating findings according to the Outcome Measures in Rheumatology Clinical Trial (OMERACT) guidelines. RESULTS: A total of 80 RA patients were included, with a mean Disease Activity Score based on 28 joints (DAS28)-ESR of 4.02. The interobserver overall agreement and concordance rate in a total of 2240 joints assessed was 81.7% (k = 0.449, p < 0.0001) for tender joints and 66% (k = 0.227, p < 0.0001) for swollen joints. The overall concordance rate was fair (Fleiss' kappa = 0.21, p = 0.027) with an overall agreement of 67.18% yet, more joints were found to be swollen by the US assessment, compared to the physical examination (43% vs 39%). CONCLUSION: In our study population, joint tenderness showed better interobserver agreement, correlation, and concordance rate than joint swelling. When comparing the US assessment to the physical examination, a fair overall concordance rate supports the need for the implementation of training sessions dedicated to standardization in rheumatology clinics.

Does healthcare regime affiliation influence the clinical outcomes of patients with rheumatoid arthritis?

BACKGROUND/PURPOSE: Adequate control of disease activity in rheumatoid arthritis (RA) depends, to a great extent, on the access to a rheumatologist. This study aimed to compare the disease outcomes of patients with RA, based on their healthcare regime affiliation. METHODS: A retrospective observational study of Colombian patients with RA in three outpatient services of different regimes: Contributory (CR, workers and their families with a monthly income above a yearly defined threshold, approximately US$ 220, who allocate a percentage of their income to financing the national health fund and to get access to healthcare services), subsidized (SR, a vulnerable population with a monthly income below the threshold, who have access to healthcare through the national health fund; comparable to the USA Medicaid population), and an excellence clinical care center (C3, access to specialized care, regardless of their healthcare affiliation regime). Data were collected from clinical records for 2 years of follow-up and included demographics, lag times between appointments, and time in high disease activity. We used the Mantel-Cox test for the analysis of time to remission/low disease activity. RESULTS: A total of 240 patients were included (80 patients per regime). At the start of follow-up, mean age was 53.7 years; 21.6% of patients were men; 79.6% of patients had established RA; 72.9% of patients had high disease activity. Patients in the CR had longer lag times between scheduled appointments (p < 0.0001). During follow-up, SR had the highest proportion of patients with high disease activity. Survival curve analysis showed no significant difference between SR and CR groups (p = 0.2903), but was significantly different compared with the C3 group (p < 0.0001). Median survival in high disease activity was greater in the SR group (293 days), followed by CR (254 days), and finally by C3 (64 days). CONCLUSION: Patients that were treated in the excellence clinical care center had better outcomes when compared with other regimes. These data support that healthcare regime may influence disease outcome in patients with RA. Key Points • Prompt access to healthcare in patients with rheumatoid arthritis is pivotal for an adequate control of the disease, for timely adjustment of treatment, and to reduce both the societal burden of the disease and its impact on individual well-being. • As an example of "structural iatrogenesis," healthcare regime affiliation appears to influence disease outcomes in patients with rheumatoid arthritis, in whom differences between regimes are observed. The most vulnerable patients appear to experience the worst outcomes. • Excellence clinical care centers for patients with rheumatoid arthritis should be implemented as an alternative to counteract structural healthcare barriers and as an approach to improve clinical outcomes through a tighter disease control.

Association of Obesity With Lower Rates of Remission in a Colombian Cohort of Patients With Rheumatoid Arthritis.

OBJECTIVES: Modifiable risk factors associated with the severity of rheumatoid arthritis have been studied, including the body mass index (BMI). The aim was to compare the evolution of disease activity during 24 months of follow-up in different initial BMI groups of patients with rheumatoid arthritis. METHOD: Patients were classified based on their initial BMI (normal weight, overweight, and obese). Data were collected during 24 months of follow-up. At 24 months, they were reclassified based on their BMI. The proportion of patients in each BMI category was calculated. The mean differences between the initial and final DAS-28 (Disease Activity Score 28) were calculated using the Kruskal-Wallis test. Results were stratified based on sex and age. Survival analysis and Mantel-Cox test for the achievement of sustained remission during follow-up were calculated. RESULTS: A total of 269 patients were included. Most patients were at the normal weight category (n = 111). Normal weight group had the highest initial score (DAS-28, 4.01). Women present higher variability in BMI and greater disease activity compared with men. Based on age group, patients between the ages 31 and 50 years are more stable in their BMI, whereas those older than 50 years had lower BMI with time. Sustained remission was achieved by 58% of patients from the normal weight group, by 57% of patients from the overweight group, and by 42% of patients from the obese group. Survival curves of the initial normal and obese groups were significantly different (p = 0.0209). CONCLUSIONS: Patients with initial obesity were less likely to achieve remission compared with patients with initial overweight or normal weight. Sex and age affects disease activity and BMI variation.

Correlation and agreement between physical and ultrasound examination after a training session dedicated to the standardization of synovitis assessment in rheumatoid arthritis patients

Abstract Objectives: Assessing disease activity in rheumatoid arthritis (RA) patients requires comprehensive quantification of tender and swollen joints. We aimed to evaluate the correlation and agreement between rheumatologists after a training session dedicated to the standardization of synovitis assessment and compare its performance with a reference imaging modality such as musculoskeletal ultrasonography (MSUS). Methods: In this cross-sectional study, a total of 28 and 10 joints in RA patients were evaluated by physical examination and ultrasound (US), respectively. After participating in a training session, individual joint assessment for tenderness and swelling was performed by three rheumatologists. MSUS examination was performed separately by an experimented radiologist in a standardized manner, evaluating findings according to the Outcome Measures in Rheumatology Clinical Trial (OMERACT) guidelines. Results: A total of 80 RA patients were included, with a mean Disease Activity Score based on 28 joints (DAS28)-ESR of 4.02. The interobserver overall agreement and concordance rate in a total of 2240 joints assessed was 81.7% (k = 0.449, p < 0.0001) for tender joints and 66% (k = 0.227, p < 0.0001) for swollen joints. The overall concordance rate was fair (Fleiss' kappa = 0.21, p = 0.027) with an overall agreement of 67.18% yet, more joints were found to be swollen by the US assessment, compared to the physical examination (43% vs 39%). Conclusion: In our study population, joint tenderness showed better interobserver agreement, correlation, and concordance rate than joint swelling. When comparing the US assessment to the physical examination, a fair overall concordance rate supports the need for the implementation of training sessions dedicated to standardization in rheumatology clinics.

Pan-American League of Associations for Rheumatology (PANLAR) capillaroscopy study group consensus for the format and content of the report in capillaroscopy in rheumatology.

OBJECTIVE: The aim of this work was to produce a consensus-based report for capillaroscopy in rheumatology to be used in daily clinical practice. METHODS: A written Delphi questionnaire regarding capillaroscopy report was developed from a literature review and expert consensus. The Delphi questionnaire was sent to an international panel including 25 rheumatologists experts in capillaroscopy, asking them to rate their level of agreement or disagreement with each statement. The exercise consisted of three online rounds and a face-to-face (live meeting) that took place in the PANLAR 2018 congress held in Buenos Aires, Argentina. RESULTS: The participants to the first, second, third, and face-to-face round were 22, 21, 21, and 16 rheumatologists, respectively. Fifty-five items were discussed in the first round, 58 in the second, 22 in the third, and 9 in the face-to-face meeting. At the end of the exercise, 46 recommendations for the capillaroscopy report in rheumatology reached a consensus. CONCLUSION: This is the first consensus-based report in capillaroscopy. It will be useful in daily clinical practice and to address the effort of the standardization in the technique. KEY POINTS: • The current lack of consensus for the capillaroscopy report makes difficult the interpretation of findings as well as follow-up of rheumatic diseases. • This study produced the first international consensus for the format and content of the naifold capillaroscopy report in rheumatology. • The report is an integral part of the capillaroscopy examination and its use in a homogeneous form can help in the correct interpretation of findings in daily practice.

Rheumatoid arthritis and systemic lupus erythematosus: Pathophysiological mechanisms related to innate immune system.

Rheumatoid arthritis and systemic lupus erythematosus are two highly prevalent autoimmune diseases that generate disability and low quality of life. The innate immune system, a long-forgotten issue in autoimmune diseases, is becoming increasingly important and represents a new focus for the treatment of these entities. This review highlights the role that innate immune system plays in the pathophysiology of rheumatoid arthritis and systemic lupus erythematosus. The role of the innate immune system in rheumatoid arthritis and systemic lupus erythematosus pathophysiology is not only important in early stages but is essential to maintain the immune response and to allow disease progression. In rheumatoid arthritis, genetic and environmental factors are involved in the initial stimulation of the innate immune response in which macrophages are the main participants, as well as fibroblast-like synoviocytes. In systemic lupus erythematosus, all the cells contribute to the inflammatory response, but the complement system is the major effector of the inflammatory process. Detecting alterations in the normal function of these cells, besides its contribution to the understanding of the pathophysiology of autoimmune diseases, could help to establish new treatment strategies for these diseases.

Vaccination Recommendations for Adults With Autoimmune Inflammatory Rheumatic Diseases in Latin America.

BACKGROUND/OBJECTIVE: Patients with autoimmune inflammatory rheumatic diseases (AIRDs) are at increased risk of contracting severe infections and suffering complications, particularly when they are receiving immunomodulating therapy. Vaccination is an important means to prevent many potential infections and thereby reduce the morbidity and mortality associated with AIRD. The purpose of this consensus document is to provide health care professionals with recommendations for the vaccination of AIRD patients who reside in Latin America. The recommendations were developed by an expert committee from the region based on a review of the literature and their clinical experience. METHODS: The Americas Health Foundation (AHF) used PubMed and EMBASE to identify clinicians and scientists with an academic or hospital affiliation and who had published in the field of adult vaccination and rheumatic diseases since 2010. As a result of this effort, AHF convened an 8-member panel of clinical and scientific experts from Latin America. Both the AHF and panel members conducted a careful literature review to identify relevant publications in the areas of adult vaccination and rheumatology, and the sum of the articles identified was provided to the entire panel. Prior to the conference, panelists were each asked to prepare a written response to a salient issue on the subject, identified by AHF. RESULTS AND CONCLUSIONS: During the conference, each response was edited by the entire group, through numerous drafts and rounds of discussion until a complete consensus on vaccination recommendations for adult patients with AIRDs was obtained, including 7 key recommendations.

Caracterización de los perfiles de expresión del factor de transcripción ikaros en enfermedades autoinmunes / Characterization of the expression profiles of the autoimmune diseases ikaros transcription factor

Introducción y objetivo: Las enfermedades autoinmunes son patologías complejas asociadas a distinos genes que no logran explicar completamente estos sindromes. Ikaros es un factor de transcripción linfoide con un alto nivel de splicing alternativo, de las cuales resultan dis-tintas isoformas, entre ellas isoformas dominantes negativas. En este estudio caracterizamos el perfil de expresión de las isoformas de Ikaros en pacientes con síndrome de Sjögren, lupus eritematoso sistémico, esclerosis sistémica y artritis reumatoide.

Guías para la inmunización del adolescente y adulto en Colombia. Documento de actualización, 2016 / Guidelines for the immunization of adolescents and adults living in Colombia. Update document, 2016

Este documento fue preparado por un grupo multidisciplinario de expertos seleccionados por la Asociación Colombiana de Infectología (ACIN) para poner al día las recomendaciones previas dadas por nuestro grupo en cuanto a la inmunización del adolescente, de la población adulta y de aquellos mayores de 60 años de edad. Para este último grupo, hemos decidido, como lo han hecho en otros países, el inmunizar a esta edad (y no después), debido a la carga de enfermedad incrementada por afecciones respiratorias y otros factores propios para América Latina y las condiciones socioeconómicas de nuestro país. Esta edición reescribe ciertos párrafos y actualiza en parte las recomendaciones hechas anteriormente y publicadas en Infectio en mayo de 2012. Las guías están orientadas al uso por aquellos que cuidan de estos pacientes y hacemos énfasis en el anciano, el inmunocomprometido y en aquellos que sufren de varias comorbilidades. Aunque en un momento dado el documento pudiera parecer incompleto, la intención deseada fue la de abarcar los recientes cambios en la administración de nuevas vacunas y otros regímenes en dosificación. Se incluye por primera vez el uso de las vacunas de 4 valencias contra la influenza. El uso de la vacuna contra el herpes zóster se discute, y se toma un cuidado especial en cuanto a la redacción del "cuándo y por qué" de la vacunación contra Streptococcus pneumoniae. En la administración de esta vacuna, el tiempo de aplicación y la secuencia asociada con la aplicación de la administración de la vacuna polisacárida de 23 valencias puede variar de acuerdo con la edad del paciente,las comorbilidades y en aquellos previamente vacunados con dicha vacuna. Finalmente, exponemos las nuevas recomendaciones de vacunación contra fiebre amarilla y dengue y le damos la bienvenida a la vacuna nonavalente contra el virus humano del papiloma.

This document was prepared by a multi-disciplinary panel of experts who have been selected by the Asociación Colombiana de Infectologia (ACIN) to revise and update previous recommendations (by our group) for the immunization of adolescents and adult population and those older than 60 years of age. For the latter group, we have chosen to move forward, like many others, and immunize them at that age because of the particular burden of disease due to respiratory conditions, and other factors strictly related to Latin America and Colombian socio-economic conditions. This edition replaces in part, updates or ads to previous recommendations published in Infectio, May 2012. The guidelines are intended to assist those caring for these patients, and emphasizes on the elderly, the immunocompromissed and on those who suffer from several co-morbidities.The contents of the guidelines could seem in complete at some point; nevertheless, they were purposefully thought as such to embrace on major changes in new vaccines or new dosin gregimens. It is included for the first time the use of cuadri-valent vaccines against influenza. The use of herpes zoster vaccine is discussed and special care is placed in the phrasing for the reader so he (she) understands the "when and why" of vaccine administration against Streptococcus pneumoniae. With pneumococcal vaccines, timing of administration may vary according to age, co-morbidities and in those previously vaccinated with the 23-polyvalent polysaccharide vaccine. There are new recommendations for the vaccination against yellow fever and dengue and we welcome the new nona-valent vaccine against the human papillomavirus.

Gastric Antral Vascular Ectasia in Systemic Sclerosis: Current Concepts.

Introduction. Gastric antral vascular ectasia (GAVE) is a rare entity with unique endoscopic appearance described as "watermelon stomach." It has been associated with systemic sclerosis but the pathophysiological changes leading to GAVE have not been explained and still remain uncertain. Methods. Databases Medline, Scopus, Embase, PubMed, and Cochrane were searched for relevant papers. The main search words were "Gastric antral vascular ectasia," "Watermelon Stomach," "GAVE," "Scleroderma," and "Systemic Sclerosis." Fifty-four papers were considered for this review. Results. GAVE is a rare entity in the spectrum of manifestations of systemic sclerosis with unknown pathogenesis. Most patients with systemic sclerosis and GAVE present with asymptomatic anemia, iron deficiency anemia, or heavy acute gastrointestinal bleeding. Symptomatic therapy and endoscopic ablation are the first-line of treatment. Surgical approach may be recommended for patients who do not respond to medical or endoscopic therapies. Conclusion. GAVE can be properly diagnosed and treated. Early diagnosis is key in the management of GAVE because it makes symptomatic therapies and endoscopic approaches feasible. A high index of suspicion is critical. Future studies and a critical review of the current findings about GAVE are needed to understand the role of this condition in systemic sclerosis.

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.

Serum endoglin levels in patients suffering from systemic sclerosis and elevated systolic pulmonary arterial pressure.

Background. Pulmonary arterial hypertension (PAH) is the main cause of morbimortality in systemic sclerosis (SSc). Increased Eng expression has been demonstrated in SSc patients. Objective. Ascertaining serum levels of Eng in SSc patients with and without elevated systolic pulmonary arterial pressure (sPAP) and comparing them with that of healthy volunteers. Methods. A cross-sectional study was carried out. A commercial ELISA kit was used for measuring serum concentrations of Eng in 60 subjects: 40 patients with SSc with and without elevated sPAP, compared to 20 healthy control subjects. Elevated sPAP was detected by echocardiogram. Results. No association between positive Eng and elevated sPAP was found when compared to the SSc without elevated sPAP group (OR = 2.85; 0.65-12.88 95% CI; P = .11); however, an association was found between positive Eng and elevated sPAP compared to healthy controls (OR = 23.22; 2.46-1050.33 95% CI; P = .001), and weak association was found between the positive Eng with SSc without elevated sPAP group compared to healthy controls (OR = 8.14, 0.8-393.74 95% CI; P = .046). Conclusion. Raised serum levels of Eng in SSc patients compared to healthy controls were found, suggesting a role for Eng in SSc vasculopathy and not just in elevated sPAP. However, prospective studies are needed to verify such observations.

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.

Systemic sclerosis: a world wide global analysis.

The objective of this study was to analyze epidemiological tendencies of systemic sclerosis (SSc) around the world in order to identify possible local variations in the presentation and occurrence of the disease. A systematic review of the literature was performed through electronic databases using the keywords "Systemic Sclerosis" and "Clinical Characteristics." Out of a total of 167 articles, 41 were included in the analysis. Significant differences in the mean age at the time of diagnosis, subsets of SSc, clinical characteristics, and presence of antibodies were found between different regions of the word. Because variations in both additive and nonadditive genetic factors and the environmental variance are specific to the investigated population, ethnicity and geography are important characteristics to be considered in the study of SSc and other autoimmune diseases.

Potential biomarkers for detecting pulmonary arterial hypertension in patients with systemic sclerosis.

Pulmonary arterial hypertension (PAH) is the major complication of systemic sclerosis (SSc) and the main cause of morbi-mortality. It is important to find predictors for this vascular problem. The objective of this study was to determine the serum levels of different biomarkers in patients with SSc and secondary PAH and to compare them with those of healthy control subjects to define their potential role as predictors of PAH. Cross-section study in which 20 patients with SSc were included. PAH was diagnosed by echocardiogram. The optical densities of endoglin (Eng), endothelin-1 (ET-1), platelet-derived growth factor (PDGF), tumoral necrosis factor alpha (TNF-alpha), Transforming growth factor beta 2 (TGF-beta2) and Interleukin 8 (IL-8) were measured in 20 patients with SSc and 20 healthy controls matched by sex. The differences found between the group of patients with PAH and the control group were (mean or median and range): ET-1 (0.20; 0.10-0.35 vs. 0.16; 0.10-0.24; P = 0.0276), IL-8 (195.7; 45.5-504 vs. 118.9; 23-299.5; P = 0.0364), TNF-alpha (0.70; 0.50-0.96 vs. 0.48; 0.38-0.65; P = 1 x 10(-8)) and Eng (0.95; 0.57-1.72 vs. 0.75; 0.57-0.89; P = 0.0028). A correlation was found between the progression of the disease and the development of Raynaud's phenomenon (Rho: 0.67 and P = 0.0011), ET-1 and Eng (Rho: 0.53 and P = 0.0196), and between IL-8 and Eng (Rho: 0.68 and P = 0.0019). In conclusions, the elevation of the serum levels of Eng and ET-1 could represent a useful tool as PAH biomarkers. Nevertheless, the diagnostic value of these markers needs to be determined by prospective studies.

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.

Acrosteolisis severa en artritis gotosa y síndrome de Down / Severe acroosteolysis in gouty arthritis and Down syndrome

La asociación de gota y síndrome de Down (SD) es poco frecuente a pesar que ambas enfermedades son comunes en la población general. Más interesante aún es el hecho que la hiperuricemia sí es una característica frecuente entre las alteraciones metabólicas del SD. A continuación se informa el caso de un paciente de sexo masculino de 35 años con SD que consultó por artritis de tres años de evolución, inicial-mente en el quinto y luego en el primer dedo del pie izquierdo. Debido a la presencia de osteolisis severa en el primer y quinto dedo en la radiografía inicial del pie, se consideró patología tumoral, lo cual, sin embargo, fue descartado por ortopedia oncológica. Posteriormente se realizó el diagnóstico de artritis gotosa al encontrar hiperuricemia y confirmación de cristales de ácido úrico en una muestra de líquido extraída de un nódulo subcutáneo encontrado en el tobillo izquierdo. Este caso hace parte de los escasos informes que relacionan el SD y la gota. Adicionalmente el desarrollo de gota en este paciente tiene características inusuales como el sitio de inicio de la enfermedad y la severidad de la presentación. No es claro porque el desarrollo de gota en pacientes con SD es infrecuente.

The association between gout and Down Syndrome (DS) is very infrequent, in spite that both diseases are common in general population. Surprisingly, hyperuricemia is a common metabolic impairment in DS. In this report we describe a 35-years old man with DS presenting with arthritis in the fifth and then in the first toe of his left foot. Severe osteolysis of the first and fifth toe was seen by radiography. Because of this, neoplasm was suspected but later ruled out by oncologic orthopedist. After we found hyperuricemia a diagnosis of gout was made, and then confirmed by examination of a sample obtained from a subcutaneous node. This report is one of the few cases previously published. In addition, development of gout in this patient has unusual features like its onset in the fifth toe and its severity at presentation. In spite that hyperuricemia is common in DS, in not clear why these patients mostly do not develop gout.