Characterization of Venezuelan patients with systemic sclerosis: a study in a tertiary hospital in Caracas.

OBJECTIVE: The aim of the study was to investigate the demographic and clinical characteristics of Venezuelan patients with systemic sclerosis (SSc) seen in a tertiary hospital. METHODS: Consecutive patients 18 years and older who fulfilled the 2013 ACR/EULAR classification criteria for SSc and who were followed up in the outpatient clinic of the Division of Rheumatology at the Hospital Universitario de Caracas were selected for the study. Demographic and clinical variables were registered at the time of inclusion using a standard protocol. RESULTS: Forty-eight SSc patients were included; 46 (95.8%) were female; the mean age was 55.1±13.7 (mean±SD) years and all were of Hispanic ethnicity. Thirty-one (64.6%) had limited SSc and 17 (35.4%) had diffuse SSc. The mean duration of disease was 13.4±11.7 (mean±SD) years, 16.74±12.99 years for limited SSc and 7.52±5.25 years for diffuse SSc (p=0.0077). Raynaud's phenomenon was the most frequent manifestation (100%), followed by arthritis (68.8%), telangiectasia (60.4%), dyspnea (60.4%), dysphagia (58.3%) and puffy hands (56.3%). The modified Rodnan Skin Score (mRSS) and the frequency of dyspnea were higher in those with diffuse as compared to limited SSc (p=0.0211 and p=0.0003, respectively). We performed high-resolution computed tomography (HRCT) of the lungs in 31 patients; 14 (45.2%) had evidence of interstitial lung disease (ILD), 11 (68.8%) with diffuse SSc (p=0.0052). The most frequent anti-nuclear antibody pattern was nucleolar, accounting for 18 (42.8%) of the cases. Anti-centromere antibodies were present in 16.7% of the cases and were associated with the limited SSc subset (p=0.0443) and with calcinosis (p=0.0020). Anti-topoisomerase antibodies were associated with ILD (p=0.0077). CONCLUSIONS: Typical clinical and serological manifestations were present in this sample of Venezuelan patients with SSc, with an expected distribution according to disease subtype. The autoantibody profile allows clinicians to identify those patients with limited forms of the disease and those without pulmonary involvement.

Characterization of Venezuelan Patients With Systemic Sclerosis: A Study in a Tertiary Hospital in Caracas.

OBJECTIVE: The aim of the study was to investigate the demographic and clinical characteristics of Venezuelan patients with systemic sclerosis (SSc) seen in a tertiary hospital. METHODS: Consecutive patients 18 years and older who fulfilled the 2013 ACR/EULAR classification criteria for SSc and who were followed up in the outpatient clinic of the Division of Rheumatology at the Hospital Universitario de Caracas were selected for the study. Demographic and clinical variables were registered at the time of inclusion using a standard protocol. RESULTS: Forty-eight SSc patients were included; 46 (95.8%) were female; the mean age was 55.1±13.7 (mean±SD) years and all were of Hispanic ethnicity. Thirty-one (64.6%) had limited SSc and 17 (35.4%) had diffuse SSc. The mean duration of disease was 13.4±11.7 (mean±SD) years, 16.74±12.99 years for limited SSc and 7.52±5.25 years for diffuse SSc (p=0.0077). Raynaud's phenomenon was the most frequent manifestation (100%), followed by arthritis (68.8%), telangiectasia (60.4%), dyspnea (60.4%), dysphagia (58.3%) and puffy hands (56.3%). The modified Rodnan Skin Score (mRSS) and the frequency of dyspnea were higher in those with diffuse as compared to limited SSc (p=0.0211 and p=0.0003, respectively). We performed high-resolution computed tomography (HRCT) of the lungs in 31 patients; 14 (45.2%) had evidence of interstitial lung disease (ILD), 11 (68.8%) with diffuse SSc (p=0.0052). The most frequent anti-nuclear antibody pattern was nucleolar, accounting for 18 (42.8%) of the cases. Anti-centromere antibodies were present in 16.7% of the cases and were associated with the limited SSc subset (p=0.0443) and with calcinosis (p=0.0020). Anti-topoisomerase antibodies were associated with ILD (p=0.0077). CONCLUSIONS: Typical clinical and serological manifestations were present in this sample of Venezuelan patients with SSc, with an expected distribution according to disease subtype. The autoantibody profile allows clinicians to identify those patients with limited forms of the disease and those without pulmonary involvement.

Characterization of Venezuelan patients with systemic sclerosis: a study in a tertiary hospital in Caracas / Caracterización de los pacientes venezolanos con esclerosis sistémica: estudio en un hospital terciario en Caracas

Objective: The aim of the study was to investigate the demographic and clinical characteristics of Venezuelan patients with systemic sclerosis (SSc) seen in a tertiary hospital. Methods: Consecutive patients 18 years and older who fulfilled the 2013 ACR/EULAR classification criteria for SSc and who were followed up in the outpatient clinic of the Division of Rheumatology at the Hospital Universitario de Caracas were selected for the study. Demographic and clinical variables were registered at the time of inclusion using a standard protocol. Results: Forty-eight SSc patients were included; 46 (95.8%) were female; the mean age was 55.1±13.7 (mean±SD) years and all were of Hispanic ethnicity. Thirty-one (64.6%) had limited SSc and 17 (35.4%) had diffuse SSc. The mean duration of disease was 13.4±11.7 (mean±SD) years, 16.74±12.99 years for limited SSc and 7.52±5.25 years for diffuse SSc (p=0.0077). Raynaud's phenomenon was the most frequent manifestation (100%), followed by arthritis (68.8%), telangiectasia (60.4%), dyspnea (60.4%), dysphagia (58.3%) and puffy hands (56.3%). The modified Rodnan Skin Score (mRSS) and the frequency of dyspnea were higher in those with diffuse as compared to limited SSc (p=0.0211 and p=0.0003, respectively). We performed high-resolution computed tomography (HRCT) of the lungs in 31 patients; 14 (45.2%) had evidence of interstitial lung disease (ILD), 11 (68.8%) with diffuse SSc (p=0.0052). The most frequent anti-nuclear antibody pattern was nucleolar, accounting for 18 (42.8%) of the cases. Anti-centromere antibodies were present in 16.7% of the cases and were associated with the limited SSc subset (p=0.0443) and with calcinosis (p=0.0020). Anti-topoisomerase antibodies were associated with ILD (p=0.0077).(AU)

Objetivo: El objetivo de este estudio fue investigar las características demográficas y clínicas de los pacientes venezolanos con esclerosis sistémica (ES) examinados en un hospital terciario. Métodos: Se seleccionaron para el estudio pacientes consecutivos mayores de 18 años, que cumplieron los criterios de la clasificación ACR/EULAR de 2013 para ES, sometidos a seguimiento en la clínica ambulatoria del Departamento de Reumatología del Hospital Universitario de Caracas. Se registraron las variables demográficas y clínicas en el momento de la inclusión, utilizando un protocolo estándar. Resultados: Se incluyó a 48 pacientes con ES, de los cuales 46 (95,8%) eran mujeres; la edad media fue de 55,1±13,7 (media±DE) años, todos ellos de etnia hispana. Treinta y uno de ellos (64,6%) tenían ES limitada y 17 (35,4%) ES difusa. La duración media de la enfermedad fue de 13,4±11,7 (media±DE) años: 16,74±12,99 años para ES limitada y 7,52±5,25 años para ES difusa (p=0,0077). El fenómeno de Raynaud fue la manifestación más frecuente (100%), seguida de la artritis (68,8%), la telangiectasia (60,4%), la disnea (60,4%), la disfagia (58,3%) y la hinchazón de manos (56,3%). La puntuación de la escala cutánea de Rodnan modificada (mRSS) y la frecuencia de la disnea fueron más altas en aquellos sujetos con ES difusa con respecto a los sujetos con ES limitada (p=0,0211 y p=0,0003, respectivamente). Realizamos una TAC de alta resolución pulmonar a 31 pacientes; 14 (45,2%) tenían evidencia de enfermedad pulmonar intersticial (EPI), 11 (68,8%) de ellos con ES difusa (p=0,0052). El patrón más frecuente de anticuerpos anti-nucleares fue de tipo nucleolar, representando 18 (42,8%) de los casos. Los anticuerpos anti-centrómeros estuvieron presentes en el 16,7% de los casos, asociándose al subconjunto de ES limitada (p=0,0443) con calcinosis (p=0,0020). Los anticuerpos anti-topoisomerasa estuvieron asociados a la EPI (p=0,0077).(AU)

Analysis of body mass index and risk factors of interstitial lung disease in systemic sclerosis patients / Análisis del índice masa corporal y factores de riesgo de neumopatía intersticial en pacientes con esclerosis sistémica

ABSTRACT Objective: To determine the risk factors and the potential relationship between body mass index (BMI) and the development of interstitial lung disease (ILD) in patients with systemic sclerosis. Methods: A cross-sectional, correlational, study with a non-probabilistic sample was conducted on consecutive patients with scleroderma seen in the Rheumatology Division at Hospital Universitario de Caracas during the period April to September 2018. Patients underwent clinical and laboratory assessments, anthropometric measurements, pulmonary function tests, and high-resolution computed axial tomography scan of the chest. Results: Of 48 patients, 95.8% were female, with a high prevalence of limited cutaneous systemic sclerosis (lcSSc) (64.6%). Underweight (BMI<18.5kg/m2) was a risk factor for the development of ILD (OR 4.60; 95% CI 1.12-18.86). There was directly proportional relationship between BMI and forced vital capacity, with no statistical significance (CC 0.23; p = 0.1180). However, other predictors of ILD included diffuse systemic sclerosis (OR 6.91,95% CI; 1.78-26.85), modified Rodnan skin score (mRSS) (OR 1.25; 95% CI; 1.04-1.30), and a speckled pattern of antinuclear antibody (OR 5.62; 95% CI; 1.33-23.62). Multivariate analysis showed that mRSS (OR 1.34; 95% CI; 1.04-1.72) and the presence of a speckled antinuclear antibody (OR 25.99; 95% CI; 1.02-16.02) were independent predictors of ILD. The percentage of BMI lost in a period of time of 2008-2018 was 14.3% (p = 0.0021), which was higher than rheumatoid arthritis (p = 0.0000), systemic lupus erythematosus (p = 0.0025), and healthy patients (p = 0.0331). Conclusion: BMI and ILD are inversely correlated. Age, underweight, higher mRSS, and speckled pattern of antinuclear antibody were risk factors for development of ILD in patients with systemic sclerosis. The mRSS could be used as a clinical predictive tool for ILD in systemic sclerosis.

RESUMEN Objetivo: Determinar los factores de riesgo y la asociación entre el índice de masa corporal (IMC) y enfermedad pulmonar intersticial difusa (EPID) en pacientes con esclerosis sistémica. Métodos: Estudio transversal, correlacional, con muestra no probabilística constituida por pacientes consecutivos con esclerosis sistémica que acudieron al Servicio de Reumatología del Hospital Universitario de Caracas durante el período abril-septiembre de 2018. Se realizó evaluación clínica, paraclínica, mediciones antropométricas, pruebas de función pulmonar y tomografía de tórax de alta resolución. Resultados: De 48 pacientes, el 95,8% fueron del sexo femenino, con mayor prevalencia de esclerosis sistémica limitada (64,6%). Un peso bajo (IMC < 18,5 kg/m2) fue un factor de riesgo de EPID (OR 4,60; IC 95% 1,12-18,86). El IMC se relacionó de forma directamente proporcional con la capacidad vital forzada sin alcanzar significación estadística (CC 0,23; p = 0,1180), sin embargo, otros predictores de EPID fueron: la esclerosis sistémica difusa (OR 6,91; IC 95% 1,78-26,85), el índice modificado de Rodnan (mRSS) (OR 1,25; IC 95% 1,04-1,30) y el patrón moteado de anticuerpos antinucleares (OR 5,62; IC 95% 1,33-23,62). El análisis multi-variado demostró asociación independiente con EPID de: mRSS (OR 1,34; IC 95% 1,04-1,72) y el patrón moteado de anticuerpos antinucleares (OR 25,99; IC 95% 1,02-16,02). El estudio del IMC durante 10 arios (2008-2018) reveló una pérdida de 14,3% (p = 0,0021), que fue mayor en comparación con pacientes con artritis reumatoide (p = 0,0000), lupus eritematoso sistémico (p = 0,0025) e individuos sanos (p = 0,0331). Conclusión: El IMC y la EPID se correlacionan de forma inversamente proporcional. La edad, un bajo peso corporal, un puntaje alto en mRSS y el patrón moteado de anticuerpos antinucleares fueron factores de riesgo para el desarrollo de EPID en pacientes con esclerosis sistémica. El mRSS podría ser utilizado como herramienta clínica predictiva de EPID en esclerosis sistémica.

Diagnóstico y manejo de la enfermedad pulmonar intersticial de la esclerosis sistémica en condiciones sanitarias desafiantes: Reporte de un caso del Hospital Universitario de Caracas / Diagnosis and management of the interstitial lung disease of systemic sclerosis in challenging public health conditions: Case report of the Caracas University Hospital

Una mujer de 51 años presentó tumefacción en ambas manos y episodios de Raynaud trifásico de tres meses de evolución. La paciente negaba tos y disnea. En el examen físico se evidenció manos tumefactas, telangiectasias faciales y lesiones en sal y pimienta. No presentaba fibrosis de piel. La determinación de anticuerpos antinucleares y antitopoisomerasa fueron positivos. Una espirometría demostró una capacidad vital forzada (CVF) de 86% del predicho. La tomografía computarizada de tórax de alta resolución (TACAR) reveló opacidades en vidrio esmerilado bilaterales, basales y subpleurales. Estos hallazgos fueron compatibles con enfermedad pulmonar intersticial no específica (EPID) secundaria a esclerosis sistémica (ES). Además, debido a la ausencia de fibrosis de piel se diagnosticó esclerodermia sine esclerodermia. Se indicaron infusiones de ciclofosfamida endovenosa en intervalos de 4 semanas, seguido de azatioprina vía oral. Este caso ilustra que el uso de pruebas de funcionalismo pulmonar como método de detección único para la EPID puede hacer que los médicos clínicos no diagnostiquen un número representativo de pacientes, y que además, la ausencia de síntomas respiratorios no descarta la enfermedad pulmonar en pacientes con CVF normal. El pronóstico sombrío de la EPID es una indicación para la búsqueda sistémica y la realización un diagnóstico precoz.

A 51-year-old woman presented with swelling in both hands and a 3-months history of triphasic Raynaud phenomenon. She denied cough and dyspnea. The physical examination was notable for swollen hands, facial telangiectasia and salt and pepper lesions. She had no skin fibrosis. The determination of antinuclear antibodies and antitopoisomerase were positive. A spirometry demonstrated a forced vital capacity of 86% of the predicted. High resolution chest computed tomography revealed bilateral, basal and subpleural ground glass opacities. These findings were consistent with the diagnosis of Scleroderma-Related Interstitial Lung Disease. Moreover, due to the absence of skin fibrosis, a diagnosis of systemic sclerosis sine scleroderma was made. Infusions of endovenous cyclophosphamide were indicated at 4-week intervals, followed by oral azathioprine. This case showed that using PFT as the single screening method for SSc-ILD may cause clinicians to miss a significant number of patients and that the absence of pulmonary symptoms does not exclude lung disease in patients with normal FVC. The poor prognosis of SSc-ILD is an indication of systematic screening for early diagnosis.