ABSTRACT
Abstract Background: Pulmonary involvement in juvenile systemic sclerosis (JSSc) is rare in children and contributes to morbimortality. This study aimed to describe the pulmonary function and clinical, radiologic, and tomographic findings in JSSc. Methods: Patients with JSSc between 5-14 years of age were included. Clinical, functional, and imaging characteristics were assessed. Patients were excluded if they showed lung disease not associated with JSSc: mixed connective tissue disease, overlap syndrome, or acute cardiopulmonary failure at the time of the study. All patients underwent physical examination, electrocardiogram, spirometry, chest X-ray, high-resolution computed tomography (HRCT) of the chest, echocardiography, lung function tests, and the 6-minute walk test (6-MWT). Descriptive statistics were employed for data analysis. Results: We studied 15 patients with the following characteristics: median age, 11 years; median since symptoms onset, 6 years; median since JSSc diagnosis and the finding of pulmonary involvement, 2 years. Lung disease was detected in 73%, interstitial lung disease (ILD) the most common affection (67%); pulmonary hypertension was found in 6.6%. 6-MWT was positive in 26.6%, forced vital capacity (FVC) was abnormal in 26.6%. No pulmonary involvement was found in four patients. Conclusions: The most frequent pulmonary affection in JSSc was ILD. Thus, early JSSc detection and periodic lung monitoring are mandatory to avoid further complications once JSSc is diagnosed.
Resumen Introducción: La afección pulmonar en la esclerosis sistémica juvenil (ESJ) es rara en niños y contribuye a la morbimortalidad. El objetivo de este estudio fue describir los hallazgos de función pulmonar, clínicos, radiológicos y tomográficos en la ESJ. Métodos: Se incluyeron pacientes con ESJ de 5-14 años de edad. Se evaluaron las características clínicas, funcionales y de imagen. No se incluyeron pacientes con enfermedades pulmonares no asociadas con ESJ en el momento del estudio: enfermedad mixta del tejido conectivo, síndrome de superposición o insuficiencia cardiopulmonar aguda. Se realizaron exploración física, electrocardiograma, espirometría, radiografía de tórax, tomografía computarizada de alta resolución de tórax, ecocardiografía, pruebas de función pulmonar y prueba de caminata de 6 minutos (PC6M). Se utilizó estadística descriptiva para el análisis de los datos. Resultados: Se estudiaron 15 pacientes con las siguientes características: mediana de edad, 11 años; mediana desde el inicio de los síntomas, 6 años; y mediana desde el diagnóstico de ESJ y hallazgo de afección pulmonar, 2 años. Se detectó enfermedad pulmonar en el 73%. La enfermedad pulmonar intersticial (EPI) fue la afección más común (67%) y se encontró hipertensión pulmonar en el 6.6%. La PC6M fue positiva en el 26.6%, y la capacidad vital forzada resultó anormal en el 26.6%. Cuatro pacientes no presentaron afección pulmonar. Conclusiones: La afección pulmonar más frecuente en la ESJ fue la EPI. La detección temprana de ESJ y la monitorización pulmonar periódica son obligatorias para evitar más complicaciones una vez diagnosticada la ESJ.
ABSTRACT
Background: Pulmonary involvement in juvenile systemic sclerosis (JSSc) is rare in children and contributes to morbimortality. This study aimed to describe the pulmonary function and clinical, radiologic, and tomographic findings in JSSc. Methods: Patients with JSSc between 5-14 years of age were included. Clinical, functional, and imaging characteristics were assessed. Patients were excluded if they showed lung disease not associated with JSSc: mixed connective tissue disease, overlap syndrome, or acute cardiopulmonary failure at the time of the study. All patients underwent physical examination, electrocardiogram, spirometry, chest X-ray, high-resolution computed tomography (HRCT) of the chest, echocardiography, lung function tests, and the 6-minute walk test (6-MWT). Descriptive statistics were employed for data analysis. Results: We studied 15 patients with the following characteristics: median age, 11 years; median since symptoms onset, 6 years; median since JSSc diagnosis and the finding of pulmonary involvement, 2 years. Lung disease was detected in 73%, interstitial lung disease (ILD) the most common affection (67%); pulmonary hypertension was found in 6.6%. 6-MWT was positive in 26.6%, forced vital capacity (FVC) was abnormal in 26.6%. No pulmonary involvement was found in four patients. Conclusions: The most frequent pulmonary affection in JSSc was ILD. Thus, early JSSc detection and periodic lung monitoring are mandatory to avoid further complications once JSSc is diagnosed.
Introducción: La afección pulmonar en la esclerosis sistémica juvenil (ESJ) es rara en niños y contribuye a la morbimortalidad. El objetivo de este estudio fue describir los hallazgos de función pulmonar, clínicos, radiológicos y tomográficos en la ESJ. Métodos: Se incluyeron pacientes con ESJ de 5-14 años de edad. Se evaluaron las características clínicas, funcionales y de imagen. No se incluyeron pacientes con enfermedades pulmonares no asociadas con ESJ en el momento del estudio: enfermedad mixta del tejido conectivo, síndrome de superposición o insuficiencia cardiopulmonar aguda. Se realizaron exploración física, electrocardiograma, espirometría, radiografía de tórax, tomografía computarizada de alta resolución de tórax, ecocardiografía, pruebas de función pulmonar y prueba de caminata de 6 minutos (PC6M). Se utilizó estadística descriptiva para el análisis de los datos. Resultados: Se estudiaron 15 pacientes con las siguientes características: mediana de edad, 11 años; mediana desde el inicio de los síntomas, 6 años; y mediana desde el diagnóstico de ESJ y hallazgo de afección pulmonar, 2 años. Se detectó enfermedad pulmonar en el 73%. La enfermedad pulmonar intersticial (EPI) fue la afección más común (67%) y se encontró hipertensión pulmonar en el 6.6%. La PC6M fue positiva en el 26.6%, y la capacidad vital forzada resultó anormal en el 26.6%. Cuatro pacientes no presentaron afección pulmonar. Conclusiones: La afección pulmonar más frecuente en la ESJ fue la EPI. La detección temprana de ESJ y la monitorización pulmonar periódica son obligatorias para evitar más complicaciones una vez diagnosticada la ESJ.
Subject(s)
Lung Diseases, Interstitial , Scleroderma, Localized , Scleroderma, Systemic , Child , Humans , Lung/diagnostic imaging , Respiratory Function Tests , Scleroderma, Systemic/complications , Scleroderma, Systemic/diagnosisABSTRACT
BACKGROUND: Small intestinal bacterial overgrowth (SIBO) affects up to 60% of patients with systemic sclerosis (SSc), and it improves with antibiotics. The addition of probiotics could lead to better results. AIMS: To evaluate the efficacy and safety of Saccharomyces boulardii (SB) versus metronidazole (M) versus M + SB for 2 months, to reduce gastrointestinal symptoms and SIBO assessed with hydrogen breath test in SSc. METHODS: An open pilot clinical trial performed in forty patients with SIBO and SSc (ACR-EULAR 2013) who signed informed consent. Three groups were assigned: M, SB, and M + SB, for 2 months. Hydrogen was measured in parts per million with a hydrogen breath test to evaluate SIBO. The National Institutes of Health Patient-Reported Outcomes Measurement Information System (NIH-PROMIS) questionnaire was applied to quantify gastrointestinal symptoms with a raw score of eight symptoms. This study is registered in ClinicalTrials.gov with the following ID: NCT03692299. RESULTS: Baseline characteristics were similar between groups. The average age was 53.2 ± 9.3 years, and the evolution of SSc was 13.5 (1-34) years. After 2 months of treatment, SIBO was eradicated in 55% of the M + SB group: 33% of SB, and 25% of M. The SB and M + SB groups had decreased diarrhea, abdominal pain, and gas/bloating/flatulence, but M remained unchanged. Reductions in expired hydrogen at 45 to 60 min were as follows: M + SB 48% and 44%, M 18% and 20%, and SB 53% and 60% at the first and second months, respectively (p < 0.01). Adverse effects were epigastric burning and constipation in M (53%) and M + SB (36%), and flatulence/diarrhea in SB (22%). CONCLUSIONS: Metronidazole treatment is partially effective in SIBO, but S. boulardii in monotherapy or in combination improves the gastrointestinal outcomes in SSc.
Subject(s)
Bacterial Infections/therapy , Intestine, Small/microbiology , Metronidazole/administration & dosage , Saccharomyces boulardii , Scleroderma, Systemic/microbiology , Scleroderma, Systemic/therapy , Adult , Anti-Bacterial Agents/administration & dosage , Bacterial Infections/diagnosis , Female , Humans , Intestine, Small/drug effects , Male , Middle Aged , Pilot Projects , Probiotics/administration & dosage , Scleroderma, Systemic/diagnosis , Treatment OutcomeABSTRACT
Autoimmune/inflammatory syndrome induced by adjuvants (ASIA) encompassing conditions linked to previous exposure to an adjuvant substance. The clinical picture is very heterogeneous, from mild to severe manifestations, including death. However, the systematic analysis of severe ASIA cases has not been performed. The aim of this study was to systematically review the literature on severe ASIA cases. A systematic review of the literature was performed investigating severe ASIA cases. All publications were identified through PubMed, EMBASE, MEDLINE and Cochrane. Articles published from 2011 to 2016 were included. Severe ASIA was arbitrarily defined as follows: major organ involvement, life-threatening conditions, intensive treatment, disability, hospitalization and outcome (survival and death). Cases described before 2011 were excluded. From 2011 to 2016, we identified 4479 ASIA cases, of them 305 fulfilled arbitrary criteria of severe ASIA including our case presentation and 11 deaths. The majority of severe ASIA cases were related to HPV vaccine, silicone, influenza vaccine and mineral oil injections. The interval from exposition to severe manifestation was from 2 days to 23 years. (1) This is the first study that analyzes all cases published on ASIA with severe manifestations. (2) The current HPV vaccine is both effective and generally safe. However, it should be noted that severe autoimmune side effects have been reported in several studies. Severe ASIA may be observed after influenza vaccines, and other vaccines. (3) Efforts should be made to discover the connection between adjuvants, autoimmunity and autoimmune diseases, because there is an increase in cases severe and life-threatening of ASIA.
