Ultrasound assessment of diaphragm and quadriceps muscles and its relationship with handgrip and respiratory muscle strength in patients with systemic sclerosis: a cross-sectional study.

BACKGROUND: Muscle dysfunction may cause disability and reduce the quality of life of patients with systemic sclerosis (SSc) when compared to healthy individuals. However, the literature on the topic is scarce and uses several criteria for assessing muscle dysfunction in this population. OBJECTIVES: To compare diaphragm and quadriceps muscle thickness, diaphragm mobility, and handgrip strength between patients with SSc and healthy individuals. METHOD: This cross-sectional study included 16 patients with SSc and 16 self-reported healthy individuals matched for age. We assessed quadriceps and diaphragm thickness and diaphragmatic mobility (ultrasound), handgrip strength (hand-held dynamometer), and respiratory muscle strength (manovacuometer). Patients also responded to the Health Assessment Questionnaire Disability Index and the International Physical Activity Questionnaire. RESULTS: Patients with SSc presented lower quadriceps thickness (p < 0.0001), diaphragmatic mobility (p = 0.01), handgrip (p < 0.0001), and respiratory muscle strength (p < 0.0001) than healthy individuals. A moderate positive correlation was observed between handgrip strength and quadriceps thickness in patients with SSc (rho = 0.576; p = 0.02). CONCLUSIONS: Patients with SSc presented reduced quadriceps thickness, diaphragmatic mobility, handgrip, and respiratory muscle strength when compared to healthy individuals Also, handgrip strength was correlated with quadriceps thickness in patients with SSc, suggesting that loss of muscle mass accompanies loss of peripheral muscle strength group of patients. Key Points • SSc patients presented reduced quadriceps thickness and diaphragmatic mobility • SSc patients have reduced handgrip and respiratory muscle strength • Lower handgrip muscle strength correlated with lower quadriceps thickness.

Esclerodermia. Manifestaciones bucomaxilofaciales, prevención y atención odontológica. Revisión narrativa / Scleroderma. Oral and maxillofacial manifestations, prevention and dental care. Narrative review

Resumen La esclerodermia pertenece a un grupo de enfermedades autoinmunes del tejido conectivo que produce inflamación, disfunción vascular y fibrosis excesiva del tejido de soporte de la piel y los órganos viscerales. A nivel bucal se presentan afecciones como la xerostomía y la microstomía, así como caries y enfermedad periodontal por dificultades en el control de la placa bacteriana. Su tratamiento odontológico requiere un manejo integral, que comprenda las patologías bucales presentes y los síntomas permanentes característicos de la enfermedad. Si bien es una patología muy infrecuente en la práctica general, es necesario enfatizar la importancia de que el odontólogo cumpla un rol multidisciplinario en el tratamiento de estos pacientes. Esta revisión narrativa tiene por objetivo describir las manifestaciones bucomaxilofaciales y el manejo odontológico del paciente con esclerodermia.

Abstract Scleroderma belongs to a group of autoimmune connective tissue diseases that causes inflammation, vascular dysfunction and excessive fibrosis of the supporting skin tissue and visceral organs. In the mouth, conditions such as xerostomia and microstomy are evident, as well as caries and periodontal disease due to difficulty in bacterial plaque control. Its dental treatment requires an integral management, which includes the oral pathologies present and the characteristic permanent symptoms of the disease. Although this pathology is not very common in the general practice, it is necessary to emphasize the importance of the dentist playing a multidisciplinary role in the treatment of these patients. This narrative review aims to describe the oral and maxillofacial manifestations and the dental management of patients with scleroderma.

Systemic scleroderma: imaging findings of diagnosis and clinical management of temporomandibular joint disorders / Esclerodermia sistêmica: diagnóstico por achados imaginológicos e manejo clínico das disfunções temporomandibulares

Scleroderma, an autoimmune disease, directly affects the production of collagen in the connective tissue. In its systemic form, the disease causes oral manifestations such as: limited mouth opening, xerostomia, periodontal disease, thickening of the periodontal ligament and bone resorption of the mandible. This case report aims to draw attention to the difficulties encountered in providing dental care to patients with scleroderma and also to highlight the imaging findings, with emphasis on the temporomandibular joints, which are of interest to dentists about the disease. In the present case, the patient presented bilateral condylar erosion, in addition to disc displacement without reduction. Due to the systemic condition of the patient, it was decided to make an individualized occlusal splint. The limitation of mouth opening is a limiting factor for the manufacture of prostheses and plates, which is why partial prostheses are indicated and are easily removed by the patient. The decisions taken have a great impact on the health and quality of life of patients in these conditions, so there is a need for multidisciplinary involvement in order to arrive at the best treatment plan. After five years of using the stabilizing plate overnight, the patient reports greater comfort and muscle relaxation upon waking up (AU)

Esclerodermia, uma doença autoimune, afeta diretamente a produção de colágeno do tecido conjuntivo. Na forma sistêmica, a doença causa manifestações bucais, como: limitação de abertura bucal, xerostomia, doença periodontal, espessamento do ligamento periodontal e reabsorção da mandíbula. Este relato de caso tem por objetivo chamar atenção para as dificuldades encontradas ao promover atendimento odontológico para pacientes com esclerodermia e também destacar os achados imaginológicos, com ênfase na articulação temporomandibular, que são da doença e de interessa ao cirurgião-dentista. No presente caso, a paciente apresentava erosão condilar bilateral, com deslocamento de disco sem redução. Devido à condição sistêmica da paciente, foi decidido confeccionar uma placa oclusal individualizada. A limitação de abertura bucal é um fator limitante para confecção de próteses e placas, por isso próteses parciais são indicadas, além de serem de fácil remoção pelo paciente. As decisões tomadas tem grande impacto na saúde e qualidade de vida de pacientes nessas condições, deste modo é necessário uma equipe multidisciplinar envolvidas para chegar no melhor plano de tratamento. Após cinco anos fazendo uso da placa estabilizadora durante a noite, a paciente relata maior conforto e relaxamento muscular ao acordar (AU)

Simpatectomía de la arteria radial y ulnar en contexto de úlcera digital secundaria a esclerosis sistémica cutánea localizada / Radial and ulnar artery sympathectomy in the context of digital ulcer secondary to localized cutaneous systemic sclerosis

SPeripheral sympathectomy is a procedure which has shown high rates of decreasing ischemic pain, recover functionality and wound healing, preventing the progression of the disease and further complications. We present a female patient with severe Raynaud´s phenomenon secondary to localized cutaneous systemic sclerosis complicated who presented digital ulcer treated with a sympathectomy of the radial and ulnar artery at the wrist level, undergoing post-operative follow-up.

Effect of crosstalk between Th17 and Th9 cells on the activation of dermal vascular smooth muscle cells in systemic scleroderma and regulation of tanshinone IIA

Abstract Background: To evaluate the effect of T-helper 17 (Th17) cells and Th9 cells on the activation of dermal vascular smooth muscle cells (DVSMCs) in systemic scleroderma (SSc) and regulation of tanshinone IIA. Methods: The expression of interleukin 17 receptor (IL-17R) and interleukin 9 receptor (IL-9R) in the skin of SSc patients was assessed by immunofluorescence. The expression of IL-9 and IL-9R mRNA in peripheral blood mononuclear cells (PBMCs) of SSc patients were detected by quantitative real-time polymerase chain reaction (qRT-PCR). The proportion of Th9 cells in PBMCs of SSc patients was sorted by flow cytometry. The effect of IL-9 on the differentiation of Th17 and IL-17 on that of Th9 was detected by flow cytometry. The proportion of Th9 and Th17 cells in SSc patients was detected by flow cytometry. The level of collagen I, III, α-SMA, IL-9R, IL-17R, JNK, P38, and ERK were analyzed using western blot (WB). Results: Th9 cells were highly expressed in SSc. IL-9 stimulated the differentiation of immature T cells into Th17 cells. IL-17 induced the differentiation of immature T cells intoTh9 cells. Tanshinone IIA inhibited the differentiation of immature T lymphocytes into Th17 and Th9. WB showed that the combined action of IL-17 and IL-9 upregulated the inflammation and proliferation of DVSMCs. Anti-IL17, anti-IL9, and tanshinone IIA inhibited the functional activation of DVSMCs. Study limitations: For Th17, Th9 and vascular smooth muscle cells, the study on the signal pathway of their interaction is not thorough enough. More detailed studies are needed to explore the mechanism of cell-cell interaction. Conclusions: The current results suggested that Th17 and Th9 cells induced the activation of DVSMCs in SSc through crosstalk in vitro, and tanshinone IIA inhibited the process.

Effect of crosstalk between Th17 and Th9 cells on the activation of dermal vascular smooth muscle cells in systemic scleroderma and regulation of tanshinone IIA.

BACKGROUND: To evaluate the effect of T-helper 17 (Th17) cells and Th9 cells on the activation of dermal vascular smooth muscle cells (DVSMCs) in systemic scleroderma (SSc) and regulation of tanshinone IIA. METHODS: The expression of interleukin 17 receptor (IL-17R) and interleukin 9 receptor (IL-9R) in the skin of SSc patients was assessed by immunofluorescence. The expression of IL-9 and IL-9R mRNA in peripheral blood mononuclear cells (PBMCs) of SSc patients were detected by quantitative real-time polymerase chain reaction (qRT-PCR). The proportion of Th9 cells in PBMCs of SSc patients was sorted by flow cytometry. The effect of IL-9 on the differentiation of Th17 and IL-17 on that of Th9 was detected by flow cytometry. The proportion of Th9 and Th17 cells in SSc patients was detected by flow cytometry. The level of collagen I, III, α-SMA, IL-9R, IL-17R, JNK, P38, and ERK were analyzed using western blot (WB). RESULTS: Th9 cells were highly expressed in SSc. IL-9 stimulated the differentiation of immature T cells into Th17 cells. IL-17 induced the differentiation of immature T cells into Th9 cells. Tanshinone IIA inhibited the differentiation of immature T lymphocytes into Th17 and Th9. WB showed that the combined action of IL-17 and IL-9 upregulated the inflammation and proliferation of DVSMCs. Anti-IL17, anti-IL9, and tanshinone IIA inhibited the functional activation of DVSMCs. STUDY LIMITATIONS: For Th17, Th9 and vascular smooth muscle cells, the study on the signal pathway of their interaction is not thorough enough. More detailed studies are needed to explore the mechanism of cell-cell interaction. CONCLUSIONS: The current results suggested that Th17 and Th9 cells induced the activation of DVSMCs in SSc through crosstalk in vitro, and tanshinone IIA inhibited the process.

Disfunción sexual en mujeres con esclerosis sistémica / Sexual dysfunction in women with systemic sclerosis

Introducción: la disfunción sexual (DS) es común entre las mujeres con enfermedades crónicas, incluyendo esclerosis sistémica (ES). Se ha asociado con características como la duración de la enfermedad, dolor, disminución de la actividad funcional, entre otras. Desde nuestro conocimiento, aún no contamos con datos locales. Objetivos: evaluar la frecuencia de DS en mujeres con ES; describir las características sociodemográficas, clínicas y psicológicas asociadas con la DS en mujeres con ES. Materiales y métodos: estudio observacional, analítico y de corte transversal. Se incluyeron mujeres de entre 20 y 59 años con diagnóstico de ES, según los criterios de clasificación del European League Against Rheumatism/American College of Rheumatology (ACR/EULAR 2013). Se excluyeron pacientes con enfermedades crónicas no controladas, otras patologías reumatológicas autoinmunes, e inactividad sexual o patología genitourinaria no relacionadas a ES en las últimas 4 semanas. La DS se evaluó con la versión en español del cuestionario índice de función sexual femenina (Female sexual function index, FSFI). Resultados: se incluyeron 56 pacientes. El 78,57% presentó DS y 19,64% era sexualmente inactiva debido a la enfermedad. Escala visual análoga (EVA) de fatiga (coeficiente β: -0,08, IC 95%: -0,14 a -0,02; p<0,01), edad (coeficiente β: -0,23, IC 95%: -0,40 a -0,05; p=0,01) y fibromialgia (coeficiente β: -11,90, IC 95%: -17,98 a -5,82; p<0,01) mostraron una asociación significativa e independiente con DS en el análisis multivariado. Conclusiones: la DS es frecuente entre las mujeres con ES, y las pacientes más jóvenes, sin fibromialgia y con menor fatiga presentaron una mejor funcionalidad sexual.

Introduction: sexual impairment (SI) is common among women with chronic diseases, including systemic sclerosis (SSc). It has been associated with characteristics such as the duration of the disease, pain, decreased functional activity, among others. To the best of our knowledge, we still do not have local data. Objectives: to evaluate the frequency of SI in women with SSc. To describe the sociodemographic characteristics, disease itself and psychological items associated with SI in women with SSc. Materials and methods: observational, analytical, cross-sectional study. We included women between 20 and 59 years diagnosed with SSc according to 2013 classification criteria ACR/EULAR. We excluded patients with uncontrolled chronic diseases or other autoimmune rheumatologic diseases and patients who, in the last 4 weeks, had dyspareunia or were sexually inactive due to causes not attributable to their disease. SI was assessed using the Spanish version of female sexual function index questionnaire (FSFI). Results: 56 patients were included. 78.57% presented SI and 19.64% of them were sexually inactive patients due to the disease. Fatigue VAS (β coefficient: -0.08, CI 95%: -0.14 to -0.02; p<0.01), age (β coefficient: -0.23, CI 95%: -0.40 to -0.05; p=0.01) and fibromyalgia (β coefficient: -11.90, CI 95%: -17.98 to -5.82; p<0.01) showed significant and independent association with SI in the multivariate analysis. Conclusions: SI is frequent among women with SSc, and younger patients, without fibromyalgia and with less fatigue have better sexual function.

Disfunción sexual en mujeres con esclerosis sistémica / Sexual dysfunction in women with systemic sclerosis

Introducción: la disfunción sexual (DS) es común entre las mujeres con enfermedades crónicas, incluyendo esclerosis sistémica (ES). Se ha asociado con características como la duración de la enfermedad, dolor, disminución de la actividad funcional, entre otras. Desde nuestro conocimiento, aún no contamos con datos locales. Objetivos: evaluar la frecuencia de DS en mujeres con ES; describir las características sociodemográficas, clínicas y psicológicas asociadas con la DS en mujeres con ES. Materiales y métodos: estudio observacional, analítico y de corte transversal. Se incluyeron mujeres de entre 20 y 59 años con diagnóstico de ES, según los criterios de clasificación del European League Against Rheumatism/American College of Rheumatology (ACR/EULAR 2013). Se excluyeron pacientes con enfermedades crónicas no controladas, otras patologías reumatológicas autoinmunes, e inactividad sexual o patología genitourinaria no relacionadas a ES en las últimas 4 semanas. La DS se evaluó con la versión en español del cuestionario índice de función sexual femenina (Female sexual function index, FSFI). Resultados: se incluyeron 56 pacientes. El 78,57% presentó DS y 19,64% era sexualmente inactiva debido a la enfermedad. Escala visual análoga (EVA) de fatiga (coeficiente ß: -0,08, IC 95%: -0,14 a -0,02; p<0,01), edad (coeficiente ß: -0,23, IC 95%: -0,40 a -0,05; p=0,01) y fibromialgia (coeficiente ß: -11,90, IC 95%: -17,98 a -5,82; p<0,01) mostraron una asociación significativa e independiente con DS en el análisis multivariado. Conclusiones: la DS es frecuente entre las mujeres con ES, y las pacientes más jóvenes, sin fibromialgia y con menor fatiga presentaron una mejor funcionalidad sexual.

Introduction: sexual impairment (SI) is common among women with chronic diseases, including systemic sclerosis (SSc). It has been associated with characteristics such as the duration of the disease, pain, decreased functional activity, among others. To the best of our knowledge, we still do not have local data. Objectives: to evaluate the frequency of SI in women with SSc. To describe the sociodemographic characteristics, disease itself and psychological items associated with SI in women with SSc. Materials and methods: observational, analytical, cross-sectional study. We included women between 20 and 59 years diagnosed with SSc according to 2013 classification criteria ACR/EULAR. We excluded patients with uncontrolled chronic diseases or other autoimmune rheumatologic diseases and patients who, in the last 4 weeks, had dyspareunia or were sexually inactive due to causes not attributable to their disease. SI was assessed using the Spanish version of female sexual function index questionnaire (FSFI). Results: 56 patients were included. 78.57% presented SI and 19.64% of them were sexually inactive patients due to the disease. Fatigue VAS (ß coefficient: -0.08, CI 95%: -0.14 to -0.02; p<0.01), age (ß coefficient: -0.23, CI 95%: -0.40 to -0.05; p=0.01) and fibromyalgia (ß coefficient: -11.90, CI 95%: -17.98 to -5.82; p<0.01) showed significant and independent association with SI in the multivariate analysis. Conclusions: SI is frequent among women with SSc, and younger patients, without fibromyalgia and with less fatigue have better sexual function.

Skin manifestations associated with systemic diseases - Part I

Abstract The skin demonstrates what is happening in the body in many diseases, as it reflects some internal processes on the surface. In this sense, skin as an organ, goes beyond its protective and barrier functions, as it provides clues for the identification of some systemic diseases. The dermatologist then raises diagnostic hypotheses for conditions related to all systems and refers them to the appropriate specialty. With easy access to examination by trained eyes and biopsies, the skin can present specific or non specific alterations on histopathology. In the first case this combination establishes the diagnosis of the disease itself. Non specific manifestations can occur in a variety of contexts and then histopathology is not specific of a particular disease. This article is divided into two parts that will cover large groups of diseases. In this first part, cutaneous manifestations of the main rheumatologic diseases are described, which are the ones with the greatest interface with dermatology. The authors also talk about vascular manifestations and granulomatous diseases. In the second part, endocrinological, hematological, oncological, cardiovascular, renal, gastrointestinal diseases, pruritus and its causes are discussed, and finally, the dermatological manifestations of SARS-CoV-2 coronavirus infection. The authors' intention is that, by using direct and easily accessible language, aim to provide practical material for consultation and improvement to all dermatologists who recognize the importance of a comprehensive assessment of their patients.

Skin manifestations associated with systemic diseases - Part I.

The skin demonstrates what is happening in the body in many diseases, as it reflects some internal processes on the surface. In this sense, skin as an organ, goes beyond its protective and barrier functions, as it provides clues for the identification of some systemic diseases. The dermatologist then raises diagnostic hypotheses for conditions related to all systems and refers them to the appropriate specialty. With easy access to examination by trained eyes and biopsies, the skin can present specific or non specific alterations on histopathology. In the first case this combination establishes the diagnosis of the disease itself. Non specific manifestations can occur in a variety of contexts and then histopathology is not specific of a particular disease. This article is divided into two parts that will cover large groups of diseases. In this first part, cutaneous manifestations of the main rheumatologic diseases are described, which are the ones with the greatest interface with dermatology. The authors also talk about vascular manifestations and granulomatous diseases. In the second part, endocrinological, hematological, oncological, cardiovascular, renal, gastrointestinal diseases, pruritus and its causes are discussed, and finally, the dermatological manifestations of SARS-CoV-2 coronavirus infection. The authors' intention is that, by using direct and easily accessible language, aim to provide practical material for consultation and improvement to all dermatologists who recognize the importance of a comprehensive assessment of their patients.

A review of hematopoietic stem cell transplantation for autoimmune diseases: multiple sclerosis, systemic sclerosis and Crohn's disease. Position paper of the Brazilian Society of Bone Marrow Transplantation

ABSTRACT Autoimmune diseases are an important field for the development of bone marrow transplantation, or hematopoietic stem cell transplantation. In Europe alone, almost 3000 procedures have been registered so far. The Brazilian Society for Bone Marrow Transplantation (Sociedade Brasileira de Transplantes de Medula Óssea) organized consensus meetings for the Autoimmune Diseases Group, to review the available literature on hematopoietic stem cell transplantation for autoimmune diseases, aiming to gather data that support the procedure for these patients. Three autoimmune diseases for which there are evidence-based indications for hematopoietic stem cell transplantation are multiple sclerosis, systemic sclerosis and Crohn's disease. The professional stem cell transplant societies in America, Europe and Brazil (Sociedade Brasileira de Transplantes de Medula Óssea) currently consider hematopoietic stem cell transplantation as a therapeutic modality for these three autoimmune diseases. This article reviews the evidence available.

A review of hematopoietic stem cell transplantation for autoimmune diseases: multiple sclerosis, systemic sclerosis and Crohn's disease. Position paper of the Brazilian Society of Bone Marrow Transplantation.

Autoimmune diseases are an important field for the development of bone marrow transplantation, or hematopoietic stem cell transplantation. In Europe alone, almost 3000 procedures have been registered so far. The Brazilian Society for Bone Marrow Transplantation (Sociedade Brasileira de Transplantes de Medula Óssea) organized consensus meetings for the Autoimmune Diseases Group, to review the available literature on hematopoietic stem cell transplantation for autoimmune diseases, aiming to gather data that support the procedure for these patients. Three autoimmune diseases for which there are evidence-based indications for hematopoietic stem cell transplantation are multiple sclerosis, systemic sclerosis and Crohn's disease. The professional stem cell transplant societies in America, Europe and Brazil (Sociedade Brasileira de Transplantes de Medula Óssea) currently consider hematopoietic stem cell transplantation as a therapeutic modality for these three autoimmune diseases. This article reviews the evidence available.

Infections and systemic sclerosis: an emerging challenge / Infecciones y esclerosis sistémica: un desafío emergente

ABSTRACT Mounting evidence has shown non-systemic sclerosis (SSc) related complications as a rising cause of hospital admission and mortality, out of which infections are among the top-five causes. Patients with SSc are at an increased risk of infection due to several features of the treatment options and to the disease itself. For instance, lung involvement is associated with a higher frequency of respiratory infections, whereas the presence of digital ulcers or calcinosis may result in skin and soft tissue infections, and even osteomyelitis. On the other hand, the growing trend towards immunomodulation and immunosuppression in patients with autoimmune diseases will place SSc patients at a higher risk of infectious complications, including opportunistic infections. A low suspicion threshold and an increasing awareness among treating specialists, particularly rheumatologists, are warranted for prevention, early diagnosis and management of infectious complications. Nonetheless, data on risk management strategies in SSc, such as vaccination and antimicrobial prophylaxis, are scarce. A narrative non-systematic review was performed to provide an update of infectious complications in patients with SSc.

RESUMEN La evidencia creciente muestra que las complicaciones no asociadas a la esclerosis sistémica (ES) son una causa cada vez más frecuente de hospitalización y mortalidad, dentro de las cuales las infecciones se encuentran entre las primeras cinco causas. Los pacientes con ES presentan un riesgo elevado de infección asociado con las opciones terapéuticas y con la enfermedad misma. Por ejemplo, el compromiso pulmonar se asocia con una mayor frecuencia de infecciones respiratorias, mientras que la presencia de úlceras digitales o calcinosis pueden resultar en infecciones de piel y tejidos blandos, incluso en osteomielitis. Por otro lado, la tendencia creciente hacia la inmunomodulación y la inmunosupresión, como tratamiento de las enfermedades autoinmunes, pondrá a estos pacientes en un mayor riesgo de infecciones, incluidas las infecciones oportunistas. Son necesarios un umbral bajo de sospecha y un alto nivel de alerta entre las especialidades tratantes, particularmente los reumatólogos, para la prevención, el diagnóstico temprano y el manejo de las complicaciones infecciosas. Sin embargo, la información respecto a estrategias de gestión de riesgo en ES, como la vacunación o la profilaxis antibiótica, es escasa. Se realizó una revisión narrativa no sistemática que presenta una actualización sobre las complicaciones infecciosas en pacientes con ES.

Effectiveness of Saccharomyces boulardii and Metronidazole for Small Intestinal Bacterial Overgrowth in Systemic Sclerosis.

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.

Estenosis de las arterias cubital y radial en pacientes con esclerosis sistémica, comparado con un grupo control / Ulnar and radial stenosis in systemic sclerosis

Objetivos: describir la frecuencia de estenosis arterial (cubital y radial) en pa-cientes con esclerosis sistémica (ES); analizar la relación entre estenosis macro-vascular y úlceras digitales. Método: se incluyeron 57 pacientes con ES, según la clasificación del Colegio Americano de Reumatología de 1980 y 21 pacientes sin ES. Se realizó ecografía doppler arterial de miembros superiores. Resultados: la estenosis en al menos una arteria cubital se objetivó en 31% de pacientes con ES (18/57) (p=0.003). Se objetivó estenosis radial en 9 de 57 pacientes con ES (15%) y en uno de los 21 controles (p=0.19). En el modelo multivariado, los predicto-res de úlceras digitales fueron inicio de Raynaud antes de los 40 años (OR 5.3 IC95% 1.54-18.22, p=0.008) y patrón tardío en la capilaroscopia (OR 4.4 IC95% 1.29-15.63, p=0.018). Conclusiones: un tercio de los pacientes ES presentó este-nosis cubital. El compromiso de los grandes vasos no se asoció a úlceras digitales.

Objectives: to describe the frequency of ulnar and radial stenosis in SSc patients. Analyze the correlation between arterial stenosis and digital ulcers. Methods: we included 57 SSc consecutive patients who fulfilled ACR 1980 classification criteria, and 21 healthy controls. An arterial ecodoppler was performed to all participants. Results: the presence of stenosis in at least one ulnar artery was observed in 18 of 57 patients with SSc (31%) and in none of the 21 controls (p=0.003). Stenosis was present in at least one radial artery in 9 of 57 SSc patients (15%) (p=0.19). In multivariate model, the best predictors of digital ulcers were age at onset of Ray-naud phenomenon before 40 years (OR 5.3 95%CI 1.54-18.22, p=0.008) and late SD pattern (OR 4.4 95%CI 1.29-15.63, p=0.018). Conclusion: in the present series, ulnar stenosis was observed frequently in SSc patients. Stenosis of large vessels was not associated with digital ulcers.

Sublingual microcirculatory alterations in patients with stable systemic sclerosis / Alteraciones microcirculatorias sublinguales en pacientes con esclerosis sistémica estable

ABSTRACT Introduction: Systemic sclerosis (SSc) is a systemic autoimmune disease characterized by fibrosis, inflammation, and microcirculatory alterations. Objective: To evaluate abnormalities in the sublingual microcirculation of SSc patients and to establish any differences compared to healthy controls. Methods: The sublingual microcirculation was determined using a Sidestream dark-field (SDF) imaging device (MicroScan; MicroVision Medical, Amsterdam, the Netherlands) in patients with SSc and controls. Results: Twelve patients with SSc (75% with diffuse cutaneous SSc) were evaluated (mean age: 52.08 ± 2.08 years). A group of 20 volunteers was used as the control. Significantly lower total capillary density (TCD) (9.2 [8.5-9.7] vs. 10.9 [9.8-12.5]) and functional capillary density (FCD) (7.0 [6.8-7.5] vs. 8.6 [7.5-9.8]) were observed in SSc patients than in healthy controls. Conclusions: SSc is related to significantly lower capillary density in the sublingual microcirculation, and the SDF imaging technique could be an alternative to nailfold video-capillaroscopy for diagnosing and following-up patients with SSc.

RESUMEN Introducción: La esclerosis sistémica (SSc) es una enfermedad autoimmune sistémica caracterizada por fibrosis, inflamación y alteraciones en la microcirculación. Objetivo: Evaluar anormalidades en la microcirculación sublingual de pacientes con diagnóstico de esclerosis sistémica y establecer diferencias en comparación con controles sanos. Métodos: Exploramos la microcirculación sublingual utilizando un dispositivo de imágenes de campo oscuro Sidestream (SDF) (Micro Scan, MicroVision Medical, Amsterdam, Holanda) en pacientes con SSc y controles. Resultados: Se evaluaron 12 pacientes con SSc estable (75% con cutánea difusa) (edad media: 52.08 ± 2.08). Un grupo de 20 voluntarios se utilizó como control. Se observó una disminución significativa en la densidad vascular total (TCD) (9.2 [8.5-9.7] vs. 10.9 [9.8-12.5]) y densidad capilar funcional (FCD) (7.0 [6.8-7.5] vs. 8.6 [7.5-9.8]) observado en pacientes con esclerosis sistémica en comparación con controles sanos. Conclusiones: La SSc se relaciona con la disminución significativa de la densidad capilar en la microcirculación sublingual, esta técnica podría ser una alternativa en pacientes críticos con esclerosis sistémica o utilizarse para seguimiento durante la hospitalización.

Análise funcional e anatômica anorretal de pacientes femininas portadoras de esclerodermia em um centro universitário de referência em desordens do assoalho pélvico / Functional and anatomical analysis of the anorectum of female scleroderma patients at a center for pelvic floor disorders

ABSTRACT BACKGROUND: Scleroderma or progressive systemic sclerosis is characterized by a chronic inflammatory process with proliferation of fibrous connective tissue and excessive deposition of collagen and extracellular matrix in the skin, smooth muscle, and viscera. The smooth muscle most involved in scleroderma is that of the esophagus, and dysphagia is the most commonly reported symptom. However, the internal anal sphincter may also be impaired by degeneration and fibrosis, leading to concomitant anal incontinence in scleroderma patients. These patients may neglect to complain about it, except when actively questioned. OBJECTIVE: To assess anorectal function and anatomy of female scleroderma patients with symptoms of anal incontinence through Cleveland Clinic Florida Fecal Incontinence Score (CCFIS), anorectal manometry and endoanal ultrasound at the outpatient clinic of colorectal and anal physiology, Clinics Hospital, University of São Paulo Medical School (HC-FMUSP). METHODS: Female scleroderma patients were prospectively assessed and questioned as to symptoms of anal incontinence. The anorectal manometry and endoanal ultrasound results were correlated with clinical data and symptoms. RESULTS: In total, 13 women were evaluated. Their mean age was 55.77 years (±16.14; 27-72 years) and their mean disease duration was 10.23 years (±6.23; 2-23 years). All had symptoms of fecal incontinence ranging from 1 to 15. Seven (53.8%) patients had fecal incontinence score no higher than 7; three (23.1%) between 8 and 13; and three (23.1%) 14 or higher, corresponding to mild, moderate, and severe incontinence, respectively. Ten (76.92%) patients had hypotonia of the internal anal sphincter. Three-dimensional endoanal ultrasound showed tapering associated with muscle atrophy of the internal sphincter in six cases and previous muscle defects in three cases. CONCLUSION: A functional and anatomical impairment of the sphincter is an important factor to assess in patients with progressive systemic sclerosis and it should not be underestimated.

RESUMO CONTEXTO: Esclerodermia ou esclerose sistêmica progressiva caracteriza-se por um processo inflamatório crônico com proliferação e fibrose do tecido conjuntivo e uma deposição excessiva de colágeno e matriz extracelular na pele, musculatura lisa e vísceras. A musculatura lisa mais envolvida é a esofágica e a disfagia é o sintoma mais comumente relatado. Entretanto, o esfíncter anal interno também pode ser acometido por essa degeneração e fibrose ocasionando incontinência anal nos pacientes portadores de esclerodermia. Isso pode ser omitido pelo paciente, exceto quando questionado de forma direta. OBJETIVO: Analisar a função e anatomia anorretal através do escore de incontinência anal de Cleveland Clinic Florida, manometria anorretal e ultrassom endoanal em pacientes do sexo feminino portadoras de esclerodermia e sintomas de incontinência anal atendidas no ambulatório de Fisiologia Colorretoanal no Hospital das Clínicas da Universidade de São Paulo (HC-FMUSP). RESULTADOS: Treze pacientes do sexo feminino foram avaliadas com média de idade de 55,77 anos (±16,14; 27-72 anos) e duração média da doença de 10,23 anos (±6,23; 2-23 anos). O índice de incontinência anal teve variação de 1-15, sendo que sete (53,8%) pacientes apresentavam índice inferior a 7; três (23,1%) entre 8 e 13; e três (23,1%) superior a 14, correspondendo à incontinência anal leve, moderada e grave, respectivamente. Dez (76,92%) pacientes apresentavam hipotonia do esfíncter anal interno. O estudo da ultrassonografia endoanal de três dimensões demonstrou afilamento com atrofia do esfíncter anal interno em seis casos e defeito muscular em três pacientes. CONCLUSÃO: O prejuízo funcional e anatômico do complexo esfincteriano anorretal é um importante fator a ser analisado em pacientes portadores de esclerose sistêmica progressiva e isso não pode ser subestimado.

Manejo prostodóntico convencional de una paciente con síndrome de CREST: reporte de caso / Conventional prosthodontics management of a patient with Crest syndrome: case report

El síndrome de CREST se caracteriza por calcinosis, síndrome de Raynaud, dismotilidad esofágica, esclerodactilia y telangiectasia. Presenta signos y síntomas en el sistema estomatognático que merecen consideración en un tratamiento. El objetivo del presente artículo fue reportar las manifestaciones orales de un caso de síndrome de CREST y describir su tratamiento protésico realizado en el Departamento de Rehabilitación Oral de la Universidad Nacional de Colombia. Se reporta una paciente mujer de 42 años, con diagnóstico de síndrome de CREST por su médico tratante, que acude a la facultad de odontología para un tratamiento restaurador. El manejo prostodóntico incluyó coronas, prótesis parcial fija metal-cerámicas y prótesis parcial removible. Los resultados obtenidos nos permiten recomendar las alternativas restaurativas convencionales similares a las usadas en un paciente sin esta condición sistémica, pero manteniendo un control estricto.

The CREST syndrome is characterized by calcinosis, Raynaud's syndrome, esophageal dysmotility, sclerodactyly, and telangiectasia. It has signs and symptoms in the stomatognathic system that deserve consideration in its treatment. The objective of this paper is to report the oral manifestations of a case of CREST syndrome and describe the prosthetic treatment performed in the Department of Oral Rehabilitation in the National University of Colombia. The case is presented of a patient female aged 42 years, diagnosed with CREST syndrome by the treating doctor. She came to the Faculty of Dentistry for restorative treatment. The prosthodontic management included metal-ceramic crowns, fixed partial denture, and removable partial denture. The results demonstrate that conventional restorative alternatives, similar to that used in a patient without this systemic condition, while maintaining strict control of it, can be recommended.

Intervenção fisioterapêutica na dor e na qualidade de vida em idosos com esclerose sistêmica. Relato de casos / Physiotherapeutic intervention on pain and quality of life of systemic sclerosis elderly patients. Case reports

RESUMO JUSTIFICATIVA E OBJETIVOS: A esclerose sistêmica é uma doença crônica, multissistêmica e autoimune, caracterizada por angiopatia disseminada em pequena e microcirculação, fenômeno de Raynoud e fibrose cutânea e de órgãos internos. A fisioterapia tem se mostrado uma alternativa eficaz no combate aos agravos osteomioarticulares causados pela doença. O objetivo deste estudo foi verificar os efeitos de um programa de intervenção fisioterapêutica na dor e na qualidade de vida de dois idosos com esclerose sistêmica. RELATOS DOS CASOS: Estudo longitudinal e intervencionista de natureza estudo de caso. Participaram do estudo dois idosos com esclerose sistêmica atendidos na Clínica de Fisioterapia da Universidade de Passo Fundo/RS. Os idosos foram submetidos a uma avaliação inicial que constituiu na coleta de dados (anamnese e exame físico-funcional) e na aplicação da escala analógica visual e do Questionário de Qualidade de Vida - Medical Outcomes Study 36 - Item Short-Form Health Survey (SF-36), traduzido e validado para o português. Após a avaliação inicial os idosos foram submetidos a um programa de intervenção baseado na cinesioterapia, com frequência de duas sessões semanais e duração de 1h por sessão, totalizando 15 sessões. De acordo com a escala analógica visual os pacientes apresentaram melhora no quadro álgico e de acordo com o SF-36 melhora na sua qualidade de vida. CONCLUSÃO: O protocolo de intervenção fisioterapêutica baseado na cinesioterapia mostrou-se uma estratégia eficaz no tratamento da dor e na melhora da qualidade de vida em idosos portadores de esclerose sistêmica.

ABSTRACT BACKGROUND AND OBJECTIVES: Systemic sclerosis is a chronic, multi-systemic and auto-immune disease, characterized by widespread angiopathy in small and microcirculation, Raynoud's phenomenon and skin and internal organs fibrosis. Physiotherapy is an effective alternative to fight musculoskeletal injuries caused by the disease. This study aimed at evaluating the effects of a physiotherapy program on pain and quality of life of two systemic sclerosis elderly patients. CASE REPORTS: Longitudinal and interventionist case study. Participated in the study two elderly patients with systemic sclerosis, treated in the Physiotherapy Clinic, Universidade de Passo Fundo/RS. Patients were submitted to baseline evaluation made up of data collection (history and physical-functional evaluation) and application of the visual analog scale and of the Quality of Life Questionnaire - Medical Outcomes Study 36 - Item Short-Form Health Survey (SF-36) translated and validated to the Portuguese language. After baseline evaluation, elderly patients were submitted to a kinesiotherapy-based intervention program, with frequency of 2 weekly sessions and duration of 1h per session, in a total of 15 sessions. According to the visual analog scale, patients have improved pain and, according to SF-36, have improved quality of life. CONCLUSION: Kinesiotherapy-based physiotherapeutic intervention protocol was an effective strategy to treat pain and improve quality of life of systemic sclerosis elderly patients.