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SUMMARY OBJECTIVE: This study aimed to evaluate the quality of sleep in individuals with systemic sclerosis and its correlation with the quality of life and disability. METHODS: This is a cross-sectional study, carried out in a tertiary service of a university hospital. Inclusion criteria were diagnosis of systemic sclerosis according to the criteria of the American College of Rheumatology/European League Against Rheumatism 2013 or the preliminary criteria of the American College of Rheumatology 1980, age ≥ 18 years; regularly monitored at the outpatient clinic of rheumatology. Clinical and demographic data of the patients were obtained through a structured interview and evaluation of the medical records. Sleep quality was assessed using the Pittsburgh Sleep Quality Index questionnaire, daytime sleepiness using the Epworth Sleepiness Scale, quality of life using 12-item short-form health survey, and disability using the scleroderma health assessment questionnaire. RESULTS: A total of 50 patients with systemic sclerosis were included, with 92% female, mean age 48.9 years, mean disease duration 8.9 years, and 60% limited cutaneous form. Most systemic sclerosis patients (84%) have poor sleep quality and 20% have excessive daytime sleepiness. There was a significant negative correlation between Pittsburgh Sleep Quality Index and the physical and mental components of the 12-item short-form health survey (r=-0.42, p=0.003 and r=-0.43, p=0.002, respectively) and a positive correlation with the scleroderma health assessment questionnaire (r=0.52, p=<0.001). CONCLUSION: This study showed that poor sleep quality is a very common finding among systemic sclerosis patients, and it negatively affects both the quality of life and the degree of disability.

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ABSTRACT Connective tissue disease-associated interstitial lung disease (CTD-ILD) represents a group of systemic autoimmune disorders characterized by immune-mediated organ dysfunction. Systemic sclerosis, rheumatoid arthritis, idiopathic inflammatory myositis, and Sjögren's syndrome are the most common CTDs that present with pulmonary involvement, as well as with interstitial pneumonia with autoimmune features. The frequency of CTD-ILD varies according to the type of CTD, but the overall incidence is 15%, causing an important impact on morbidity and mortality. The decision of which CTD patient should be investigated for ILD is unclear for many CTDs. Besides that, the clinical spectrum can range from asymptomatic findings on imaging to respiratory failure and death. A significant proportion of patients will present with a more severe and progressive disease, and, for those, immunosuppression with corticosteroids and cytotoxic medications are the mainstay of pharmacological treatment. In this review, we summarized the approach to diagnosis and treatment of CTD-ILD, highlighting recent advances in therapeutics for the various forms of CTD.

RESUMO Doença pulmonar intersticial associada à doença do tecido conjuntivo (DPI-DTC) representa um grupo de distúrbios autoimunes sistêmicos caracterizados por disfunção de órgãos imunomediada. Esclerose sistêmica, artrite reumatoide, miosite inflamatória idiopática e síndrome de Sjögren são as DTC mais comuns que apresentam acometimento pulmonar, bem como pneumonia intersticial com achados autoimunes. A frequência de DPI-DTC varia de acordo com o tipo de DTC, mas a incidência total é de 15%, causando um impacto importante na morbidade e mortalidade. A decisão sobre qual paciente com DTC deve ser investigado para DPI não é clara para muitas DTC. Além disso, o espectro clínico pode variar desde achados assintomáticos em exames de imagem até insuficiência respiratória e morte. Parte significativa dos pacientes apresentará doença mais grave e progressiva, e, para esses pacientes, imunossupressão com corticosteroides e medicamentos citotóxicos são a base do tratamento farmacológico. Nesta revisão, resumimos a abordagem do diagnóstico e tratamento de DPI-DTC, destacando os recentes avanços na terapêutica para as diversas formas de DTC.

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Background: Systemic sclerosis associated-interstitial lung disease (SSc-ILD) show a high mortality. The factors associated SSc-ILD have shown variability in different populations. There are few studies in Mexican mestizos. Objective: To analyze the epidemiological, clinical and paraclinical factors associated with SSc-ILD. Material and methods: Cross-sectional study, where patients > 18 years of age with a diagnosis of SSc according to EULAR/ACR 2013 criteria and diagnosis of ILD by forced vital capacity (FVC) < 70% and > 5% of affected lung area on tomography were included. The strength of association of the factors for ILD was measured by odds ratio (OR) with 95% confidence intervals (95% CI). The significant variables were analyzed by multiple logistic regression with adjustment. Results: Of the 80 patients with SSc, 38 (47.5%) had ILD. Risk factors such as smoking, higher activity index, lower FVC, anti-topoisomerase I antibodies, and protective factors such as a limited SSc, early capillaroscopic pattern, and anti-centromere antibodies (ACA) were associated with ILD. In the multivariate analysis, a higher activity index with OR 4.17, (95% CI 2.01-8.65) persisted as a risk factor, while ACA with OR 0.17, (95% CI 0.03-0.85) persisted as a protective factor associated with SSc-ILD. Conclusions: A higher index of activity and ACA persisted as factors associated with SSc-ILD.

Introducción: la enfermedad pulmonar intersticial (EPI) está relacionada a una mayor mortalidad en esclerosis sistémica (ES). Los factores asociados a su presentación han mostrado variabilidad en diferentes poblaciones. Pocos estudios se han realizado en mestizos mexicanos. Objetivo: analizar los factores clínicos y paraclínicos asociados a EPI en pacientes con ES. Material y métodos: estudio retrospectivo de casos y controles en el que se incluyeron pacientes > 18 años con diagnóstico de ES, según criterios EULAR/ACR 2013 y EPI por capacidad vital forzada (CVF) < 70% y > 5% de área pulmonar efectada en tomografía. La fuerza de asociación de los factores para EPI se midió por razón de momios (RM) con intervalos de confianza del 95% (IC95%). Las variables significativas se analizaron por regresión logística múltiple con ajuste. Resultados: de los 80 pacientes con ES, 38 (47.5%) tenían EPI. Factores de riesgo como tabaquismo, mayor índice de actividad, menor CVF, anticuerpos antitopoisomerasa I y factores protectores como variedad limitada de ES, patrón capilaroscópico temprano y anticuerpos anticentrómero estuvieron asociados a EPI. En el análisis multivariado un mayor índice de actividad con RM 4.17, (IC95%: 2.01-8.65) persistió como un factor de riesgo; en cambio, los anticuerpos anticentrómero con RM 0.17, (IC95%: 0.03-0.85) persistieron como un factor protector asociado a EPI. Conclusiones: un mayor índice de actividad y la presencia anticuerpos anticentrómero persistieron como factores asociados a la presencia de EPI en ES.

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La esclerosis sistémica sin escleroderma es una forma no común de compromiso sistémico de esta enfermedad, con ausencia de manifestaciones cutáneas. Presentamos el caso de una mujer de 71 años con índice de masa corporal (IMC) en 16,9 kg/m2, con historia de hipotiroidismo y estreñimiento crónico, quien acude a emergencia por dolor y distensión abdominal, asociado a náuseas. Se realizó una tomografía abdomino-pélvica encontrándose cámara gástrica colapsada, asas intestinales con algunos segmentos distendidos, con signos de fecalización de asa delgada. La colonoscopía describió dolicocolon severo. Se observaron placas induradas en codo derecho y en ambos glúteos. Los exámenes auxiliares: ANA 1/640 patrón centromérico, anticuerpos anticentrómero = 227 U/mL (valor normal - VN: 0-25), Anti SCL-70 = 1,9 U/mL (VN 0-25) y anti RNP = 0,1 U/mL (VN 0-25). La capilaroscopía de borde ungueal a 200x mostró megacapilares. El diagnóstico clínico fue esclerosis sistémica sin esclerodermia. Se inició tratamiento con dieta rica en fibra y nutrición parenteral por 1 semana. La evolución de la paciente fue favorable.

Systemic sclerosis sine Scleroderma is an uncommon form of systemic compromise of this disease, in the abscense of cutaneous manifestations. We present the case of a 71 years-old woman, body mass index (BMI) in 16.9 kg/m2, with history of hypothyroidism and chronic constipation, who goes to the emergency with pain, abdominal bloating, and nausea. An abdominopelvic tomography was made, reporting a collapse of the gastric chamber, some segments of intestinal loops distended with signs of fecalization in the small intestine. A colonoscopy described severe dolicocolon. We observed indurated plaques in left elbow and in both buttocks. Lab tests: ANA 1/640 with anticentromeric pattern, anticentromere antibodies = 227 U/mL (0-25), Anti-SCL-70 = 1.9 U/mL (0-25), anti-RNP = 0.1 U/mL (0-25). Peri-ungueal cappilaroscopy 200x showed mega capillaries. Clinical diagnosis was systemic sclerosis sine scleroderma. We initiated treatment with a high-fiber diet and parenteral nutrition for a week. The patient evolution was favorable.

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BACKGROUND: Systemic Sclerosis (SSc) patients may need hand surgery. OBJECTIVE: To develop a screening tool for rheumatologists to identify potential candidates with systemic sclerosis for hand surgery, optimizing referrals. METHODS: A pilot cross-sectional study from January 2015 to December 2016. SAMPLE SIZE: 51 participants. INCLUSION CRITERIA: ≥ 18 years old, meeting the 2013 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) classification criteria for SSc and hand impairment. DATA COLLECTED: age, sex, race, disease duration, SSc subtypes, vasodilator use, skin thickness, finger stiffness, presence of Digital Ulcers (DU) and/or calcinosis, presence of Raynaud's Phenomenon (RP) attacks, health status and disability, disease status, pain intensity and functional status of the hands. Data were analyzed by a multivariate logistic regression model. RESULTS: Fulfillment of surgical criteria: 68.8%. The surgical group had higher scores on the HAQ-DI (1.39 vs. 0.96, p = 0.032) and CHFS (25.0 vs. 12.0, p = 0.005) questionnaires, and a higher frequency of DU (91.43% vs. 18.75%, p < 0.0010), calcinosis (60.0% vs. 0.0%, p < 0.001), use of vasodilators (100.0% vs. 75.0%, p = 0.007) and digital stiffness (28.57% vs. 0.0%, p = 0.017). The presence of DU increased the chance of surgical indication by 46.2 times (ORIC 95% = 8.23 to 259.49). The statistical model showed good accuracy (86.3%, p < 0.001), sensitivity (91.4%), and specificity (81.2%). CONCLUSION: The presence of DU in SSc could be used as a screening feature for early identification and referral of potential candidates for hand surgery.

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Purpose/objectives: Systemic sclerosis (SSc) is a rare chronic autoimmune disease characterized by vascular abnormalities and connective tissue disorders. In 2021, the multidisciplinary team of the university hospital recognized a demand for oral health information among SSc patients. This study aims to describe the development of an SSc oral health booklet and its validation by people with SSc. Methods: For the development of the booklet, the project creators employed the action research methodology involving ten stages. SSc patients evaluated the material by filling out a digital form. Results: One hundred and thirty-one people with SSc evaluated the booklet. It received an average score of 9.73 (SD: 0.80) in relevance, 9.82 (SD: 0.47) in explicitness, and 9.49 (SD: 0.93) in aesthetics. The final version was emailed to the participants and posted on social media. Innovation: This is the first booklet that deeply addresses these issues in Portuguese and constitutes a soft technology to instruct and assist patients with SSc. Conclusion: The production of high-quality educational materials on oral health for patients with SSc is necessary. The online distribution increased its reach and had great importance in the context of the COVID-19 pandemic.

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Background: COVID-19 brought with it unknowns related to systemic sclerosis. Objective: To know the clinical evolution and prognosis of COVID-19 in a cohort of patients with systemic sclerosis. Methods: During the pandemic, we had digital contact with a cohort of 197 patients with SSc. If they had any condition that met the suspected definition of COVID-19, they underwent polymerase chain reaction testing for SARS-CoV-2; they were treated on an outpatient or hospital basis without interfering with their treatment. They followed their evolution every 24 hours until they became asymptomatic or died. Results: Thirteen patients (6.6%), nine diffuse cutaneous (dcSSc), and four limited cutaneous (lcSSc) developed COVID-19 during nine months of follow-up. The immunosuppressants used at the time of the disease were: mycophenolate mofetil, methotrexate, and prednisone, in low doses. Seven patients had interstitial lung disease (ILD). The main symptoms were chest pain, cough, dyspnea, dysgeusia, and anosmia, 1 with mild symptoms without pneumonia, 11 with mild pneumonia, and one with severe pneumonia that required hospital management. Only one (7.7%) presented severe pneumonia, was hospitalized, and died. Conclusions: COVID-19 disease in patients with SSc can be overcome in most cases, even when they are ILD and were using immunosuppressants at the time of infection with the SARS-CoV-2 virus.

Introducción: la COVID-19 trajo consigo incógnitas relacionadas con la esclerosis sistémica, enfermedad de baja prevalencia asociada a neumopatía intersticial difusa (NID). Objetivo: conocer la evolución clínica y el pronóstico de la COVID-19 en una cohorte de pacientes con esclerosis sistémica (ES). Métodos: se analizó una serie de 13 casos procedentes de una cohorte de 197 pacientes con ES en seguimiento vía digital. Cuando los pacientes cumplieron con la definición sospechosa de COVID-19 se solicitó prueba de reacción en cadena de polimerasa para SARS-CoV-2. Todos los pacientes recibieron seguimiento durante su atención ambulatoria u hospitalaria, sin interferir con su tratamiento cada 24 horas hasta quedar asintomáticos o fallecer. Resultados: de 197 pacientes, trece (6.6%) enfermaron de COVID-19 de edad 57 años (RIC: 52-63), cutáneos difusa (ESD) y 4 limitada (ESL) en lapso de 9 meses. Once presentaron neumonía leve (84%), una neumonía grave con fallecimiento intrahospitalario (7.7%). La oximetría media al ambiente se mantuvo en SO2 90% (88-92%). Casi todos usaban inmunosupresores (84%) al momento de enfermar: micofenolato de mofetilo, metotrexato, prednisona en dosis bajas. Siete (53%) tenían enfermedad pulmonar intersticial (EPI) previa. Principalmente manifestaron disnea (67.5%), dolor torácico, tos, disgeusia y anosmia. Conclusiones: es posible que al momento del contagio con el virus SARS-CoV-2 los inmunosupresores permitieran una menor respuesta inflamatoria sistémica, evitando un peor pronóstico, incluso en quienes tienen enfermedad intersticial previa.

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Introduction: Nailfold videocapillaroscopy is a non-invasive tool for the assessment of peripheral microcirculation, the main indication is the study of Raynaud's phenomenon, poorly standardized outside of this context. There is no clear information in real-life about the rea-sons for referral, the presence of clinical findings of autoimmune diseases, the frequency of patterns of autoantibodies, and specific capillaroscopic findings. Objective: The purpose of this survey is to describe the sociodemographic, clinical, paraclinical, and angioscopy findings of a cohort of subjects referred to a capillaroscopy service in North-western Colombia. Methods: A retrospective study was conducted, from 2015 to 2018. Categorical variables were expressed in frequency and percentage and quantitative variables in mean and standard deviation or median with interquartile range, depending on the distribution of the data. Results: A total of 318 capillaroscopies were performed for the first time. The main referral reason was Raynaud's phenomenon (n = 134; 42.1%). The most frequent baseline capillaroscopic pattern found was normal (n = 123; 38.7%). Of the 12 capillaroscopies that presented a non-specific pattern at a 6-month follow-up, only one (8.3%) progressed to a scleroderma pattern. In the subjects with systemic sclerosis, the most frequent clinical finding was sclerodactyly (n = 34; 37.8%), and 42/44 individuals (95.4%) had positive antinuclear antibodies; the most frequent pattern was centromere (n = 27; 64.3%) Conclusions: In a real-world setting, the main referral reason for capillaroscopy was Raynaud's phenomenon; more than a third of the subjects had normal capillaroscopic findings. Sclerodactyly was the most frequent clinical finding in patients with scleroderma capillaroscopic pattern.

Introducción: La videocapilaroscopia del lecho ungular es una herramienta no invasiva para la evaluación de la microcirculación periférica; la indicación principal es el estudio del fenómeno de Raynaud. Luego de una revisión de la literatura, no hay información clara sobre los motivos de remisión, presencia de hallazgos clínicos de enfermedades autoinmunes, frecuencia de patrones de autoanticuerpos y hallazgos capilaroscópicos específicos. Objetivo: Describir los hallazgos sociodemográficos, clínicos, paraclínicos y capilaroscópicos de sujetos remitidos a un servicio de capilaroscopia en el noroccidente colombiano. Métodos: Estudio retrospectivo de 2015 a 2018. Las variables categóricas se expresaron en frecuencias absolutas y porcentajes, y las variables cuantitativas en media y desviación estándar o mediana con rango intercuartílico, dependiendo de la distribución de los datos. Resultados: Se realizaron 318 capilaroscopias por primera vez. El principal motivo de remisión fue el fenómeno de Raynaud (n = 134; 42,1%). El patrón capilaroscópico basal más frecuente fue el normal (n = 123; 38,7%). De las 12 capilaroscopias que presentaron un patrón no específico en un seguimiento de seis meses, solo una (8,3%) progresó a un patrón de esclerodermia. En los sujetos con esclerosis sistémica, el hallazgo clínico más frecuente fue la esclerodactilia (n = 34; 37,8%), y 42/44 individuos (95,4%) tenían anticuerpos antinucleares positivos; el patrón más frecuente fue el centromérico (n = 27; 64,3%). Conclusiones: La razón principal de remisión para realizar una capilaroscopia fue el fenómeno de Raynaud; más de un tercio de los sujetos tenían hallazgos capilaroscópicos normales. La esclerodactilia fue el hallazgo clínico más frecuente en pacientes con patrón capilaroscópico de esclerodermia.

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SUMMARY OBJECTIVE: Cardiovascular disease risk prediction in scleroderma is important. In this study of scleroderma patients, the aim was to investigate the relationship between cardiac myosin-binding protein-C, sensitive troponin T, and trimethylamine N-oxide and cardiovascular disease risk with the Systematic COronary Risk Evaluation 2 model of the European Society of Cardiology. METHODS: Systematic COronary Risk Evaluation 2 risk groups of 38 healthy controls and 52 women with scleroderma were evaluated. Cardiac myosin-binding protein-C, sensitive troponin T, and trimethylamine N-oxide levels were analyzed with commercial ELISA kits. RESULTS: In scleroderma patients, cardiac myosin-binding protein-C and trimethylamine N-oxide levels were higher than healthy controls but sensitive troponin T was not (p<0.001, p<0.001, and p=0.274, respectively). Out of 52 patients, 36 (69.2%) were at low risk, and the other 16 (30.8%) patients were at high-moderate risk with the Systematic COronary Risk Evaluation 2 model. At the optimal cutoff values, trimethylamine N-oxide could discriminate high-moderate risk with sensitivity 76%, specificity 86% and cardiac myosin-binding protein-C with sensitivity 75%, specificity 83%. Patients with high trimethylamine N-oxide levels (≥10.28 ng/mL) could predict high-moderate- Systematic COronary Risk Evaluation 2 risk 15 times higher than those with low trimethylamine N-oxide (<10.28 ng/mL) levels (odds ratio [OR]: 15.00, 95%CI 3.585-62.765, p<0.001). Similarly, high cardiac myosin-binding protein-C (≥8.29 ng/mL) levels could predict significantly higher Systematic COronary Risk Evaluation 2 risk than low cardiac myosin-binding protein-C (<8.29 ng/mL) levels (OR: 11.00, 95%CI 2.786-43.430). CONCLUSION: Noninvasive cardiovascular disease risk prediction indicators in scleroderma, cardiac myosin-binding protein-C, and trimethylamine N-oxide could be recommended to distinguish between high-moderate risk and low risk with the Systematic COronary Risk Evaluation 2 model.

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Abstract Background Systemic Sclerosis (SSc) patients may need hand surgery. Objective To develop a screening tool for rheumatologists to identify potential candidates with systemic sclerosis for hand surgery, optimizing referrals. Methods A pilot cross-sectional study from January 2015 to December 2016. Sample size: 51 participants. Inclusion criteria: ≥ 18 years old, meeting the 2013 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) classification criteria for SSc and hand impairment. Data collected: age, sex, race, disease duration, SSc subtypes, vasodilator use, skin thickness, finger stiffness, presence of Digital Ulcers (DU) and/or calcinosis, presence of Raynaud's Phenomenon (RP) attacks, health status and disability, disease status, pain intensity and functional status of the hands. Data were analyzed by a multivariate logistic regression model. Results Fulfillment of surgical criteria: 68.8%. The surgical group had higher scores on the HAQ-DI (1.39 vs. 0.96, p =0.032) and CHFS (25.0 vs. 12.0, p =0.005) questionnaires, and a higher frequency of DU (91.43% vs. 18.75%, p <0.0010), calcinosis (60.0% vs. 0.0%, p <0.001), use of vasodilators (100.0% vs. 75.0%, p =0.007) and digital stiffness (28.57% vs. 0.0%, p =0.017). The presence of DU increased the chance of surgical indication by 46.2 times (ORIC 95% = 8.23 to 259.49). The statistical model showed good accuracy (86.3%, p <0.001), sensitivity (91.4%), and specificity (81.2%). Conclusion The presence of DU in SSc could be used as a screening feature for early identification and referral of potential candidates for hand surgery.