RESUMO
BACKGROUND: Early triage, the search for new therapies, and closer monitoring of patients with systemic sclerosis before their lung function irreversibly deteriorates are urgent concerns. Because it is an independent predictor of systemic sclerosis-related mortality, the 6-min walk test is a potentially useful tool to evaluate outcomes, along with pulmonary function and computed tomography. This study aimed to establish a reference value for the 6-min walking distance in women with diffuse cutaneous systemic sclerosis-associated interstitial lung disease that takes into account the effects of muscle and lung function. METHODS: This was a cross-sectional study in which 69 women with systemic sclerosis underwent the 6-min walk test, Health Assessment Questionnaire-Disability Index, pulmonary function, handgrip strength test, and quadriceps strength test. FINDINGS: The mean 6-min walking distance was 447 ± 78 m, and 43.5% of the participants did not reach 80% of their predicted value. 6-min walking distance correlated positively with quadriceps strength (r = 0.418, P = 0.0004), forced vital capacity (r = 0.306, P = 0.011), pulmonary diffusion (r = 0.360, P = 0.002), maximum inspiratory pressure (r = 0.268, P = 0.029), and maximum expiratory pressure (r = 0.288, P = 0.019) and negatively with age (r = -0.378, P = 0.001), body mass index (r = -0.248, P = 0.039), and Health Assessment Questionnaire-Disability Index (r = -0.438, P = 0.0001). In the multiple linear regression analysis, quadriceps strength, body mass index, pulmonary diffusion, age, and maximum expiratory pressure explained 72% of the 6-min walking distance variability. INTERPRETATION: Muscle function and, to a lesser extent, lung function are key contributors in determining the reference value for the 6-min walking distance in women with diffuse cutaneous systemic sclerosis-associated interstitial lung disease.
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Doenças Pulmonares Intersticiais , Esclerodermia Difusa , Escleroderma Sistêmico , Humanos , Feminino , Teste de Esforço/métodos , Força da Mão , Esclerodermia Difusa/complicações , Estudos Transversais , Valores de Referência , Doenças Pulmonares Intersticiais/complicações , Força Muscular/fisiologia , Escleroderma Sistêmico/complicações , CaminhadaRESUMO
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.
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Humanos , Escleroderma Sistêmico , Doença de Crohn , Transplante de Medula Óssea , Transplante de Células-Tronco Hematopoéticas , Esclerodermia Difusa , Esclerose MúltiplaRESUMO
BACKGROUND: The reproducibility and reliability of the modified Rodnan's Skin Score (mRSS) are debated due to investigator-related subjectivity. Here, we evaluate if durometry correlates with mRSS in patients with diffuse systemic sclerosis (SSc). METHODS: This cross-sectional study was conducted from December 2018 to June 2019, including 58 diffuse SSc patients. Two certified researchers, blind to each other's scores, performed the mRSS, followed by durometry at 17 predefined skin sites. For durometry and mRSS, individual scores per skin site were registered. Durometry and mRSS results measured by each researcher, as well as scores from different researchers, were compared. Skin thickness measurements from forearm skin biopsies were available in a subset of the patients, for comparisons. Statistical analyses included Cohen's Kappa Coefficient, Intraclass Correlation Coefficient, Kendall's Coefficient and Spearman's test. RESULTS: Mean (standard deviation, SD) patient age was 44.8 (12.9) years, and 88% were female. Inter-rater agreement varied from 0.88 to 0.99 (Intraclass correlation coefficient) for durometry, and 0.54 to 0.79 (Cohen's Kappa coefficient) for mRSS, according to the specific evaluated sites. When data were compared with skin thickness assessed in forearm biopsies, durometry correlated better with skin thickness than mRSS. CONCLUSION: Durometry may be considered as an alternative method to quantify skin involvement in patients with diffuse SSc. The strong inter-rater agreement suggests that the method may be useful for the assessment of patients by multiple researchers, as in clinical trials.
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Esclerodermia Difusa , Pele , Biópsia , Estudos Transversais , Antebraço/patologia , Humanos , Esclerodermia Difusa/diagnóstico , Esclerodermia Difusa/patologia , Pele/patologiaRESUMO
Abstract The heart and lung are target organs in systemic sclerosis (SSc) and similar symptoms (dyspnea and cough) may make the differential diagnosis between the two lesions difficult. In addition, complete atrioventricular block (CAVB) is a rare complication of this disease. This case report is about a patient with SSc and pulmonary fibrosis who was admitted to the emergency room with CAVB, heart failure (HF) and progressive worsening of the underlying disease.
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Humanos , Masculino , Pessoa de Meia-Idade , Fibrose Pulmonar/complicações , Esclerodermia Difusa/complicações , Bloqueio Atrioventricular/complicações , Fibrose Pulmonar/diagnóstico , Tosse , Esclerodermia Difusa/diagnóstico , Esclerodermia Difusa/tratamento farmacológico , Diagnóstico Precoce , Diagnóstico Diferencial , Dispneia , Bloqueio Atrioventricular/diagnóstico , Hipertensão PulmonarRESUMO
OBJECTIVE: To evaluate the frequency of anti-collagen type V in humans with early systemic sclerosis (SSc) compared to defined SSc patients and healthy controls, since collagen type V was shown to be overexpressed in early SSc patients' skin and there is no data concerning the presence of this antibody in early stages of human SSc. Experimental studies showed that animal models immunized with collagen type V developed a disease similar to human systemic sclerosis (SSc), with antibodies production, mainly in early stages post-immunization. METHODS: Eighty-one female SSc patients were included and divided into two groups: early-SSc (18 patients-EULAR Preliminary Criteria) and defined-SSc (63 patients-ACR Criteria 1980). The control group consisted of 19 healthy women age-matched to Early-SSc group. Anti-collagen type V was performed by ELISA. Data was analyzed by appropriate tests. RESULTS: The prevalence of anti-collagen type V in early-SSc, defined-SSc and control groups was respectively 33, 17 and 5% (p = 0.07). SSc patients with anti-collagen type V had shorter disease duration compared to those without this antibody (8.8 ± 5.1 vs. 14.7 ± 8.9, p = 0.006). Likewise, early-SSc patients with anti-collagen V also had a shorter disease duration than patients negative for this antibody (4.6 ± 2.2 vs. 9.7 ± 5.2, p = 0.04). No association with clinical subsets or scleroderma antibodies specificities was observed (p > 0.05). CONCLUSION: The production of anti-collagen type V in SSc seems to be an early event independent of other antibodies specificities. Further studies are necessary to determine if the underlying mechanism for this chronology involves a primary immune response to abnormal expression of collagen type V.
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Anticorpos/análise , Colágeno Tipo V/imunologia , Esclerodermia Difusa/imunologia , Esclerodermia Limitada/imunologia , Adulto , Fatores Etários , Anticorpos Antinucleares/análise , Biomarcadores/análise , Estudos de Casos e Controles , Colágeno Tipo V/análise , Estudos Transversais , Feminino , Humanos , Pessoa de Meia-Idade , Esclerodermia Localizada/imunologiaRESUMO
Radius and tibia bone microarchitecture, analyzed through a high-resolution peripheral quantitative computed tomography, were significantly impaired in female patients with diffuse systemic sclerosis compared with healthy controls. Acroosteolysis, quality of life-grip strength, hand disability, and disease duration were significantly associated with this bone deterioration. INTRODUCTION: The effect of diffuse systemic sclerosis (dSSc) on the bone is not completely understood. The objective of this study was to analyze the volumetric bone mineral density (vBMD), microarchitecture, and biomechanical parameters at the distal radius and tibia using high-resolution peripheral quantitative computed tomography (HR-pQCT, XtremeCT) in female patients with dSSc and identify clinical and laboratory variables associated with these parameters. METHODS: Thirty-eight women with dSSc and 76 healthy controls were submitted to HR-pQCT at the distal radius and tibia. Clinical and laboratory findings, bone mineral density(BMD), nailfold capillaroscopy (NC), total passive range of motion(ROM), and quality of life (health assessment questionnaire-HAQ) were associated with HR-pQCT (Scanco Medical AG, Brüttisellen, Switzerland) parameters. Multiple linear regression models adjusted for clinical and laboratory variables, ROM and HAQ, were performed. RESULTS: Density, microarchitecture, and biomechanical parameters at the distal radius and tibia were significantly impaired in dSSc patients compared with healthy controls (p < 0.001). Multiple linear regression models showed that lower trabecular density (Tb.vBMD) (radius R2 = 0.561, p = 0.002; and tibia R2 = 0.533, p = 0.005), and lower trabecular number (Tb.N) (tibia R2 = 0.533, p = 0.005) were significantly associated with acroosteolysis. Higher trabecular separation (Tb.Sp) was associated with disease duration and higher HAQ-grip strength (radius R2 = 0.489, p = 0.013), while cortical density (Ct.vBMD) was associated with ROM (radius R2 = 0.294, p = 0.002). CONCLUSION: Bone microarchitecture in patients with dSSc, analyzed through HR-pQCT, showed impairment of trabecular and cortical bone at distal radius and tibia. Variables associated with hand involvement (acroosteolysis, quality of life-grip strength, and ROM) and disease duration may be considered prognostic factors of this bone impairment.
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Densidade Óssea/fisiologia , Rádio (Anatomia)/fisiopatologia , Esclerodermia Difusa/fisiopatologia , Tíbia/fisiopatologia , Acro-Osteólise/etiologia , Acro-Osteólise/fisiopatologia , Adolescente , Adulto , Antropometria/métodos , Fenômenos Biomecânicos/fisiologia , Estudos de Casos e Controles , Feminino , Articulações dos Dedos/fisiopatologia , Força da Mão/fisiologia , Humanos , Angioscopia Microscópica , Pessoa de Meia-Idade , Qualidade de Vida , Rádio (Anatomia)/diagnóstico por imagem , Amplitude de Movimento Articular/fisiologia , Esclerodermia Difusa/complicações , Esclerodermia Difusa/diagnóstico por imagem , Tíbia/diagnóstico por imagem , Tomografia Computadorizada por Raios X , Adulto JovemRESUMO
INTRODUCTION: Systemic sclerosis (SSc) is a multisystemic autoimmune disease. Few studies have focused on the outcomes of SSC patients who require intensive care unit (ICU) admission, largely due to the absence of protocols for the optimal management of this disease during an ICU stay. OBJECTIVES: This study aimed to describe the outcomes of a series of SSc patients admitted to the ICU at a single center in Cali, Colombia. METHODS: Case series of SSc patients admitted to the ICU were reviewed. The main outcome was ICU mortality. Statistical analysis was performed with measures of central tendency and proportions. RESULTS: All the patients (n = 14) were female and either middle-aged or elderly; 9 (64%) were diagnosed with diffuse cutaneous sclerosis, and the remaining 5 patients with limited cutaneous sclerosis. Some were readmitted; therefore, the total number of ICU admissions was 21. The principal causes of ICU admissions were non-SSc-related causes (n = 15 [71.4%]). The respiratory system was the most involved on ICU admissions. The ICU mortality rate was 43% (n = 6). CONCLUSIONS: The severity of the disease at ICU admission and comorbidity are independently associated with ICU-related mortality. Furthermore, the optimal management of SSc patients includes accurate detection of SSc-associated organ involvement. More studies involving this category of patients are needed to establish the best effective protocols.
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Cuidados Críticos , Doenças Respiratórias , Esclerodermia Difusa , Esclerodermia Limitada , Idoso , Protocolos Clínicos/normas , Colômbia/epidemiologia , Comorbidade , Cuidados Críticos/métodos , Cuidados Críticos/normas , Cuidados Críticos/estatística & dados numéricos , Feminino , Necessidades e Demandas de Serviços de Saúde , Humanos , Unidades de Terapia Intensiva/estatística & dados numéricos , Masculino , Pessoa de Meia-Idade , Readmissão do Paciente/estatística & dados numéricos , Doenças Respiratórias/epidemiologia , Doenças Respiratórias/terapia , Estudos Retrospectivos , Esclerodermia Difusa/diagnóstico , Esclerodermia Difusa/mortalidade , Esclerodermia Difusa/terapia , Esclerodermia Limitada/diagnóstico , Esclerodermia Limitada/mortalidade , Esclerodermia Limitada/terapiaRESUMO
STUDY DESIGN: This study used a quasi-experimental design where patients were evaluated before and after participation in the self-management program. INTRODUCTION: Hands are commonly affected in systemic sclerosis (SSc). Strategies to maintain or improve hand function are indicated upon diagnosis and throughout the course of the disease. PURPOSE OF THE STUDY: The purpose of this study was to develop and evaluate a home-based program for hands in patients with SSc. METHODS: A home-based self-management program that consisted of concise instructions about SSc and hand exercises was developed and evaluated in a group of patients with SSc during 8 weeks. Primary outcome measures were hand pain (Visual Analogue Scale) and hand function (Cochin Hand Function Scale). Secondary outcome measures were disability (Scleroderma Health Assessment Questionnaire), finger motion (delta finger-to-palm), grip strength, tip and key pinch strength, Raynaud phenomenon and digital ulcers impact, quality of life (Short Form Health Survey). For comparisons between different times analysis of variance for repeated measures was used. To calculate the effect size (ES), the Cohen's test was performed. To evaluate skin moisturizing and warming habits before and after intervention, the McNemar test was used. Statistical significance was set at P ≤ .05. RESULTS: Twenty-two SSc patients (19 women: 3 men; 16 limited scleroderma: 6 diffuse scleroderma) completed the program. Significant improvements were noted for hand pain (3.97 vs 2.21, ES: 0.69), Cochin Hand Function Scale (19.24 vs 12.48, ES: 0.48), Scleroderma Health Assessment Questionnaire (0.95 vs 0.48, ES: 1.01), delta finger-to-palm (92.86 vs 106.33, ES: 0.40), grip strength (14.43 vs 19, ES: 0.58), tip pinch strength (2.49 vs 4.18, ES: 1.15), key pinch strength (4.01 vs 5.22, ES: 0.76), Raynaud phenomenon impact (0.94 vs 0.47, ES: 0.75), Short Form Health Survey-role physical (47.38 vs 60.14, ES: 0.61), physical functioning (34.62 vs 61.9, ES: 0.18), social functioning (60.71 vs 75.6, ES: 0.64), bodily pain (50.55 vs 63.38, ES: 0.58), vitality (45.95 vs 62, ES: 2.22), mental health (56.62 vs 72.38, ES: 0.84) moisturizing, and cold avoidance habits. Patients considered the program easy to follow with no adverse effects related to exercises. DISCUSSION: We developed a home based hand care program to be offered to SSc patients. Improvements in hand function, strength, disability, motion, and overall quality of life were independent of age, income, education level, disease duration, and skin score. Our findings support those of other studies that reported the benefits of hand exercises in SSc. Some study limitations include the lack of a control group, the small number of subjects and the short-time follow up. CONCLUSIONS: This home-based program for patients with SSc improved hand pain, function, mobility, and strength at the end of 8 weeks. Patient adherence and sustained efficacy is still to be determined.