Composición corporal en una serie de pacientes con esclerosis sistémica / Body Composition in a Series of Systemic Sclerosis Patients

Introducción: La esclerosis sistémica es una enfermedad de etiología desconocida crónica, caracterizada por fibrosis tisular, disfunción vascular y autoinmunidad. Entre otros síntomas, las alteraciones gastrointestinales son responsables del riesgo de malnutrición. De manera que la evaluación de la composición corporal es fundamental. Objetivo: Caracterizar la composición corporal de los pacientes con esclerosis sistémica. Métodos: Se realizó un estudio descriptivo y transversal de pacientes que se atienden en la consulta protocolizada del Servicio de Reumatología del Hospital Hermanos Ameijeiras. La muestra quedó conformada por 90 pacientes con diagnóstico confirmado de esclerosis sistémica, atendidos entre los años de 2019 y 2021. Resultados: Dentro de las manifestaciones clínicas, predominó en estos pacientes la forma difusa. Se observó que a mayor tiempo de la evolución se presentaban más alteraciones gastrointestinales y úlceras digitales. Más de la mitad de los pacientes tenían sobrepeso u obesidad y se encontraron valores altos y muy altos de grasa corporal. A mayor índice de masa corporal, mayores valores de grasa corporal, grasa visceral y menor masa magra. Los pacientes con bajo índice de masa corporal tenían una tendencia significativa a presentar alteraciones gastrointestinales y no se evidenciaron diferencias en los porcentajes de grasa visceral ni de grasa corporal sin embargo, el porciento de la masa magra fue significativamente inferior. Conclusiones: Se caracterizó la identificación temprana y detallada de esta enfermedad para disminuir la mortalidad y mejorar el pronóstico de estos pacientes, mediante el análisis de la composición corporal(AU)

Introduction: Systemic sclerosis is a chronic disease of unknown etiology, characterized by tissue fibrosis, vascular dysfunction and autoimmunity. Among other symptoms, gastrointestinal disturbances are responsible for the risk of malnutrition. In this sense, the evaluation of body composition is fundamental. Objective: To characterize the body composition of patients with systemic sclerosis. Methods: A descriptive and cross-sectional study was carried out in patients assisted in the protocolized consultation of the Rheumatology Service of Hospital Hermanos Ameijeiras. The sample was made up of 90 patients with confirmed diagnosis of systemic sclerosis who were treated from 2019 to 2021. Results: Diffuse form predominated in these patients as the clinical manifestations. It was observed that the longer the evolution, the more gastrointestinal alterations and digital ulcers were presented. More than half of the patients were overweight or obese, as high and very high values of body fat were found. The higher the body mass index, the higher the values of body fat, visceral fat and lower lean mass. Patients with low body mass index had significant tendency to present gastrointestinal alterations and no differences were found in the percentages of visceral fat or body fat. However, the percentage of lean mass was significantly lower. Conclusions: The early and detailed identification of this disease was characterized to reduce mortality and improve the prognosis of these patients through the analysis of body composition(AU)

Paul Klee y la esclerosis sistémica / Paul Klee and systemic sclerosis

Paul Klee representa uno de los pilares del arte de vanguardia del principio del siglo XX. Produjo una ingente cantidad de obras artísticas en sus 60 años de vida. La esclerosis sistémica se manifestó cinco años antes de morir, pero no por ello redujo su actividad creadora. En este artículo se analiza su historia y su enfermedad que tanto influyeron en su obra, y que lo convirtieron en ejemplo paradigmático de resiliencia ante la adversidad.

Paul Klee represents one of the pillars of avant-garde art of the early 20th century. He produced an enormous amount of artistic works in the sixty years of his life. Systemic sclerosis appeared five years before he died, but this did not reduce his creative activity. This article analyzes his life and his illness that so influenced his work and that is a paradigmatic example of resilience in the face of adversity.

Is exposure to environmental factors associated with a characteristic clinical and laboratory profile in systemic sclerosis? A retrospective analysis.

To identify environmental factors (EF) in a large cohort of patients with systemic sclerosis (SSc) analyzing their clinical and laboratory presentation. A cohort of consecutive patients attended at a single Brazilian SSc outpatient clinic was analyzed regarding EF. Data were analyzed according to clinical, demographic and laboratory characteristics, as well as SSc subtype. In a cohort of 662 patients, 70 (10.6%) had known previous exposure to EF, predominantly organic solvents (51.4%), silica (20%), silicone (12.9%) and pesticides (11.4%). In the SSc cohort, patients with EF had a significantly higher frequency of male gender (p < 0.01), African-Brazilian ethnicity (p = 0.01), myopathy (p = 0.02), and pigmentary disorders (p = 0.04), with shorter disease duration (p = 0.01). When SSc subtypes were analyzed separately, there was positive association with male gender in limited (p < 0.01) and diffuse (p < 0.01) SSc, as well as African-Brazilian ethnicity (p = 0.04), severe interstitial lung disease (p < 0.01), myopathy (p = 0.02) and SD pattern at nailfold capillaroscopy (p = 0.01) in limited SSc, and negative association with esophageal hypomotility (p < 0.01) and ANA positivity (p = 0.02) in diffuse SSc. Multiple regression analyses showed that myopathy was independently associated with previous exposure to EF (OR = 2.09; 95% CI 1.15-3.82), especially silica exposure (OR = 3.09; 95% CI 1.67-5.73). This study showed that SSc patients with previous exposure to EF may have some specific clinical characteristics, mainly a higher frequency of myopathy, also showing more severe ILD, preferably in male and African-Brazilian patients, associated with a lower frequency of ANA positivity.

Evaluation of the association between KIR polymorphisms and systemic sclerosis: a meta-analysis

Abstract Background: The results of investigations on the association between killer cell immunoglobulin-like receptor (KIR) gene polymorphisms and the risk of systemic sclerosis (SSc) are inconsistent. To comprehensively evaluate the influence of KIR polymorphisms on the risk of SSc, this meta-analysis was performed. Methods: A systematic literature search was performed in electronic databases including Scopus and PubMed/ MEDLINE to find all available studies involving KIR gene family polymorphisms and SSc risk prior to July 2019. Pooled odds ratios (ORs) and their corresponding 95% confidence intervals (CIs) were measured to detect associations between KIR gene family polymorphisms and SSc risk. Results: Five articles, comprising 571 patients and 796 healthy participants, evaluating the KIR gene family polymorphisms were included in the final meta-analysis according to the inclusion and exclusion criteria, and 16 KIR genes were assessed. None of the KIR genes were significantly associated with the risk of SSc. Conclusions: The current meta-analysis provides evidence that KIR genes might not be potential risk factors for SSc risk.(AU)

Pulmonary restriction secondary to cutaneous sclerosis caused by chronic graft versus host disease: Case Report / [Restricción pulmonar secundaria a esclerosis cutánea por enfermedad injerto contra huésped crónica: Reporte de Caso.]

Chronic graft versus host disease (GVHD) is a major complication of the allogeneic stem cell transplant. One of most frequent manifestations of GVHD is the cutaneous compromise with the sclerodermatous variety being the most severe. We considered that the restrictive respiratory compromise and its evolution are not well characterized. We described the functional respiratory alterations of a patient with sclerodermatous chronic GVHD and considered differential diagnosis of pulmonary restriction in this type of patient. We reported the case of a 21-year-old woman with pulmonary restriction secondary to cutaneous sclerosis which was caused by chronic GVHD. This report illustrates the importance of utilizing both functional respiratory tests and diagnosis images to accurately characterize the cause of the respiratory compromise. We believe that the functional alterations described in this case could be caused by the cutaneous disorder found.

La enfermedad injerto contra huésped crónica (EICH) es una complicación mayor del trasplante de células progenitoras alogénico. Una de sus manifestaciones más frecuentes es el compromiso cutáneo, siendo su variedad esclerodermiforme la más severa. El compromiso respiratorio, predominantemente restrictivo y su evolución no han sido bien caracterizados. El caso describe las alteraciones funcionales respiratorias de una paciente con EICH cutánea crónica tipo esclerodermiforme en dos momentos de su evolución y considera los diagnósticos diferenciales que producen restricción en estos pacientes. Se presenta el caso de una mujer de 21 años con restricción pulmonar secundaria a esclerosis cutánea por EICH. Este caso ilustra la relevancia de la utilización conjunta de las pruebas funcionales respiratorias y de los métodos diagnósticos por imágenes para caracterizar adecuadamente el compromiso respiratorio de estos pacientes. A nuestro juicio las alteraciones funcionales respiratorias descriptas pueden ser explicadas por el trastorno cutáneo hallado.

Impacto social de una conducta terapéutica diferente para la esclerosis sistémica / Social Impact of a New Therapeutic Conduct for the Systemic Sclerosis

Se realizó una revisión sobre la esclerosis sistémica con un enfoque filosófico social de carácter crítico e interdisciplinar, siguiendo una lógica investigativa. En el orden científico se obtiene una conducta terapéutica diferente y posiblemente más eficaz desde etapas tempranas, tiene como base el estadio clínico y compromiso órgano específico; en lo social evita complicaciones y muerte, en lo económico representa una ventaja para el paciente y familiares, para el sistema de salud, al minimizar costos por recursos de medios diagnósticos, otras terapéuticas aplicadas: estadías hospitalarias; en la comunidad científica constituyen una herramienta de trabajo para la atención especializada de pacientes esclerodérmicos. Se concluyó que una sustentación teórica sólida acerca de las ventajas de la terapéutica, genera nuevo conocimiento científico para su validación práctica, interdisciplinar las ramas del saber científico con enfoque histórico actual logran una visión social integral de un problema de la ciencia y la tecnología para soluciones sostenibles.(AU)

A review was made on systemic sclerosis with a social philosophical focus of a critical and interdisciplinary nature, following research logic. In the scientific order a different and possibly more effective therapeutic behavior from the early stages is obtained, based on the clinical stage and specific organ commitment. Regarding social approach it avoids complications and death, in the economic one it represents an advantage for the patient and relatives, minimizing costs of diagnostic means resources; another applied therapy constitutes hospital stays; in the scientific community it is a working tool for the specialized care of scleroderma patients. The authors concluded that a concrete theoretical support on the advantages of therapeutics, generates new scientific knowledge for its practical validation, interdisciplinary branches of scientific knowledge with current historical approach achieve a comprehensive social vision of a problem of science and technology for sustainable solutions.

Systemic sclerosis and silica exposure: a rare association in a large Brazilian cohort.

The objective of this study is to describe the characteristics of patients with Erasmus syndrome (ES) in a large SSc Brazilian cohort. Nine hundred and forty-seven SSc patients attended at the Scleroderma Outpatient Clinic at two academic medical centers in Brazil and classified as SSc according to the ACR/EULAR criteria were retrospectively studied. Information on demographics, clinical, and laboratory features was obtained by chart review. ES patients had their HLA class II characterized by PCR-SSO method as available. Among the 947 SSc patients studied, nine (0.9 %) had ES. These ES patients were predominantly male (78 %) and smokers (68 %) and presented diffuse SSc (67 %). Mean time of occupational exposure to silica was 13.7 years, with mean age at onset of 47 years. Previous history of tuberculosis was referred by 33 % of the ES patients. All the ES patients presented Raynaud's phenomenon, esophageal involvement, and interstitial lung disease (ILD). Antinuclear antibodies were present in all the ES patients, while anti-topoisomerase I was positive in 44 % and no patient had anticentromere antibody. Three different HLA-DQB alleles (0506, 0305, and 0303) were observed. Compared to non-ES cases, patients with ES were associated with male gender (p < 0.001), diffuse SSc (p < 0.05), ILD (p < 0.05), positive anti-topoisomerase I antibodies (p < 0.05), and death (p < 0.05). Multivariate analysis did not confirm that silicosis is an independent risk factor for SSc. To conclude, ES was rare in this large SSc cohort, although associated with a bad prognosis.

Atualização na etiopatogênese da esclerose sistêmica / Update on etiopathogenesis of systemic sclerosis

A Esclerose Sistêmica (ES) é uma doença autoimune de etiologia multifatorial, desencadeada pela combinação de fatores genéticos e ambientais. Sua variada expressão clínica resulta da complexa interação fisiopatogênica de três elementos principais: a vasculopatia proliferativa, a desregulação imunológica e a deposição e remodelamento anormais da matriz extracelular (MEC), da qual resulta a fibrose característica da doença. Eventos fisiopatogênicos precoces parecem ser a lesão endotelial e o desequilíbrio no reparo vascular, com a ativação de células endoteliais, do sistema imune e das plaquetas, com a liberação de múltiplos mediadores, como as citocinas proinflamatórias TH2 e os fatores de crescimento, desencadeando uma sequência de eventos simultâneos ou em cascata que envolve diversas vias de sinalização intracelular. O resultado mais importante desses eventos é a hiperativação dos fibroblastos, as principais células efetoras da fibrose, as quais passam a produzir grandes quantidades de constituintes da MEC e a secretar múltiplos fatores de crescimento e citocinas que perpetuam o processo. Neste artigo apresentamos uma revisão dos principais fatores potencialmente implicados na etiologia da ES e revisitamos os conhecimentos atuais sobre os mais importantes mecanismos envolvidos no desenvolvimento das lesões características da doença. O melhor entendimento desses mecanismos fisiopatogênicos possibilita identificar potenciais alvos terapêuticos, o que pode resultar em avanços no manejo dessa complexa e debilitante doença.

Systemic Sclerosis (SSc) is an autoimmune disease of multifactorial etiology, triggered by a combination of genetic and environmental factors. Its varied clinical expression results from the complex physiopathogenic interaction of three main elements: proliferative vasculopathy, immune dysregulation and abnormal deposition and remodeling of the extracellular matrix (ECM), of which the characteristic disease fibrosis is the result. Early physiopathogenic events appear to be endothelial injury and imbalance in vascular repair with the activation of endothelial cells, the immune system and platelets, with the release of multiple mediators such as TH2 proinflammatory cytokines and growth factors, triggering a sequence of simultaneous or cascading events that involve several intracellular signaling pathways. The most important result of these events is the hyperactivation of fibroblasts, the main effector cells of fibrosis, which will then produce large amounts of ECM constituents and secrete multiple growth factors and cytokines that perpetuate the process. In this article we review the main factors potentially involved in the etiology of SSc and reexamine the current knowledge about the most important mechanisms involved in the development of lesions that are characteristic of the disease. A better understanding of these physiopathogenic mechanisms will help identify potential therapeutic targets, which may result in advances in the management of this complex and debilitating disease.

Erasmus syndrome: silicosis and systemic sclerosis.

The silicosis is the pneumoconiosis more frequent, resulting from the inhalation of silica or silicates containing mineral dust, mainly characterized by irreversible lung fibrosis. It is associated with the development of other diseases, including pulmonary tuberculosis, lung cancer and autoimmune diseases. The connective tissue disease after exposure to silica occurs usually after 15 years of initial exposure.The Erasmus syndrome describes the association of systemic sclerosis following exposure to silica with or without silicosis. The authors report two cases of patients with diagnosis of silicosis who developed systemic sclerosis.

Síndrome de Erasmus: silicose e esclerose sistêmica / Erasmus syndrome: silicosis and systemic sclerosis

A silicose é a pneumoconiose mais frequente, decorrente da inalação de sílica ou de poeiras minerais contendo silicatos, caracterizada principalmente pela fibrose pulmonar de caráter irreversível. Está associada com o desenvolvimento de outras doenças, incluindo tuberculose pulmonar, câncer pulmonar e doenças autoimunes. A doença do tecido conjuntivo posterior à exposição à sílica ocorre geralmente após 15 anos do início da exposição. A síndrome de Erasmus refere-se ao desenvolvimento de esclerose sistêmica em indivíduos previamente expostos à sílica e que apresentavam ou não silicose. Os autores relatam dois casos de pacientes com diagnóstico de silicose que desenvolveram esclerose sistêmica.

The silicosis is the pneumoconiosis more frequent, resulting from the inhalation of silica or silicates containing mineral dust, mainly characterized by irreversible lung fi brosis. It is associated with the development of other diseases, including pulmonary tuberculosis, lung cancer and autoimmune diseases. The connective tissue disease after exposure to silica occurs usually after 15 years of initial exposure.The Erasmus syndrome describes the association of systemic sclerosis following exposure to silica with or without silicosis. The authors report two cases of patients with diagnosis of silicosis who developed systemic sclerosis.

[Scleroderma: an update on the pathogenesis and treatment]. / Etiopatogenia y tratamiento de la esclerodermia. Conceptos actuales.

Scleroderma is a multiorganic disease characterized by inflammatory, vascular and sclerotic changes in skin and internal organs. It is considered as a tripartite disease, associated to autoimmune, fibroblast and endothelial defect, due to genetic, environmental and infectious factors. This disease can be classified in systemic and localized form. The Raynaud phenomenon occurs in 90% of the patients with the diagnosis. It explains the microcirculation involvement and the reduction in the number of capillaries. Malformation of nail bed capillaries is readily demonstrated by nail bed microscopy and has been shown to correlate both with disease severity and with degree of internal organ involvement. The MRSS-51 validates the skin involvement and has the main predictive value to determine the patient survival. MRSS-51 should not be considered as an activity disease parameter or used to validate the effectiveness of treatment. Nowadays, multiple treatment alternatives exist for scleroderma disease; however these treatments offer poor results for the cutaneous manifestations.

Update on etiopathogenesis of systemic sclerosis.

Systemic Sclerosis (SSc) is an autoimmune disease of multifactorial etiology, triggered by a combination of genetic and environmental factors. Its varied clinical expression results from the complex physiopathogenic interaction of three main elements: proliferative vasculopathy, immune dysregulation and abnormal deposition and remodeling of the extracellular matrix (ECM), of which the characteristic disease fibrosis is the result. Early physiopathogenic events appear to be endothelial injury and imbalance in vascular repair with the activation of endothelial cells, the immune system and platelets, with the release of multiple mediators such as TH2 proinflammatory cytokines and growth factors, triggering a sequence of simultaneous or cascading events that involve several intracellular signaling pathways. The most important result of these events is the hyperactivation of fibroblasts, the main effector cells of fibrosis, which will then produce large amounts of ECM constituents and secrete multiple growth factors and cytokines that perpetuate the process. In this article we review the main factors potentially involved in the etiology of SSc and reexamine the current knowledge about the most important mechanisms involved in the development of lesions that are characteristic of the disease. A better understanding of these physiopathogenic mechanisms will help identify potential therapeutic targets, which may result in advances in the management of this complex and debilitating disease.

Systemic sclerosis sine scleroderma associated with antiphospholipid syndrome.

The antiphospholipid syndrome (APS) can be primary, when it occurs alone, or secondary, when it is associated with another autoimmune disease, mainly systemic lupus erythematosus and rarely other autoimmune diseases. Cases described in literature (Medline 1966 to December 2009) associate the presence of antiphospholipid antibodies with the presence of APS and systemic sclerosis (SS). Currently, however, no cases of the SS variant sine scleroderma with APS have been described. In this study, the authors describe the case of a patient with APS characterised by thrombosis of the retinal veins, in May 2006, the presence of lupus anticoagulant and an anticardiolipin IgG antibody. In May 2007, this patient developed Raynaud's phenomenon, a lack of oesophageal motility and nailfold capillaroscopy with a scleroderma pattern. The patient was positive for the anti-centromere antibody but lacked any evidence of cutaneous thickening or involvement. In summary, the authors describe the first case of a patient with APS associated with SS sine scleroderma.

Etiopatogenia de la esclerosis sistémica progresiva / Etiopathogenesis of systemic sclerosis

La esclerosis sistémica progresiva (ESP) es una enfermedad autoinmune crónica del tejido conectivo. Se caracteriza por una alteración vascular inicial, una respuesta inmune alterada con producción de autoanticuerpos y un exceso de síntesis y depósito de fibras de colágeno en la piel y tejido conectivo. La activación y el daño endotelial son eventos tempranos en la patogenia de la enfermedad; sin embargo, el factor gatillante continúa siendo desconocido. Se piensa que el evento principal sería la interacción entre eventos autoinmunes y cambios vasculares tempranos, lo cual resulta en la generación de fibroblastos activados considerados como las células efectoras de la enfermedad. Se reconocen dos subgrupos clínicos de ESP: la variedad cutánea limitada y la variedad cutánea difusa, las cuales presentan distintos patrones de compromiso orgánico, autoanticuerpos, evolución y sobrevida.

Progressive systemic sclerosis is a chronic autoimmune disease of connective tissue. It is characterized by early vascular changes, altered immune response with production of auto-antibodies, and excessive synthesis and deposition of collagen fibers in the skin and connective tissue. Activation and endothelial cell damage are early events in the pathogenesis of the disease, but the precise triggering event(s) remain elusive. The main event would be the interaction between autoimmune events early vascular changes, resulting in the generation of activated fibroblasts, regarded as effector cells of the disease. There are two major subgroups of SSc, the limited cutaneous and the diffuse cutaneous variety, which have distinct patterns of organ involvement, self-auto-antibodies, evolution and survival.

Miocardiopatía restrictiva y comunicación interauricular en un paciente con esclerodermia / Restrictive cardiomyopathy and atrial septal defect in a patient with scleroderma

Se presentó un paciente con esclerodermia que se diagnosticó a partir de las manifestaciones cardiovasculares de la enfermedad: palpitaciones, disnea de esfuerzo, inflamación de manos y pies e incluso la cara. Clínicamente se manifestó fenómeno de Raynaud y litiasis renal. Eléctricamente, fibrilación auricular de respuesta rápida y por la ecocardiogiografia, patrón de miocardiopatia restrictiva, hipertensión pulmonar y comunicación interauricular tipo fosa oval (AU)

A patient was reported with a diagnosis of scleroderma based on the cardiovascular manifestations of the disease: palpitation, exertional dyspnea, swelling of the hands and feet and even the face. Clinical manifestations were Raynaud's phenomenon and renal lithiasis. Electrically, rapid response atrial fibrillation, and ecocardiogiographically, a pattern of restrictive cardiomyopathy, pulmonary hypertension and fossa ovalis atrial septal defect (AU)

Miocardiopatía restrictiva y comunicación interauricular en un paciente con esclerodermia / Restrictive cardiomyopathy and atrial septal defect in a patient with scleroderma

Se presentó un paciente con esclerodermia que se diagnosticó a partir de las manifestaciones cardiovasculares de la enfermedad: palpitaciones, disnea de esfuerzo, inflamación de manos y pies e incluso la cara. Clínicamente se manifestó fenómeno de Raynaud y litiasis renal. Eléctricamente, fibrilación auricular de respuesta rápida y por la ecocardiogiografia, patrón de miocardiopatia restrictiva, hipertensión pulmonar y comunicación interauricular tipo fosa oval

A patient was reported with a diagnosis of scleroderma based on the cardiovascular manifestations of the disease: palpitation, exertional dyspnea, swelling of the hands and feet and even the face. Clinical manifestations were Raynaud's phenomenon and renal lithiasis. Electrically, rapid response atrial fibrillation, and ecocardiogiographically, a pattern of restrictive cardiomyopathy, pulmonary hypertension and fossa ovalis atrial septal defect

Systemic sclerosis: environmental factors.

Epidemiological evidence for the association between environmental and occupational risk factors and systemic sclerosis (SSc) has been extensively analyzed. Such exposures are frequently of long duration, and the inadequate classification of the type of exposure and other confounding variables may bias their estimated association with SSc. Environmental factors could be classified as occupational (silica, organic solvents), infectious (bacterial, viral), and non-occupational/non-infectious (drugs, pesticides, silicones). Understanding the link between environmental risk factors and the development of SSc is limited, due to the phenotypic and pathogenic heterogeneity of patients and disease, respectively, and also due to poor ability to assess environmental exposures quantitatively and the role of the gene-environment interactions in this disease. Global collaboration could increase the chance for a better use of the data obtained from a limited number of cases and also limited resources. Normalization and validation of biomarkers and questionnaires could also be very useful to reliably quantify environmental exposures.

Esclerose sistêmica difusa grave: relato de caso em homem / Severe diffuse systemic sclerosis: a case report in man

Homem de 64 anos se apresentou no serviço de emergência relatando espessamento progressivo da pele, lombalgia, disfagia e lesões necróticas dolorosas em quirodáctilos. Foi feito o diagnóstico de esclerose sistêmica difusa grave, com insuficiência renal crônica, fibrose pulmonar e miocardiopatia secundárias a esclerodermia. São discutidas a manifestação grave dessa doença, suas complicações e apresentação no sexo masculino.

A 64-year-old male presented at the emergency unit complaining of progressive hardening of the skin, lumbago, dysphagia and painful necrotic lesions in the fingers. He was diagnosed with severe diffuse systemic sclerosis and chronic kidney failure, pulmonary fibrosis, and myocardiopathy secondary to scleroderma. Here we discuss the severe manifestation of this disease, its complications, and its presentation in the male sex.

Systemic sclerosis: a world wide global analysis.

The objective of this study was to analyze epidemiological tendencies of systemic sclerosis (SSc) around the world in order to identify possible local variations in the presentation and occurrence of the disease. A systematic review of the literature was performed through electronic databases using the keywords "Systemic Sclerosis" and "Clinical Characteristics." Out of a total of 167 articles, 41 were included in the analysis. Significant differences in the mean age at the time of diagnosis, subsets of SSc, clinical characteristics, and presence of antibodies were found between different regions of the word. Because variations in both additive and nonadditive genetic factors and the environmental variance are specific to the investigated population, ethnicity and geography are important characteristics to be considered in the study of SSc and other autoimmune diseases.