Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 100
Filtrar
1.
J Rheumatol ; 2021 Nov 15.
Artigo em Inglês | MEDLINE | ID: mdl-34782448

RESUMO

OBJECTIVE: There is still a great deal to learn about the influence of gender in systemic sclerosis (SSc). In this respect, national registries provide large and homogeneous patient cohorts for analytical studies. We therefore investigated a wide-ranging and well-characterized SSc series with the aim of identifying gender differences in disease expressions, with a special focus on demographic, clinical and serological characteristics. METHODS: A multicenter SSc cohort of 2,281 patients, 247 men, was recruited in the Italian SPRING (Systemic Sclerosis PRogression INvestiGation) registry. Demographic data, disease manifestations, serological profile and internal organ involvement were compared. RESULTS: The overall female/male ratio was 8.2/1. Female/male ratios for limited SSc, diffuse SSc and sine SSc subsets were 8.7/1, 4.9/1, and 10.7/1 respectively. A shorter Raynaud's onset to SSc diagnosis, an increased prevalence of diffuse cutaneous subset, renal crisis, and digital ulcers were found in males, while a significant higher percentage of sicca syndrome, serum ANA, anti-ENA, anti-La/SSB, and anti-CENP-1 was detected in the female group. Males exhibited lower left ventricular ejection fraction, higher prevalence of conduction blocks, arrhythmias, ground glass and honeycombing. Moreover, forced vital capacity and total lung capacity were medially lower in men than in women. Finally, males were more frequently treated with immunosuppressive drugs. CONCLUSION: Our study further supports the presence of several gender-related differences in SSc patients. These differences were pronounced as regards the severity of cutaneous, peripheral vascular and cardiopulmonary involvement for male patients, whereas an increased prevalence of sicca syndrome and a specific autoantibody profile characterize the female gender.

2.
Ann Med Surg (Lond) ; 71: 102984, 2021 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-34840747

RESUMO

Systemic sclerosis is a connective tissue disease with cutaneous involvement. Clinical manifestations result from the balance of inflammations/autoimmunity process and fibrogenesis. Patients suffer from skin ulcers, non-ulcerative lesions including digital pitting scars, telangiectasias, subungual hyperkeratosis, abrasions, fissures, and subcutaneous calcinosis. A review about the pathophysiology of the disease, the physical examination of the patients, the instrumental assessment, and possible treatments is performed.

3.
J Autoimmun ; 125: 102744, 2021 Nov 10.
Artigo em Inglês | MEDLINE | ID: mdl-34781162

RESUMO

Autoimmune systemic diseases (ASD) may show impaired immunogenicity to COVID-19 vaccines. Our prospective observational multicenter study aimed to evaluate the seroconversion after the vaccination cycle and at 6-12-month follow-up, as well the safety and efficacy of vaccines in preventing COVID-19. The study included 478 unselected ASD patients (mean age 59 ± 15 years), namely 101 rheumatoid arthritis (RA), 38 systemic lupus erythematosus (SLE), 265 systemic sclerosis (SSc), 61 cryoglobulinemic vasculitis (CV), and a miscellanea of 13 systemic vasculitis. The control group included 502 individuals from the general population (mean age 59 ± 14SD years). The immunogenicity of mRNA COVID-19 vaccines (BNT162b2 and mRNA-1273) was evaluated by measuring serum IgG-neutralizing antibody (NAb) (SARS-CoV-2 IgG II Quant antibody test kit; Abbott Laboratories, Chicago, IL) on samples obtained within 3 weeks after vaccination cycle. The short-term results of our prospective study revealed significantly lower NAb levels in ASD series compared to controls [286 (53-1203) vs 825 (451-1542) BAU/mL, p < 0.0001], as well as between single ASD subgroups and controls. More interestingly, higher percentage of non-responders to vaccine was recorded in ASD patients compared to controls [13.2% (63/478), vs 2.8% (14/502); p < 0.0001]. Increased prevalence of non-response to vaccine was also observed in different ASD subgroups, in patients with ASD-related interstitial lung disease (p = 0.009), and in those treated with glucocorticoids (p = 0.002), mycophenolate-mofetil (p < 0.0001), or rituximab (p < 0.0001). Comparable percentages of vaccine-related adverse effects were recorded among responder and non-responder ASD patients. Patients with weak/absent seroconversion, believed to be immune to SARS-CoV-2 infection, are at high risk to develop COVID-19. Early determination of serum NAb after vaccination cycle may allow to identify three main groups of ASD patients: responders, subjects with suboptimal response, non-responders. Patients with suboptimal response should be prioritized for a booster-dose of vaccine, while a different type of vaccine could be administered to non-responder individuals.

4.
J Autoimmun ; 124: 102727, 2021 11.
Artigo em Inglês | MEDLINE | ID: mdl-34601207

RESUMO

Systemic sclerosis (SSc) is a connective tissue disease secondary to three cardinal pathological features: immune-system alterations, diffuse microangiopathy, and fibrosis involving the skin and internal organs. The etiology of SSc remains quite obscure; it may encompass multiple host genetic and environmental -infectious/chemical-factors. The present review focused on the potential role of environmental agents in the etiopathogenesis of SSc based on epidemiological, clinical, and laboratory investigations previously published in the world literature. Among infectious agents, some viruses that may persist and reactivate in infected individuals, namely human cytomegalovirus (HCMV), human herpesvirus-6 (HHV-6), and parvovirus B19 (B19V), and retroviruses have been proposed as potential causative agents of SSc. These viruses share a number of biological activities and consequent pathological alterations, such as endothelial dysfunction and/or fibroblast activation. Moreover, the acute worsening of pre-existing interstitial lung involvement observed in SSc patients with symptomatic SARS-CoV-2 infection might suggest a potential role of this virus in the overall disease outcome. A variety of chemical/occupational agents might be regarded as putative etiological factors of SSc. In this setting, the SSc complicating silica dust exposure represents one of the most promising models of study. Considering the complexity of SSc pathogenesis, none of suggested causative factors may explain the appearance of the whole SSc; it is likely that the disease is the result of a multifactorial and multistep pathogenetic process. A variable combination of potential etiological factors may modulate the appearance of different clinical phenotypes detectable in individual scleroderma patients. The in-deep investigations on the SSc etiopathogenesis may provide useful insights in the broad field of human diseases characterized by diffuse microangiopathy or altered fibrogenesis.


Assuntos
COVID-19/complicações , Infecções por Citomegalovirus/complicações , Exposição Ocupacional/efeitos adversos , Infecções por Parvoviridae/complicações , Infecções por Retroviridae/complicações , Infecções por Roseolovirus/complicações , SARS-CoV-2 , Escleroderma Sistêmico/etiologia , Citomegalovirus , Herpesvirus Humano 6 , Humanos , Parvovirus B19 Humano , Retroviridae , Escleroderma Sistêmico/virologia
5.
Adv Skin Wound Care ; 2021 Oct 06.
Artigo em Inglês | MEDLINE | ID: mdl-34629385

RESUMO

ABSTRACT: A novel SARS-CoV-2 infection, causing the disease designated as COVID-19 principally affecting the respiratory tract, lung at alveolar and interstitial levels, has recently emerged. Systemic sclerosis (SSc) is an autoimmune connective disease characterized by vascular abnormalities and diffuse and progressive fibrosis of the skin and internal organs. Raynaud phenomenon occurs in virtually all patients affected by SSc and in most cases represents the onset symptom of the disease, since it could appear several years before the overt illness. Although the exact pathophysiologic pathways leading to RP and SSc are still unknown, several infectious agents, especially viruses, have been suggested as possible triggering factors. Here authors describe the first case of a Raynaud phenomenon secondary to Systemic sclerosis following SARS-CoV2 infection.

6.
Curr Pharm Des ; 27(41): 4245-4252, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34477509

RESUMO

BACKGROUND: The Covid-19 pandemic may have a deleterious impact on patients with autoimmune systemic diseases (ASD) due to their deep immune-system alterations. OBJECTIVE: This study aims to investigate the prevalence of symptomatic Covid-19 and its correlations with both organ involvement and ongoing treatments in a large series of Italian ASD patients during the first wave of pandemic. METHODS: Our multicenter telephone 6-week survey included 3,029 unselected ASD patients enrolled at 36 tertiary referral centers of northern, central, and southern Italian macro-areas with different diffusion of the pandemic. Symptomatic SARS-CoV-2 infection was classified as definite Covid-19 (presence of symptoms plus positive oral/nasopharyngeal swabs) or highly suspected Covid-19 (highly suggestive symptoms, in the absence of a swab testing). RESULTS: A significantly higher prevalence of definite plus highly suspected Covid-19 compared to the Italian general population was detected in the whole ASD series (p=.000), as well as in patients from the three macro-areas (p=.000 in all). Statistically higher prevalence of Covid-19 was also found in connective tissue diseases compared to chronic arthritis subgroup (p=.000) and in ASD patients with pre-existing interstitial lung involvement (p=.000). Patients treated with either conventional disease-modifying anti-rheumatic drugs (DMARDs) and/or biological DMARDs showed a significantly lower prevalence of Covid-19 (p=.000 in both). Finally, scleroderma patients undergoing low-dose aspirin showed a significantly lower rate of Covid-19 compared to those without (p=0.003). CONCLUSION: The higher prevalence of Covid-19 in ASD patients, along with the significant correlations with important clinical features and therapeutic regimens, suggests the need to develop targeted prevention/management strategies during the current pandemic wave.


Assuntos
Doenças Autoimunes , COVID-19 , Doenças Reumáticas , Doenças Autoimunes/tratamento farmacológico , Doenças Autoimunes/epidemiologia , Humanos , Pulmão , Pandemias , Doenças Reumáticas/tratamento farmacológico , Doenças Reumáticas/epidemiologia , SARS-CoV-2
7.
J Pers Med ; 11(6)2021 Jun 20.
Artigo em Inglês | MEDLINE | ID: mdl-34203014

RESUMO

Systemic Sclerosis (SSc) is a chronic disease associated with a 1.5-fold increase in cancer risk, including lung cancer, hematological malignancies, and breast cancer (BC). This is a retrospective study aiming to explore the clinical and pathological features of BC developed by SSc patients. A total of 54.5% of patients developed BC before SSc (median interval: 5 years), whereas 45.5% of patients developed BC after SSc (median delay: 8 years). A total of 93.1% of patients were diagnosed with an early stage tumor. Among invasive carcinomas, 70.8% presented with a low Mib1, 8.3% with a tubular histotype, and 42.8% with a Luminal A-like phenotype. A total of 66.6% of patients underwent breast-conserving surgery and 55.5% RT. A total of 40% of patients developed interstitial lung disease after RT and 20% diffuse cutaneous SSc. The cause of death of the six deceased patients was PAH. A significant association was observed between the use of immunosuppressive therapy and diffuse skin extension, negative ACA, positive Anti-Scl-70, and interstitial lung disease, but not BC status. SSc patients developed BC at a good prognosis, suggesting a de-escalation strategy of cancer therapies. In particular, ionizing radiation and chemotherapeuticals should be limited to higher-risk cases. Finally, proper screening is mandatory in order to allow for early cancer detection in SSc patients.

9.
Clin Med Insights Circ Respir Pulm Med ; 15: 11795484211001349, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33795941

RESUMO

Objective: COVID-19 pandemic represents a serious health emergency that severely compromised our Public Health system, resulting in a rapid and forced reorganization and involved the management of chronic diseases too. The Scleroderma Unit of Modena and Reggio Emilia follows more than 600 patients suffering from systemic sclerosis (SSc) and recently became the referral center (HUB) in Emilia-Romagna for this rare connective tissue disease. The aim of the present study was to evaluate the extent by which the lockdown and the pandemic has impacted the activity of admissions to Scleroderma Unit of Modena and Reggio Emilia. Methods: Our daily clinical activity is characterized by outpatient visits, videocapillaroscopy exam, ulcers treatment, therapeutic infusions in day hospital regimen, multidisciplinary visits following our dedicated SSc care pathway, and clinical trials. Our activity has been quickly rescheduled to ensure the proper assistance to our SSc patients during the COVID-19 pressure. Results: The use of telemedicine has certainly assured a robust continuity of health care. Furthermore, telephone pre-triage, nurse/medical triage, proper physical distancing and use of PPE/DPI allowed us to re-organize and continue SSc daily activity. Specifically, therapeutic infusions in day hospital regimen and outpatient visits, including ulcers treatment, was guaranteed and maximized. Conclusion: The management of scleroderma patients by an expert specialist reference center is crucial in order to ensure continuity of care and pursue the best SSc practice.

11.
Case Reports Plast Surg Hand Surg ; 8(1): 18-22, 2021 Feb 12.
Artigo em Inglês | MEDLINE | ID: mdl-33628864

RESUMO

Here we describe the case of a 60-year-old-woman with systemic sclerosis sent to our Scleroderma Unit to treat digital stumps. The stumps were successfully treated with autologous fat grafting (crown-shape infiltration). Our technique of autologous lipotransfer improved wound healing in a scleroderma patient with stump-digital ulcers where all other options failed.

13.
Dermatol Reports ; 13(3): 9075, 2021 Nov 17.
Artigo em Inglês | MEDLINE | ID: mdl-35003566

RESUMO

Systemic sclerosis (SSc) is a complex autoimmune and up to 50% of patients develop digital ulcers. We revised fifty consecutive patients with SSc-related digital ulcers (DUs) who referred to our Scleroderma Unit. Thirty-five of them who showed clear signs of DUs infection underwent to cutaneous swab and microbiological data collection. We performed 87 cutaneous swabs overall. DUs were recurrent in 58% of the patients and multiple in 60% of patients. Fourty-four swabs (53%) were positive for Staphylococcus aureus (13% Methicillin-Resistant), 9 (10%) were positive for Pseudomonas aeruginosa, and then the others less frequently isolated. Nine patients (25%) needed hospitalization. Our data support a patient-tailored approached to DUs, particularly those infected. Selfhygiene and asepsis during dressing procedures are mandatory. Patient must be trained to avoid dangerous behaviors and reduce the risk of infection.

15.
Clin Rheumatol ; 39(11): 3163-3170, 2020 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-32979101

RESUMO

Resilience is defined as "the capacity of individuals to cope successfully with significant change or adversity". The challenge posed by the COVID-19 pandemic may potentially represent an overwhelmingly stressful event for patients with chronic diseases. Aim of our study was to investigate the levels of resilience in individuals with inflammatory arthritis living in Emilia Romagna, the third hardest-hit Italian region during the ongoing COVID-19 pandemic. To this purpose, we developed a survey consisting of four different sections assessing demographic characteristics, the 14-item resilience scale (RS14) and questionnaires evaluating depression and anxiety. Consecutive patients with inflammatory arthritis were recruited over a short time frame immediately after the end of national lockdown and compared with control individuals from the general population. One hundred twenty-two patients and 173 controls were included. Levels of resilience, as measured by RS14 score, were significantly higher in patients with inflammatory arthritis (82.6 ± 14.0 vs 79.0 ± 12.8, p = 0.018). After stratification for gender, the difference in RS14 score was maintained in women (p = 0.045), but not in men (p = 0.252). High resilience, defined as having a RS14 score > 90, was significantly more prevalent in patients than in controls (30% vs 16%, p = 0.009). In arthritis patients, no significant differences in RS14 were observed after stratification for specific diagnosis, age, or disease duration and activity. Our findings suggest that patients with inflammatory arthritis may be more resilient than the general population towards unexpected stressful events such as the ongoing COVID-19 pandemic. Key Points • Living with inflammatory arthritis may foster resilience. • After COVID-19, patients with inflammatory arthritis were more resilient than the general population.


Assuntos
Adaptação Psicológica , Ansiedade/psicologia , Artrite Reumatoide/psicologia , Infecções por Coronavirus , Depressão/psicologia , Pandemias , Pneumonia Viral , Resiliência Psicológica , Espondiloartropatias/psicologia , Estresse Psicológico/psicologia , Adulto , Idoso , Artrite Psoriásica/psicologia , Betacoronavirus , COVID-19 , Estudos de Casos e Controles , Feminino , Humanos , Itália , Masculino , Pessoa de Meia-Idade , SARS-CoV-2 , Fatores Sexuais
16.
Clin Rheumatol ; 39(11): 3195-3204, 2020 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-32852623

RESUMO

INTRODUCTION: Covid-19 infection poses a serious challenge for immune-compromised patients with inflammatory autoimmune systemic diseases. We investigated the clinical-epidemiological findings of 1641 autoimmune systemic disease Italian patients during the Covid-19 pandemic. METHOD: This observational multicenter study included 1641 unselected patients with autoimmune systemic diseases from three Italian geographical areas with different prevalence of Covid-19 [high in north (Emilia Romagna), medium in central (Tuscany), and low in south (Calabria)] by means of telephone 6-week survey. Covid-19 was classified as (1) definite diagnosis of Covid-19 disease: presence of symptomatic Covid-19 infection, confirmed by positive oral/nasopharyngeal swabs; (2) highly suspected Covid-19 disease: presence of highly suggestive symptoms, in absence of a swab test. RESULTS: A significantly higher prevalence of patients with definite diagnosis of Covid-19 disease, or with highly suspected Covid-19 disease, or both the conditions together, was observed in the whole autoimmune systemic disease series, compared to "Italian general population" (p = .030, p = .001, p = .000, respectively); and for definite + highly suspected diagnosis of Covid-19 disease, in patients with autoimmune systemic diseases of the three regions (p = .000, for all comparisons with the respective regional general population). Moreover, significantly higher prevalence of definite + highly suspected diagnosis of Covid-19 disease was found either in patients with various "connective tissue diseases" compared to "inflammatory arthritis group" (p < .000), or in patients without ongoing conventional synthetic disease-modifying anti-rheumatic drugs treatments (p = .011). CONCLUSIONS: The finding of a higher prevalence of Covid-19 in patients with autoimmune systemic diseases is particularly important, suggesting the need to develop valuable prevention/management strategies, and stimulates in-depth investigations to verify the possible interactions between Covid-19 infection and impaired immune-system of autoimmune systemic diseases. Key Points • Significantly higher prevalence of Covid-19 is observed in a large series of patients with autoimmune systemic diseases compared to the Italian general population, mainly due to patients' increased susceptibility to infections and favored by the high exposure to the virus at medical facilities before the restriction measures on individual movement. • The actual prevalence of Covid-19 in autoimmune systemic diseases may be underestimated, possibly due to the wide clinical overlapping between the two conditions, the generally mild Covid-19 disease manifestations, and the limited availability of virological testing. • Patients with "connective tissue diseases" show a significantly higher prevalence of Covid-19, possibly due to deeper immune-system impairment, with respect to "inflammatory arthritis group". • Covid-19 is more frequent in the subgroup of autoimmune systemic diseases patients without ongoing conventional synthetic disease-modifying anti-rheumatic drugs, mainly hydroxyl-chloroquine and methotrexate, which might play some protective role against the most harmful manifestations of Covid-19.


Assuntos
Doenças Autoimunes/epidemiologia , Infecções por Coronavirus/epidemiologia , Pneumonia Viral/epidemiologia , Doenças Reumáticas/epidemiologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Antirreumáticos/uso terapêutico , Artrite Psoriásica/tratamento farmacológico , Artrite Psoriásica/epidemiologia , Artrite Psoriásica/fisiopatologia , Artrite Reumatoide/tratamento farmacológico , Artrite Reumatoide/epidemiologia , Artrite Reumatoide/fisiopatologia , Doenças Autoimunes/tratamento farmacológico , Doenças Autoimunes/fisiopatologia , Betacoronavirus , COVID-19 , Infecções por Coronavirus/fisiopatologia , Dermatomiosite/tratamento farmacológico , Dermatomiosite/epidemiologia , Dermatomiosite/fisiopatologia , Feminino , Glucocorticoides/uso terapêutico , Humanos , Itália/epidemiologia , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Lúpus Eritematoso Sistêmico/epidemiologia , Lúpus Eritematoso Sistêmico/fisiopatologia , Masculino , Pessoa de Meia-Idade , Pandemias , Pneumonia Viral/fisiopatologia , Doenças Reumáticas/tratamento farmacológico , Doenças Reumáticas/fisiopatologia , SARS-CoV-2 , Escleroderma Sistêmico/tratamento farmacológico , Escleroderma Sistêmico/epidemiologia , Escleroderma Sistêmico/fisiopatologia , Síndrome de Sjogren/tratamento farmacológico , Síndrome de Sjogren/epidemiologia , Síndrome de Sjogren/fisiopatologia , Espondilite Anquilosante/tratamento farmacológico , Espondilite Anquilosante/epidemiologia , Espondilite Anquilosante/fisiopatologia , Doenças do Tecido Conjuntivo Indiferenciado/tratamento farmacológico , Doenças do Tecido Conjuntivo Indiferenciado/epidemiologia , Doenças do Tecido Conjuntivo Indiferenciado/fisiopatologia
17.
Ann Rheum Dis ; 79(9): 1210-1217, 2020 09.
Artigo em Inglês | MEDLINE | ID: mdl-32606043

RESUMO

OBJECTIVE: To prospectively investigate whether differences in pulmonary vasculature exist in systemic sclerosis (SSc) and how they are distributed in patients with different pulmonary function. METHODS: Seventy-four patients with SSc undergoing chest CT scan for interstitial lung disease (ILD) screening or follow-up were prospectively enrolled. A thorough clinical, laboratory and functional evaluation was performed the same day. Chest CT was spirometry gated at total lung capacity and images were analysed by two automated software programs to quantify emphysema, ILD patterns (ground-glass, reticular, honeycombing), and pulmonary vascular volume (PVV). Patients were divided in restricted (FVC% <80, DLco%<80), isolated DLco% reduction (iDLco- FVC%≥80, DLco%<80) and normals (FVC%≥80, DLco%≥80). Spearman ρ, Mann-Whitney tests and logistic regressions were used to assess for correlations, differences among groups and relationships between continuous variables. RESULTS: Absolute and lung volume normalised PVV (PVV/LV) correlated inversely with functional parameters and positively with all ILD patterns (ρ=0.75 with ground glass, ρ=0.68 with reticular). PVV/LV was the only predictor of DLco at multivariate analysis (p=0.007). Meanwhile, the reticular pattern prevailed in peripheral regions and lower lung thirds, PVV/LV prevailed in central regions and middle lung thirds. iDLco group had a significantly higher PVV/LV (2.2%) than normal (1.6%), but lower than restricted ones (3.8%). CONCLUSIONS: Chest CT in SSc detects a progressive increase in PVV/LV as DLco decreases. Redistribution of perfusion to less affected lung regions rather than angiogenesis nearby fibrotic lung may explain the results. Further studies to ascertain whether the increase in PVV/LV reflects a real increase in blood volume are needed.


Assuntos
Doenças Pulmonares Intersticiais/diagnóstico por imagem , Pulmão/irrigação sanguínea , Escleroderma Sistêmico/diagnóstico por imagem , Espirometria/estatística & dados numéricos , Tomografia Computadorizada por Raios X/estatística & dados numéricos , Adulto , Feminino , Humanos , Modelos Logísticos , Pulmão/diagnóstico por imagem , Doenças Pulmonares Intersticiais/etiologia , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Testes de Função Respiratória , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/fisiopatologia , Espirometria/métodos , Estatísticas não Paramétricas , Tomografia Computadorizada por Raios X/métodos , Capacidade Vital
18.
Aesthetic Plast Surg ; 44(5): 1820-1832, 2020 10.
Artigo em Inglês | MEDLINE | ID: mdl-32632623

RESUMO

BACKGROUND: Systemic sclerosis is a connective tissue disease. Skin involvement of the mouth and hand may compromise function and quality of life. Autologous fat grafting has been described as a specific treatment of these clinical features. We report the results of our prospective study designed to treat and prevent skin complications in systemic sclerosis. MATERIALS AND METHODS: We treated 25 patients with mouth and/or hand involvement (microstomia, xerostomia, skin sclerosis, Raynaud's phenomenon and long-lasting digital ulcers) with autologous fat grafting, according to the Coleman's technique, around the mouth and/or at the base of each finger. The surgical procedures were repeated in each patient every 6 months for a total of two or three times. Clinical data were collected before the first surgery and again 6 months after each surgical procedure. Pain, skin thickness, saliva production and disability were assessed with validated tests. RESULTS: Overall we performed 63 autologous fat grafting sessions (either on the mouth, on the hands or on both anatomical areas). Results at 6 moths after the last session included improvement of xerostomia evaluated with a sialogram, reduction of the skin tension around the mouth and, in the hands, reduction of the Raynaud phenomenon as well as skin thickness. Pain was reduced while the perception of disability improved. Digital ulcers healed completely in 8/9 patients. CONCLUSIONS: Our results confirm the efficacy and safety of autologous fat grafting for the treatment of skin complications and digital ulcers due to systemic sclerosis. In addition, the patients' subjective well-being improved. Level of evidence V This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .


Assuntos
Qualidade de Vida , Escleroderma Sistêmico , Tecido Adiposo , Humanos , Estudos Prospectivos , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/cirurgia , Transplante Autólogo , Resultado do Tratamento
19.
Clin Exp Rheumatol ; 38 Suppl 125(3): 40-47, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32301427

RESUMO

OBJECTIVES: Systemic sclerosis (SSc) is a severe multiple-organ disease characterised by unpredictable clinical course, inadequate response to treatment, and poor prognosis. National SSc registries may provide large and representative patients cohorts required for descriptive and prognostic studies. Therefore, the Italian Society for Rheumatology promoted the registry SPRING (Systemic sclerosis Progression INvestiGation). METHODS: The SPRING is a multi-centre rheumatological cohort study encompassing the wide scleroderma spectrum, namely the primary Raynaud's phenomenon (pRP), suspected secondary RP, Very Early Diagnosis of Systemic Sclerosis (VEDOSS), and definite SSc. Here we describe the demographic and clinical characteristics of a population of 2,028 Italian patients at the initial phase of enrolment, mainly focusing on the cohort of 1,538 patients with definite SSc. RESULTS: Definite SSc showed a significantly higher prevalence of digital ulcers, capillaroscopic 'late' pattern, oesophageal and cardio-pulmonary involvement compared to VEDOSS, as expected on the basis of the followed classification criteria. The in-depth analysis of definite SSc revealed that male gender, diffuse cutaneous subset, and anti-Scl70 seropositivity were significantly associated with increased prevalence of the most harmful disease manifestations. Similarly, patients with very short RP duration (≤1 year) at SSc diagnosis showed a statistically increased prevalence of unfavourable clinico-serological features. CONCLUSIONS: Nationwide registries with suitable subsetting of patients and follow-up studies since the prodromal phase of the disease may give us valuable insights into the SSc natural history and main prognostic factors.


Assuntos
Doença de Raynaud , Escleroderma Sistêmico , Estudos de Coortes , Humanos , Itália , Masculino , Angioscopia Microscópica , Sistema de Registros
20.
Int J Rheum Dis ; 23(5): 681-692, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32314542

RESUMO

OBJECTIVES: Data on macrovascular involvement in systemic sclerosis (SSc) are still debatable. The aim of this study was to estimate its prevalence and possible determinants in a large cohort. METHODS: One hundred and fifty-five outpatients with SSc were enrolled. Data about disease characteristics and cardiovascular risk factors were collected and patients underwent ecocolor Doppler ultrasonography of arteries of the neck and lower (LL) and upper (UL) limbs. RESULTS: Mean age was 57.9 ± 14.5 years and most were female (88.4%) with a limited subset (63.2%). Mean disease duration was 11.4 ± 8.1 years. Twenty-three (14.8%) had hypertension, 7 (4.8%) diabetes, 64 (41.3%) hypercholesterolemia and 63 (40.6%) were active/past smokers. Seventy-nine (49%) patients had plaques at carotids, 49 (32.9%) at LL and 7 (4.9%) at UL. In multivariate analysis, patients with carotid plaques had more often a limited pattern (P = .001), patients with distal LL plaques pulmonary arterial hypertension (P = .006) and patients with proximal LL plaques lower diffusing capacity for carbon monoxide adjusted to hemoglobin and its ratio to alveolar volume (P = .004). In patients with UL plaques traditional cardiovascular risk factors were not more common, while forced vital capacity was lower (P = .023). Finally, upper limb and proximal LL plaques were as common in early disease patients as in longstanding ones, although the former were younger. CONCLUSIONS: This study shows that macrovascular involvement is quite common in SSc and that some disease characteristics linked to microvascular involvement are associated with atherosclerotic plaques, which can be present even in early disease. Our study suggests that a complete evaluation of macrocirculation is mandatory for rheumatologists treating SSc patients.


Assuntos
Artérias/diagnóstico por imagem , Extremidade Inferior/irrigação sanguínea , Escleroderma Sistêmico/diagnóstico por imagem , Ultrassonografia Doppler em Cores , Extremidade Superior/irrigação sanguínea , Doenças Vasculares/diagnóstico por imagem , Adulto , Idoso , Artérias Carótidas/diagnóstico por imagem , Feminino , Humanos , Itália/epidemiologia , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Prevalência , Escleroderma Sistêmico/epidemiologia , Doenças Vasculares/epidemiologia
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA
...