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1.
Autoimmun Rev ; 20(3): 102755, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-33476823

RESUMO

Systemic sclerosis (SSc) is a rare chronic autoimmune disease characterized by vasculopathy, dysregulation of innate and adaptive immune responses, and progressive fibrosis. SSc remains an orphan disease, with high morbity and mortality in SSc patients. The mesenchymal stromal cells (MSC) demonstrate in vitro and in vivo pro-angiogenic, immuno-suppressive, and anti-fibrotic properties and appear as a promising stem cell therapy type, that may target the key pathological features of SSc disease. This review aims to summarize acquired knowledge in the field of :1) MSC definition and in vitro and in vivo functional properties, which vary according to the donor type (allogeneic or autologous), the tissue sources (bone marrow, adipose tissue or umbilical cord) or inflammatory micro-environment in the recipient; 2) preclinical studies in various SSc animal models , which showed reduction in skin and lung fibrosis after MSC infusion; 3) first clinical trials in human, with safety and early efficacy results reported in SSc patients or currently tested in several ongoing clinical trials.


Assuntos
Transplante de Células-Tronco Mesenquimais , Células-Tronco Mesenquimais , Escleroderma Sistêmico , Animais , Fibrose , Humanos , Escleroderma Sistêmico/patologia , Escleroderma Sistêmico/terapia , Pele/patologia
2.
J Clin Rheumatol ; 27(1): 31-33, 2021 Jan 01.
Artigo em Inglês | MEDLINE | ID: mdl-33347032

RESUMO

BACKGROUND: The coronavirus disease (COVID-19) pandemic and its subsequent effects on health care systems have significantly impacted the management of chronic rheumatic diseases, including systemic sclerosis (SSc). METHODS: In this context, a 25-item anonymized e-survey was posted on the Twitter and Facebook e-groups and pages of various scleroderma organizations and patient communities to assess the problems faced by patients with SSc during the pandemic, with a focus on effects on the disease, drug procurance, continuity of medical care, and prevalent fears among patients. RESULTS: Of the 291 participants (median age of 55 [43.5-63] years, 93.8% females), limited systemic sclerosis was the most common diagnosis (42.3%). Many patients experienced problems attributable to the COVID-19 pandemic (119, 40.9%), of which 46 (38.7%) required an increase in medicines, and 12 (10.1%) of these needed hospitalizations for disease-related complications. More than one-third (36.4%) were on glucocorticoids or had underlying cardiovascular risks (39%) that would predispose them to severe COVID-19.A significant proportion (38.1%) faced hurdles in procuring medicines or experienced disruption in physiotherapy sessions (24.7%). One-quarter (24.1%) felt it was difficult to contact their specialist, whereas another 7.2% were unable to do so. Contracting COVID-19 was the most prevalent fear (71.5%), followed by infection in the family (61.9%), and a flare of the disease (45.4%). Most respondents preferred teleconsultations (55.7%) over hospital visits in the pandemic period. CONCLUSION: The results of the patient survey suggest that the COVID-19 pandemic has affected many patients with SSc and may translate to poorer outcomes in this population in the postpandemic period.


Assuntos
/epidemiologia , Acesso aos Serviços de Saúde , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/terapia , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Pandemias , Inquéritos e Questionários
3.
JAMA Netw Open ; 3(12): e2029917, 2020 12 01.
Artigo em Inglês | MEDLINE | ID: mdl-33315114

RESUMO

Importance: Patients with autoimmune disease and lung cancer pose a multidisciplinary treatment challenge, particularly with the advent of immunotherapy. However, the association between autoimmune disease and lung cancer survival is largely unknown. Objective: To determine the association between autoimmune disease and lung cancer survival. Design, Setting, and Participants: Retrospective cohort study between 2003 and 2019 at a single academic medical center (Northwestern University). A query of the Northwestern Medicine Enterprise Data Warehouse identified 349 patients with lung cancer and several autoimmune diseases. Types of lung cancers included small cell, adenocarcinoma, squamous cell carcinoma, non-small cell not otherwise specified, and large cell carcinoma. Autoimmune diseases included rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis, mixed connective tissue disease, myositis, and Sjögren syndrome. Inclusion criteria were biopsy-confirmed lung cancer, autoimmune diagnosis confirmed by a rheumatologist, and death or an encounter listed in the electronic medical record within 2 years of study end. A control group of patients with biopsy-proven lung cancer but without autoimmune disease was identified. Data analysis was conducted from March to July 2020. Exposure: Presence of autoimmune disease. Main Outcomes and Measures: Overall survival and progression-free survival in patients with autoimmune disease. The hypothesis was that patients with autoimmune disease would have worse progression-free survival and overall survival compared with patients in the control group. Results: Of the original 349 patients, 177 met inclusion criteria. Mean (SD) age at lung cancer diagnosis was 67.0 (10.0) years and 136 (76.8%) were women. Most common autoimmune diseases were rheumatoid arthritis (97 [54.8%]), systemic sclerosis (43 [24.3%]), and systemic lupus erythematous (15 [8.5%]). Most common lung cancers were adenocarcinoma (99 [55.9%]), squamous cell carcinoma (29 [16.4%]), and small cell lung cancer (17 [9.6%]). A total of 219 patients (mean [SD] age at diagnosis, 65.9 [4.1] years; 173 [79.0%]) were identified as having lung cancer without autoimmune disease and included in the control cohort. Compared with patients in the control group, patients with autoimmune disease experienced no difference in overall survival (log-rank P = .69). A total of 126 patients (69.5%) with autoimmune disease received standard of care vs 213 patients (97.3%) in the control group (P < .001). No individual autoimmune disease was associated with worse prognosis, even among patients with underlying interstitial lung disease. Conclusions and Relevance: Compared with institutional controls, patients with autoimmune disease experienced no difference in survival despite the fact that fewer patients in this group received standard-of-care treatment. No individual autoimmune disease was associated with worse prognosis. Future multicenter prospective trials are needed to further evaluate autoimmune disease and lung cancer survival.


Assuntos
Neoplasias Pulmonares , Pulmão/patologia , Idoso , Artrite Reumatoide/epidemiologia , Artrite Reumatoide/fisiopatologia , Artrite Reumatoide/terapia , Autoimunidade , Biópsia/métodos , Biópsia/estatística & dados numéricos , Comorbidade , Registros Eletrônicos de Saúde/estatística & dados numéricos , Feminino , Humanos , Pesquisa Interdisciplinar , Neoplasias Pulmonares/imunologia , Neoplasias Pulmonares/mortalidade , Neoplasias Pulmonares/patologia , Neoplasias Pulmonares/terapia , Lúpus Eritematoso Sistêmico/epidemiologia , Lúpus Eritematoso Sistêmico/fisiopatologia , Masculino , Estadiamento de Neoplasias , Noroeste dos Estados Unidos/epidemiologia , Prognóstico , Estudos Retrospectivos , Escleroderma Sistêmico/epidemiologia , Escleroderma Sistêmico/fisiopatologia , Escleroderma Sistêmico/terapia , Padrão de Cuidado/organização & administração , Padrão de Cuidado/estatística & dados numéricos , Análise de Sobrevida
4.
Medicine (Baltimore) ; 99(31): e21431, 2020 Jul 31.
Artigo em Inglês | MEDLINE | ID: mdl-32756151

RESUMO

RATIONALE: The use of autologous hematopoietic stem cell transplantation (AHSCT) for autoimmune diseases has become the first indication for transplant in nonmalignant disease. Mucormycosis is a rare invasive infection with increasing incidence in patients treated with AHSCT. We report the first case of pulmonary mucormycosis following AHSCT for systemic sclerosis (SSc). PATIENT CONCERNS: A 24-year-old woman with rapidly progressive diffuse cutaneous SSc presented with an acute respiratory distress syndrome 6 days after AHSCT. DIAGNOSES: The results of clinical and computed tomography scan were consistent with pulmonary mucormycosis and the diagnosis was confirmed by a positive Mucorales Polymerase Chain Reaction on a peripheral blood sample. INTERVENTIONS AND OUTCOMES: Early antifungal therapy by intravenous amphotericin B provided rapid improvement within 4 days and sustained recovery after 2 years of follow-up. LESSONS: With the progressively increasing use of AHSCT and other stem cell therapy for treatment of severe SSc and other autoimmune diseases, the potential onset of rare post-transplant fungal infections, such as mucormycosis, requires careful patient monitoring and better awareness of early initiation of adequate therapy.


Assuntos
Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Mucormicose/etiologia , Esclerodermia Difusa/etiologia , Escleroderma Sistêmico/terapia , Transplante Autólogo/efeitos adversos , Doença Aguda , Administração Intravenosa , Assistência ao Convalescente , Anfotericina B/administração & dosagem , Anfotericina B/uso terapêutico , Antifúngicos/administração & dosagem , Antifúngicos/uso terapêutico , Feminino , Transplante de Células-Tronco Hematopoéticas/métodos , Humanos , Pneumopatias Fúngicas/diagnóstico por imagem , Pneumopatias Fúngicas/microbiologia , Pneumopatias Fúngicas/patologia , Mucorales/genética , Esclerodermia Difusa/patologia , Transplante Autólogo/métodos , Resultado do Tratamento , Adulto Jovem
5.
J Am Acad Orthop Surg ; 28(16): e686-e695, 2020 Aug 15.
Artigo em Inglês | MEDLINE | ID: mdl-32769717

RESUMO

Scleroderma is derived from Latin meaning hard skin. It is an uncommon, noninflammatory connective tissue disorder characterized by increased fibrosis of the skin and in certain variants, multiple other organ systems. Scleroderma involves a spectrum of pathologic changes and anatomic involvement. It can be divided into localized and systemic scleroderma. Hand involvement is common and can include calcium deposits within the soft tissues, digital ischemia, and joint contracture. Nonsurgical management consists of lifestyle modifications, biofeedback, therapy for digital stiffness/contracture, and various pharmacologic medications. When nonsurgical measures are unsuccessful, certain surgical options may be indicated, each with their inherent advantages and pitfalls. Patients with scleroderma who are undergoing surgical intervention pose unique difficulties because of their poorly vascularized tissue and deficient soft-tissue envelopes, thus increasing their susceptibility to wound healing complications and infection. Some subgroups of patients are frequently systemically ill, and specific perioperative measures should be considered to reduce their surgical risk. The spectrum of hand manifestations seen in patients with scleroderma will be reviewed with the focus on evaluation and management.


Assuntos
Mãos , Procedimentos Ortopédicos/métodos , Esclerodermia Localizada/cirurgia , Escleroderma Sistêmico/cirurgia , Calcinose , Mãos/patologia , Mãos/cirurgia , Humanos , Comunicação Interdisciplinar , Equipe de Assistência ao Paciente , Esclerodermia Localizada/diagnóstico , Esclerodermia Localizada/patologia , Esclerodermia Localizada/terapia , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/patologia , Escleroderma Sistêmico/terapia
7.
Medicine (Baltimore) ; 99(28): e21267, 2020 Jul 10.
Artigo em Inglês | MEDLINE | ID: mdl-32664182

RESUMO

BACKGROUND: Systemic sclerosis (SSc) is 1 of the most complex systemic autoimmune diseases.Accumulating evidence suggests that gut microbiota affect the development and function of the immune system and may play a role in the pathogenesis of autoimmune diseases. This new paradigm raises the possibility that many diseases result, at least partially, from microbiota-related dysfunction. This understanding invites the investigation of fecal microbiota transplantation (FMT) in the treatment of SSc. However, no study has specifically and systematically investigated the efficacy and safety of FMT in the treatment of SSc. Thus, this study will systematically and comprehensively appraise the efficacy and safety of FMT in the treatment of SSc. METHODS: We will search the following sources without restrictions for date, language, or publication status: PubMed, Web of Science,Cochrane Central Register of Controlled Trials (CENTRAL) Cochrane Library, EMBASE and China National Knowledge Infrastructure. We will apply a combination of Medical Subject Heading (MeSH) and free-text terms incorporating database-specific controlled vocabularies and text words to implement search strategies. We will also search the ongoing trials registered in the World Health Organization's International Clinical Trials Registry Platform. Besides, the previous relevant reviews conducted on FMT for SSc and reference lists of included studies will also be searched. RESULTS: This study will provide a reliable basis for the treatment of SSc with FMT. CONCLUSIONS: The findings will be an available reference to evaluate the efficacy and safety of FMT in the treatment of SSc. REGISTRATION NUMBER: INPLASY202060019.


Assuntos
Transplante de Microbiota Fecal/métodos , Escleroderma Sistêmico/terapia , Adolescente , Adulto , Feminino , Microbioma Gastrointestinal/fisiologia , Humanos , Masculino , Metanálise como Assunto , Pessoa de Meia-Idade , Projetos de Pesquisa , Escleroderma Sistêmico/microbiologia , Revisões Sistemáticas como Assunto , Resultado do Tratamento , Adulto Jovem
8.
J Psychosom Res ; 135: 110132, 2020 08.
Artigo em Inglês | MEDLINE | ID: mdl-32521358

RESUMO

OBJECTIVE: Contagious disease outbreaks and related restrictions can lead to negative psychological outcomes, particularly in vulnerable populations at risk due to pre-existing medical conditions. No randomised controlled trials (RCTs) have tested interventions to reduce mental health consequences of contagious disease outbreaks. The primary objective of the Scleroderma Patient-centered Intervention Network COVID-19 Home-isolation Activities Together (SPIN-CHAT) Trial is to evaluate the effect of a videoconference-based program on symptoms of anxiety. Secondary objectives include evaluating effects on symptoms of depression, stress, loneliness, boredom, physical activity, and social interaction. METHODS: The SPIN-CHAT Trial is a pragmatic RCT that will be conducted using the SPIN-COVID-19 Cohort, a sub-cohort of the SPIN Cohort. Eligible participants will be SPIN-COVID-19 Cohort participants without a positive COVID-19 test, with at least mild anxiety (PROMIS Anxiety 4a v1.0 T-score ≥ 55), not working from home, and not receiving current counselling or psychotherapy. We will randomly assign 162 participants to intervention groups of 7 to 10 participants each or waitlist control. We will use a partially nested RCT design to reflect dependence between individuals in training groups but not in the waitlist control. The SPIN-CHAT Program includes activity engagement, education on strategies to support mental health, and mutual participant support. Intervention participants will receive the 4-week (3 sessions per week) SPIN-CHAT Program via videoconference. The primary outcome is PROMIS Anxiety 4a score immediately post-intervention. ETHICS AND DISSEMINATION: The SPIN-CHAT Trial will test whether a brief videoconference-based intervention will improve mental health outcomes among at-risk individuals during contagious disease outbreak.


Assuntos
Ansiedade/prevenção & controle , Infecções por Coronavirus/psicologia , Promoção da Saúde/métodos , Pneumonia Viral/psicologia , Escleroderma Sistêmico/terapia , Infecções por Coronavirus/epidemiologia , Infecções por Coronavirus/prevenção & controle , Humanos , Pandemias/prevenção & controle , Assistência Centrada no Paciente , Pneumonia Viral/epidemiologia , Pneumonia Viral/prevenção & controle , Avaliação de Programas e Projetos de Saúde , Projetos de Pesquisa , Medição de Risco , Isolamento Social/psicologia , Comunicação por Videoconferência
9.
Clin Rheumatol ; 39(7): 2043-2047, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32514674

RESUMO

COVID-19 is a world health emergency which may inevitably affect the management of a complex autoimmune disease such as systemic sclerosis (SSc). Several SSc patients are frail and, in this pandemic, need a careful protection. The COVID-19 infection might complicate the clinical scenario of interstitial lung disease (ILD) in SSc because it determines a severe pneumonia characterized by radiological features similar to SSc-ILD. The striking CT similarities between the 2 diseases make it difficult to distinguish a worsening of SSc-ILD from COVID-19-ILD superinfection. Moreover, other aspects, like isolation during lock down, may cause a significant psychological stress which will pile up on the already difficult contact with the patients for a routine check-up. Moreover, the drug shortage is a real problem in these times. For these reasons, the rheumatologist in daily clinical practice should carefully differentiate the possible COVID-19 infection in order to optimize the patient management. Therefore, the challenge in everyday life will be to achieve in due time the differential diagnosis as well as the long-term psychological impact.Key Points• SSc patients should be encouraged to continue their chronic therapy; in case of immunosuppressive therapy it must be discontinued for safety in case of COVID-19 infection.• Psychological support must be guaranteed to every SSc patients.• COVID-19 pneuminia is hard to distinguish from an interstitial lung disease due to SSc lung involvment.• Data sharing is fundamental for an optimal managment of SSc patients during COVID-19 pandemia.


Assuntos
Infecções por Coronavirus , Imunossupressores/uso terapêutico , Doenças Pulmonares Intersticiais , Pandemias , Pneumonia Viral/diagnóstico , Escleroderma Sistêmico , Betacoronavirus/isolamento & purificação , Comorbidade , Infecções por Coronavirus/complicações , Infecções por Coronavirus/epidemiologia , Infecções por Coronavirus/fisiopatologia , Infecções por Coronavirus/terapia , Diagnóstico Diferencial , Humanos , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/etiologia , Administração dos Cuidados ao Paciente/métodos , Pneumonia Viral/complicações , Pneumonia Viral/epidemiologia , Pneumonia Viral/fisiopatologia , Pneumonia Viral/terapia , Angústia Psicológica , Escleroderma Sistêmico/epidemiologia , Escleroderma Sistêmico/psicologia , Escleroderma Sistêmico/terapia , Isolamento Social/psicologia , Tomografia Computadorizada por Raios X/métodos
10.
Autoimmun Rev ; 19(8): 102583, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32553611

RESUMO

Systemic sclerosis (SSc) is a rare chronic disease of unknown etiology characterized by vascular abnormalities and fibrosis involving the skin and internal organs, especially the gastrointestinal tract, lung, heart and kidneys. Although the disease was historically stratified according to the extent of skin involvement, more recent approaches place more emphasis on patterns and extent of internal organ involvement. Despite numerous clinical trials, disease-modifying treatment options are still limited resulting in persistent poor quality of life and high mortality. This review provides an overview of autoantibodies in SSc and novel approaches to stratify the disease into clinically actionable subsets.


Assuntos
Autoanticorpos , Escleroderma Sistêmico , Autoanticorpos/sangue , Autoanticorpos/imunologia , Humanos , Qualidade de Vida , Escleroderma Sistêmico/imunologia , Escleroderma Sistêmico/patologia , Escleroderma Sistêmico/terapia
11.
Ann Rheum Dis ; 79(8): 1084-1089, 2020 08.
Artigo em Inglês | MEDLINE | ID: mdl-32409324

RESUMO

BACKGROUND: Autologous haematopoietic stem cell transplantation (HSCT) improves survival in systemic sclerosis (SSc) with poor prognosis, but is hampered by treatment-related mortality (TRM). OBJECTIVE: To evaluate event-free survival (EFS), TRM, response to treatment, disease progression and patient characteristics associated with events. METHODS: All patients treated with HSCT for SSc in The Netherlands until 2017 (n=92) were included. Data on skin involvement (modified Rodnan skin score (mRSS), pulmonary function (forced vital capacity (FVC) and diffusion capacity of the lungs for carbon monoxide (DLCO)), extent of interstitial lung disease on high-resolution CT using Goh scores and left ventricular ejection fraction (LVEF) were collected at baseline, 1, 2 and 5 years. Occurrence of events, defined as death or major organ failure, were collected until 2019. As control, a comparison between patients treated with cyclophosphamide (CYC) and patients with HSCT who participated in the Autologous Stem Cell Transplantation International Scleroderma (ASTIS) trial was performed. RESULTS: Median follow-up was 4.6 years. EFS estimates at 5, 10 and 15 years were 78%, 76% and 66%, respectively. Twenty deaths occurred. Mean FVC, DLCO, mRSS and Goh scores all improved significantly. Disease progression occurred in 22 patients. Frequency of TRM decreased over time and occurred more often in males. Events were independently associated with male sex, LVEF <50% and older age. In ASTIS, patients treated with HSCT (n=23) 7 events occurred versus 13 in the CYC group (n=22). CONCLUSION: Our data confirm long-term efficacy of HSCT in improving survival, skin and lung involvement in SSc. Male sex, lower LVEF and older age at baseline were identified as risk factors for events.


Assuntos
Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Transplante de Células-Tronco Hematopoéticas/mortalidade , Escleroderma Sistêmico/terapia , Adulto , Idoso , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Países Baixos , Intervalo Livre de Progressão , Fatores de Risco , Escleroderma Sistêmico/mortalidade , Transplante Autólogo/efeitos adversos
12.
Clin Rheumatol ; 39(7): 2025-2029, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32406001

RESUMO

The coronavirus disease 2019 (COVID-19), the result of an infection with the new virus, SARS-CoV-2, is rapidly spreading worldwide. It is largely unknown whether the occurrence of COVID-19 in patients with rheumatic immune diseases has some specific manifestations, or makes them more prone to rapidly progress into severe COVID-19. In this case report, we describe the clinical features of 5 rheumatic immune disease patients with the concomitant presence of COVID-19. Amongst these patients, 4 had rheumatoid arthritis (RA) and 1 had systemic sclerosis (SSc). Two patients had a history of close contact with a COVID-19 patient. The age of the patients ranged between 51 and 79 years. Fever (80%), cough (80%), dyspnea (40%), and fatigue (20%) were the most common presenting symptoms. Laboratory investigations revealed leukopenia and lymphopenia in 2 patients. In all the patients, chest computerized tomography (CT) revealed patchy ground glass opacities in the lungs. During the hospital stay, the condition of two patients remained the same (i.e., mild COVID-19), two patients progressed to the severe COVID-19, and one patient worsened to the critically ill COVID-19. These patients were treated with antiviral agents for COVID-19, antibiotics for secondary bacterial infections, and immunomodulatory agents for rheumatic immune diseases. All the patients responded well, were cured of COVID-19, and subsequently discharged.


Assuntos
Antivirais/uso terapêutico , Artrite Reumatoide , Infecções por Coronavirus , Imunomodulação , Pandemias , Pneumonia Viral , Escleroderma Sistêmico , Idoso , Artrite Reumatoide/diagnóstico , Artrite Reumatoide/epidemiologia , Artrite Reumatoide/terapia , Betacoronavirus/isolamento & purificação , Contagem de Células Sanguíneas/métodos , Infecções por Coronavirus/diagnóstico , Infecções por Coronavirus/epidemiologia , Infecções por Coronavirus/imunologia , Infecções por Coronavirus/terapia , Estado Terminal/terapia , Progressão da Doença , Feminino , Humanos , Pulmão/diagnóstico por imagem , Masculino , Pessoa de Meia-Idade , Pneumonia Viral/diagnóstico , Pneumonia Viral/epidemiologia , Pneumonia Viral/imunologia , Pneumonia Viral/terapia , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/epidemiologia , Escleroderma Sistêmico/terapia , Avaliação de Sintomas/métodos , Tomografia Computadorizada por Raios X/métodos , Resultado do Tratamento
13.
Clin Rheumatol ; 39(7): 2063-2065, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32462423

RESUMO

COVID-19 outbreak has quickly spread worldwide, causing a high pressure on the health-care system. In Italy, from March 8, 2020, all the deferrable clinical activities have been suspended to increase the health care offer for COVID-19 patients. The hospital organization has been modified also in order to assure non-COVID-19 patients assistance. The Scleroderma Unit of ASST Pini-CTO Hospital, in Milan, in the region mostly hit by SARS-CoV-2 in Italy, follows more than 600 patients affected by systemic sclerosis (SSc). Patients with SSc need a close follow-up with a regular screening of organ involvement and frequent intravenous treatments. All SSc patients have been educated about ministerial directives to limit COVID-19 spread. The organization of our Scleroderma Unit has been quickly rethought to assure SSc patients assistance in safety for them and for health-care workers during urgent visits or infusion therapies. Using electronic way of communication with frequent virtual contact and guarantying home deliveries of some therapies, we allowed a continuity of care also outside the Hospital.


Assuntos
Infecções por Coronavirus , Procedimentos Clínicos , Unidades Hospitalares/organização & administração , Controle de Infecções/organização & administração , Pandemias , Pneumonia Viral , Escleroderma Sistêmico , Betacoronavirus/isolamento & purificação , Comorbidade , Infecções por Coronavirus/epidemiologia , Infecções por Coronavirus/prevenção & controle , Procedimentos Clínicos/organização & administração , Procedimentos Clínicos/tendências , Gerenciamento Clínico , Humanos , Itália/epidemiologia , Inovação Organizacional , Pandemias/prevenção & controle , Pneumonia Viral/epidemiologia , Pneumonia Viral/prevenção & controle , Reumatologia/métodos , Escleroderma Sistêmico/epidemiologia , Escleroderma Sistêmico/terapia
14.
Ann Rheum Dis ; 79(6): 724-726, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-32349982

RESUMO

Due to the frequent presence of interstitial lung disease and widespread use of immunosuppressive treatment, systemic sclerosis (SSc) patients may be considered at risk for a more severe disease course and higher mortality when they develop Severe Acute Respiratory Syndrome - Coronavirus - 2 (SARS-CoV-2) virus infection. Therefore, with World Scleroderma Foundation endorsement, experts from different specialties including rheumatology, virology and clinical immunology gathered virtually to answer to the main practical clinical questions regarding SARS-CoV-2 infection coming from both patients and physicians. This preliminary advice is aligned with other national and international recommendations, adapted for SSc patients.


Assuntos
Infecções por Coronavirus/tratamento farmacológico , Infecções por Coronavirus/fisiopatologia , Pneumonia Viral/tratamento farmacológico , Pneumonia Viral/fisiopatologia , Escleroderma Sistêmico/terapia , Escleroderma Sistêmico/virologia , Anticorpos Monoclonais Humanizados/uso terapêutico , Betacoronavirus , Infecções por Coronavirus/epidemiologia , Humanos , Imunossupressores/administração & dosagem , Imunossupressores/efeitos adversos , Doenças Pulmonares Intersticiais/imunologia , Doenças Pulmonares Intersticiais/virologia , Pandemias , Pneumonia Viral/epidemiologia , Pneumonia Viral/virologia , /virologia , Escleroderma Sistêmico/epidemiologia , Escleroderma Sistêmico/imunologia
15.
PLoS One ; 15(5): e0232739, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32437393

RESUMO

OBJECTIVES: Systemic sclerosis (SSc) is an auto-immune, multi organ disease marked by severe gastrointestinal (GI) involvement and gut dysbiosis. Here, we aimed to determine the safety and efficacy of fecal microbiota transplantation (FMT) using commercially-available anaerobic cultivated human intestinal microbiota (ACHIM) in SSc. METHODS: Ten patients with SSc were randomized to ACHIM (n = 5) or placebo (n = 5) in a double-blind, placebo-controlled 16-week pilot. All patients had mild to severe upper and lower GI symptoms including diarrhea, distention/bloating and/or fecal incontinence at baseline. Gastroduodenoscopy transfer of ACHIM or placebo was performed at weeks 0 and 2. Primary endpoints were safety and clinical efficacy on GI symptoms assessed at weeks 4 and 16. Secondary endpoints included changes in relative abundance of total, immunoglobulin (Ig) A- and IgM-coated fecal bacteria measured by 16s rRNA sequencing. RESULTS: ACHIM side effects were mild and transient. Two placebo controls experienced procedure-related serious adverse events; one developed laryngospasms at week 0 gastroduodenoscopy necessitating study exclusion whilst one encountered duodenal perforation during gastroduodenoscopy at the last study visit (week 16). Decreased bloating, diarrhea and/or fecal incontinence was observed in four of five patients in the FMT group (week 4 or/and 16) and in two of four in the placebo group (week 4 or 16). Relative abundance, richness and diversity of total and IgA-coated and IgM-coated bacteria fluctuated more after FMT, than after placebo. CONCLUSIONS: FMT of commercially-available ACHIM is associated with gastroduodenoscopy complications but reduces lower GI symptoms by possibly altering the gut microbiota in patients with SSc.


Assuntos
Transplante de Microbiota Fecal , Escleroderma Sistêmico/microbiologia , Escleroderma Sistêmico/terapia , Bactérias , Método Duplo-Cego , Ácidos Graxos/metabolismo , Incontinência Fecal/etiologia , Transplante de Microbiota Fecal/efeitos adversos , Fezes/química , Feminino , Humanos , Imunoglobulina A/metabolismo , Imunoglobulina M/metabolismo , Complexo Antígeno L1 Leucocitário/metabolismo , Masculino , Pessoa de Meia-Idade , Projetos Piloto , Placebos , Resultado do Tratamento
16.
Adv Exp Med Biol ; 1253: 375-403, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32445102

RESUMO

Scleroderma (systemic sclerosis; SSc) is a complex and highly heterogeneous multisystem rheumatic disease characterized by vascular abnormality, immunologic derangement, and excessive deposition of extracellular matrix (ECM) proteins. To date, the etiology of this life-threatening disorder remains not fully clear. More and more studies show epigenetic modifications play a vital role. The aberrant epigenetic status of certain molecules such as Fli-1, BMPRII, NRP1, CD70, CD40L, CD11A, FOXP3, KLF5, DKK1, SFRP1, and so on contributes to the pathogenesis of progressive vasculopathy, autoimmune dysfunction, and tissue fibrosis in SSc. Meanwhile, numerous miRNAs including miR-21, miR-29a, miR-196a, miR-202-3p, miR-150, miR-let-7a, and others are involved in the process. In addition, the abnormal epigenetic biomarker levels of CD11a, Foxp3, HDAC2, miR-30b, miR-142-3p, miR-150, miR-5196 in SSc are closely correlated with disease severity. In this chapter, we not only review new advancements on the epigenetic mechanisms involved in the pathogenesis of SSc and potential epigenetic biomarkers, but also discuss the therapeutic potential of epigenetic targeting therapeutics such as DNA methylation inhibitors, histone acetylase inhibitors, and miRNA replacement.


Assuntos
Epigênese Genética , Escleroderma Sistêmico/genética , Escleroderma Sistêmico/terapia , Biomarcadores , Humanos , MicroRNAs , Escleroderma Sistêmico/patologia , Escleroderma Sistêmico/fisiopatologia
17.
Clin Exp Dermatol ; 45(6): 679-684, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32449205

RESUMO

Paediatric morphoea is a debilitating fibrosing disorder of uncertain aetiology, affecting the skin and subcutaneous tissues. Defining optimum management strategies in paediatric morphoea remains an ongoing challenge, owing to the varied presentations and a relative paucity of paediatric-specific studies. We performed a literature search on PubMed, MEDLINE and Google Scholar, using keywords such as 'pediatric morphea', 'juvenile localised scleroderma' and 'juvenile systemic sclerosis'. Relevant studies, including randomized trials, reviews of standard current guidelines and original research articles, were selected and results analysed before summarizing them. In Part 1 of this review, we described the epidemiology, aetiopathogenesis and clinical classification; in this part, we discuss the diagnosis, markers of disease activity, management and natural history in paediatric morphoea.


Assuntos
Corticosteroides/uso terapêutico , Fototerapia , Esclerodermia Localizada/diagnóstico , Esclerodermia Localizada/terapia , Biomarcadores , Criança , Humanos , Metotrexato/uso terapêutico , Ácido Micofenólico/uso terapêutico , Esclerodermia Localizada/patologia , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/terapia
18.
Z Rheumatol ; 79(5): 429-436, 2020 Jun.
Artigo em Alemão | MEDLINE | ID: mdl-32322977

RESUMO

There have been three randomized controlled trials on autologous hematopoietic stem cell transplantation (AHSCT) in systemic sclerosis (SSc) that demonstrated significant superiority with respect to survival, improvement of cutaneous fibrosis, lung function and quality of life compared to standard treatment; however, these advantages must be carefully weighed against the transplantation-related risks. For this reason, an expert group from the stem cell therapy working party of the German Society for Rheumatology (DGRh) has now developed recommendations for the use of AHSCT in SSc. Based on the high-quality evidence, AHSCT is considered as the standard option for the treatment of selected SSc patients. Potential candidates for AHSCT are those with early, rapidly progressive, diffuse cutaneous SSc with visceral manifestations who have not yet developed severe damage to internal organs. A close cooperation between rheumatologists and transplantation centers is crucial for optimizing patient selection and treatment outcomes.


Assuntos
Transplante de Células-Tronco Hematopoéticas , Reumatologia , Escleroderma Sistêmico , Alemanha , Humanos , Qualidade de Vida , Reumatologia/normas , Escleroderma Sistêmico/terapia , Transplante Autólogo
19.
Pediatrics ; 145(5)2020 05.
Artigo em Inglês | MEDLINE | ID: mdl-32269136

RESUMO

Systemic sclerosis sine scleroderma (ssSSc) is a rare variant of systemic sclerosis, with only one pediatric case reported in the medical literature to date. Pulmonary arterial hypertension as the presenting feature of ssSSc is extremely rare, even in adults, and so far has never been reported in children. We report, for the first time, a case of pediatric ssSSc in a 3-year-old girl, who presented with interstitial lung disease and pulmonary hypertension. The patient was prescribed early aggressive pulmonary vasodilators combined with anti-inflammatory medications. The clinical response was good, and her current condition at 12 years of age is remarkable, considering the high mortality rates reported in adults. We underscore the importance of early aggressive treatment in future cases of similar presentation.


Assuntos
Hipertensão Arterial Pulmonar/complicações , Hipertensão Arterial Pulmonar/diagnóstico por imagem , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/diagnóstico por imagem , Cateterismo Cardíaco/métodos , Criança , Pré-Escolar , Feminino , Humanos , Hipertensão Arterial Pulmonar/terapia , Escleroderma Sistêmico/terapia
20.
Lancet Respir Med ; 8(3): 304-320, 2020 03.
Artigo em Inglês | MEDLINE | ID: mdl-32113575

RESUMO

Systemic sclerosis is an autoimmune connective tissue disease, which is characterised by immune dysregulation and progressive fibrosis that typically affects the skin, with variable internal organ involvement. It is a rare condition that affects mostly young and middle-aged women, resulting in disproportionate morbidity and mortality. Currently, interstitial lung disease is the most common cause of death among patients with systemic sclerosis, with a prevalence of up to 30% and a 10-year mortality of up to 40%. Interstitial lung disease is more common among African Americans and in people with the diffuse cutaneous form of systemic sclerosis or anti-topoisomerase 1 antibodies. Systemic sclerosis-associated interstitial lung disease most commonly presents with dyspnoea, cough, and a non-specific interstitial pneumonia pattern on CT scan, with a minority of cases fulfilling the criteria for usual interstitial pneumonia. The standard therapy has traditionally been combinations of immunosuppressants, particularly mycophenolate mofetil or cyclophosphamide. These immunosuppressants can be supplemented by targeted biological and antifibrotic therapies, whereas autologous haematopoietic stem-cell transplantation and lung transplantation are reserved for refractory cases.


Assuntos
Doenças Pulmonares Intersticiais/etiologia , Escleroderma Sistêmico/complicações , Progressão da Doença , Feminino , Humanos , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/patologia , Doenças Pulmonares Intersticiais/terapia , Masculino , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/patologia , Escleroderma Sistêmico/terapia
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