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1.
Hautarzt ; 72(4): 337-348, 2021 Apr.
Artigo em Alemão | MEDLINE | ID: mdl-33740079

RESUMO

Systemic lupus erythematosus (SLE) is a highly variable disease driven by the tendency to form very different types of antibody. This sometimes leads to an exaggerated respect for the complexity of the disease symptoms and to uncertainty in dealing with affected patients; however, nowadays the most important measures in the diagnosis and management of the disease can be broken down to a manageable extent. In this respect, the new recommendations of the European League Against Rheumatism (EULAR) on SLE and the recommendations on lupus nephritis jointly developed by EULAR and the European Renal Association/European Dialysis and Transplant Association (ERA/EDTA) are helpful, as are the new classification criteria from EULAR and the American College of Rheumatology (ACR). In this article the core points of these publications are summarized and contemporary SLE management is presented. As a rule, SLE can be effectively treated when managed in this way.


Assuntos
Lúpus Eritematoso Sistêmico , Doenças Reumáticas , Reumatologia , Humanos , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/terapia , Estados Unidos
2.
Medicine (Baltimore) ; 100(13): e20866, 2021 Apr 02.
Artigo em Inglês | MEDLINE | ID: mdl-33787566

RESUMO

INTRODUCTION: DOCK8 deficiency is a primary immunodeficiency characterized by recurrent infections, severe allergic disease, and autoimmunity. Here, we report a patient with DOCK8 deficiency that was initially presented as systemic lupus erythematosus (SLE) without recurrent infections and treated with hematopoietic stem cell transplantation (HSCT). PATIENT CONCERNS: A 16-month-old boy with a previous history of eczema developed high fever and hand and foot swelling. Over time, multiple purpura, oral ulcers, and oliguria developed with a persistent fever. His laboratory findings showed anemia, thrombocytopenia, and coagulopathy with a high level of C-reactive protein (CRP). No definite pathogens were identified. The complement fractions C3, C4, and CH50 were low. Autoantibodies including antinuclear antibody (ANA) and anti-ds DNA antibody were positive. He definitively satisfied the 2015 ACR/SLICC revised criteria for the diagnosis of SLE (7 points out of 16); therefore, he was treated with a steroid. Lupus nephritis was confirmed by renal biopsy later. Considering the early-onset SLE, partial exome sequencing was performed. DIAGNOSIS: One heterozygous missense variant, c.5536A>G (p.Lys1846Glu), which was inherited from his father, and heterozygous deletion of exon 1 to 8 inherited from his mother were found. Through the results of the genetic testing, the patient was confirmed to have DOCK8 deficiency. INTERVENTIONS: At the age of 28 months, he received haploidentical HSCT from his mother as a donor. OUTCOMES: Laboratory findings including complement fractions C3, C4, CH50, anti-ds DNA antibody, and the ANA became normal after HSCT. Currently, at 12 months post-HSCT, he is doing well, without any autoimmune features or infections. CONCLUSIONS: DOCK8 deficiency can be presented as autoimmune disease such as SLE. Encountering a child diagnosed with SLE at a very young age, pediatricians should consider immunodeficiency syndrome including DOCK8 deficiency.


Assuntos
Fatores de Troca do Nucleotídeo Guanina/deficiência , Transplante de Células-Tronco Hematopoéticas/métodos , Lúpus Eritematoso Sistêmico/terapia , Doenças da Imunodeficiência Primária/terapia , Humanos , Lactente , Lúpus Eritematoso Sistêmico/congênito , Lúpus Eritematoso Sistêmico/imunologia , Masculino , Doenças da Imunodeficiência Primária/genética , Doenças da Imunodeficiência Primária/imunologia
3.
Cochrane Database Syst Rev ; 3: CD007478, 2021 03 09.
Artigo em Inglês | MEDLINE | ID: mdl-33687069

RESUMO

BACKGROUND: Lupus erythematosus is an autoimmune disease with significant morbidity and mortality. Cutaneous disease in systemic lupus erythematosus (SLE) is common. Many interventions are used to treat SLE with varying efficacy, risks, and benefits. OBJECTIVES: To assess the effects of interventions for cutaneous disease in SLE. SEARCH METHODS: We searched the following databases up to June 2019: the Cochrane Skin Specialised Register, CENTRAL, MEDLINE, Embase, Wiley Interscience Online Library, and Biblioteca Virtual em Saude (Virtual Health Library). We updated our search in September 2020, but these results have not yet been fully incorporated. SELECTION CRITERIA: We included randomised controlled trials (RCTs) of interventions for cutaneous disease in SLE compared with placebo, another intervention, no treatment, or different doses of the same intervention. We did not evaluate trials of cutaneous lupus in people without a diagnosis of SLE. DATA COLLECTION AND ANALYSIS: We used standard methodological procedures expected by Cochrane. Primary outcomes were complete and partial clinical response. Secondary outcomes included reduction (or change) in number of clinical flares; and severe and minor adverse events. We used GRADE to assess the quality of evidence. MAIN RESULTS: Sixty-one RCTs, involving 11,232 participants, reported 43 different interventions. Trials predominantly included women from outpatient clinics; the mean age range of participants was 20 to 40 years. Twenty-five studies reported baseline severity, and 22 studies included participants with moderate to severe cutaneous lupus erythematosus (CLE); duration of CLE was not well reported. Studies were conducted mainly in multi-centre settings. Most often treatment duration was 12 months. Risk of bias was highest for the domain of reporting bias, followed by performance/detection bias. We identified too few studies for meta-analysis for most comparisons. We limited this abstract to main comparisons (all administered orally) and outcomes. We did not identify clinical trials of other commonly used treatments, such as topical corticosteroids, that reported complete or partial clinical response or numbers of clinical flares. Complete clinical response Studies comparing oral hydroxychloroquine against placebo did not report complete clinical response. Chloroquine may increase complete clinical response at 12 months' follow-up compared with placebo (absence of skin lesions) (risk ratio (RR) 1.57, 95% confidence interval (CI) 0.95 to 2.61; 1 study, 24 participants; low-quality evidence). There may be little to no difference between methotrexate and chloroquine in complete clinical response (skin rash resolution) at 6 months' follow-up (RR 1.13, 95% CI 0.84 to 1.50; 1 study, 25 participants; low-quality evidence). Methotrexate may be superior to placebo with regard to complete clinical response (absence of malar/discoid rash) at 6 months' follow-up (RR 3.57, 95% CI 1.63 to 7.84; 1 study, 41 participants; low-quality evidence). At 12 months' follow-up, there may be little to no difference between azathioprine and ciclosporin in complete clinical response (malar rash resolution) (RR 0.83, 95% CI 0.46 to 1.52; 1 study, 89 participants; low-quality evidence). Partial clinical response Partial clinical response was reported for only one key comparison: hydroxychloroquine may increase partial clinical response at 12 months compared to placebo, but the 95% CI indicates that hydroxychloroquine may make no difference or may decrease response (RR 7.00, 95% CI 0.41 to 120.16; 20 pregnant participants, 1 trial; low-quality evidence). Clinical flares Clinical flares were reported for only two key comparisons: hydroxychloroquine is probably superior to placebo at 6 months' follow-up for reducing clinical flares (RR 0.49, 95% CI 0.28 to 0.89; 1 study, 47 participants; moderate-quality evidence). At 12 months' follow-up, there may be no difference between methotrexate and placebo, but the 95% CI indicates there may be more or fewer flares with methotrexate (RR 0.77, 95% CI 0.32 to 1.83; 1 study, 86 participants; moderate-quality evidence). Adverse events Data for adverse events were limited and were inconsistently reported, but hydroxychloroquine, chloroquine, and methotrexate have well-documented adverse effects including gastrointestinal symptoms, liver problems, and retinopathy for hydroxychloroquine and chloroquine and teratogenicity during pregnancy for methotrexate. AUTHORS' CONCLUSIONS: Evidence supports the commonly-used treatment hydroxychloroquine, and there is also evidence supporting chloroquine and methotrexate for treating cutaneous disease in SLE. Evidence is limited due to the small number of studies reporting key outcomes. Evidence for most key outcomes was low or moderate quality, meaning findings should be interpreted with caution. Head-to-head intervention trials designed to detect differences in efficacy between treatments for specific CLE subtypes are needed. Thirteen further trials are awaiting classification and have not yet been incorporated in this review; they may alter the review conclusions.


Assuntos
Fármacos Dermatológicos/uso terapêutico , Imunossupressores/uso terapêutico , Lúpus Eritematoso Sistêmico/terapia , Dermatopatias/terapia , Idade de Início , Azatioprina/uso terapêutico , Viés , Fatores Biológicos/uso terapêutico , Cloroquina/efeitos adversos , Cloroquina/uso terapêutico , Técnicas Cosméticas , Ciclosporina/uso terapêutico , Fármacos Dermatológicos/efeitos adversos , Exantema , Feminino , Humanos , Hidroxicloroquina/efeitos adversos , Hidroxicloroquina/uso terapêutico , Lúpus Eritematoso Cutâneo/classificação , Lúpus Eritematoso Cutâneo/diagnóstico , Lúpus Eritematoso Cutâneo/terapia , Lúpus Eritematoso Sistêmico/classificação , Lúpus Eritematoso Sistêmico/complicações , Masculino , Medicina Tradicional Chinesa , Metotrexato/efeitos adversos , Metotrexato/uso terapêutico , Placebos/uso terapêutico , Qualidade de Vida , Ensaios Clínicos Controlados Aleatórios como Assunto , Dermatopatias/etiologia , Exacerbação dos Sintomas
4.
Rheumatology (Oxford) ; 60(3): 1474-1479, 2021 03 02.
Artigo em Inglês | MEDLINE | ID: mdl-33677595

RESUMO

OBJECTIVES: We aimed to estimate what proportion of people with SLE attending UK rheumatology clinics would be categorized as being at high risk from coronavirus disease 2019 (COVID-19) and therefore asked to shield, and explore what implications this has for rheumatology clinical practice. METHODS: We used data from the British Society for Rheumatology multicentre audit of SLE, which included a large, representative cross-sectional sample of patients attending UK Rheumatology clinics with SLE. We calculated who would receive shielding advice using the British Society for Rheumatology's risk stratification guidance and accompanying scoring grid, and assessed whether ethnicity and history of nephritis were over-represented in the shielding group. RESULTS: The audit included 1003 patients from 51 centres across all 4 nations of the UK. Overall 344 (34.3%) patients had a shielding score ≥3 and would have been advised to shield. People with previous or current LN were 2.6 (1.9-3.4) times more likely to be in the shielding group than people with no previous LN (P < 0.001). Ethnicity was not evenly distributed between the groups (chi-squared P < 0.001). Compared with White people, people of Black ethnicity were 1.9 (1.3-2.8) and Asian 1.9 (1.3-2.7) times more likely to be in the shielding group. Increased risk persisted after controlling for LN. CONCLUSION: Our study demonstrates the large number of people with SLE who are likely to be shielding. Implications for clinical practice include considering communication across language and cultural differences, and ways to conduct renal assessment including urinalysis, during telephone and video consultations for patients who are shielding.


Assuntos
/prevenção & controle , Lúpus Eritematoso Sistêmico/terapia , Aceitação pelo Paciente de Cuidados de Saúde/estatística & dados numéricos , Padrões de Prática Médica/estatística & dados numéricos , Quarentena/estatística & dados numéricos , Reumatologia/estatística & dados numéricos , Adulto , Estudos Transversais , Feminino , Humanos , Lúpus Eritematoso Sistêmico/virologia , Nefrite Lúpica/terapia , Nefrite Lúpica/virologia , Masculino , Auditoria Médica , Pessoa de Meia-Idade , Análise de Regressão , Telemedicina/estatística & dados numéricos , Reino Unido/epidemiologia
5.
Autoimmun Rev ; 20(4): 102775, 2021 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-33609790

RESUMO

Immune cells play essential roles in metabolic homeostasis and thus, undergo analogous changes in normal physiology (e.g., puberty and pregnancy) and in various metabolic and immune diseases. An essential component of this close relationship between the two is sex differences. Many autoimmune diseases, such as systemic lupus erythematous and multiple sclerosis, feature strikingly increased prevalence in females, whereas in contrast, infectious diseases, such as Ebola and Middle East Respiratory Syndrome, affect more men than women. Therefore, there are fundamental aspects of metabolic homeostasis and immune functions that are regulated differently in males and females. This can be observed in sex hormone-immune interaction where androgens, such as testosterone, have shown immunosuppressive effects whilst estrogen is on the opposite side of the spectrum with immunoenhancing facilitation of mechanisms. In addition, the two sexes exhibit significant differences in metabolic regulation, with estrous cycles in females known to induce variability in traits and more pronounced metabolic disease phenotype exhibited by males. It is likely that these differences underlie both the development of metabolic and autoimmune diseases and the response to current treatment options. Sexual dimorphism in immunometabolism has emerged to become an area of intense research, aiming to uncover sex-biased effector molecules in the various metabolic tissues and immune cell types, identify sex-biased cell-type-specific functions of common effector molecules, and understand whether the sex differences in metabolic and immune functions influence each other during autoimmune pathogenesis. In this review, we will summarize recent findings that address these critical questions of sexual dimorphism in immunometabolism as well as their translational implications for the clinical management of autoimmune diseases.


Assuntos
Doenças Autoimunes , Lúpus Eritematoso Sistêmico , Autoimunidade , Feminino , Humanos , Lúpus Eritematoso Sistêmico/terapia , Masculino , Medicina de Precisão , Gravidez , Caracteres Sexuais
6.
Med Clin North Am ; 105(2): 341-353, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-33589107

RESUMO

Management of women with rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), and obstetric antiphospholipid syndrome (APS) during pregnancy presents unique clinical challenges. Women with both RA and SLE can have disease flares during pregnancy, leading to pregnancy complications, such as preeclampsia, small-for-gestational-age infants, and preterm delivery. Disease should be under control prior to conception. Women with obstetric APS need to be anticoagulated during pregnancy. Many but not all antirheumatic medications can be used during pregnancy and lactation.


Assuntos
Síndrome Antifosfolipídica , Artrite Reumatoide , Lúpus Eritematoso Sistêmico , Administração dos Cuidados ao Paciente/métodos , Complicações na Gravidez , Síndrome Antifosfolipídica/diagnóstico , Síndrome Antifosfolipídica/terapia , Artrite Reumatoide/diagnóstico , Artrite Reumatoide/terapia , Feminino , Humanos , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/terapia , Gravidez , Complicações na Gravidez/diagnóstico , Complicações na Gravidez/terapia , Resultado da Gravidez , Gravidez de Alto Risco
7.
Autoimmun Rev ; 20(2): 102736, 2021 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-33333233

RESUMO

The B cell activating factor (BAFF), or B lymphocyte stimulator (BLyS), is a B cell survival factor which supports autoreactive B cells and prevents their deletion. BAFF expression is closely linked with autoimmunity and is enhanced by genetic alterations and viral infections. Furthermore, BAFF seems to be involved in adipogenesis, atherosclerosis, neuro-inflammatory processes and ischemia reperfusion (I/R) injury. BAFF is commonly overexpressed in Systemic Lupus Erythematosus (SLE) and strongly involved in the pathogenesis of the disease. The relationship between BAFF levels, disease activity and damage accrual in SLE is controversial, but growing evidence is emerging on its role in renal involvement. Belimumab, a biologic BAFF inhibitor, has been the first biologic agent licensed for SLE therapy so far. As Rituximab (RTX) has been shown to increase BAFF levels following B cell depletion, the combination therapy of RTX plus belimumab (being evaluated in two RCT) seems to be a valuable option for several clinical scenarios. In this review we will highlight the growing body of evidence of immune and non-immune related BAFF expression in experimental and clinical settings.


Assuntos
Lúpus Eritematoso Sistêmico , Viroses , Autoimunidade , Fator Ativador de Células B , Linfócitos B , Humanos , Lúpus Eritematoso Sistêmico/terapia
8.
BMJ Case Rep ; 13(12)2020 Dec 29.
Artigo em Inglês | MEDLINE | ID: mdl-33376090

RESUMO

Autoimmune hepatitis (AIH) is an autoimmune liver disease characterised by the presence of autoantibodies including antinuclear antibodies, anti-smooth muscle antibody and hypergammaglobulinaemia. Systemic lupus erythematosus (SLE) is a systemic disease that can affect multiple organs. Coexistence of AIH and SLE as an overlap syndrome occurs in about 1%-2.6% of the AIH cases. Since both conditions share common autoimmune features, their coexistence can pose a diagnostic dilemma which can result in a delay in treatment. We present here a challenging case of a middle-aged woman with AIH in remission who later developed new-onset fatigue, pleural effusion and splenomegaly.


Assuntos
Autoanticorpos/sangue , Hepatite Autoimune , Imunossupressores/administração & dosagem , Lúpus Eritematoso Sistêmico , Derrame Pleural , Esplenomegalia , Biópsia/métodos , Comorbidade , Diagnóstico Diferencial , Progressão da Doença , Feminino , Hepatite Autoimune/diagnóstico , Hepatite Autoimune/epidemiologia , Hepatite Autoimune/imunologia , Hepatite Autoimune/terapia , Humanos , Fígado/patologia , Testes de Função Hepática/métodos , Lúpus Eritematoso Sistêmico/epidemiologia , Lúpus Eritematoso Sistêmico/imunologia , Lúpus Eritematoso Sistêmico/fisiopatologia , Lúpus Eritematoso Sistêmico/terapia , Linfadenopatia/diagnóstico , Linfadenopatia/etiologia , Pessoa de Meia-Idade , Derrame Pleural/diagnóstico , Derrame Pleural/etiologia , Esplenomegalia/diagnóstico por imagem , Esplenomegalia/etiologia , Tomografia Computadorizada por Raios X/métodos , Resultado do Tratamento
9.
Med. clín (Ed. impr.) ; 155(11): 494-501, dic. 2020. tab
Artigo em Espanhol | IBECS | ID: ibc-198342

RESUMO

El lupus eritematoso sistémico (LES) es una enfermedad autoinmunitaria multisistémica compleja, con una gran heterogeneidad en su presentación clínica y una alta morbimortalidad. Aunque su pronóstico ha mejorado de forma notable a lo largo de las últimas décadas, aún siguen existiendo necesidades no cubiertas en esta enfermedad. En esta actualización realizamos una puesta al día de los principales avances en esta enfermedad en los últimos años. Concretamente revisamos los nuevos criterios de clasificación propuestos por la Liga Europea de Reumatología y el Colegio Americano de Reumatología, la búsqueda de nuevos biomarcadores, las nuevas definiciones de remisión y de baja actividad de la enfermedad, así como las estrategias actuales de abordaje y tratamiento del LES y la situación actual de las nuevas terapias


Systemic lupus erythematosus (SLE) is a complex autoimmune multisystemic disease of great clinical heterogeneity and significant potential morbidity and mortality. Although the outlook for patients with SLE has greatly improved, many unmet needs remain. In this review we aim to summarize the most relevant data on SLE that have emerged in recent years. In particular we discuss the new classification criteria from the European League Against Rheumatism and American College of Rheumatology, new biomarkers, novel definitions of remission and low lupus disease activity and what has emerged on new drugs and new therapeutic strategies


Assuntos
Humanos , Lúpus Eritematoso Sistêmico/epidemiologia , Técnica Indireta de Fluorescência para Anticorpo/métodos , Prognóstico , Lúpus Eritematoso Sistêmico/prevenção & controle , Lúpus Eritematoso Sistêmico/terapia , Biomarcadores , Anticorpos Antinucleares/análise
10.
Yonsei Med J ; 61(11): 951-957, 2020 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-33107238

RESUMO

PURPOSE: To compare the clinical characteristics and renal outcomes between patients who initially had lupus nephritis (LN) at the onset of systemic lupus erythematosus (SLE) (initial-onset LN) and those who developed LN within 5 years after SLE onset (early-onset LN). MATERIALS AND METHODS: SLE patients with biopsy-proven LN were retrospectively reviewed. The clinical parameters and renal outcomes were compared between initial-onset and early-onset LN groups. We used Cox regression analysis to estimate risk of worse renal outcomes according to the onset time of LN. RESULTS: Of all 136 LN patients, 92 (67.6%) and 44 (32.4%) patients were classified into the initial-onset and early-onset LN groups, respectively. The initial-onset LN group had higher prevalences of class IV LN (54.3% vs. 34.1%, p=0.027), impaired renal function (34.8% vs. 11.4%, p=0.004), microscopic hematuria (73.9% vs. 54.5%, p=0.024), and higher urine protein/creatinine ratio [4626.1 (2180.0-6788.3) mg/g vs. 2410.0 (1265.0-5168.5) mg/g, p=0.006] at LN diagnosis. Renal relapse (46.3% vs. 25.7%, p=0.039) and progression to chronic kidney disease (CKD) or end-stage renal disease (ESRD) were more common (24.4% vs. 8.3%, p=0.042) in the initial-onset LN group. In Cox regression analysis, the initial-onset LN group had higher risks of renal relapse [adjusted hazard ratio (HR) 3.56, 95% confidence interval (CI) 1.51-8.35, p=0.004] and progression to CKD or ESRD (adjusted HR 4.57, 95% CI 1.03-20.17, p=0.045), compared with the early-onset LN group. CONCLUSION: Patients with LN at SLE onset may have more severe renal presentations and experience worse renal outcomes than those who develop LN within 5 years.


Assuntos
Falência Renal Crônica/etiologia , Rim/fisiopatologia , Lúpus Eritematoso Sistêmico/patologia , Nefrite Lúpica/patologia , Adulto , Biópsia , Progressão da Doença , Feminino , Humanos , Lúpus Eritematoso Sistêmico/terapia , Nefrite Lúpica/complicações , Nefrite Lúpica/terapia , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Índice de Gravidade de Doença , Fatores de Tempo , Adulto Jovem
11.
Semin Arthritis Rheum ; 50(5): 1150-1157, 2020 10.
Artigo em Inglês | MEDLINE | ID: mdl-32927376

RESUMO

BACKGROUND: Systemic lupus erythematosus (SLE) is a chronic disease characterised by autoimmunity and increased susceptibility to infections. COVID-19 is a systemic viral disease currently spreading as a pandemic. Little is known about the impact of COVID-19 in patients with SLE. OBJECTIVE: to acquire information on the impact of COVID-19 in SLE. METHODS: A 26-item anonymous questionnaire investigating demographics, SLE clinical features, COVID-19 diagnoses and changes in treatments and daily habits was administered to patients with SLE from three referral centres through www.surveymonkey.com over 10 days. Data from the survey were compared to those from published estimates about the general population. RESULTS: Four-hundred-seventeen patients responded to the survey. More than 60% of subjects complained of symptoms that are also associated to COVID-19. Fourteen COVID-19 diagnoses (five confirmed by polymerase chain reaction) were reported, in contrast to a 0.73% prevalence of confirmed cases in Lombardy. One hospitalisation was reported. Fever, anosmia, dry cough, a self-reported history of neuropsychiatric SLE and a recent contact with confirmed COVID-19 cases were more strongly associated with COVID-19, as were symptoms and lower compliance to behavioural preventive measures in patients' contacts. No protective effect was seen in subjects on hydroxychloroquine. CONCLUSION: COVID-19 morbidity might only moderately be increased in most patients with SLE, although limited information can be inferred on more severe cases. Hydroxychloroquine apparently seems not to confer protection to infection per se, although other beneficial roles cannot be excluded. Containment policies and behavioural preventive measures could have a major role in limiting the impact of COVID-19 in patients with SLE.


Assuntos
Controle de Doenças Transmissíveis/métodos , Infecções por Coronavirus , Hidroxicloroquina/uso terapêutico , Lúpus Eritematoso Sistêmico , Pandemias , Pneumonia Viral , Isolamento Social/psicologia , Avaliação de Sintomas , Adulto , Antirreumáticos/uso terapêutico , Betacoronavirus , Técnicas de Laboratório Clínico/métodos , Infecções por Coronavirus/diagnóstico , Infecções por Coronavirus/epidemiologia , Infecções por Coronavirus/prevenção & controle , Feminino , Humanos , Itália/epidemiologia , Lúpus Eritematoso Sistêmico/epidemiologia , Lúpus Eritematoso Sistêmico/terapia , Masculino , Pessoa de Meia-Idade , Pandemias/prevenção & controle , Cooperação do Paciente/estatística & dados numéricos , Pneumonia Viral/epidemiologia , Pneumonia Viral/prevenção & controle , Prevalência , Pesquisa Qualitativa , Avaliação de Sintomas/métodos , Avaliação de Sintomas/estatística & dados numéricos
12.
Autoimmun Rev ; 19(9): 102612, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32668290

RESUMO

"Rhupus" or "rhupus syndrome" is a poorly described and underdiagnosed disease in which features of both rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE) appear in the same patient, most often sequentially. The SLE-related involvement is usually mild, dominated by hematological abnormalities and skin, serosal and renal involvement. The natural history of rhupus arthritis follows an RA-like pattern and can progress towards typical inflammatory erosions, deformations and disability. Despite the lack of consensus on the definition of rhupus and on its place in the spectrum of autoimmunity, a growing number of studies are pointing towards a true overlap between RA and SLE. However, the inclusion criteria employed in the literature during the last 4 decades are heterogeneous, making the already rare cohorts and case reports difficult to analyze. Because of this heterogeneity and due to the rarity of the disease, the prevalence, pathophysiology and natural history as well as the radiological and immunological profiles of rhupus are poorly described. Moreover, since there is no validated therapeutic strategy, treatment is based on clinicians' experience and on the results of a few studies. We herein present a systematic literature review to analyze the clinical and laboratory data of all reported rhupus patients and to provide up-to-date information about recent advances in the understanding of the pathophysiological mechanisms, diagnostic tools and treatment options.


Assuntos
Artrite Reumatoide/complicações , Lúpus Eritematoso Sistêmico/complicações , Artrite Reumatoide/diagnóstico , Artrite Reumatoide/fisiopatologia , Artrite Reumatoide/terapia , Humanos , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/fisiopatologia , Lúpus Eritematoso Sistêmico/terapia , Prevalência , Síndrome
13.
Ann Intern Med ; 172(11): ITC81-ITC96, 2020 06 02.
Artigo em Inglês | MEDLINE | ID: mdl-32479157

RESUMO

Systemic lupus erythematosus (lupus) is characterized by aberrant activity of the immune system, leading to variable clinical symptoms. Lupus is more prevalent in African American women and women in other ethnic minority groups. Diagnosing, treating, and identifying novel therapies for lupus is challenging because of its genetic and phenotypic heterogeneity. Lupus nephritis is the most common target-organ manifestation and requires individualized care to minimize toxicity. A multidisciplinary approach to caring for pregnant patients with lupus is essential to optimize outcomes.


Assuntos
Lúpus Eritematoso Sistêmico/diagnóstico , Diagnóstico Diferencial , Humanos , Lúpus Eritematoso Sistêmico/patologia , Lúpus Eritematoso Sistêmico/terapia
14.
Immunol Med ; 43(4): 171-178, 2020 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-32374660

RESUMO

We report a case of incipient systemic lupus erythematosus (SLE) that rapidly progressed to complete atrioventricular block (cAVB). A 20-year-old man was admitted with facial erythema, painless oral aphtha, polyarthritis, and myalgia of each extremity. On admission, he developed first-degree atrioventricular block, pericarditis, pleuritis, renal failure, hemophagocytic lymphohistiocytosis, neurophychiatric SLE (left cerebellar infarction), and Staphylococcus aureus bacteremia. He was subsequently diagnosed with SLE based on several positive findings on immunological tests (including positive for antinuclear antibody). Despite immediate glucocorticoid pulse therapy and plasma exchange (PE) along with antibiotic, he developed cAVB that required temporary pacing on day 2. Because it was thought that hypercytokinemia exacerbated pericarditis, which progressed to myocarditis and cAVB, we decided to PE and cytokine-adsorbing therapy with AN69ST-continuous hemodiafiltration (CHDF). Other than renal failure, his organ dysfunctions improved with the multidisciplinary therapy. CAVB improved and temporary pacing was no longer required on day 11. Even a first-degree atrioventricular block can rapidly progress to cAVB; therefore, strict attention to electrocardiogram is necessary in severe SLE cases. When presenting with organ dysfunctions caused by hypercytokinemia such as severe SLE cases or SLE with severe infection cases, use of the combination of PE and AN69ST-CHDF might be beneficial.


Assuntos
Bloqueio Atrioventricular/etiologia , Bloqueio Atrioventricular/terapia , Hemodiafiltração/métodos , Lúpus Eritematoso Sistêmico/complicações , Linfo-Histiocitose Hemofagocítica/etiologia , Linfo-Histiocitose Hemofagocítica/terapia , Troca Plasmática/métodos , Adulto , Antibacterianos/uso terapêutico , Citocinas/isolamento & purificação , Glucocorticoides/administração & dosagem , Humanos , Lúpus Eritematoso Sistêmico/terapia , Masculino , Desintoxicação por Sorção/métodos , Resultado do Tratamento , Adulto Jovem
15.
Z Rheumatol ; 79(5): 437-445, 2020 Jun.
Artigo em Alemão | MEDLINE | ID: mdl-32322976

RESUMO

Mesenchymal stromal or stem cells (MSC) possess strong immunomodulatory properties. Due to their impressive potential to differentiate into various cell types they are capable of inducing mechanisms of tissue repair. Experimental data have demonstrated impaired MSC function in several rheumatic diseases in vitro; however, the relevance of these phenomena for the pathogenesis of rheumatic disorders has not been convincingly demonstrated. Nevertheless, allogeneic MSC transplantation (MSCT), and possibly autologous MSCT as well, could prove to be an interesting instrument for the treatment of autoimmune rheumatic diseases. The first clinical trials have demonstrated positive effects in systemic lupus erythematosus, systemic sclerosis and Sjogren's syndrome; however, questions regarding the long-term benefits and safety as well as the best source, the optimal cultivation technique and the most effective way of application of MSC are still unanswered.


Assuntos
Doenças Autoimunes , Transplante de Células-Tronco Mesenquimais , Doenças Reumáticas , Doenças Autoimunes/terapia , Humanos , Lúpus Eritematoso Sistêmico/terapia , Células-Tronco Mesenquimais , Doenças Reumáticas/terapia
17.
Am J Case Rep ; 21: e921299, 2020 Apr 14.
Artigo em Inglês | MEDLINE | ID: mdl-32284523

RESUMO

BACKGROUND Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by autoantibody production leading to inflammation in multiple organs; it commonly affects young women in their child-bearing years. Clinical manifestations are diverse and range from mild arthritis to diffuse alveolar hemorrhage (DAH). DAH is a rare and devastating complication of SLE that carries a mortality rate of up to 50%, despite aggressive therapy. CASE REPORT A 21-year-old primigravida at 16 weeks gestation presents with a productive cough, rash, sore throat, and high-grade fever. Chest x-ray suggested multifocal pneumonia. Patient deteriorated despite antibiotics and intravenous (IV) fluids. She developed worsening anemia, leukopenia, and thrombocytopenia. Autoimmune workup was positive for Coombs, antinuclear antibody, anti-smith antibody, and hypocomplementemia. Skin biopsy was consistent with SLE. SLE vasculitis was suspected. She required mechanical intubation for rapid respiratory deterioration, with CT thorax suggesting ARDS. Bronchoscopy was done and confirmed DAH. Her course was further complicated with retinopathy and acute pancreatitis associated with SLE. She was treated with IV steroids, IV cyclophosphamide, and plasmapheresis, with significant clinical improvement and successful extubation. She delivered a healthy baby at 32 weeks gestation. CONCLUSIONS Early recognition and initiation of treatment is critical to survival in DAH and requires a high index of clinical suspicion. Treatment includes high-dose steroids, cyclophosphamide, and plasma exchange. Pregnancy increases the risk of adverse outcome in SLE. Seven cases of DAH in pregnant patients with SLE have been reported. Here, we report a catastrophic presentation of DAH, acute pancreatitis, and retinopathy in a pregnant patient with newly diagnosed SLE.


Assuntos
Lúpus Eritematoso Sistêmico/complicações , Insuficiência de Múltiplos Órgãos/etiologia , Complicações na Gravidez/etiologia , Vasculite/etiologia , Feminino , Hemorragia/diagnóstico por imagem , Hemorragia/etiologia , Hemorragia/terapia , Humanos , Pneumopatias/diagnóstico por imagem , Pneumopatias/etiologia , Pneumopatias/terapia , Lúpus Eritematoso Sistêmico/terapia , Insuficiência de Múltiplos Órgãos/terapia , Gravidez , Complicações na Gravidez/diagnóstico por imagem , Complicações na Gravidez/terapia , Alvéolos Pulmonares/diagnóstico por imagem , Alvéolos Pulmonares/patologia , Vasculite/diagnóstico por imagem , Vasculite/terapia , Adulto Jovem
18.
Qual Life Res ; 29(9): 2415-2423, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32270369

RESUMO

PURPOSE: This study aimed to assess mental health status (depression, anxiety, and stress) and explore factors associated with the disease-specific quality of life among Systemic Lupus Erythematosus (SLE) patients in Thailand. METHODS: This cross-sectional study used an online convenience sampling of 650 SLE patients who were registered members of the Thailand SLE Club. The study survey comprised of demographic information, health history, Depression, Anxiety, Stress Scale (DASS), and Lupus Quality of Life Scale (LupusQoL). RESULTS: The survey response rate was 61.2%. Out of 344 respondents, most were female (96.9%). The scores were suggestive of the presence of mild depression and stress, but moderate anxiety. The higher depression, anxiety, and stress levels were associated with lower education and income (r = - .14 to - .29, p < .01) and higher number of SLE symptoms (r = .17 to .33, p < .05). Better quality of life was significantly related to lower number of symptoms, lower levels of stress/anxiety/depression, higher education, and better income. Also, the longer the patients were kept out of the hospital (last hospitalization), the better their quality of life. By using hierarchical multiple regression, four predictors of the quality of life were identified; the number of symptoms, stress, anxiety, and depression. These predictors combined explained 51% of the variance, F(5,108) = 24.34, p < .001, adjusted R2 = .51. CONCLUSIONS: To improve the quality of life, SLE patients should focus on self-management of their symptoms. Health care providers should include SLE self-care health education in the plan of care. They also should use multidisciplinary approach in order to provide holistic treatment, including psychological care.


Assuntos
Ansiedade/psicologia , Depressão/psicologia , Lúpus Eritematoso Sistêmico/psicologia , Qualidade de Vida/psicologia , Estresse Psicológico/psicologia , Adulto , Transtornos de Ansiedade/psicologia , Estudos de Casos e Controles , Estudos Transversais , Feminino , Humanos , Internet , Lúpus Eritematoso Sistêmico/terapia , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Inquéritos e Questionários , Tailândia
19.
Z Rheumatol ; 79(4): 351-358, 2020 May.
Artigo em Alemão | MEDLINE | ID: mdl-32232548

RESUMO

BACKGROUND: For the first time, the European League Against Rheumatism (EULAR) recommendations for the management of systemic lupus erythematosus (SLE) include a treat to target (T2T) strategy, naming remission as the main target. The aim is to improve the long-term survival, prevent damage and optimize the health-related quality of life. Compared to rheumatoid arthritis (RA) for which the T2T approach is already widely used, establishing T2T in SLE remains challenging due to its heterogeneity and limited treatment options. OBJECTIVE: The aim of this article is to outline the current status of the T2T concept for SLE and to identify challenges and particularities. A special focus is placed on the use of activity scores and the importance of steroids in targeted treatment of SLE. RESULTS AND CONCLUSION: The use of T2T for SLE remains a challenge. With the definitions of the lupus low disease activity states (LLDAS) and the definitions of remission in SLE (DORIS) a big step forward has been taken on the road towards the ideal treatment target; however, many aspects of the "treat" remain unclear. As an example, due to a lack of data there are no explicit strategies for steroid reduction although it is often mandatory to achieve the targets. The T2T also includes much more than only immunosuppressive treatment and antimalarial agents, as disease damage which has already occurred and damage due to drugs, concomitant phenomena (such as depression) and comorbidities as well as measures for secondary prophylaxis must also be included.


Assuntos
Lúpus Eritematoso Sistêmico , Indução de Remissão , Artrite Reumatoide , Progressão da Doença , Humanos , Lúpus Eritematoso Sistêmico/terapia , Qualidade de Vida , Indução de Remissão/métodos , Índice de Gravidade de Doença , Resultado do Tratamento
20.
Artigo em Inglês | MEDLINE | ID: mdl-32231129

RESUMO

INTRODUCTION: Over 400,000 slaves were taken from Africa and brought to Charleston, South Carolina, as part of the transatlantic slave trade during the 18th and 19th centuries. Due to these negative historical events, the healthcare of African Americans in Charleston may be compromised in regard to chronic illnesses and other conditions affecting minorities, such as lupus. MATERIALS AND METHODS: The current study used an ethnographic approach to obtain the perspectives of lupus patients with the goal of identifying gaps within current research. In addition to patient perspectives, the geographical location of Charleston, South Carolina was considered through inquiries around culture, community, advocacy, and client/patient interaction to establish a narrative for the themes that emerged. RESULTS: The eleven major themes identified were connectedness, knowledge, experience with lupus, compliance, clinical trial participation, career and planning for the future, visits, access to resources, lifestyle, transition from child to adult care, and an overarching theme of self-management. CONCLUSION: Understanding healthcare perceptions and decision-making among culturally diverse populations, particularly those who have been defined by centuries of substandard care, marginalization, exploitation, and distrust, is critical to the development of culturally tailored interventions designed to improve patient outcomes and reduce health disparities.


Assuntos
Tomada de Decisões , Assistência à Saúde/história , Disparidades em Assistência à Saúde/história , Lúpus Eritematoso Sistêmico/terapia , Adulto , Afro-Americanos , Criança , Feminino , Pesquisa sobre Serviços de Saúde , História do Século XXI , Humanos , Lúpus Eritematoso Sistêmico/etnologia , Participação do Paciente , South Carolina , Transição para Assistência do Adulto
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