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1.
Beijing Da Xue Xue Bao Yi Xue Ban ; 52(5): 892-896, 2020 Oct 18.
Artigo em Chinês | MEDLINE | ID: mdl-33047725

RESUMO

OBJECTIVE: To measure the level of serum Semaphorin 3A (Sema3A) and to analyze the relationship between serum Sema3A and systemic lupus erythematosus (SLE) with thrombocytopenia. METHODS: The concentration of serum Sema3A was detected by enzyme-linked immuno sorbent assay (ELISA) in 170 SLE patients, 50 Sjögren's syndrome (SS) patients, 19 hypersplenism (HS) patients and 150 healthy controls (HC). Based on the presence of thrombocytopenia and whether the thrombocytopenia was in remission, the SLE patients were divided into three groups: SLE with thrombocytopenia (41 cases), SLE with thrombocytopenia remission (28 cases), and SLE without thrombocytopenia (101 cases). According to whether there was thrombocytopenia, the SS patients were divided into SS with thrombocytopenia (18 cases) and SS without thrombocytopenia (32 cases). The 28 SLE patients who underwent bone marrow aspiration biopsy were divided into two groups from the aspect of whether the bone marrow hyperplasia was normal (19 cases) or low (9 cases), as well as from the aspect of whether the maturity disturbance of megakaryocyte was positive (8 cases) or negative (20 cases). The serum Sema3A levels in SLE, SS, HS with HC were compared, meanwhile, the correlation between serum Sema3A level and platelet (PLT) in the patients with different diseases analyzed. RESULTS: (1) Serum Sema3A levels in SLE were significantly lower than in HC [(3.84±2.76) µg/L vs. (6.96±2.62) µg/L, P < 0.001], serum Sema3A levels in SS were also obviously lower than in HC [(4.35±3.57) µg/L vs. (6.96±2.62) µg/L, P < 0.001], and in HS it was lower than HC at a certain extant [(5.67±2.26) µg/L vs. (6.96±2.62) µg/L, P=0.041]. (2) Serum Sema3A levels in SLE were slightly lower than in SS, but there was no significant difference [(3.84±2.76) µg/L vs. (4.35±3.57) µg/L, P=0.282]. However, when compared with HS, serum Sema3A levels in SLE were significantly lower [(3.84±2.76) µg/L vs. (5.67±2.26) µg/L, P=0.006]. (3) Serum Sema3A concentration in SLE with thrombocytopenia was significantly lower than in SLE with thrombocytopenia remission [(1.28±1.06) µg/L vs. (3.83±2.65) µg/L, P < 0.001], and in SLE patients without thrombocytopenia [(1.28±1.06) µg/L vs. (4.87±2.60) µg/L, P < 0.001]. There was no significant difference between SLE with thrombocytopenia remission and SLE without thrombocytopenia [(3.83±2.65) µg/L vs. (4.87±2.600 µg/L, P=0.123]. Serum Sema3A concentration in SLE with thrombocytopenia was slightly lower than in SS with thrombocytopenia, but there was no significant difference [(1.28±1.06) µg/L vs. (1.68±1.11) µg/L, P=0.189]. (4) Strong positive correlations were found between serum Sema3A and PLT in SLE (r=0.600, P < 0.001). Positive correlations were also found between serum Sema3A and PLT in SS (r=0.573, P < 0.001). However, there was no such correlation showed in HS patients (P=0.393). (5) There was no significant difference of serum Sema3A concentration in SLE whether the bone marrow hyperplasia was normal or low. And the same situation appeared in the patients whether the maturity disturbance of megakaryocyte was positive or negative (P>0.05). CONCLUSION: Serum Sema3A was significantly reduced in SLE patients, and it was highly correlated with the blood damage. Similar conclusions could be drawn in patients with SS. The serum level of Sema3A was generally decreasing in desmosis which merged thrombocytopenia, and was obviously positive correlated with platelet counts.


Assuntos
Lúpus Eritematoso Sistêmico , Síndrome de Sjogren , Trombocitopenia , Ensaio de Imunoadsorção Enzimática , Humanos , Lúpus Eritematoso Sistêmico/complicações , Semaforina-3A , Trombocitopenia/etiologia
2.
Medicine (Baltimore) ; 99(35): e21888, 2020 Aug 28.
Artigo em Inglês | MEDLINE | ID: mdl-32871918

RESUMO

Systemic lupus erythematosus (SLE) is a prototypic autoimmune disease with considerable genetic predisposition. Nucleotide-binding oligomerization domain-like receptor family pyrin domain-containing protein 3 (NLRP3) is crucial for the innate immunity and implicated in SLE pathogenesis. Accordingly, we conducted a case-control study to find the association of NLRP3 variations with SLE susceptibility and disease activity.Three single nucleotide polymorphisms of NLRP3 (rs3806268, rs4612666, and rs10754558) were genotyped in 400 SLE patients and 400 healthy controls; the patients were further divided into mild-to-moderate or high disease activity subgroup. Serum cytokines, complements, and autoantibodies were also detected.We found that rs4612666 TT genotype conferred a higher risk of severe disease activity with adjusted odds ratio = 2.08, P = .02 and adjusted odds ratio  = 2.34, P = .01 in the codominant and recessive model, respectively. Nevertheless, there was no association between the 3 single nucleotide polymorphisms of NLRP3 gene and SLE susceptibility. In addition, C4 decreased significantly in rs3806268 GG (P < .001) and rs4612666 TT genotype carriers (P = .03). A higher trend of interleukin-1ß and interleukin-γ release were identified in rs3806268 AA and rs10754558 CC genotype carriers, respectively.NLRP3 polymorphisms are associated with SLE disease activity and hypocomplementemia. Interleukin-1ß and interleukin-γ levels in SLE patients are correlated with NLRP3 variants as well.


Assuntos
Predisposição Genética para Doença , Lúpus Eritematoso Sistêmico/genética , Proteína 3 que Contém Domínio de Pirina da Família NLR/genética , Polimorfismo de Nucleotídeo Único , Adolescente , Adulto , Idoso , Grupo com Ancestrais do Continente Asiático/genética , Biomarcadores/sangue , Estudos de Casos e Controles , Complemento C4/metabolismo , Feminino , Estudos de Associação Genética , Humanos , Subunidade gama Comum de Receptores de Interleucina/sangue , Interleucina-1beta/sangue , Lúpus Eritematoso Sistêmico/sangue , Lúpus Eritematoso Sistêmico/imunologia , Masculino , Pessoa de Meia-Idade , Fator de Necrose Tumoral alfa/sangue , Adulto Jovem
3.
RMD Open ; 6(2)2020 09.
Artigo em Inglês | MEDLINE | ID: mdl-32878994

RESUMO

OBJECTIVES: Patients with inflammatory rheumatic diseases (IRD) infected with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) may be at risk to develop a severe course of COVID-19. The influence of immunomodulating drugs on the course of COVID-19 is unknown. To gather knowledge about SARS-CoV-2 infections in patients with IRD, we established a registry shortly after the beginning of the pandemic in Germany. METHODS: Using an online questionnaire (www.COVID19-rheuma.de), a nationwide database was launched on 30 March 2020, with appropriate ethical and data protection approval to collect data of patients with IRD infected with SARS-CoV-2. In this registry, key clinical and epidemiological parameters-for example, diagnosis of IRD, antirheumatic therapies, comorbidities and course of the infection-are documented. RESULTS: Until 25 April 2020, data from 104 patients with IRD infected with SARS-CoV-2 were reported (40 males; 63 females; 1 diverse). Most of them (45%) were diagnosed with rheumatoid arthritis, 59% had one or more comorbidities and 42% were treated with biological disease-modifying antirheumatic drugs. Hospitalisation was reported in 32% of the patients. Two-thirds of the patients already recovered. Unfortunately, 6 patients had a fatal course. CONCLUSIONS: In a short time, a national registry for SARS-CoV2-infected patients with IRD was established. Within 4 weeks, 104 cases were documented. The registry enables to generate data rapidly in this emerging situation and to gain a better understanding of the course of SARS-CoV2-infection in patients with IRD, with a distinct focus on their immunomodulatory therapies. This knowledge is valuable for timely information of physicians and patients with IRD, and shall also serve for the development of guidance for the management of patients with IRD during this pandemic.


Assuntos
Antirreumáticos/uso terapêutico , Produtos Biológicos/uso terapêutico , Infecções por Coronavirus/fisiopatologia , Pneumonia Viral/fisiopatologia , Sistema de Registros , Doenças Reumáticas/tratamento farmacológico , Adulto , Idoso , Idoso de 80 Anos ou mais , Artrite Psoriásica/complicações , Artrite Psoriásica/tratamento farmacológico , Artrite Reumatoide/complicações , Artrite Reumatoide/tratamento farmacológico , Betacoronavirus , Infecções por Coronavirus/complicações , Infecções por Coronavirus/mortalidade , Feminino , Alemanha , Granulomatose com Poliangiite/complicações , Granulomatose com Poliangiite/tratamento farmacológico , Hospitalização , Humanos , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Pandemias , Pneumonia Viral/complicações , Pneumonia Viral/mortalidade , Polimialgia Reumática/complicações , Polimialgia Reumática/tratamento farmacológico , Prognóstico , Doenças Reumáticas/complicações , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/tratamento farmacológico , Índice de Gravidade de Doença , Espondilite Anquilosante/complicações , Espondilite Anquilosante/tratamento farmacológico , Adulto Jovem
4.
N Engl J Med ; 383(12): 1149-1155, 2020 09 17.
Artigo em Inglês | MEDLINE | ID: mdl-32937047

RESUMO

Daratumumab, a human monoclonal antibody that targets CD38, depletes plasma cells and is approved for the treatment of multiple myeloma. Long-lived plasma cells are implicated in the pathogenesis of systemic lupus erythematosus because they secrete autoantibodies, but they are unresponsive to standard immunosuppression. We describe the use of daratumumab that induced substantial clinical responses in two patients with life-threatening lupus, with the clinical responses sustained by maintenance therapy with belimumab, an antibody to B-cell activating factor. Significant depletion of long-lived plasma cells, reduction of interferon type I activity, and down-regulation of T-cell transcripts associated with chronic inflammation were documented. (Supported by the Deutsche Forschungsgemeinschaft and others.).


Assuntos
ADP-Ribosil Ciclase 1/antagonistas & inibidores , Anticorpos Monoclonais/uso terapêutico , Imunossupressores/uso terapêutico , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Glicoproteínas de Membrana/antagonistas & inibidores , Plasmócitos/efeitos dos fármacos , ADP-Ribosil Ciclase 1/metabolismo , Adulto , Anticorpos Monoclonais Humanizados/uso terapêutico , Creatinina/sangue , Creatinina/urina , Regulação para Baixo , Feminino , Humanos , Interferon Tipo I/antagonistas & inibidores , Quimioterapia de Manutenção , Glicoproteínas de Membrana/metabolismo , Pessoa de Meia-Idade , Proteinúria , Linfócitos T/efeitos dos fármacos , Linfócitos T/metabolismo
5.
Zhong Nan Da Xue Xue Bao Yi Xue Ban ; 45(4): 469-475, 2020 Apr 28.
Artigo em Inglês, Chinês | MEDLINE | ID: mdl-32879074

RESUMO

Wernekink commissure syndrome (WCS) is very rare. Four patients with WCS, admitted to our hospital from April to May 2018, were chosen for this study, and their clinical manifestations, imaging features, and etiology were retrospectively analyzed based on the literatures. All patients with WCS manifested as bilateral cerebellar ataxia such as symmetrical limb and trunk ataxia, but the degree of ataxia was asymmetrical distribution based on the anatomy. Dysarthria was the main and constant clinical manifestation of the syndrome. Ophthalmoplegia had great variability, and WCS with oculomotor nerve palsy may be considered as atypical WCS. The incidence of olivary degeneration and palatine myoclonus is relatively low, which may be related to the difference in the reported time intervals of cases. Changes in consciousness and emotion may be the characteristic of neglected WCS, which should be paid more attention. Cerebral infarction is the main etiology of WCS. We reported that cerebral infarction and WCS was the first symptom in a patient with systemic lupus erythematosus (SLE). We should pay more attention to special etiology in diagnosis and treatment of WCS.


Assuntos
Ataxia Cerebelar , Lúpus Eritematoso Sistêmico , Infarto Cerebral , Humanos , Estudos Retrospectivos , Síndrome
6.
Curr Opin Rheumatol ; 32(6): 572-582, 2020 11.
Artigo em Inglês | MEDLINE | ID: mdl-32890029

RESUMO

PURPOSE OF REVIEW: The purpose of this review is highlighting the most recent evidence on the clinical efficacy and toxicity of antimalarials in systemic lupus erythematosus (SLE). RECENT FINDINGS: New data confirm the effects of antimalarials in preventing SLE activity, damage and infections and in decreasing mortality. An important reduction in use of health resources is related to continued antimalarial use. Hydroxychloroquine (HCQ) may prevent preeclampsia in pregnant women with SLE. HCQ ocular toxicity is infrequent and could be associated with blood levels. Gastrointestinal and skin toxicity are underrecognized and could influence adherence. Prolongation of QT interval is extremely unusual with HCQ. Doses of HCQ of 200 mg/day seem to offer a good efficacy/toxicity balance. HCQ protection against herpes zoster and Pneumocystis jirovecii infection has been shown. On the contrary, HCQ prescription by doctors and adherence by patients are both under recommended standards. The recent coronavirus disease 2019 pandemic has resulted in a significant shortage of HCQ in many countries with possible consequences in the correct treatment of lupus patients. SUMMARY: Recent evidence reinforces the central role of HCQ in SLE therapy. The reduction in activity, damage accrual and mortality is consistent across studies, countries and ethnical groups. On the contrary, and despite the well established beneficial effects of prolonged regular HCQ therapy, many SLE patients do never take this drug or it is eventually stopped in the setting of severe flares, pregnancy or presumed toxicity. Every effort must be made to assure the correct prescription of HCQ and not to withdraw the drug unless unequivocal signs of toxicity are present.


Assuntos
Antimaláricos/uso terapêutico , Betacoronavirus , Infecções por Coronavirus/epidemiologia , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Pandemias , Pneumonia Viral/epidemiologia , Comorbidade , Infecções por Coronavirus/tratamento farmacológico , Feminino , Humanos , Lúpus Eritematoso Sistêmico/epidemiologia , Pneumonia Viral/tratamento farmacológico , Resultado do Tratamento
7.
Clin Exp Rheumatol ; 38(5): 822-833, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32940208

RESUMO

OBJECTIVES: This research aimed to investigate the level of peripheral blood circular RNAs (circRNAs) from systemic lupus erythematosus (SLE) patients with renal involvement (SLE+RI) to identify novel biomarkers for SLE+RI screening. METHODS: circRNAs expression in peripheral blood from 3 SLE+RI patients, 3 SLE patients without renal involvement (SLE-RI) and 3 healthy controls (HC) were performed by microarray. All upregulated expressed circRNAs coming from "circBase" between the three groups were determined by real time-quantitative polymerase chain reaction (qRT-PCR) in SLE+RI, SLE-RI, HC, neprhritis without SLE (NWS) and rheumatoid arthritis (RA) patients. The diagnostic value of these circRNAs for SLE+RI was evaluated by receiver operating characteristic (ROC) curve. A 15-day follow-up was evaluated in 7 newly diagnosed SLE+RI patients to investigate the level change of these circRNAs after treatment. RESULTS: We confirmed that the level of hsa_circ_0082688, hsa_circ_0082689 and hsa_circ_0008675 were significantly elevated in SLE+RI patients with respect to the SLE-RI, RA, NWS patients and the HC. The level of hsa_circ_0082688, hsa_circ_0082689 and hsa_circ_0008675 were associated with C4, anti-dsDNA, anti-nucleosome. The level of hsa_circ_0008675 was associated with C3, and the level of hsa_circ_0082688 and hsa_circ_0008675 were associated with treatment. ROC curve analysis suggested that hsa_circ_0082688-hsa_circ_0008675 had significant value in the diagnosis of new-onset SLE+RI patients than the controls (new-onset SLE-RI patients, RA patients, NWS patients and HC) with an area under the curve of 0.925, sensitivity of 79.17% and specificity of 96.64%. CONCLUSIONS: This study suggests that peripheral blood hsa_circ_0082688-hsa_circ_0008675 level in SLE+RI patients is upregulated and may also serve as a potential biomarker for SLE+RI patient diagnosis and treatment.


Assuntos
Artrite Reumatoide , Lúpus Eritematoso Sistêmico , Biomarcadores , Humanos , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/genética , RNA/genética , RNA Circular , Curva ROC
8.
Am J Case Rep ; 21: e927304, 2020 Sep 26.
Artigo em Inglês | MEDLINE | ID: mdl-32978364

RESUMO

BACKGROUND This case series describes 5 patients with SARS-CoV-2 infection and COVID-19 in Ecuador who had been treated with hydroxychloroquine for systemic lupus erythematosus (SLE) prior to their COVID-19 illness. CASE REPORT Case #1 reports a 29-year-old woman who had been treated with 200 mg of hydroxychloroquine per day for 1 year and presented with flu-like symptoms, chest pain, fever, odynophagia, asthenia, dry cough, and chills. Case #2 was a 34-year-old woman whose treatment for SLE included 200 mg of hydroxychloroquine per day since 2017. She arrived at the clinic with a dry cough, asthenia, and myalgias. Case #3 was a 24-year-old woman who had been using 200 mg of hydroxychloroquine per day since 2010. She presented with asthenia, myalgias, headaches, hypogeusia, and anosmia. Case #4 was a 39-year-old woman taking 200 mg of hydroxychloroquine every day for SLE who presented with dyspnea, chest pain, odynophagia, hypogeusia, anosmia, diarrhea, and fever. Case #5 was a 46-year-old woman who had been taking 200 mg of hydroxychloroquine since 2019. She came to our hospital complaining of chest pain, fever, and dyspnea. In all 5 patients, SARS-CoV-2 infection was confirmed with a nasopharyngeal SARS-CoV-2 reverse transcription-polymerase chain reaction (RT-PCR) test using the Cepheid/GeneXpert system. CONCLUSIONS All 5 of our patients with SLE who were taking hydroxychloroquine presented with SARS-CoV-2 infection and symptoms of COVID-19. This case series provides support for a lack of prevention of COVID-19 by hydroxychloroquine.


Assuntos
Infecções por Coronavirus/prevenção & controle , Hidroxicloroquina/administração & dosagem , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Pandemias/prevenção & controle , Pneumonia Viral/prevenção & controle , Adulto , Técnicas de Laboratório Clínico , Infecções por Coronavirus/diagnóstico , Infecções por Coronavirus/epidemiologia , Dispneia/diagnóstico , Dispneia/etiologia , Equador , Serviço Hospitalar de Emergência , Feminino , Febre/diagnóstico , Febre/etiologia , Humanos , Pessoa de Meia-Idade , Pandemias/estatística & dados numéricos , Pneumonia Viral/diagnóstico , Pneumonia Viral/epidemiologia , RNA Viral/análise , Reação em Cadeia da Polimerase em Tempo Real/métodos , Medição de Risco , Amostragem , Falha de Tratamento , Adulto Jovem
9.
Medicine (Baltimore) ; 99(33): e21736, 2020 Aug 14.
Artigo em Inglês | MEDLINE | ID: mdl-32872060

RESUMO

RATIONALE: Pilot studies have reported that patients with systemic lupus erythematosus (SLE) appear more likely to develop into neoplasia, especially lymphatic hyperplasia diseases. To our knowledge, this is the first case report of the concomitant onset of SLE and primary breast diffuse large B-cell lymphoma (PB-DLBCL). PATIENT CONCERNS: We reported an unusual case of the occurrence of primary breast diffuse large B-cell lymphoma in a 25-year-old female patient who had been diagnosed with SLE and treated with immunosuppressive drugs for about 4 years. She presented a 7-week history of a painless mass above the left breast and no history suggestive of any nipple discharge, fever, and weight loss. DIAGNOSIS: Ultrasonography of the breast showed that there was 1 mass in the left breast. After breast mass surgical resection, histopathological examinations were performed and revealed that it was primary breast diffuse large B-cell lymphoma. INTERVENTIONS: Treatment strategy with vincristine and dexamethasone was used to improve symptoms. However, the patient's renal function deteriorated and the blood potassium rose continuously and she and their family members refused the follow-up treatments. OUTCOMES: The patient died 8 months after she was discharged from the hospital. LESSONS: PB-DLBCL is a rare occurrence in SLE patients. Therefore, a careful examination is very important in SLE cohort, as activity of the disease and malignancy may mimic each other. Meanwhile, when symptoms cannot be explained or insensitive to treatment, the occurrence of malignant tumors must be highly considered.


Assuntos
Neoplasias da Mama/complicações , Mama/patologia , Falência Renal Crônica/etiologia , Lúpus Eritematoso Sistêmico/complicações , Linfoma Difuso de Grandes Células B/complicações , Adulto , Neoplasias da Mama/diagnóstico por imagem , Neoplasias da Mama/patologia , Neoplasias da Mama/terapia , Evolução Fatal , Feminino , Humanos , Linfoma Difuso de Grandes Células B/diagnóstico por imagem , Linfoma Difuso de Grandes Células B/patologia , Linfoma Difuso de Grandes Células B/terapia , Radiografia , Ultrassonografia
10.
Orv Hetil ; 161(31): 1293-1301, 2020 08.
Artigo em Húngaro | MEDLINE | ID: mdl-32750018

RESUMO

INTRODUCTION: Lupus nephritis is the most severe complication of systemic lupus erythematosus (SLE), its development and the effectiveness of immunosuppressive therapy substantially influence patients' quality of life and survival. AIM: In this retrospective observational investigation, the long term-outcome of patients with lupus nephritis, followed at the St. Margit Hospital Immunonephrological Outpatient Clinic, was evaluated. RESULTS: Between 1997 December 1 and 2019 April 30, 73 patients (age 33.7 ± 15 years, 82% female, 18% male) were under care with median observation of 119 [between 3-264] months. At diagnosis, eGFR showed 68 [7-120] ml/min, proteinuria was 2800 [23-16812] mg/day; 10 patients needed dialysis treatment acutely. Renal biopsy, performed in 68 patients, proved proliferative lupus nephritis in 55 and pure membranous lupus nephritis in 6 patients. Administering combined immunosuppressive therapy, complete remission was achieved in 50 and partial remission in 21 cases; one or repeated relapses developed in 28 subjects. Two patients, by the time they got under our care, had already required chronic dialysis, and in the long term, three more patients progressed to end-stage renal disease requiring renal replacement therapy. Renal function stabilized in all other participants, clinical activity of SLE, SLEDAI score, complement levels and immunserology results improved significantly. CONCLUSIONS: Lupus nephritis can be effectively treated by combined induction and prolonged maintenance immunosuppression, but to prevent progression of the disease, long-term care is necessary by co-operation of nephrologist and immunologist. To provide adequate prevention and therapy of the SLE's multiorgan involvement and also the potential complications of immunosuppression, multidisciplinary team is needed with all specialists who may facilitate these patients' complex care. For the long-term management of patients with lupus nephritis, the nephrologists have to be responsible, and the multidisciplinary teams also have to be under their direction. Orv Hetil. 2020; 161(31): 1293-1301.


Assuntos
Imunossupressores/uso terapêutico , Lúpus Eritematoso Sistêmico/complicações , Nefrite Lúpica/tratamento farmacológico , Adulto , Idoso , Feminino , Humanos , Imunossupressores/administração & dosagem , Assistência de Longa Duração , Nefrite Lúpica/imunologia , Nefrite Lúpica/psicologia , Masculino , Pessoa de Meia-Idade , Qualidade de Vida , Estudos Retrospectivos , Resultado do Tratamento
11.
Medicine (Baltimore) ; 99(31): e21467, 2020 Jul 31.
Artigo em Inglês | MEDLINE | ID: mdl-32756168

RESUMO

BACKGROUND: This study aimed to evaluate the scleral thickness and corneal parameters of patients with systemic lupus erythematosus (SLE). METHODS: Forty-seven eyes of 47 SLE patients and 44 eyes of healthy controls were included in this cross-sectional study. Anterior segment optical coherence tomography (AS-OCT) was used to measure the corneal and scleral thickness. Scleral thickness (ST) was measured based upon the segmentation at 1000 to 5000 µm from the scleral spur. Pentacam HR was used to measure corneal parameters. RESULTS: There was no statistically significant difference between SLE group and control group according to age and sex (P > .05). The ST measurements at all distances from scleral spur were found to be thicker in patients with SLE (P < .05). Central corneal thickness (CCT), cornea volume (CV), corneal densitometry (CD), and peripheral corneal thickness (PCT) measurements were similar between the groups (P > .05). CONCLUSION: ST was thicker in SLE patients compared with healthy controls. AS-OCT seems helpful in selecting optimal sites for pharmaceutical or surgical intervention in SLE patients, since it shows thickness variations in anterior sclera.


Assuntos
Córnea/diagnóstico por imagem , Lúpus Eritematoso Sistêmico/complicações , Esclera/diagnóstico por imagem , Tomografia de Coerência Óptica/métodos , Adulto , Estudos de Casos e Controles , Córnea/patologia , Paquimetria Corneana/métodos , Estudos Transversais , Densitometria , Feminino , Voluntários Saudáveis , Humanos , Lúpus Eritematoso Sistêmico/patologia , Masculino , Pessoa de Meia-Idade , Esclera/patologia , Turquia/epidemiologia
13.
Artigo em Russo | MEDLINE | ID: mdl-32790983

RESUMO

The comorbidity of neuromyelitis optica spectrum disorder (NMOSD) and systemic lupus erythematosus (SLE) is a poorly studied problem. The issues of the pathogenetic relationship between these diseases, timely diagnosis of their co-existence in one patient, disease course and therapeutic approaches are the most relevant. The authors summarize current views on the state of the problem and analyze three clinical cases of NMOSD and SLE comorbidity including the diagnostic issues and therapeutic approaches.


Assuntos
Lúpus Eritematoso Sistêmico/epidemiologia , Neuromielite Óptica , Aquaporina 4 , Autoanticorpos , Comorbidade , Humanos
14.
Rheumatol Int ; 40(10): 1593-1598, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32794113

RESUMO

OBJECTIVE: To describe clinical characteristics of patients with rheumatic and musculoskeletal diseases (RMDs) and immunosuppressive therapies with Coronavirus disease 2019 (COVID-19) at an academic rheumatology center in Madrid and to identify baseline variables associated with a severe infection requiring hospitalization. METHODS: We identified SARS-CoV-2 positive cases by polymerase chain reaction performed at our center within an updated RMDs database in our clinic. Additional RMDs patients were identified when they contacted the clinic because of a positive infection. Data extraction included diagnosis, demographics, immunosuppressive treatment, comorbidities, and laboratory tests. Comparisons between patients with or without hospitalization were performed. Multivariate logistic regression was used to analyze associations between baseline variables and need for hospitalization. RESULTS: A total of 62 patients with COVID-19 and underlying RMDs were identified by April 24, 2020. Median age was 60.9 years, and 42% men. Forty-two patients required hospitalization; these were more frequently men, older and with comorbidities. There were no statistically significant between-group differences for rheumatologic diagnosis and for baseline use of immunosuppressive therapy except for glucocorticoids that were more frequent in hospitalized patients. Total deaths were 10 (16%) patients. In multivariate analysis, male sex (odds ratio [OR], 8.63; p = 0.018), previous lung disease (OR, 27.47; p = 0.042), and glucocorticoids use (> 5 mg/day) (OR, 9.95; p = 0.019) were significantly associated to hospitalization. CONCLUSION: Neither specific RMD diagnoses or exposures to DMARDs were associated with increased odds of hospitalization. Being male, previous lung disease and exposure to glucocorticoids were associated with higher odds of hospitalization in RMDs patients.


Assuntos
Artrite Psoriásica/tratamento farmacológico , Artrite Reumatoide/tratamento farmacológico , Infecções por Coronavirus/fisiopatologia , Glucocorticoides/uso terapêutico , Hospitalização/estatística & dados numéricos , Imunossupressores/uso terapêutico , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Pneumonia Viral/fisiopatologia , Idoso , Antibacterianos/uso terapêutico , Anticorpos Monoclonais Humanizados/uso terapêutico , Antirreumáticos/uso terapêutico , Antivirais/uso terapêutico , Artrite Psoriásica/complicações , Artrite Psoriásica/epidemiologia , Artrite Reumatoide/complicações , Artrite Reumatoide/epidemiologia , Doenças Autoimunes/complicações , Doenças Autoimunes/tratamento farmacológico , Doenças Autoimunes/epidemiologia , Azitromicina/uso terapêutico , Betacoronavirus , Comorbidade , Infecções por Coronavirus/complicações , Infecções por Coronavirus/tratamento farmacológico , Infecções por Coronavirus/mortalidade , Infecções por Coronavirus/terapia , Combinação de Medicamentos , Feminino , Humanos , Hidroxicloroquina/uso terapêutico , Modelos Logísticos , Lopinavir/uso terapêutico , Pneumopatias/epidemiologia , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/epidemiologia , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Pandemias , Pneumonia Viral/complicações , Pneumonia Viral/mortalidade , Pneumonia Viral/terapia , Estudos Retrospectivos , Ritonavir/uso terapêutico , Índice de Gravidade de Doença , Fatores Sexuais , Espanha/epidemiologia
15.
Medicine (Baltimore) ; 99(34): e21909, 2020 Aug 21.
Artigo em Inglês | MEDLINE | ID: mdl-32846857

RESUMO

BACKGROUND: Systemic Lupus Erythematosus (SLE) is a diffuse connetive tissue disease, which is difficult to be conquered. However, the traditional Chinese medicine is significant in the treatment. And the Chinese medicine tripterygium and its plant extraction can help us to overcome this disease, to some extent. METHODS: The deadline should be from inception to February 2020 by computer from the databases: the Cochrane Library, Pubmed, Embase, Web of Science in English and the Chinese National Knowledge Infrastructure, Wanfang Database, Chinese Biomedical Literature Database and Chinese Science, Chinese Traditional Medicine Database, Chinese Science and Technology Periodical Database in Chinese. Included criteria are randomized controlled trials. The primary outcomes are the clinical symptoms, systemic lupus erythematosus disease activity index and quality of life questionnaire (the top 10 frequency). We will use RevMan 5.0 statistical software for data synthesis, sensitivity analysis, meta regression, subgroup analysis, and risk of bias assessment. The publish bias will be assessed by a funnel plot and the funnel plot symmetries will be evaluated by Begg and Egger tests. We will use the Grading of Recommendations Assessment, Development and Evaluation system to assess the quality of evidence. RESULTS: This article will give a protocol for meta analysis which can make sure the efficacy and side effect of the tripterygium and its plant extraction for SLE. CONCLUSION: The efficacy and side effect of the tripterygium and its plant extraction for SLE will be evaluated. ETHICS AND DISSEMINATION: Without personal information involved, ethical approval and informed consent form is no need. The review will be submitted to a peer-reviewed journal prospectively to spread our findings. PROSPERO REGISTRATION NUMBER: PROSPERO CRD42020176444.


Assuntos
Medicamentos de Ervas Chinesas/uso terapêutico , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Extratos Vegetais/uso terapêutico , Tripterygium/química , Humanos , Lúpus Eritematoso Sistêmico/psicologia , Medicina Tradicional Chinesa/métodos , Qualidade de Vida , Ensaios Clínicos Controlados Aleatórios como Assunto , Resultado do Tratamento , Tripterygium/efeitos adversos
17.
Zh Nevrol Psikhiatr Im S S Korsakova ; 120(7. Vyp. 2): 97-106, 2020.
Artigo em Russo | MEDLINE | ID: mdl-32844638

RESUMO

Neuromyelitis optica spectrum disorders (NMOSD) - autoimmune condition characterized by an inflammatory lesions mainly of the spinal cord with the development of longitudinally extensive transverse myelitis (LETM) and/or involvement of the optic nerve with the development of usually bilateral optical neuritis (ON). In recent years, there has been increased awareness that NMOSD can be combined with other autoimmune diseases, including myasthenia gravis (MG), systemic lupus erythematosus (SLE) et al. The simultaneous presence of several autoimmune diseases in one patient can adversely affect the course of each of the diseases, causing the so-called mutual burden or «overlap syndrome¼. In this article, we describe our own clinical observation of a 51-year-old woman of European origin who developed acute relapsing TM seropositive for AQP4-IgG, by 23 years after the diagnosis of generalized MG seropositive for antibodies to acetylcholine receptors (AChR-Ab) and the occurrence of SLE, criterially confirmed, several months after the initial TM attack. During the fourth TM attack, partial positive dynamics was achieved only against the background of the combined use of intravenous methylprednisolone (pulse therapy), high-volume plasma exchange, rituximab and cyclophosphamide. The NMOSD is a rare disease leading to severe disability. In patients with MG, when symptoms of damage to the central nervous system appear, an analysis should be performed for AQP4-IgG and possibly for antibodies to myelin glycoprotein of oligodendrocytes (MOG-Ab), as well as markers characteristic of systemic connective tissue diseases (SCTD). In patients with STDD, when symptoms of involvement nervous systemappear, testing for AQP4-IgG (and, if necessary, for MOG-Ab) should be performed to exclude NMOSD, as well as AChR-Ab (and, if necessary, antibodies against muscle specific kinase (MuSK-Ab)) to exclude MG.


Assuntos
Lúpus Eritematoso Sistêmico , Miastenia Gravis , Mielite Transversa , Neuromielite Óptica , Aquaporina 4 , Autoanticorpos , Feminino , Humanos , Pessoa de Meia-Idade , Recidiva Local de Neoplasia
18.
J Assoc Physicians India ; 68(5): 18-21, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32610860

RESUMO

Objectives: Infections are a major cause of morbidity and mortality in patients of Systemic Lupus erythematosus (SLE). We therefore aimed to determine the spectrum of infections in patients of SLE, find a correlation between various disease parameters and the severity and outcome of infections and to compare the outcome between different modalities of immunosuppressive therapy. Methods: A cross-sectional study was carried out by including all the diagnosed patients of Systemic lupus erythematosus (based on SLICC criteria[1]) aged 12 years and above who developed infections during the study period of 18 months. Immunocompromised patients because of coexisting diseases like diabetes, retroviral disease and cancer patients on immunosuppression were excluded. 139 cases of infections were identified in 104 patients of SLE during the study period. Results: 92 patients had one episode of infection, 19 patients had two episodes and 3 patients contracted infections thrice during the study period. The mean age of the sample population was 29.45 ± 7.9 years. 21-30 years age group constituted 51.75% (59/114) of the patients. 61/139 infections (44%) were bacterial, 22/139 (16%) fungal, 20/139 (14%) viral and 4/139 (3%) parasitic. Tuberculosis was the most common infection (40/139, 28.78%). Lower respiratory tract was the most common site of infections found in the study (37/139, 26.62%). 75/139 (54%) were major infections. 50% tuberculous infections were extrapulmonary. The mean duration of SLE until the time of infection was 35.84 ± 53.80 months. SLE Disease Activity Index (SLEDAI) was ≥ 5 in 92.09% of cases. End organ damage was found in 82.7% (115 cases) and amongst them, renal lupus was found in 110/115 cases. No association was found between end organ damage and severity or outcome of infections. In 89 cases patients were on prednisolone alone, in 29 cases patients were additionally on Cyclophosphamide or had received it in the past, and in 15 cases Mycophenolate mofetil (MMF) with prednisolone while in 6 cases all the three drugs had been given at some point of time. Tuberculosis was the most common infection amongst all the groups. The mean daily dose of prednisolone was 19.62 ± 16.04 mg/day. The mean cumulative steroid dose in patients of our study was 9165.76 ± 7833.72 mg. Central nervous system infections occurred more in patients who had received Cyclophosphamide (p = 0.01). Five deaths occurred due to life threatening infections and all of them were either on high dose prednisolone or on cytotoxic drugs (Cyclophosphamide/Mycophenolate mofetil [MMF]). There was no association between treatment modality and severity and outcome of infection. Conclusion: SLE patients are predisposed to various minor as well as lifethreatening infections. It is essential to prevent infections by screening, reducing exposure to sources or contacts of infection and minimizing the exposure to immunosuppressive agents while controlling disease activity.


Assuntos
Lúpus Eritematoso Sistêmico , Adulto , Criança , Estudos Transversais , Ciclofosfamida , Humanos , Imunossupressores , Ácido Micofenólico , Índice de Gravidade de Doença , Adulto Jovem
19.
BMC Infect Dis ; 20(1): 470, 2020 Jul 02.
Artigo em Inglês | MEDLINE | ID: mdl-32615937

RESUMO

BACKGROUND: Strongyloidiasis is a gastrointestinal parasitic infection caused by percutaneous infection with Strongyloides stercoralis. Digestive symptoms such as diarrhea and abdominal pain are the main manifestation, but serious infections such as septicemia, purulent meningitis, and bacterial pneumonia may occur in individuals harboring human T-lymphotropic virus type 1 (HTLV-1) or who are immunocompromised. Although coinfection with Strongyloides stercoralis and HTLV-1 can lead to chronic strongyloidiasis and a disseminated form of the disease, there is a high rate of response to the anthelmintic ivermectin. CASE PRESENTATION: We report a case of strongyloidiasis infection syndrome that was difficult to differentiate from immune reconstitution inflammatory syndrome (IRIS) for various reasons. The patient had been treated with the corticosteroids tacrolimus (Tac) and mycophenolate mofetil (MMF) for systemic lupus erythematosus (SLE) with lupus nephritis and pancytopenia. When the steroid was reduced, she developed cytomegalovirus (CMV) enteritis, and her respiratory status rapidly deteriorated immediately after the withdrawal of Tac and MMF. It was difficult to distinguish immune reconstitution inflammatory syndrome from strongyloidiasis infection syndrome because stool cultures were negative and eosinophils were not increased. Bronchoscopy revealed viable Strongyloides, leading to a diagnosis of strongyloidiasis infection syndrome, but the patient died despite treatment. CONCLUSIONS: Both corticosteroid therapy and HTLV-1 infection can be associated with a decrease of eosinophils, despite the presence of parasitic infection. In conclusion, even if multiple culture tests are negative, the risk of parasitic infection should be assessed in patients receiving immunosuppressants and steroids even in non-endemic areas.


Assuntos
Infecções por Citomegalovirus/complicações , Citomegalovirus/imunologia , Infecções por HTLV-I/complicações , Vírus Linfotrópico T Tipo 1 Humano/isolamento & purificação , Imunossupressão/efeitos adversos , Imunossupressores/uso terapêutico , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Strongyloides stercoralis/isolamento & purificação , Estrongiloidíase/complicações , Idoso , Animais , Anti-Helmínticos/uso terapêutico , Antivirais/uso terapêutico , Coinfecção/tratamento farmacológico , Citomegalovirus/isolamento & purificação , Infecções por Citomegalovirus/diagnóstico , Infecções por Citomegalovirus/tratamento farmacológico , Infecções por Citomegalovirus/virologia , Evolução Fatal , Feminino , Ganciclovir/uso terapêutico , Infecções por HTLV-I/diagnóstico , Infecções por HTLV-I/tratamento farmacológico , Infecções por HTLV-I/virologia , Humanos , Hospedeiro Imunocomprometido , Imunossupressores/efeitos adversos , Ivermectina/uso terapêutico , Estrongiloidíase/diagnóstico , Estrongiloidíase/tratamento farmacológico , Estrongiloidíase/parasitologia , Síndrome
20.
Nat Commun ; 11(1): 3294, 2020 07 03.
Artigo em Inglês | MEDLINE | ID: mdl-32620744

RESUMO

Systemic lupus erythematosus (SLE) is mediated by autoreactive antibodies that damage multiple tissues. Genome-wide association studies (GWAS) link >60 loci with SLE risk, but the causal variants and effector genes are largely unknown. We generated high-resolution spatial maps of SLE variant accessibility and gene connectivity in human follicular helper T cells (TFH), a cell type required for anti-nuclear antibodies characteristic of SLE. Of the ~400 potential regulatory variants identified, 90% exhibit spatial proximity to genes distant in the 1D genome sequence, including variants that loop to regulate the canonical TFH genes BCL6 and CXCR5 as confirmed by genome editing. SLE 'variant-to-gene' maps also implicate genes with no known role in TFH/SLE disease biology, including the kinases HIPK1 and MINK1. Targeting these kinases in TFH inhibits production of IL-21, a cytokine crucial for class-switched B cell antibodies. These studies offer mechanistic insight into the SLE-associated regulatory architecture of the human genome.


Assuntos
Predisposição Genética para Doença/genética , Estudo de Associação Genômica Ampla/métodos , Lúpus Eritematoso Sistêmico/genética , Polimorfismo de Nucleotídeo Único , Regiões Promotoras Genéticas/genética , Linfócitos T Auxiliares-Indutores/metabolismo , Autoanticorpos/imunologia , Autoanticorpos/metabolismo , Células Cultivadas , Mapeamento Cromossômico/métodos , Perfilação da Expressão Gênica/métodos , Humanos , Células Jurkat , Lúpus Eritematoso Sistêmico/imunologia , Proteínas Serina-Treonina Quinases/genética , Proteínas Proto-Oncogênicas c-bcl-6/genética , Interferência de RNA , Receptores CXCR5/genética , Linfócitos T Auxiliares-Indutores/imunologia
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