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1.
Braz J Med Biol Res ; 54(10): e11355, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34287582

RESUMO

The etiology of subacute combined degeneration (SCD) of the spinal cord is closely associated with vitamin B12 (VitB12) deficiency. The clinical manifestations of SCD are complex and vary substantially. Due to some SCD patients with atypical manifestations and concomitant autoimmune disorders, the probability of misdiagnosis and missed diagnosis is still relatively high in the early stage. We report the cases of two patients who were missed or misdiagnosed at another hospital because of the normal initial VitB12 level and partial overlap of clinical manifestations, finally diagnosed as SCD with atypical manifestations and concomitant autoimmune disorders, pharyngeal-cervical-brachial Guillain-Barre syndrome in Case 1 and SCD with autoimmune thyroiditis in Case 2. After undergoing corresponding treatment, death was reported in Case 1 and improvement in Case 2. Analysis of the clinical manifestations and investigation of the underlying pathogenesis in such patients could help improve the rate of early diagnosis and allow timely treatment of SCD, thereby preventing disease progression and poor clinical outcomes.


Assuntos
Doenças Autoimunes , Degeneração Combinada Subaguda , Doenças Autoimunes/complicações , Doenças Autoimunes/diagnóstico , Humanos , Imageamento por Ressonância Magnética , Medula Espinal , Degeneração Combinada Subaguda/complicações , Degeneração Combinada Subaguda/diagnóstico , Degeneração Combinada Subaguda/patologia , Vitamina B 12
2.
Mol Immunol ; 137: 105-113, 2021 09.
Artigo em Inglês | MEDLINE | ID: mdl-34242919

RESUMO

Underlying mechanisms of multi-organ manifestations and exacerbated inflammation in COVID-19 are yet to be delineated. The hypothesis of SARS-CoV-2 triggering autoimmunity is gaining attention and, in the present study, we have identified 28 human proteins harbouring regions homologous to SARS-CoV-2 peptides that could possibly be acting as autoantigens in COVID-19 patients displaying autoimmune conditions. Interestingly, these conserved regions are amongst the experimentally validated B cell epitopes of SARS-CoV-2 proteins. The reported human proteins have demonstrated presence of autoantibodies against them in typical autoimmune conditions which may explain the frequent occurrence of autoimmune conditions following SARS-CoV-2 infection. Moreover, the proposed autoantigens' widespread tissue distribution is suggestive of their involvement in multi-organ manifestations via molecular mimicry. We opine that our report may aid in directing subsequent necessary antigen-specific studies, results of which would be of long-term relevance in management of extrapulmonary symptoms of COVID-19.


Assuntos
Autoantígenos/imunologia , Doenças Autoimunes/complicações , COVID-19/etiologia , Epitopos de Linfócito B/imunologia , SARS-CoV-2/imunologia , Autoanticorpos/imunologia , Doenças Autoimunes/imunologia , Doenças Autoimunes/virologia , Autoimunidade/imunologia , COVID-19/imunologia , COVID-19/patologia , Humanos , Mimetismo Molecular/imunologia
3.
Arch Soc Esp Oftalmol (Engl Ed) ; 96(7): 347-352, 2021 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-34217471

RESUMO

INTRODUCTION: The objective of these study is to know the characteristics of COVID-19 in patients with uveitis associated with Systemic Autoimmune Disease (SAD) through telematic survey. MATERIAL AND METHODS: Internal Medicine Society and Group of Systemic Autoimmune disease conducted a telematic survey of patients with SAD to learn about the characteristics of COVID-19 in this population. RESULTS: A total of 2,789 patients answered the survey, of which 28 had a diagnosis of uveitis associated with SAE. The majority (82%) were female and caucasian (82%), with a mean age of 48 years. The most frequent SAEs were Behçet's disease followed by sarcoidosis and systemic lupus erythematosus. 46% of the patients were receiving corticosteroid treatment at a mean prednisone dose of 11 mg/day. Regarding infection, 14 (50%) patients reported symptoms compatible with SARS-CoV-2 infection. RT-PCR was performed on the nasopharyngeal smear in two patients and in one of them (4%) it was positive. CONCLUSIONS: Both asymptomatic and symptomatic COVID-19 patients with ASD-associated UNI had received similar immunosuppressive treatment.


Assuntos
Doenças Autoimunes/complicações , COVID-19/complicações , Uveíte/etiologia , Adulto , Idoso , Estudos Transversais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Autorrelato
5.
Zhejiang Da Xue Xue Bao Yi Xue Ban ; 50(2): 222-228, 2021 04 25.
Artigo em Inglês | MEDLINE | ID: mdl-34137220

RESUMO

Oral lichen planus (OLP) is a chronic inflammatory oral mucosal disease with unclear etiology. Autoimmune thyroid diseases (AITD) is a type of autoimmune disease characterized by increased thyroid-specific antibodies. In recent years, more and more studies have found that the incidence of AITD is increased in OLP patients. The occurrence and development of OLP and AITD may be related to the expression of thyroid autoantigen in oral keratinocytes, the imbalance of thyroid hormone (Th)1/Th2 and Th17/Treg cell subsets, the abnormal quantity and function of follicular helper T cells and chemokines and the specific killing ability of CD8 T cells to target cells. In this article, the possible immune mechanisms involved in the coexistence of OLP and AITD are reviewed to provide insights for the diagnosis, treatment and prevention of these two diseases from the perspective of immunology.


Assuntos
Doenças Autoimunes , Doença de Hashimoto , Líquen Plano Bucal , Doenças Autoimunes/complicações , Humanos , Líquen Plano Bucal/complicações , Mucosa Bucal , Células Th17
6.
BMJ Case Rep ; 14(5)2021 May 11.
Artigo em Inglês | MEDLINE | ID: mdl-33975851

RESUMO

A 62-year-old woman was referred to our department for further investigation of anaemia. Blood test showed macrocytic anaemia. Oesophagogastroduodenoscopy (OGD) revealed proximal-predominant gastric atrophy and flat elevated lesion in the gastric body. Several days after OGD, she complained of gait disturbance and was diagnosed with subacute combined degeneration of the spinal cord. Furthermore, laboratory tests showed positive for both anti-parietal cell and anti-intrinsic factor antibodies, as well as increased serum gastrin level and decreased pepsinogen I level, which confirmed the diagnosis of autoimmune gastritis (AIG). Anaemia and neurological symptoms were improved after vitamin B12 supplementation. Subsequently, the patient underwent gastric endoscopic submucosal dissection; histopathological examination revealed gastric adenoma. AIG can cause gastric neoplasms and vitamin B12 deficiency, with the latter resulting in pernicious anaemia and neurological disorders. These diseases are treatable but potentially life-threatening. This case highlights the importance of early diagnosis of AIG and proper management of its comorbidities.


Assuntos
Adenoma , Doenças Autoimunes , Gastrite , Neoplasias Gástricas , Degeneração Combinada Subaguda , Deficiência de Vitamina B 12 , Adenoma/patologia , Doenças Autoimunes/complicações , Doenças Autoimunes/diagnóstico , Doenças Autoimunes/patologia , Feminino , Gastrite/complicações , Gastrite/diagnóstico , Gastrite/patologia , Humanos , Pessoa de Meia-Idade , Medula Espinal/patologia , Neoplasias Gástricas/complicações , Neoplasias Gástricas/diagnóstico , Neoplasias Gástricas/cirurgia , Deficiência de Vitamina B 12/complicações , Deficiência de Vitamina B 12/diagnóstico , Deficiência de Vitamina B 12/tratamento farmacológico
7.
Obstet Gynecol ; 137(6): e177-e197, 2021 Jun 01.
Artigo em Inglês | MEDLINE | ID: mdl-34011892

RESUMO

ABSTRACT: The purpose of this Committee Opinion is to offer guidance about indications for and timing and frequency of antenatal fetal surveillance in the outpatient setting. Antenatal fetal surveillance is performed to reduce the risk of stillbirth. However, because the pathway that results in increased risk of stillbirth for a given condition may not be known and antenatal fetal surveillance has not been shown to improve perinatal outcomes for all conditions associated with stillbirth, it is challenging to create a prescriptive list of all indications for which antenatal fetal surveillance should be considered. This Committee Opinion provides guidance on and suggests surveillance for conditions for which stillbirth is reported to occur more frequently than 0.8 per 1,000 (the false-negative rate of a biophysical profile) and which are associated with a relative risk or odds ratio for stillbirth of more than 2.0 compared with pregnancies without the condition. Table 1 presents suggestions for the timing and frequency of testing for specific conditions. As with all testing and interventions, shared decision making between the pregnant individual and the clinician is critically important when considering or offering antenatal fetal surveillance for individuals with pregnancies at high risk for stillbirth or with multiple comorbidities that increase the risk of stillbirth. It is important to emphasize that the guidance offered in this Committee Opinion should be construed only as suggestions; this guidance should not be construed as mandates or as all encompassing. Ultimately, individualization about if and when to offer antenatal fetal surveillance is advised.


Assuntos
Morte Fetal/prevenção & controle , Seleção de Pacientes , Cuidado Pré-Natal/normas , Natimorto , Transtornos Relacionados ao Uso de Substâncias/complicações , Assistência Ambulatorial , Anemia Falciforme/complicações , Doenças Autoimunes/complicações , Anormalidades Congênitas/diagnóstico por imagem , Tomada de Decisão Compartilhada , Complicações do Diabetes/complicações , Feminino , Retardo do Crescimento Fetal/diagnóstico por imagem , Movimento Fetal , Humanos , Hipertensão/complicações , Nefropatias/complicações , Idade Materna , Doenças Placentárias/terapia , Poli-Hidrâmnios/terapia , Gravidez , Gravidez de Alto Risco , Gravidez Múltipla , Fatores de Risco , Doenças da Glândula Tireoide/complicações , Fatores de Tempo
8.
Front Immunol ; 12: 666114, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34012450

RESUMO

Patients with chronic inflammatory diseases are often treated with immunosuppressants and therefore are of particular concern during the SARS-CoV-2 pandemic. Serological tests will improve our understanding of the infection and immunity in this population, unless they tests give false positive results. The aim of this study was to evaluate the specificity of SARS-Cov-2 serological assays using samples from patients with chronic inflammatory diseases collected prior to April 2019, thus defined as negative. Samples from patients with multiple sclerosis (MS, n=10), rheumatoid arthritis (RA, n=47) with or without rheumatoid factor (RF) and/or anti-cyclic citrullinated peptide antibodies (anti-CCP2) and systemic lupus erythematosus (SLE, n=10) with or without RF, were analyzed for SARS-CoV-2 antibodies using 17 commercially available lateral flow assays (LFA), two ELISA kits and one in-house developed IgG multiplex bead-based assay. Six LFA and the in-house validated IgG assay correctly produced negative results for all samples. However, the majority of assays (n=13), gave false positive signal for samples from patients with RA and SLE. This was most notable in samples from RF positive RA patients. No false positive samples were detected in any assay using samples from patients with MS. Poor specificity of commercial serological assays could possibly be, at least partly, due to interfering antibodies in samples from patients with chronic inflammatory diseases. For these patients, the risk of false positivity should be considered when interpreting results of the SARS-CoV-2 serological assays.


Assuntos
Doenças Autoimunes/complicações , Teste Sorológico para COVID-19 , COVID-19/diagnóstico , SARS-CoV-2/isolamento & purificação , Adolescente , Adulto , Idoso , Anticorpos Antivirais/sangue , Autoanticorpos/sangue , Doenças Autoimunes/imunologia , COVID-19/complicações , COVID-19/imunologia , Reações Falso-Positivas , Feminino , Humanos , Imunoensaio , Imunoglobulina G/sangue , Imunoglobulina M/sangue , Masculino , Pessoa de Meia-Idade , Fator Reumatoide/sangue , SARS-CoV-2/imunologia , Adulto Jovem
9.
Clin Rheumatol ; 40(9): 3807-3815, 2021 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-33813619

RESUMO

OBJECTIVES: To compare cardiac function and systolic dyssynchrony of fetuses not exposed to and those exposed to maternal autoimmune antibodies using two-dimensional speckle tracking echocardiography (2DSTE). METHODS: An observational study of 52 fetuses, 18 from mothers with autoimmune antibodies (anti-SSA/Ro60, anti-Ro52 or/and anti-SSB/La) and 34 from healthy mothers without antibodies, was conducted. Maternal baseline characteristics, fetoplacental Doppler parameters, and conventional echocardiographic data were prospectively collected. Systolic global and regional longitudinal strain of left and right ventricle (LV and RV) and the time to peak strain of regional myocardium were measured using 2DSTE. We also calculated the differences in time to peak strain between the LV free wall and RV free wall (two-chamber dyssynchrony, 2C-DYS) and the LV dyssynchrony between the septum and LV free wall (one-chamber dyssynchrony, 1C-DYS). RESULTS: There were no significant differences in conventional systolic and diastolic functional parameters for the LV and RV. No effect modification was demonstrated in a myocardial deformation analysis. However, 1C-DYS was significantly more prolonged in the maternal autoimmune disease group (19.50 [8.00 to 29.25] vs. 28.50 [13.50 to 39.25], P = 0.042). CONCLUSIONS: LV systolic mechanical dyssynchrony in fetuses of mothers with autoimmune antibodies suggests in-utero subclinical damage of the cardiac conduction system. Key points • The left ventricular systolic dyssynchrony was significantly more prolonged in the maternal autoimmune disease (AD) fetuses. • Subclinical damage to the left ventricular conduction system of the fetal heart in maternal AD was observed. • Systolic and diastolic functional of the left and right ventricle were preserved in fetuses exposed to maternal autoimmune disease.


Assuntos
Doenças Autoimunes , Disfunção Ventricular Esquerda , Doenças Autoimunes/complicações , Doenças Autoimunes/diagnóstico por imagem , Diástole , Ecocardiografia , Feto , Humanos , Recém-Nascido , Sístole , Disfunção Ventricular Esquerda/diagnóstico por imagem
10.
Int J Mol Sci ; 22(6)2021 Mar 19.
Artigo em Inglês | MEDLINE | ID: mdl-33808574

RESUMO

COVID-19 is without any doubt the worst pandemic we have faced since the H1N1 virus outbreak. Even if vaccination against SARS-CoV-2 infection is becoming increasingly available, a more feasible approach for COVID-19 prevention and therapy is still needed. Evidence of a pathological link between metabolic diseases and severe forms of COVID-19 has stimulated critical reflection and new considerations. In particular, an abnormal immune response observed in certain patients with SARS-CoV-2 infection suggested possible common predisposing risk factors with autoimmune diseases such as Type 1 Diabetes (T1D). Correct supplementation with dietary factors may be key to preventing and counteracting both the underlying metabolic impairment and the complications of COVID-19. A set of agents may inhibit the cytokine storm and hypercoagulability that characterize severe COVID-19 infection: vitamin D3, omega-3 polyunsaturated fatty acids, polyphenols like pterostilbene, polydatin and honokiol, which can activate anti-inflammatory and antioxidant sirtuins pathways, quercetin, vitamin C, zinc, melatonin, lactoferrin and glutathione. These agents could be highly beneficial for subjects who have altered immune responses. In this review, we discuss the antiviral and metabolic effects of these dietary factors and propose their combination for potential applications in the prevention and treatment of COVID-19. Rigorous studies will be fundamental for validating preventive and therapeutic protocols that could be of assistance to mitigate disease progression following SARS-CoV-2 infection.


Assuntos
Doenças Autoimunes/dietoterapia , COVID-19/dietoterapia , Dieta , Doenças Metabólicas/dietoterapia , Doenças Autoimunes/complicações , COVID-19/complicações , Síndrome da Liberação de Citocina/dietoterapia , Síndrome da Liberação de Citocina/etiologia , Progressão da Doença , Humanos , Doenças Metabólicas/complicações , Trombofilia/dietoterapia , Trombofilia/etiologia
11.
BMC Infect Dis ; 21(1): 366, 2021 Apr 17.
Artigo em Inglês | MEDLINE | ID: mdl-33865323

RESUMO

BACKGROUND: Over the past decades, Klebsiella pneumoniae (K. pneumoniae) infections have been increasing and affected immunocompromised patients nosocomially and communally, with extended-spectrum ß-lactamase (ESBL) production becoming a major concern. Patients with rheumatic autoimmune diseases, mostly receiving immunosuppressive therapy, are vulnerable to various infections, including K. pneumoniae. However, few have investigated K. pneumoniae infections in this specific population. This study aimed to identify factors associated with ESBL production and mortality of K. pneumoniae pneumonia among patients with rheumatic autoimmune diseases in the Emergency Department. METHODS: We retrospectively investigated patients with rheumatic diseases who were diagnosed with K. pneumoniae pneumonia. The diagnosis of K. pneumoniae pneumonia was based on clinical manifestations, radiological findings and microbiological testing results. Prognostic factors and risk factors for ESBL production were determined with univariate and multivariate logistic regression analysis. Empirical therapy and antimicrobial susceptibility data were also collected. RESULTS: Of 477 K. pneumoniae pneumonia patients, 60 were enrolled into this study. The in-hospital mortality was 28.3%. Septic shock, ICU admission, the need for mechanical ventilation and change of antibiotics due to clinical deterioration, all related to mortality, were included as unfavorable clinical outcomes. Multivariate analysis suggested that ESBL production (OR, 6.793; p = 0.012), initial PCT ≥ 0.5 ng/ml (OR, 5.024; p = 0.033) and respiratory failure at admission (OR, 4.401; p = 0.046) predicted increased mortality. ESBL production was significantly associated with dose of corticosteroids (OR, 1.033; p = 0.008) and CMV viremia (OR, 4.836; p = 0.032) in patients with rheumatic autoimmune diseases. Abnormal leukocyte count (OR, 0.192; p = 0.036) was identified as a protective factor of ESBL-producing K. pneumoniae pneumonia. The most commonly used empirical antibiotic was ceftazidime, while most isolates showed less resistance to carbapenems and amikacin in susceptibility testing. CONCLUSIONS: K. pneumoniae pneumonia could be life-threatening in patients with rheumatic autoimmune diseases. Our findings suggested that ESBL production, initial PCT ≥ 0.5 ng/ml and respiratory failure at admission were independent factors associated with poor prognosis. Dose of corticosteroids and CMV viremia, predicting ESBL production in K. pneumoniae pneumonia, may help make individualized antibiotic decisions in clinical practice.


Assuntos
Doenças Autoimunes/epidemiologia , Infecções por Klebsiella/epidemiologia , Klebsiella pneumoniae/isolamento & purificação , Pneumonia Bacteriana/epidemiologia , Doenças Reumáticas/epidemiologia , Corticosteroides/uso terapêutico , Adulto , Idoso , Antibacterianos/uso terapêutico , Doenças Autoimunes/complicações , Doenças Autoimunes/tratamento farmacológico , Doenças Autoimunes/microbiologia , China/epidemiologia , Farmacorresistência Bacteriana/efeitos dos fármacos , Feminino , Mortalidade Hospitalar , Humanos , Imunossupressores/uso terapêutico , Infecções por Klebsiella/tratamento farmacológico , Infecções por Klebsiella/etiologia , Infecções por Klebsiella/microbiologia , Masculino , Pessoa de Meia-Idade , Pneumonia Bacteriana/complicações , Pneumonia Bacteriana/etiologia , Estudos Retrospectivos , Doenças Reumáticas/complicações , Doenças Reumáticas/tratamento farmacológico , Doenças Reumáticas/microbiologia , Fatores de Risco , beta-Lactamases/biossíntese
12.
Medicine (Baltimore) ; 100(15): e24889, 2021 Apr 16.
Artigo em Inglês | MEDLINE | ID: mdl-33847609

RESUMO

RATIONALE: Anti-myelin oligodendrocyte protein antibody-associated disease (MOGAD) is a new disease entity with various clinical phenotypes. MOGAD often present with recurrent optic neuritis (ON), and it can also develop as a compartment of neuromyelitis optica spectrum disorder (NMOSD). Moreover, multiple autoantibodies such as an anti-myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA) had been reported in the serum of patients with NMOSD. PATIENT CONCERNS: We report an 86-year-old woman with a 2-year history of microscopic polyangiitis (MPA). The patient had a rapid loss of vision in her left eye. No abnormal findings were observed on her left fundus, and she tested negative for MPO-ANCA upon admission. However, anti-MOG antibodies were observed in the patient's serum and cerebrospinal fluid. DIAGNOSIS: A diagnosis of MOGAD complicated with MPA was made. INTERVENTIONS: The patient received twice steroid pulse therapy and oral azathioprine as maintenance therapy. OUTCOMES: Her vision rapidly recovered, and no subsequent relapse was observed during the 8-month observation period. CONCLUSION: To the best of our knowledge, this is the first case of MOGAD complicated with MPA, and steroid pulse therapy and azathioprine therapy were effective for ON caused by MOGAD.


Assuntos
Doenças Autoimunes/complicações , Glicoproteína Mielina-Oligodendrócito/imunologia , Neurite Óptica/complicações , Idoso de 80 Anos ou mais , Doenças Autoimunes/tratamento farmacológico , Cegueira/etiologia , Feminino , Humanos , Imunossupressores/uso terapêutico , Neurite Óptica/tratamento farmacológico
13.
Rev Med Suisse ; 17(733): 697-701, 2021 Apr 07.
Artigo em Francês | MEDLINE | ID: mdl-33830702

RESUMO

Small fiber neuropathy (SFN) causes damage to small-calibre nerve fibers (unmyelinated C fibers and myelinated A-delta fibers). The symptoms of SFN usually are sensitive including paresthesia, dysesthesia or burning pain, and protopathic deficits, sometimes associated with dysautonomia. The causes of SFN can be classified in six main groups: idiopathic, toxic, metabolic, immunological, infectious and hereditary. In this article, we present the diagnostic approach to SFN, the most common autoimmune aetiologies, as well as elements of their therapeutic management.


Assuntos
Doenças Autoimunes , Neuropatia de Pequenas Fibras , Doenças Autoimunes/complicações , Doenças Autoimunes/diagnóstico , Humanos , Dor , Neuropatia de Pequenas Fibras/diagnóstico , Neuropatia de Pequenas Fibras/etiologia
14.
Med Clin (Barc) ; 156(12): 615-621, 2021 06 25.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-33836859

RESUMO

Autoimmune rheumatic diseases are inflammatory disorders that can involve multiple organs, including the heart. The high risk of cardiovascular pathology in these patients is not only due to traditional cardiovascular risk factors, but also to chronic inflammation and autoimmunity. All cardiac structures may be affected during the course of systemic autoimmune diseases (valves, the conduction system, the myocardium, endocardium and pericardium, and coronary arteries), and the cardiac complications have a variety of clinical manifestations. As these are all associated with an unfavourable prognosis, it is essential to detect subclinical cardiac involvement in asymptomatic systemic autoimmune disease patients and begin adequate management and treatment early. In this review, we examine the multiple cardiovascular manifestations in patients with rheumatological disorders and available management strategies.


Assuntos
Doenças Autoimunes , Doenças Cardiovasculares , Cardiopatias , Doenças Reumáticas , Doenças Autoimunes/complicações , Doenças Autoimunes/diagnóstico , Doenças Autoimunes/epidemiologia , Autoimunidade , Doenças Cardiovasculares/epidemiologia , Doenças Cardiovasculares/etiologia , Cardiopatias/diagnóstico , Cardiopatias/etiologia , Humanos , Miocárdio , Doenças Reumáticas/complicações
15.
J Autoimmun ; 120: 102632, 2021 06.
Artigo em Inglês | MEDLINE | ID: mdl-33812171

RESUMO

OBJECTIVE: COVID-19 has substantial morbidity and mortality. We studied whether hospitalized patients with COVID-19 and chronic inflammatory diseases experienced worse outcomes compared to patients hospitalized with COVID-19 without chronic inflammatory diseases. METHODS: Danish nationwide registers were used to establish a cohort of hospitalized patients with COVID-19 and inflammatory bowel diseases (IBD), rheumatoid arthritis (RA), spondyloarthropathy (SpA), or psoriatic arthritis (PsA) (exposed), and a control cohort without these diseases (unexposed) between March 1, 2020, and October 31, 2020. We compared median length of hospital stay, used median regression models to estimate crude and adjusted differences. When estimating crude and adjusted odds ratio (OR) for continuous positive airway pressure (CPAP) and mechanical ventilation, in-hospital death, 14-day and 30-day mortality, we used logistic regression models. RESULTS: We identified 132 patients with COVID-19 and IBD, RA, SpA, or PsA, and 2811 unexposed admitted to hospital with COVID-19. There were no differences between exposed and unexposed regarding length of hospital stay (6.8 days vs. 5.5 days), need for mechanical ventilation (7.6% vs. 9.4%), or CPAP (11.4% vs. 8.8%). Adjusted OR for in-hospital death was 0.71 (95% CI 0.42-1.22), death after 14-days 0.70 (95% CI 0.42-1.16), and death after 30-days 0.68 (95% CI 0.41-1.13). CONCLUSION: Hospitalized patients with COVID-19 and chronic inflammatory diseases did not have statistically significant increased length of hospital stay, had same need for mechanical ventilation, and CPAP. Mortality was similar in hospitalized patients with COVID-19 and chronic inflammatory diseases, compared to patients hospitalized with COVID-19 and no chronic inflammatory diseases.


Assuntos
Doenças Autoimunes/mortalidade , COVID-19/mortalidade , Mortalidade Hospitalar , Tempo de Internação , Sistema de Registros , SARS-CoV-2 , Idoso , Idoso de 80 Anos ou mais , Doenças Autoimunes/complicações , Doenças Autoimunes/terapia , COVID-19/etiologia , COVID-19/terapia , Doença Crônica , Estudos de Coortes , Dinamarca/epidemiologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Respiração Artificial , Fatores de Risco
16.
Clin Rheumatol ; 40(9): 3755-3763, 2021 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-33646447

RESUMO

OBJECTIVE: To compare Pneumocystis jirovecii pneumonia (PJP) risk between patients with autoimmune rheumatic diseases (ARD) and the general population METHODS: We identified patients with ARD recorded in the National Health Insurance Research Database of Taiwan from 2002 to 2015 and randomly selected a comparison cohort from the general population matched for age and sex. We analyzed PJP risk stratified by sex, age, comorbidities, and medications using Cox proportional hazard model. RESULTS: We enrolled 103,117 patients with ARD. PJP risk significantly increased in patients with any ARD and with each individual ARD like rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), Sjogren's syndrome (SjS), polymyositis and dermatomyositis (PM/DM), systemic sclerosis (SSc), and systemic vasculitis. Patients with PM/DM showed prominent risk with incidence rate of 12.47/100,000 patient year (95% confidence interval (CI), 32.16-86.70). In a time-dependent Cox proportional hazard model with comorbidities and medications as covariates, PM/DM, SSc, SLE, and SjS significantly increased adjusted hazard ratios (aHR) of 5.40, 5.12, 4.09, and 3.64, respectively (95% CI of 2.82-10.35, 2.16-12.13, 2.41-6.95, and 2.06-6.42, respectively). AHR after adjusting for male sex, cancer, human immunodeficiency virus infection (HIV), and interstitial lung disease also significantly increased. Use of daily oral steroid dose of >10 mg conferred the highest risk followed by mycophenolate. Use of injected steroids, cyclophosphamide, biological agents, methotrexate, and cyclosporine conferred a significantly higher risk. CONCLUSION: Underlying ARD significantly predisposes patients to PJP, with PM/DM posing the highest threat. In addition to underlying disease, comorbidities and concomitant immunosuppressants are major risks. The strongest risk is recent daily steroid dose of >10 mg. Mycophenolate seems to be a more prominent risk factor than cyclophosphamide. Key Points • Autoimmune rheumatic diseases (ARD) significantly increased the overall risk of PJP, and so did each individual ARD. • Use of steroids, mycophenolate, cyclophosphamide, biological agents, methotrexate, and cyclosporine all significantly increased risk of PJP. • Male, elderly, malignancy, HIV, and interstitial lung disease are also related to increased risk of PJP. • Underlying ARD, comorbidities, and use of immunosuppressant should all be considered in determining the overall risk of PJP.


Assuntos
Artrite Reumatoide , Doenças Autoimunes , Lúpus Eritematoso Sistêmico , Pneumocystis carinii , Pneumonia por Pneumocystis , Doenças Reumáticas , Idoso , Doenças Autoimunes/complicações , Doenças Autoimunes/epidemiologia , Humanos , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/epidemiologia , Masculino , Pneumonia por Pneumocystis/complicações , Pneumonia por Pneumocystis/epidemiologia , Doenças Reumáticas/complicações , Doenças Reumáticas/epidemiologia
18.
Int J Mol Sci ; 22(5)2021 Feb 25.
Artigo em Inglês | MEDLINE | ID: mdl-33669083

RESUMO

Phelan McDermid syndrome (PMcD) is a neurogenetic disease associated with haploinsufficiency of the SHANK3 gene due to a spectrum of anomalies in the terminal region of the long arm of chromosome 22. SHANK3 is the abbreviation for SH3 domain and ankyrin repeat-containing protein, a gene that encodes for proteins of the postsynaptic density (PSD) of excitatory synapses. This PSD is relevant for the induction and plasticity of spine and synapse formation as a basis for learning processes and long-term potentiation. Individuals with PMcD present with intellectual disability, muscular hypotonia, and severely delayed or absent speech. Further neuropsychiatric manifestations cover symptoms of the autism spectrum, epilepsy, bipolar disorders, schizophrenia, and regression. Regression is one of the most feared syndromes by relatives of PMcD patients. Current scientific evidence indicates that the onset of regression is variable and affects language, motor skills, activities of daily living and cognition. In the case of regression, patients normally undergo further diagnostics to exclude treatable reasons such as complex-focal seizures or psychiatric comorbidities. Here, we report, for the first time, the case of a young female who developed progressive symptoms of regression and a dystonic-spastic hemiparesis that could be traced back to a comorbid multiple sclerosis and that improved after treatment with methylprednisolone.


Assuntos
Doenças Autoimunes/tratamento farmacológico , Transtornos Cromossômicos/complicações , Metilprednisolona/administração & dosagem , Esclerose Múltipla/complicações , Regressão Psicológica , Anormalidades Múltiplas/genética , Anormalidades Múltiplas/fisiopatologia , Administração Intravenosa , Adulto , Transtorno do Espectro Autista/complicações , Doenças Autoimunes/líquido cefalorraquidiano , Doenças Autoimunes/complicações , Doenças Autoimunes/imunologia , Deleção Cromossômica , Transtornos Cromossômicos/líquido cefalorraquidiano , Transtornos Cromossômicos/diagnóstico por imagem , Transtornos Cromossômicos/genética , Cromossomos Humanos 21-22 e Y/genética , Cromossomos Humanos Par 22/genética , Feminino , Humanos , Imageamento por Ressonância Magnética , Esclerose Múltipla/líquido cefalorraquidiano , Proteínas do Tecido Nervoso/genética , Deleção de Sequência , Punção Espinal
19.
Rheumatol Int ; 41(5): 911-920, 2021 05.
Artigo em Inglês | MEDLINE | ID: mdl-33683393

RESUMO

Data on therapy of COVID-19 in immunocompetent and immunosuppressed children are scarce. We aimed to explore management strategies of pediatric rheumatologists. All subscribers to international Pediatric Rheumatology Bulletin Board were invited to take part in an online survey on therapeutic approaches to COVID-19 in healthy children and children with autoimmune/inflammatory diseases (AID). Off-label therapies would be considered by 90.3% of the 93 participating respondents. In stable patients with COVID-19 on oxygen supply (stage I), use of remdesivir (48.3%), azithromycin (26.6%), oral corticosteroids (25.4%) and/or hydroxychloroquine (21.9%) would be recommended. In case of early signs of "cytokine storm" (stage II) or in critically ill patients (stage III) (a) anakinra (79.5% stage II; 83.6% stage III) or tocilizumab (58.0% and 87.0%, respectively); (b) corticosteroids (oral 67.2% stage II, intravenously 81.7% stage III); (c) intravenous immunoglobulins (both stages 56.5%); or (d) remdesivir (both stages 46.7%) were considered. In AID, > 94.2% of the respondents would not support a preventive adaptation of the immunomodulating therapy. In case of mild COVID-19, more than 50% of the respondents would continue pre-existing treatment with immunoglobulins (100%), hydroxychloroquine (94.2%), anakinra (79.2%) or canakinumab (72.5%), or tocilizumab (69.8%). Long-term corticosteroids would be reduced by 26.9% (< = 2 mg/kg/d) and 50.0% (> 2 mg/kg/day), respectively, with only 5.8% of respondents voting to discontinue the therapy. Conversely, more than 75% of respondents would refrain from administering cyclophosphamide and anti-CD20-antibodies. As evidence on management of pediatric COVID-19 is incomplete, continuous and critical expert opinion and knowledge exchange is helpful.


Assuntos
Antirreumáticos/uso terapêutico , Antivirais/uso terapêutico , Doenças Autoimunes/tratamento farmacológico , COVID-19/tratamento farmacológico , Reumatologia/métodos , Doenças Autoimunes/complicações , COVID-19/epidemiologia , Estudos de Casos e Controles , Criança , Humanos , Imunomodulação , Pandemias , SARS-CoV-2 , Inquéritos e Questionários
20.
Clin J Gastroenterol ; 14(3): 918-922, 2021 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-33743140

RESUMO

Autoimmune pancreatitis is a rare, distinct and increasingly recognized form of chronic inflammatory pancreatic disease secondary to an underlying autoimmune mechanism. We report on a 14-year-old boy who developed autoimmune pancreatitis, while he was under treatment with eltrombopag for chronic immune thrombocytopenia. Therapy with corticosteroids resulted in complete remission of both. This is the first report on the co-occurrence of autoimmune pancreatitis and chronic immune thrombocytopenia in childhood, and clinicians should be aware of this rare association, because early diagnosis and therapy of autoimmune pancreatitis may prevent severe complications.


Assuntos
Doenças Autoimunes , Pancreatite Autoimune , Pancreatite , Púrpura Trombocitopênica Idiopática , Adolescente , Doenças Autoimunes/complicações , Doença Crônica , Humanos , Masculino , Pâncreas , Pancreatite/complicações , Púrpura Trombocitopênica Idiopática/complicações , Púrpura Trombocitopênica Idiopática/tratamento farmacológico
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