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2.
Am J Nurs ; 120(8): 32-37, 2020 08.
Artigo em Inglês | MEDLINE | ID: mdl-32665509

RESUMO

If undiagnosed and untreated, pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection (PANDAS) can suddenly and drastically disrupt the lives of previously healthy children and their families. The key to prompt diagnosis of PANDAS and its appropriate treatment is provider awareness that streptococcal infection may present with neuropsychiatric symptoms. The authors discuss the signs and symptoms that characterize PANDAS, as well as its presumed pathogenesis, and illustrate, through a composite case history, a symptom presentation, diagnostic journey, treatment course, and recovery that is representative of many PANDAS cases.


Assuntos
Doenças Autoimunes/diagnóstico , Transtorno Obsessivo-Compulsivo/diagnóstico , Infecções Estreptocócicas/diagnóstico , Antibacterianos/uso terapêutico , Anti-Inflamatórios não Esteroides/uso terapêutico , Antipsicóticos/uso terapêutico , Doenças Autoimunes/tratamento farmacológico , Doenças Autoimunes/epidemiologia , Doenças Autoimunes/fisiopatologia , Criança , Feminino , Humanos , Masculino , Transtorno Obsessivo-Compulsivo/tratamento farmacológico , Transtorno Obsessivo-Compulsivo/epidemiologia , Transtorno Obsessivo-Compulsivo/fisiopatologia , Pais/educação , Educação de Pacientes como Assunto , Prevalência , Prognóstico , Infecções Estreptocócicas/tratamento farmacológico , Infecções Estreptocócicas/epidemiologia , Infecções Estreptocócicas/fisiopatologia
3.
Autoimmun Rev ; 19(8): 102588, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32540447

RESUMO

The commercial tests currently available as second-level tests to detect ANA sub-specificities are generally used independently from the ANA immunofluorescence (IIF) pattern. The aim of this study was to evaluate the efficacy of the use of a customizable pattern-oriented antigenic panel by immunoblot (IB) using the International Consensus on ANA Patterns (ICAP) classification scheme, in order to introduce a novel and updated autoimmune diagnostic flowchart. 710 sera referred for routine ANA testing were selected on the basis of the ANA pattern according to the ICAP nomenclature (nuclear speckled AC-2,4,5; nucleolar AC-8,9,10,29; cytoplasmic speckled AC-18,19,20) and on an IIF titer ≥1:320. They were then assayed by three experimental IB assays using a panel of selected antigens. ICAP-oriented IB detected 515 antibody reactivities vs. 457 of traditional anti-ENA in the nuclear speckled pattern group, 108 vs. 28 in the nucleolar pattern group, and 43 vs. 34 in the cytoplasmic speckled pattern. This pilot study may lead the way for a new approach introducing an ICAP pattern-oriented follow up testing as a valid alternative to the existing standard panels, thus enabling more patients with autoimmune rheumatic disease to be accurately diagnosed.


Assuntos
Algoritmos , Anticorpos Antinucleares , Doenças Autoimunes , Técnicas e Procedimentos Diagnósticos , Immunoblotting , Anticorpos Antinucleares/sangue , Doenças Autoimunes/sangue , Doenças Autoimunes/diagnóstico , Técnica Indireta de Fluorescência para Anticorpo , Humanos , Immunoblotting/normas , Projetos Piloto
5.
J Autoimmun ; 112: 102502, 2020 08.
Artigo em Inglês | MEDLINE | ID: mdl-32527675

RESUMO

BACKGROUND: Whether patients with autoimmune rheumatic diseases (ARD) have a higher risk for SARS-CoV-2 infection (COVID-19) and how SARS-CoV-2 pandemic impacts on adherence to therapy has not been fully elucidated. We assessed the rate and clinical presentation of COVID-19, and adherence to therapy in a large cohort of patients with ARD followed-up in a tertiary University-Hospital in Northeast Italy. METHODS: Between April 9th and April 25th, 2020, after SARS-CoV-2 infection peak, a telephone survey investigating the impact of COVID-19 on patients with systemic lupus erythematosus (SLE), systemic sclerosis (SSc), rheumatoid arthritis (RA), ANCA-associated vasculitis (AAV), and idiopathic inflammatory myopathies (IIM) was administered. Demographics, disease activity status, therapy, occupational exposure, and adherence to social distancing advise were also collected. RESULTS: 916 patients (397 SLE, 182 AAV, 176 SSc, 111 RA, 50 IIM) completed the survey. 148 patients developed at least one symptom compatible with COVID-19 (cough 96, sore throat 64, fever 64, arthromyalgias 59, diarrhea 26, conjunctivitis 18, ageusia/hyposmia, 18). Among the 916 patients, 65 (7.1%) underwent SARS-CoV-2 nasopharyngeal swab (18 symptomatic and 47 asymptomatic), 2 (0.21%) tested positive, a proportion similar to that observed in the general population of the Veneto region. No deaths occurred. 31 patients (3.4%) withdrew ≥1 medication, mainly immunosuppressants or biologics. Adoption of social distancing was observed by 860 patients (93.9%), including 335 (36.6%) who adopted it before official lockdown. CONCLUSIONS: COVID-19 incidence seems to be similar in our cohort compared to the general population. Adherence to therapy and to social distancing advise was high.


Assuntos
Doenças Autoimunes/tratamento farmacológico , Betacoronavirus , Infecções por Coronavirus/tratamento farmacológico , Imunossupressores/administração & dosagem , Pneumonia Viral/tratamento farmacológico , Doenças Reumáticas/tratamento farmacológico , Adulto , Idoso , Doenças Autoimunes/diagnóstico , Doenças Autoimunes/virologia , Infecções por Coronavirus/diagnóstico , Infecções por Coronavirus/patologia , Feminino , Humanos , Itália , Masculino , Pessoa de Meia-Idade , Pandemias , Pneumonia Viral/diagnóstico , Pneumonia Viral/patologia , Doenças Reumáticas/diagnóstico , Doenças Reumáticas/virologia
6.
S D Med ; 73(6): 266-269, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-32580259

RESUMO

IgG4-related disease (IgG4RD) is a chronic immune mediated condition primarily affecting the hepato-pancreatico-biliary system. We report a case of IgG4RD with extensive pancreatic and hepatic involvement masquerading as metastatic pancreatic malignancy posing a diagnostic and therapeutic dilemma.


Assuntos
Doenças Autoimunes , Doença Relacionada a Imunoglobulina G4 , Pancreatite , Doenças Autoimunes/diagnóstico , Humanos , Imunoglobulina G , Doença Relacionada a Imunoglobulina G4/diagnóstico , Pâncreas , Pancreatite/diagnóstico
8.
J Vis Exp ; (159)2020 05 29.
Artigo em Inglês | MEDLINE | ID: mdl-32538917

RESUMO

Islet autoantibodies (IAbs) are widely used in type 1 diabetes (T1D) diagnosis and prediction. Four major IAbs to insulin (IAA), glutamate decarboxylase-65 (GADA), insulinoma antigen-2 (IA-2A), and zinc transporter-8 (ZnT8A) are equally important in disease prediction. Presently, up to 40% of patients diagnosed with T1D go on to develop other autoimmune disorders. Unfortunately, current screening methods using a single autoantibody for measurement are laborious and inefficient for large scale screening studies. We recently developed a simple multiplexed electrochemiluminescence (ECL) assay to address these current issues. The assay combines all 7 autoantibody tests into one well. Each well includes three IAbs (IAA, GADA, and IA-2A), autoantibodies to thyroid peroxidase (TPOA) and thyroid globulin (ThGA) to detect autoimmune thyroid disease, autoantibodies to tissue transglutaminase (TGA) for celiac disease, and autoantibodies to interferon alpha (IFNαA) for autoimmune polyglandular syndrome-1 (APS-1); all of which screen for T1D and other relevant autoimmune diseases, simultaneously. The multiplex ECL assay is based on the single ECL assay platform, but instead uses the multiplex plate combining multiple autoantibody assays, up to 10, into a single well. The main difference from the single ECL assay is that each antibody-antigen complex formed in the fluid-phase is restrained onto a specific spot on each well through a linker system on the multiplex plate. The 7-Plex ECL assay, in the present study, is validated against standard radio-binding assays (RBA) and single ECL assays, using a large cohort of newly diagnosed T1D patients and age-matched healthy controls, resulting in excellent assay sensitivity and specificity.


Assuntos
Doenças Autoimunes/diagnóstico , Diabetes Mellitus Tipo 1/diagnóstico , Ensaios de Triagem em Larga Escala/métodos , Feminino , Humanos , Masculino
9.
Presse Med ; 49(2): 104021, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-32437843

RESUMO

Interstitial lung diseases encompass a broad range of numerous individual conditions, some of them characterized histologically by fibrosis, especially idiopathic pulmonary fibrosis, nonspecific interstitial pneumonia, chronic hypersensitivity pneumonia, interstitial lung disease associated with connective tissue diseases, and unclassifiable interstitial lung disease. The diagnostic approach relies mainly on the clinical evaluation, especially assessment of the patient's demographics, history, smoking habits, occupational or domestic exposures, use of drugs, and on interpretation of high-quality HRCT of the chest. Imaging is key to the initial diagnostic approach, and often can confirm a definite diagnosis, particularly a diagnosis of idiopathic pulmonary fibrosis when showing a pattern of usual interstitial pneumonia in the appropriate context. In other cases, chest HRCT may orientate toward an alternative diagnosis and appropriate investigations to confirm the suspected diagnosis. Autoimmune serology helps diagnosing connective disease. Indications for bronchoalveolar lavage and for lung biopsy progressively become more restrictive, with better considerations for their discriminate value, of the potential risk associated with the procedure, and of the anticipated impact on management. Innovative techniques and genetics are beginning to contribute to diagnosing interstitial lung disease and to be implemented routinely in the clinic. Multidisciplinary discussion, enabling interaction between pulmonologists, chest radiologists, pathologists and often other healthcare providers, allows integration of all information available. It increases the accuracy of diagnosis and prognosis prediction, proposes a first-choice diagnosis, may suggest additional investigations, and often informs the management. The concept of working diagnosis, which can be revised upon additional information being made available especially longitudinal disease behaviour, helps dealing with diagnostic uncertainty inherent to interstitial lung diseases and facilitates management decisions. Above all, the clinical approach and how thoroughly the patient's history and possible exposures are assessed determine the possibility of an accurate diagnosis.


Assuntos
Doenças Pulmonares Intersticiais/diagnóstico , Alveolite Alérgica Extrínseca/diagnóstico , Doenças Autoimunes/diagnóstico , Biópsia , Lavagem Broncoalveolar , Doenças do Tecido Conjuntivo/complicações , Doenças do Tecido Conjuntivo/diagnóstico , Humanos , Fibrose Pulmonar Idiopática/diagnóstico , Pulmão/patologia , Doenças Pulmonares Intersticiais/etiologia , Doenças Pulmonares Intersticiais/patologia , Exame Físico , Fibrose Pulmonar/diagnóstico , Radiografia Torácica , Testes de Função Respiratória , Tomografia Computadorizada por Raios X
10.
Hautarzt ; 71(7): 542-552, 2020 Jul.
Artigo em Alemão | MEDLINE | ID: mdl-32435817

RESUMO

BACKGROUND AND OBJECTIVES: Treatment of severe dermatological autoimmune diseases and toxic epidermal necrolysis (TEN) with high-dose intravenous immunoglobulin (IVIg) is a well-established procedure in dermatology. As treatment with IVIg is usually considered for rare clinical entities or severe cases, the use of immunoglobulin is not generally based on data from randomized controlled trials usually required for evidence-based medicine. Since the indications for the use of IVIg are rare, it is unlikely that such studies will be available in the foreseeable future. Because first-line use is limited by the high costs of IVIg, the first clinical guidelines on the use of IVIg in dermatological conditions were established in 2008 and renewed in 2011. METHODS: The European guidelines presented here were prepared by a panel of experts nominated by the European Dermatology Forum (EDF) and European Academy of Dermatology and Venereology (EADV). The guidelines were developed to update the indications for treatment currently considered effective and to summarize the evidence for the use of IVIg in dermatological autoimmune diseases and TEN. RESULTS AND CONCLUSION: The current guidelines represent consensual expert opinions and definitions on the use of IVIg reflecting current published evidence and are intended to serve as a decision-making tool for the use of IVIg in dermatological diseases.


Assuntos
Doenças Autoimunes/tratamento farmacológico , Dermatologia/normas , Imunoglobulinas Intravenosas/administração & dosagem , Guias de Prática Clínica como Assunto , Dermatopatias/tratamento farmacológico , Doenças Autoimunes/diagnóstico , Dermatologia/métodos , Relação Dose-Resposta a Droga , Europa (Continente) , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Dermatopatias/diagnóstico , Síndrome de Stevens-Johnson
11.
Hautarzt ; 71(5): 359-364, 2020 May.
Artigo em Alemão | MEDLINE | ID: mdl-32253451

RESUMO

Autoinflammatory syndromes are characterized by an exaggerated activation of the innate immune system and frequently present with skin symptoms. In contrast to autoimmune disorders no specific autoantibodies or autoreactive immune cells are detected. Thus, the diagnosis is usually difficult and can only be made by a careful interpretation of anamnestic, clinical and laboratory parameters. In some hereditary autoimmune syndromes specific genetic mutations are described and can be helpful for the diagnosis. For treatment of these disorders both classic immunomodulatory drugs and specific cytokine inhibitors are used, mainly directed against interleukin­1. Long-term therapy is generally required.


Assuntos
Doenças Autoimunes/imunologia , Doenças Hereditárias Autoinflamatórias , Hidradenite Supurativa/imunologia , Pioderma Gangrenoso/diagnóstico , Dermatopatias/imunologia , Doenças Autoimunes/diagnóstico , Citocinas , Hidradenite Supurativa/diagnóstico , Humanos , Interleucina-1 , Dermatopatias/etiologia , Dermatopatias/patologia , Síndrome
12.
Hautarzt ; 71(5): 342-358, 2020 May.
Artigo em Alemão | MEDLINE | ID: mdl-32303770

RESUMO

Systemic autoinflammatory diseases are characterized by a spontaneous chronic inflammatory reaction mediated by the innate immunity. The inflammatory processes involve many organs including the skin. Diagnosis remains a challenge despite new molecular genetic methods, but early diagnosis is crucial for the prevention of long-term complications such as amyloidosis. Skin manifestations are often observed early in the course of the disease and can be decisive in the diagnosis.


Assuntos
Doenças Autoimunes/diagnóstico , Doenças Hereditárias Autoinflamatórias/diagnóstico , Humanos , Imunidade Inata , Inflamação , Pele
13.
Praxis (Bern 1994) ; 109(5): 355-359, 2020.
Artigo em Alemão | MEDLINE | ID: mdl-32233757

RESUMO

Muscle Complaints - When to Evaluate Further? Abstract. Muscle complaints are frequently reported symptoms which can occur in the context of numerous and not always primary muscle diseases. Further diagnostic steps are not always warranted. The presence of distinct warning symptoms represents the base for further decision making. Warning symptoms include among others myalgia/weakness/atrophy/hypertrophy of distinct muscle groups, fasciculations, dysphagia, bulbar signs, abnormal reflexes, signs for a systemic autoimmune disorder/connective tissue disease or a persisting CK elevation (>1000 U/l) even in asymptomatic patients. Depending on the indication important diagnostic steps include lab tests, MRI, E(N)MG, specific endurance tests, muscle biopsy or genetic tests.


Assuntos
Doenças Autoimunes , Doenças Musculares , Atrofia , Doenças Autoimunes/complicações , Doenças Autoimunes/diagnóstico , Biópsia , Humanos , Músculo Esquelético , Doenças Musculares/diagnóstico , Mialgia/etiologia
15.
Zhonghua Er Ke Za Zhi ; 58(3): 218-222, 2020 Mar 02.
Artigo em Chinês | MEDLINE | ID: mdl-32135594

RESUMO

Objective: To explore the clinical phenotype, immunological features, pathogenesis and gene variation of a case with A20 haploinsufficiency (HA20). Methods: A patient diagnosed with tumor necrosis factor α-induced protein 3 (TNFAIP3) mutated HA20 was admitted into Shenzhen Children's Hospital in May,2019.The clinical data was analyzed. Flow cytometry was used to detect the patient's peripheral blood lymphocyte subsets, and also, the percentage of follicular helper T cell (TFH) cells in the patient and thirteen healthy controls. After the construction of empty vector, wild-type and mutant plasmid vectors, a wild-type or mutant overexpression system of the TNFAIP3 gene was established in 293T cells and Hela cells. Then, the expression level of A20 in 293T cells and the expression of inhibitor K binding α (IKBα) in green fluorescent protein (GFP)+Hela cells before and after tumor necrosis factor α (TNF-α) stimulation were measured, to verify the pathogenicity of this variation. Results: A 5 years and 11 months old boy, presented with recurrent oral ulcer, abdominal pain, joint swelling and arthralgia. Oral ulcer, chronic skin rashes, knee joint swelling were observed. The levels of inflammatory markers were increased. Colonoscopy showed congestion of mucosa and multiple ulcers in terminal ileum and ileocecus. The absolute number of naive B cells was 124×10(6) cells/L (reference range 147×10(6)-431×10(6) cells/L), accounting for 0.430 of the total B cells (reference range 0.484-0.758). Compared to healthy controls (0.016-0.071), the percentage of TFH cells in CD4(+)T cells was much lower (0.008).A heterozygous mutation of TNFAIP3 gene (c.909_913 del, p.L303fs) was identified by genetic analysis. In vitro study showed that truncated A20 protein was expressed in TNFAIP3 mutant overexpressed 293T cells, which verified the pathogenicity of this variation. Besides, after TNF-α stimulation, the degradation rate of IkBα protein in mutant overexpressed Hela cells (35%) was between the other two groups (15% in the wild-type group and 57% in the non-loaded group). Conclusions: This case with HA20 due to a de novo TNFAIP3 gene mutation presents with early onset Behcet-like autoinflammatory syndrome. This variation leads to expression of truncated A20 protein, enhanced degradation of IkBα, and further activation of nuclear factor κB signaling pathway.


Assuntos
Doenças Autoimunes , Haploinsuficiência , NF-kappa B , Proteína 3 Induzida por Fator de Necrose Tumoral alfa , Doenças Autoimunes/diagnóstico , Doenças Autoimunes/genética , Pré-Escolar , Haploinsuficiência/genética , Células HeLa , Heterozigoto , Humanos , Masculino , Fenótipo , Proteína 3 Induzida por Fator de Necrose Tumoral alfa/genética
16.
Muscle Nerve ; 61(6): 791-796, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-32133669

RESUMO

INTRODUCTION: Small-fiber neuropathy is rare in children. It has been associated with several autoimmune disorders, but there are no reports of an autoinflammatory etiology. METHODS: The data of four children/adolescents presenting with erythromelalgia and neuropathic pain from 2014 to 2019 were collected retrospectively from the electronic database of a pediatric medical center. RESULTS: Results of clinical and/or electrophysiological evaluation excluded large nerve fiber involvement. Skin biopsy results confirmed small-fiber neuropathy. According to genetic analysis, two patients were heterozygous and one was homozygous for mutations in the familial Mediterranean fever (MEFV) gene. Behcet disease was diagnosed in the fourth patient. Treatment with anti-interleukin-1 agents, intravenous immunoglobulin, and glucocorticoids was beneficial. DISCUSSION: The diagnosis of small-fiber neuropathy should be considered in children/adolescents presenting with erythromelalgia. A thorough investigation is required to reveal the underlying disorder. Clinicians should be alert to the peripheral neurological manifestations of autoinflammatory syndromes because effective treatments are available.


Assuntos
Eritromelalgia/complicações , Eritromelalgia/diagnóstico , Neuropatia de Pequenas Fibras/complicações , Neuropatia de Pequenas Fibras/diagnóstico , Adolescente , Doenças Autoimunes/complicações , Doenças Autoimunes/diagnóstico , Doenças Autoimunes/fisiopatologia , Criança , Eritromelalgia/fisiopatologia , Feminino , Humanos , Inflamação/complicações , Inflamação/diagnóstico , Inflamação/fisiopatologia , Estudos Retrospectivos , Neuropatia de Pequenas Fibras/fisiopatologia , Síndrome
17.
BMC Neurol ; 20(1): 44, 2020 Feb 03.
Artigo em Inglês | MEDLINE | ID: mdl-32013931

RESUMO

BACKGROUND: Autoimmune encephalitis is characterized by neuropsychiatric symptoms associated with brain inflammation. The differential is usually broad and Psychiatry often collaborates with Neurology in diagnostic clarification and symptom management. At least 40% of neuroencephalitis cases are of unknown etiology which adds to difficulties in making the right diagnosis and deciding on the appropriate treatment (Granerod et al., Lancet Infect Dis 10:835-44, 2010). The aim of this case series was to present four cases with complicated psychiatric symptomatology and isolated neurologic signs and symptoms, evaluated at a large tertiary medical center and treated for suspected autoimmune encephalitis, demonstrating the complexity of diagnosis and treatment. CASE PRESENTATION: Four diagnostically challenging and heterogeneous cases displayed clinical symptomatology suggestive of autoimmune encephalitis. All cases presented with neurologic and psychiatric symptoms, but had negative autoantibody panels, normal or inconclusive magnetic resonance imaging results and non-specific cerebrospinal fluid changes. All were challenged with immunosuppressive/immunomodulatory treatments with overall poor response rates. CONCLUSIONS: There is a heterogeneous presentation of autoimmune encephalitis in pediatric populations. In the absence of positive findings on testing, individuals who do not meet proposed criteria for seronegative encephalitis may be misdiagnosed, and/or may not respond adequately to treatment. In those cases, comprehensive evaluation and stringent application of consensus guidelines is necessary.


Assuntos
Doenças Autoimunes/diagnóstico , Encefalite/diagnóstico , Adolescente , Autoanticorpos/imunologia , Criança , Feminino , Humanos , Lactente , Imagem por Ressonância Magnética , Masculino , Transtornos Mentais/etiologia
18.
BMJ Case Rep ; 13(2)2020 Feb 05.
Artigo em Inglês | MEDLINE | ID: mdl-32029513

RESUMO

Statin-induced necrotising autoimmune myopathy (SINAM) is a rare disease characterised by proximal muscle weakness and elevated creatine kinase levels that is usually in the thousands. Anti-3-hydroxy-3-methyl glutaryl co-enzyme A reductase (HMGCR) antibodies are associated with SINAM. Autoimmune hepatitis (AIH) is an inflammatory disease of the liver that is usually of unknown aetiology but can also be associated with concurrent extrahepatic autoimmune disorders. We are reporting a case of biopsy proven AIH associated with SINAM in a patient presenting with oropharyngeal dysphagia. The patient had elevated anti-HMGCR antibodies and anti-smooth muscle antibodies. SINAM and AIH were confirmed by muscle biopsy and liver biopsy, respectively. The patient had complete resolution of his symptoms and complete normalisation of his liver function tests after 6 months of the treatment.


Assuntos
Doenças Autoimunes/diagnóstico , Transtornos de Deglutição/etiologia , Hepatite Autoimune/diagnóstico , Inibidores de Hidroximetilglutaril-CoA Redutases/efeitos adversos , Miosite/diagnóstico , Doenças Autoimunes/induzido quimicamente , Hepatite Autoimune/complicações , Humanos , Masculino , Pessoa de Meia-Idade , Miosite/induzido quimicamente
19.
BMC Neurol ; 20(1): 1, 2020 Jan 03.
Artigo em Inglês | MEDLINE | ID: mdl-31900128

RESUMO

OBJECTIVE: To investigate the clinical characteristics and prognosis of anti-gamma-aminobutyric acid-B (GABA-B) receptor encephalitis. METHODS: This retrospective study enrolled nineteen patients with anti-GABA-B receptor encephalitis. Clinical manifestations, radiological and electroencephalogram features, treatment and outcomes were collected and analyzed. The neurological function was evaluated according to the modified Rankin Scale (mRS). RESULTS: There were eleven patients in the favorable-prognosis group (mRS ≤ 2) and eight patients in the poor-prognosis group (mRS > 2). In the favorable-prognosis group, clinical symptoms included memory deterioration (n = 10; 90.9%), epileptic seizures (n = 9; 81.8%), psychiatric disorders (n = 9; 81.8%), and conscious disturbance (n = 5; 45.5%); magnetic resonance imaging (MRI) indicated an involvement of the limbic system in three (27.3%) cases in this group. Lung cancer was detected in one patient (9.1%). After an average follow-up period of 11.7 months, four (36.4%) patients were cured, and seven (63.6%) patients showed significant improvements. In the poor-prognosis group, all patients presented with memory deterioration, epileptic seizures, psychiatric disorders, and conscious disturbance; five (62.5%) patients had convulsive status epilepticus, and five (62.5%) patients developed respiratory failure; MRI indicated an involvement of the limbic system in seven (87.5%) cases. Malignant tumors were detected in five (62.5%) patients. After an average follow-up period of 14.8 months, seven (87.5%) patients died and one (12.5%) patient remained dependent in daily life. CONCLUSIONS: The clinical manifestations of anti-GABA-B receptor encephalitis include epileptic seizures, cognitive impairment and psychiatric disorders. Patients with convulsive status epilepticus or respiratory failure have poor outcomes. In anti-GABA-B receptor encephalitis, limbic system involvement is associated with a poor prognosis in and radiological examinations can reflect disease progression. Early diagnosis and appropriate treatment should be highlighted.


Assuntos
Doenças Autoimunes , Encefalite , Receptores de GABA-B/imunologia , Doenças Autoimunes/complicações , Doenças Autoimunes/diagnóstico , Doenças Autoimunes/fisiopatologia , China , Encefalite/complicações , Encefalite/diagnóstico , Encefalite/fisiopatologia , Humanos , Prognóstico , Estudos Retrospectivos , Convulsões/etiologia
20.
Curr Allergy Asthma Rep ; 20(1): 4, 2020 01 28.
Artigo em Inglês | MEDLINE | ID: mdl-31993777

RESUMO

PURPOSE OF REVIEW: Progestogen hypersensitivity (PH) is a condition which typically occurs in women in childbearing years with a spectrum of symptoms ranging from urticaria with or without angioedema, dermatitis to systemic anaphylaxis. Herein, a clinical case of PH is presented followed by a discussion on the evaluation, diagnosis, and management of PH. RECENT FINDINGS: Progestogen hypersensitivity (a.k.a. "autoimmune progesterone dermatitis") symptoms are associated with exogenous progestin exposure (e.g., contraceptive medicines, in vitro fertilization therapy) or endogenous progesterone from progesterone surges during the luteal phase of the menstrual cycle and pregnancy. This condition can be difficult to recognize due to its heterogeneous clinical presentation. The mechanism of PH is believed to be primarily IgE-mediated; however, less commonly other immune responses may be involved. There is now a useful progesterone specific IgE immunoassay to assist in diagnosis and well-defined treatment algorithms that can be used to successfully manage PH. The epidemiology of PH is still poorly elucidated but is likely to be encountered by clinicians and especially allergists given the extensive use of oral contraceptives and increased use of supra-physiologic doses of progesterone required to support pregnancy in IVF. Including PH in the differential diagnosis of women presenting with cyclic hypersensitivity will accelerate diagnosis and successful management of this condition.


Assuntos
Anafilaxia/induzido quimicamente , Doenças Autoimunes/induzido quimicamente , Dispositivos Intrauterinos Medicados/efeitos adversos , Progesterona/efeitos adversos , Progestinas/efeitos adversos , Urticária/induzido quimicamente , Anafilaxia/diagnóstico , Anafilaxia/tratamento farmacológico , Antialérgicos/uso terapêutico , Doenças Autoimunes/diagnóstico , Doenças Autoimunes/tratamento farmacológico , Dermatite/diagnóstico , Dermatite/tratamento farmacológico , Dessensibilização Imunológica , Remoção de Dispositivo , Ensaio de Imunoadsorção Enzimática , Feminino , Humanos , Imunoglobulina E/sangue , Omalizumab/uso terapêutico , Urticária/diagnóstico , Urticária/tratamento farmacológico , Adulto Jovem
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