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1.
Rinsho Ketsueki ; 60(10): 1449-1454, 2019.
Artigo em Japonês | MEDLINE | ID: mdl-31695006

RESUMO

The clinical course of chronic lymphocytic leukemia (CLL) is often complicated by autoimmune cytopenia (AIC). Here we report a case of a 69-year-old woman with CLL complicated by monoclonal immunoglobulin deposition disease (MIDD) and AIC. The patient was diagnosed with CLL at the age of 63 years and treated with chemotherapy including rituximab and/or fludarabine owing to the development of anemia, multiple lymphadenopathy, and B symptoms at the age of 66 years. Furthermore, pancytopenia and renal failure developed at the age of 68 years. Consequently, the patient was admitted owing to dyspnea. Because of no apparent signs of CLL progression, we concluded that pancytopenia was due to AIC (autoimmune granulocytopenia and thrombocytopenia) and renal anemia. MIDD was diagnosed based on renal histology and detection of IgM and λ chain immunoglobulin deposits on glomeruli and tubules, which were presumed to be derived from CLL cells. The patient was treated with ibrutinib in order to reduce monoclonal protein levels. AIC improved concurrently with IgM reduction 1 month later. Supportive care, involving transfusion and granulocyte colony-stimulating factor, was not required approximately 3 months after initiating ibrutinib treatment. In contrast, MIDD did not improve and maintenance hemodialysis was required. Owing to its antitumor and immunomodulatory effects, ibrutinib may contribute to improve CLL-associated AIC.


Assuntos
Anticorpos Monoclonais , Doenças Autoimunes/tratamento farmacológico , Leucemia Linfocítica Crônica de Células B/complicações , Pirazóis/uso terapêutico , Pirimidinas/uso terapêutico , Idoso , Feminino , Humanos , Rituximab
3.
Recurso na Internet em Português | LIS - Localizador de Informação em Saúde, LIS-bvsms | ID: lis-LISBR1.1-46830

RESUMO

Os pênfigos são doenças relativamente raras caracterizados pela formação de bolhas na pele e, às vezes, também nas mucosas (como boca, garganta, olhos, nariz e região genital de homens e mulheres).


Assuntos
Pênfigo , Doenças Autoimunes , Penfigoide Bolhoso
4.
Zhong Nan Da Xue Xue Bao Yi Xue Ban ; 44(8): 878-884, 2019 Aug 28.
Artigo em Chinês | MEDLINE | ID: mdl-31570674

RESUMO

OBJECTIVE: To assess the value of immunohistochemical analysis for expressions of C3d, C4d, IgG, IgG4, and CD123 in the diagnosis of autoimmune skin diseases.
 Methods: We investigated the expressions of C3d, C4d, IgG, IgG4, and CD123 in paraffin-embedded, formalin-fixed tissues from 27 lupus erythematosus cases, including 8 discoid lupus erythematosus (DLE) cases, 4 subacute cutaneous lupus erythematosus (SCLE) cases, and 15 systemic lupus erythematosus (SLE) cases. Tissues from 15 dermatomyositis (DM) cases, 15 bullous pemphigoid (BP) cases, and 15 pemphigus cases were examined by immunohistochemical analysis. The differences in expression rates of C3d, C4d, IgG, IgG4, and CD123 between immunohistochemical staining and direct immunofluorescence were compared in the diagnosis of these diseases.
 Results: In the lupus erythematosus group, the positive rates of C3d and C4d deposited along the dermoepidermal junction were 85.2% and 51.9%, respectively. In the dermatomyositis group, the positive rates of C3d and C4d deposited along the dermoepidermal junction were 40% and 0, respectively. The expressions of C3d and C4d in lupus erythematosus tissues were significantly higher than those in DM tissues (P<0.05). The expression of CD123 protein in skin lesions of the lupus group was significantly higher than that in the DM group (P<0.05). In the BP group, the positive rates of C3d and C4d deposited along the dermoepidermal junction were 100% and 86.7%, respectively. In the pemphigus group, the positive rates of C3d and C4d deposited in the intercellular space of keratinocytes were 100% and 60%, respectively. The expressions of IgG and IgG4 in pemphigus tissues were higher than those in BP tissues (P<0.05). And the ratios of IgG4 to IgG in the pemphigus group was significantly higher than that in the BP group (P<0.05).
 Conclusion: The assays of C3d and C4d define an important diagnostic adjunct in evaluation of lupus erythematosus, BP and pemphigus. In some cases, it may even replace the direct immunofluorescence as a diagnostic adjunct. The expression of CD123 possesses certain clinical significance for the differential diagnosis of lupus erythematosus, and IgG4 and IgG expressions have adjunctive diagnostic significance for pemphigus.


Assuntos
Doenças Autoimunes , Lúpus Eritematoso Sistêmico , Pênfigo , Complemento C3d , Proteínas do Sistema Complemento , Humanos , Imunoglobulina G , Subunidade alfa de Receptor de Interleucina-3
5.
Medicine (Baltimore) ; 98(41): e17348, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31593084

RESUMO

Immune checkpoint inhibitors (ICIs) like cytotoxic T-lymphocyte-associated protein 4 (anti-CTLA4) and programmed death cell protein 1 (anti-PD1) have revolutionized cancer treatment. As ICI use becomes widespread, more immune-related adverse events (irAE's) are being reported. Our aim was to investigate the frequency and nature of new irAE's as well as report the frequency of flare-ups of pre-existing autoimmune conditions occurring after ICI therapy.We performed a retrospective chart review of all patients treated for cancer with anti-PD1 or anti-CTLA4 or combination therapy at our tertiary care center from January 2014 to April 2016. Demographic data, cancer type and stage, irAE's (new immune disorders and disease flares of pre-existing autoimmune disorders on ICI therapy), and drug treatment information were extracted.We identified 220 patients treated with ICI therapy during the study period out of which 27% (60/220) developed irAE's. 11% in anti-CTLA4 group and 16% among anti-PD1 treated patients developed irAE's. IrAE's resulted in discontinuation of cancer therapy in 28% of those who developed irAE's. 21.4% had a flare of their autoimmune disease but only 1 required discontinuation of immunotherapy.IrAE's are an important emerging clinical disease entity for specialists to be aware of. Our study shows that ICI's can be safely used in patients with pre-existing autoimmune conditions with close monitoring. However, there is still a large unmet need to have a better understanding of how to systematically evaluate and manage patients with irAE's as well as for identifying the predictors of irAE's.


Assuntos
Antineoplásicos Imunológicos/efeitos adversos , Doenças do Sistema Imunitário/induzido quimicamente , Imunoterapia/efeitos adversos , Melanoma/tratamento farmacológico , Neoplasias/tratamento farmacológico , Adulto , Idoso , Idoso de 80 Anos ou mais , Doenças Autoimunes/induzido quimicamente , Doenças Autoimunes/imunologia , Antígeno CTLA-4/antagonistas & inibidores , Feminino , Humanos , Doenças do Sistema Imunitário/imunologia , Masculino , Melanoma/imunologia , Pessoa de Meia-Idade , Neoplasias/imunologia , Receptor de Morte Celular Programada 1/antagonistas & inibidores , Estudos Retrospectivos
6.
Zhonghua Gan Zang Bing Za Zhi ; 27(8): 643-647, 2019 Aug 20.
Artigo em Chinês | MEDLINE | ID: mdl-31594084

RESUMO

Objective: To understand the basic information of anti-mitochondrial antibody (anti-AMA)-positive patients after initial diagnosis, and to set groundwork for further exploring the clinical significance of AMA in various diseases. Methods: Demographic data and related clinical information recorded through the Information System of Peking University People's Hospital from January 2013 to December 2016 were collected. Patients whose AMA and/or AMA-M2 first- tested as positive were recorded. Complications were classified according to the International Classification of Diseases. Results: A total of 1323 AMA positive cases were discovered for the first time. Among them, 78.0% were women, and the age of initial diagnosis was 56.8 ± 16.0 years. The first three initially diagnosed departments were rheumatology and immunology (37.4%), liver Disease (15.9%) and hematology (15.9%) relevant to musculoskeletal and connective tissue diseases (45.2%), hematology and hematopoietic organs and immune diseases (30.6%) and circulatory system diseases (29.7%). There were 297 newly confirmed cases of primary biliary cholangitis (PBC); accounting for 89.2% of women, and the age of initial diagnosis was 60.1 ± 12.4 years. The top three departments of initially diagnosed as PBC were liver disease (37.7%), rheumatology (33.0%) and gastroenterology (15.2%), of which 39.7% had musculoskeletal and connective tissue diseases, 27.9% had circulatory diseases, and 24.9 % were combined with endocrine and metabolic diseases. Conclusion: Besides PBC and other autoimmune diseases, AMA and / or AMA-M2 positivity can be observed in a variety of diseases in several clinical departments, and its clinical significance remains to be further clarified.


Assuntos
Autoanticorpos/sangue , Colangite/diagnóstico , Cirrose Hepática Biliar/diagnóstico , Adulto , Idoso , Doenças Autoimunes , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Mitocôndrias/imunologia
7.
Adv Exp Med Biol ; 1172: 63-78, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31628651

RESUMO

The co-stimulation and co-inhibition signal pathways, immune checkpoints, are among the central mechanisms to regulate the T-cell immunity. Optimal signals involve intricate interactions of numerous ligands and receptors. Manipulation of these signals offers great clinical opportunities and has revolutionized the cancer treatment therapies. The 2018 Nobel Prize in Physiology or Medicine was awarded to James P. Allison and Tasuku Honjo in recognition of their discovery of cancer immunotherapy by inhibition of immune checkpoint molecules. Despite the landmark discovery in cancer immunotherapy, the efforts to harness immunity against cancer are also restricted by the limited knowledge on the co-stimulation and co-inhibition signaling networks. Understanding the structures of these molecules, in particular, tackling the interaction paradigms from the structural perspective, help to provide more accurate insights into the signaling mechanisms, which may further facilitate the development of novel biologics and improve the efficacy of the existing biologics against these targets. Here we review our current understanding on the structures of these co-stimulatory and co-inhibitory molecules. Specifically, we focus on the structural basis of several checkpoint molecules among the CD28-B7 family and discuss the therapeutic drugs against these targets for the treatment of human cancers, autoimmune disorders, and transplantation.


Assuntos
Antígenos CD28 , Linfócitos T , Doenças Autoimunes , Antígenos CD28/química , Antígenos CD28/imunologia , Humanos , Imunoterapia , Neoplasias/terapia , Transplante de Órgãos , Transdução de Sinais/imunologia , Linfócitos T/imunologia
8.
Adv Exp Med Biol ; 1172: 79-96, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31628652

RESUMO

The Interleukin (IL)-10 cytokine family includes IL-10, IL-19, IL-20, IL-22, IL-24, and IL-26, which are considered as Class 2α-helical cytokines. IL-10 is the most important cytokine in suppressing pro-inflammatory responses in all kinds of autoimmune diseases and limiting excessive immune responses. Due to protein structure homology and shared usage of receptor complexes as well as downstream signaling pathway, other IL-10 family cytokines also show indispensable functions in immune regulation, tissue homeostasis, and host defense. In this review, we focus on immune functions and structures of different cytokines in this family and try to better understand how their molecular mechanisms connect to their biological functions. The molecular details regarding their actions also provide useful information in developing candidate immune therapy reagents for a variety of diseases.


Assuntos
Interleucina-10 , Doenças Autoimunes/imunologia , Humanos , Imunoterapia , Interleucina-10/química , Interleucina-10/imunologia , Transdução de Sinais/imunologia , Relação Estrutura-Atividade
9.
Adv Exp Med Biol ; 1172: 97-117, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31628653

RESUMO

The IL-17 family in humans consists of six distinct cytokines (IL-17A-F) that can interact with five IL-17 receptors (IL-17RA-E). The interaction between these cytokines and their receptors are critical in mediating host defenses while also making major contributions to inflammatory and autoimmune responses as demonstrated through both in vitro and in vivo experiments as well as human clinical trials. Inhibition of the IL-17A/IL-17RA interaction by monoclonal antibodies has also displayed remarkable efficacies in clinical trials against psoriasis and other autoimmune diseases. Recently, we and others reported the identification and characterization of both small-molecule and peptide IL-17A antagonists. These non-antibody IL-17A antagonists can effectively and selectively disrupt the IL-17A/IL-17RA complex and may provide alternative modalities to treat IL-17-related autoimmune and inflammatory diseases. This chapter summarizes the reported crystal structures of the IL-17 cytokines, their complexes with IL-17RA, and their complexes with both monoclonal antibodies as well as small-molecule and peptide antagonists.


Assuntos
Interleucina-17 , Receptores de Interleucina-17 , Animais , Anticorpos Monoclonais/química , Anticorpos Monoclonais/metabolismo , Doenças Autoimunes/imunologia , Cristalização , Humanos , Interleucina-17/antagonistas & inibidores , Interleucina-17/química , Interleucina-17/imunologia , Receptores de Interleucina-17/antagonistas & inibidores , Receptores de Interleucina-17/química , Receptores de Interleucina-17/imunologia
10.
Postepy Biochem ; 65(3): 217-223, 2019 10 01.
Artigo em Polonês | MEDLINE | ID: mdl-31643169

RESUMO

Transposable elements (TEs) are the sequences that are able to "jump" across the genome. They are found in virtually all organisms including human. Although in human, the majority of TEs lost their ability to autonomous transposition, they make up almost half of our genome, and played important roles in genome evolution. Fast progress in deep sequencing and functional analysis has revealed the importance of domes­ticated copies of transposable elements, including their regulatory sequences, transcripts and proteins in normal cells functioning. However, a growing numer of evidence suggest the involvment of TEs in development and progression of autoimmune and neurodegenerative disaeses as well as in many types of cancer. In this review we summarize the current state of knowledge about the LTR retroelements: endogenous retroviruses (ERVs) and Ty3/Gypsy retrotransposons, and their role in human organism.


Assuntos
Genoma Humano/genética , Retroelementos/genética , Doenças Autoimunes/genética , Retrovirus Endógenos/genética , Evolução Molecular , Humanos , Neoplasias/genética , Doenças Neurodegenerativas/genética , Sequências Repetidas Terminais/genética
11.
An Bras Dermatol ; 94(4): 399-404, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31644610

RESUMO

BACKGROUND: The Autoimmune Bullous Disease Quality of Life (ABQOL) and the Treatment of Autoimmune Bullous Disease Quality of Life (TABQOL) questionnaires proved to be reliable tools that measure the disease and treatment burden. OBJECTIVES: We aimed to assess the ABQOL and TABQOL in the Arabic population. METHODS: The English questionnaires were translated into the Arabic language by a certified translation agency. Eighty autoimmune bullous disease (AIBD) patients were included in this study. Patients were asked to answer 2 questionnaires. After 1 week the same patients were asked to answer the same questionnaires again. RESULTS: The age of the patients ranged from 19 to 81 years (mean=46), 19 males, 61 females. The ABQOL ranged from 0-37 (mean=16.4±9.2). The TABQOL ranged from 2-43 (mean=21.5±9.4). Test-retest reliability was acceptable, Cronbach's alpha was 0.76 for ABQOL and 0.74 for TABQOL. There was no significant correlation between the age of the patients and ABQOL, r =-0.2, p value was 0.183. There was a significant negative correlation between the age of the patients and the TABQOL, r=-0.2, p value was 0.039. There was a significant negative correlation between the education of the patients and the TABQOL, r=-0.3, p value was 0.007. STUDY LIMITATIONS: Small sample size of some AIBDs and patients with severe disease. CONCLUSION: Objective and valuable measurements such as ABQOL and TABQOL are now available to help physicians understand their patient's distress and should be used in every patient with AIBD. Younger and less educated patients appear to have more effects on their QOL from the treatments.


Assuntos
Doenças Autoimunes/fisiopatologia , Doenças Autoimunes/terapia , Qualidade de Vida , Dermatopatias Vesiculobolhosas/fisiopatologia , Dermatopatias Vesiculobolhosas/terapia , Inquéritos e Questionários/normas , Adulto , Idoso , Idoso de 80 Anos ou mais , Estudos Transversais , Egito , Feminino , Humanos , Linguagem , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Reprodutibilidade dos Testes , Índice de Gravidade de Doença , Dermatopatias Vesiculobolhosas/imunologia , Fatores de Tempo , Resultado do Tratamento , Tunísia , Adulto Jovem
12.
Mol Biol (Mosk) ; 53(5): 849-859, 2019.
Artigo em Russo | MEDLINE | ID: mdl-31661483

RESUMO

T cells play a key role in adaptive immunity reactions, recognizing antigens using variable TCRs. Functional TCR subunit genes are formed by somatic rearrangement, and some of the resulting TCRs recognize autoantigens, the body's own molecules. The autoreactive T cells that carry such TCRs pose a threat of inducing immune reactions against their own organism. In the course of the immune system's development, some autoreactive T lymphocytes are eliminated by apoptosis, some differentiate into immunosuppressive regulatory T cells, which support immunological tolerance to autoantigens, and the rest fall into a non-functional state of anergy. Suppression of effector T cells by regulatory T cells is mediated by immunosuppressive cytokines and costimulatory molecules, depletion of stimulating IL-2, removal of autoreactive peptides together with MHC molecules, and in other ways. Impairment of self-tolerance leads to autoimmune diseases. However, the loss of immunological tolerance can be employed in tumor treatment, allowing immunotherapy and the use of the potential of autoreactive effector T cells. The fact that the efficacious immunotherapy of tumors is often accompanied by adverse autoimmune reactions currently seems to be the inevitable price paid by using this approach.


Assuntos
Autoantígenos/imunologia , Epitopos/imunologia , Linfócitos T/imunologia , Doenças Autoimunes/imunologia , Humanos , Imunoterapia , Neoplasias/imunologia , Neoplasias/terapia , Receptores de Antígenos de Linfócitos T/imunologia , Tolerância a Antígenos Próprios/imunologia
13.
Sichuan Da Xue Xue Bao Yi Xue Ban ; 50(4): 471-477, 2019 Jul.
Artigo em Chinês | MEDLINE | ID: mdl-31642221

RESUMO

OBJECTIVE: To determinethe diagnostic valuesand reliabilityof cardiac magnetic resonance tissue tracking (CMR-TT) derived two-dimensional(2D) and three-dimensional(3D) strains in assessing experimental autoimmunity myocarditis (EAM) in rats. METHODS: 20 Lewis rats were randomly divided into model and control groups. The animal model of autoimmune myocarditis was induced by injecting porcine cardiac myosin into the footpads of the rats.On day 35, all of the rats were examined using the 7.0T CMR cine scan. The cardiac function and global strain of the left ventricular of the rats were analyzed with specific cardiac post-processing. The rats were then sacrificed and myocardial samples were taken and stained with HE and Masson. The diagnostic values of the strain parameters were assessed by receiver operating characteristic (ROC) curves with the pathological results as diagnostic criteria.The reliability of the strain parameters were tested using interclass correlation coefficient (ICC), coefficients of variation (CV) and Bland-Altman. RESULTS: No abnormal pathological changes in myocardial cells were found in the control group. Myocarditis was successfully induced in all of the rats in the model group, showing myocardial fiber arrangement disorder, degeneration, necrosis, inflammatory cell infiltration and interstitial fibrosis. The ROC showed that 2D global strain parameters possessed higher diagnostic values than 3D strain parameters. The 2D had an area under the curve (AUC) of 0.96 in global circumferential strain (GCS), 0.95 in global radial strain (GRS), and 0.90 in global longitudinal strain (GLS), compared with 0.87 GCS, 0.85 GRS, and 0.77 GLS in the 3D, respectively.The reliability of the 2D strain parameters was high, except for inter-observer 2D GRS(ICC=0.893). The 3D strain parameters had lower reliability (ICCs:0.421-0.79) than the 2D strain parameters (ICCs:0.893-0.986). CONCLUSION: The diagnostic values of 2D strain parameters are higher than 3D strain parameters in diagnosing myocarditis.


Assuntos
Doenças Autoimunes/diagnóstico por imagem , Imagem Cinética por Ressonância Magnética , Miocardite/diagnóstico por imagem , Animais , Modelos Animais de Doenças , Distribuição Aleatória , Ratos , Ratos Endogâmicos Lew , Reprodutibilidade dos Testes , Suínos , Função Ventricular Esquerda
15.
Sheng Wu Yi Xue Gong Cheng Xue Za Zhi ; 36(5): 755-762, 2019 Oct 25.
Artigo em Chinês | MEDLINE | ID: mdl-31631623

RESUMO

Autoimmune pancreatitis (AIP) is a unique subtype of chronic pancreatitis, which shares many clinical presentations with pancreatic ductal adenocarcinoma (PDA). The misdiagnosis of AIP often leads to unnecessary pancreatic resection. 18F-FDG positron emission tomography/ computed tomography (PET/CT) could provide comprehensive information on the morphology, density, and functional metabolism of the pancreas at the same time. It has been proved to be a promising modality for noninvasive differentiation between AIP and PDA. However, there is a lack of clinical analysis of PET/CT image texture features. Difficulty still remains in differentiating AIP and PDA based on commonly used diagnostic methods. Therefore, this paper studied the differentiation of AIP and PDA based on multi-modality texture features. We utilized multiple feature extraction algorithms to extract the texture features from CT and PET images at first. Then, the Fisher criterion and sequence forward floating selection algorithm (SFFS) combined with support vector machine (SVM) was employed to select the optimal multi-modality feature subset. Finally, the SVM classifier was used to differentiate AIP from PDA. The results prove that texture analysis of lesions helps to achieve accurate differentiation of AIP and PDA.


Assuntos
Adenocarcinoma/diagnóstico por imagem , Doenças Autoimunes/diagnóstico por imagem , Neoplasias Pancreáticas/diagnóstico por imagem , Pancreatite/diagnóstico por imagem , Algoritmos , Diagnóstico Diferencial , Fluordesoxiglucose F18 , Humanos , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons , Máquina de Vetores de Suporte
16.
Sheng Li Xue Bao ; 71(5): 769-782, 2019 Oct 25.
Artigo em Chinês | MEDLINE | ID: mdl-31646331

RESUMO

Autoimmune diseases are a kind of chronic diseases with unclear etiology, which has the characteristics of repetition and difficulty to cure completely. Aerobic exercise, as an effective intervention method for chronic diseases, has also received extensive attention in the field of the prevention and treatment of autoimmune diseases. In this paper, the effects of aerobic exercise on immune system and autoimmune diseases in recent years are reviewed, and the related mechanisms are discussed. It is pointed out that aerobic exercise can improve the homeostasis of immune environment by affecting the number and function of immune cells, inhibit the systemic inflammatory response of the body, and then delay the occurrence and development of autoimmune diseases.


Assuntos
Doenças Autoimunes/prevenção & controle , Exercício , Sistema Imunitário , Homeostase , Humanos
17.
Zhonghua Jie He He Hu Xi Za Zhi ; 42(9): 700-704, 2019 Sep 12.
Artigo em Chinês | MEDLINE | ID: mdl-31484245

RESUMO

Objective: To explore the effect of pirfenidone in fibrotic interstitial pneumonia with autoimmune features (IPAF) after treatment with corticosteroids and immunosuppressants. Methods: We conducted a retrospective analysis of 2 adult patients with IPAF in the Peking Union Medical College Hospital. As their fibrotic interstitial lung disease failed to improve with further treatment with corticosteroids and immunosuppressants, they were treated with pirfenidone based on corticosteroids and immunosuppressants. Their clinical, chest radiological data and prognosis were collected and relevant literatures were reviewed. Results: One patient was a 43 year old female, the other was a 53 year old male. IPAF was diagnosed with their classic clinical, serological and radiological features. They were partially responded to corticosteroids and immunosuppressants at the initial period. Pirfenidone was suggested for them as their lung fibrosis was not improved further with immunosuppressive therapy. After 4-5 months treatment with pirfenidone, based on corticosteroids and immunosuppressant administration, their clinical and radiological manifestations improved significantly. Conclusions: Pirfenidone might be a good add-on choice for fibrotic IPAF when the disease did not respond well to corticosteroids and immunosuppressants.


Assuntos
Anti-Inflamatórios não Esteroides/uso terapêutico , Doenças Autoimunes/diagnóstico , Fibrose Pulmonar Idiopática/tratamento farmacológico , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/tratamento farmacológico , Piridonas/uso terapêutico , Corticosteroides/uso terapêutico , Adulto , Feminino , Humanos , Fibrose Pulmonar Idiopática/diagnóstico , Imunossupressores/uso terapêutico , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Resultado do Tratamento
18.
Brain Nerve ; 71(9): 1003-1012, 2019 Sep.
Artigo em Japonês | MEDLINE | ID: mdl-31506402

RESUMO

We present a case of a 73-year-old female who developed subacute memory disturbance, reduced consciousness and quadriparesis following pernicious anemia. Brain magnetic resonance imagings (MRI) in diffusion weighted, T2 weighted and fluid attenuated inversion recovery (FLAIR) images revealed hyperintensities in bilateral frontal, parietal, temporal and occipital cortices, left thalamus, bilateral splenium of corpus callosum, and bilateral subcortical white matters. Brain gadolinium enhanced T1 weighted MRI revealed very slight post-contrast enhancement lesions in the right posterior temporal region and bilateral parietal regions. Serum was negative for anti- aquaporin (AQP) 4 antibody, anti-glutamic acid decarboxylase (GAD) antibody and anti-voltage-gated potassium channel (VGKC) antibody, and cerebrospinal fluid (CSF) was negative for anti- N-methyl-D-aspartate (NMDA) receptor antibody. CSF analysis showed slight protein elevation with normal cellular content. No evidence of neoplasm was observed using whole-body 18 F -fluorodeoxyglucose- positron emission tomography/computed tomography. Pathological findings of the left frontal lesion revealed perivascular and scattered parechymal T-lymphocytic infiltration, and astrogliosis without vascular hyalinization. Patient achieved partial recovery during two intraveneous pulse methylprednisolone treatments, and exacerbation afterwards. After the third intraveneous pulse methylprednisolone treatment, remission is sustained for six years. This case can be regarded as autoantibody-negative but probable autoimmune encephalitis with the features of nonparaneoplastic panencephalitis and treatable dementia. Nonparaneoplastic autoimmune panencephalitis with widespread multifocal brain lesions on brain MRI is extremely rare, with exception of anti- NMDA receptor antibody encephalitis.


Assuntos
Anemia Perniciosa/complicações , Doenças Autoimunes/etiologia , Demência/etiologia , Encefalite/etiologia , Idoso , Doenças Autoimunes/tratamento farmacológico , Encéfalo/diagnóstico por imagem , Encéfalo/patologia , Demência/tratamento farmacológico , Encefalite/tratamento farmacológico , Feminino , Seguimentos , Humanos , Imagem por Ressonância Magnética
19.
Isr Med Assoc J ; 21(7): 480-486, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31507125

RESUMO

BACKGROUND: Serum rheumatoid factors are autoantibodies of different isotypes directed against the Fc fraction of immunoglobulin G (IgG) and represent paradigmatic autoantibodies that have been largely used in clinical practice for decades. Traditionally IgG has been associated with rheumatoid arthritis and more recently included also in the classification criteria for SjÓ§gren's syndrome. Researchers have established that rheumatoid factors are positive in a variety of infectious, autoimmune, and neoplastic disorders, thus requiring a comprehensive evaluation of seropositive patients. Of note, hepatitis B and C viruses represent a crossroad that includes the high rheumatoid factor seroprevalence and chronic inflammatory disease, as well as progression to non-Hodgkin's lymphomas. Chronic antigen stimulation is the likely common ground of these processes and rheumatoid factors may represent mere bystanders or drivers of pathology. Mixed cryoglobulinemia and lymphoproliferative disease are prime examples of the deleterious effects of rheumatoid factor-B cell activity, possibly associated with hepatitis B and C. More importantly, they show a clear association in a physiological host response to infection, chronic inflammation, and the slide toward autoimmunity and malignancy. The association between hepatitis B and C infections and the appearance of serum rheumatoid factors is further supported by prevalence data, which support a coexistence of these markers in a significant proportion of cases, with viral infections being frequent causes of rheumatoid factors in patients without a rheumatic condition. We provide a comprehensive overview of the known connections between hepatitis B and C infections and rheumatoid factors.


Assuntos
Hepatite B/imunologia , Hepatite C/imunologia , Fator Reumatoide/imunologia , Autoanticorpos/imunologia , Doenças Autoimunes/imunologia , Autoimunidade/imunologia , Crioglobulinemia/imunologia , Humanos , Transtornos Linfoproliferativos/imunologia , Neoplasias/imunologia , Fator Reumatoide/sangue
20.
Isr Med Assoc J ; 21(7): 487-490, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31507126

RESUMO

BACKGROUND: Recurrent pericarditis is a state of repetitive inflammation of the pericardium with intervals of remission. The etiology of recurrent pericarditis is still largely unknown, yet most causes are presumed to be immune mediated. Genetic factors, including human leukocyte antigen (HLA) haplotypes, can be involved in dysregulation of the immune system and as a predisposition to several autoimmune conditions, including recurrent pericarditis. Several diseases are frequently associated with such manifestations. They include systemic lupus erythematosus, familial Mediterranean fever, and tumor necrosis factor receptor-associated periodic syndrome. However, idiopathic recurrent pericarditis remains the most frequently observed clinical condition and the conundrum of this disease still needs to be solved.


Assuntos
Doenças Autoimunes/genética , Predisposição Genética para Doença , Pericardite/fisiopatologia , Febre Familiar do Mediterrâneo/genética , Febre Familiar do Mediterrâneo/imunologia , Antígenos HLA/genética , Haplótipos , Humanos , Lúpus Eritematoso Sistêmico/genética , Lúpus Eritematoso Sistêmico/imunologia , Pericardite/genética , Pericardite/imunologia , Recidiva
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