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1.
Orphanet J Rare Dis ; 18(1): 24, 2023 Feb 01.
Artigo em Inglês | MEDLINE | ID: mdl-36726161

RESUMO

BACKGROUND: Autoantibodies against C1-inhibitor (C1-INH-Ab) have a diagnostic value in acquired angioedema due to C1-inhibitor deficiency (C1-INH-AAE), even though antibodies can circulate in complexes, which can be undetectable by proven methods. Our aim was to measure C1-INH/C1-INH-Ab complexes (CAC) and investigate their connection to C1-INH-Ab and the changes in their titer over time. RESULTS: 19 patients were diagnosed with C1-INH-AAE in the Hungarian Angioedema Center of Reference and Excellence; 79% of them had an underlying disease. Samples were examined with a newly developed in-house complex ELISA method. Patients with high C1-INH-Ab titer had a CAC titer which did not exceed the normal level and the ones with high CAC titer had a C1-INH-Ab titer which did not exceed the normal level. In case of those patients who had C1-INH-Ab and CAC of the same type of immunoglobulin, the increasing titer of C1-INH-Ab went together with the decreasing level of CAC and vice versa. CAC titer was already increased before the diagnosis of the underlying disease. CONCLUSIONS: Free circulating and complex antibodies are in a dynamically changing equilibrium. CAC measurements can help to predict the development of an underlying disease. The efficiency of the treatment for underlying disease can be monitored by the decreasing CAC titers. Our results show that the CAC can be of important additional information besides the complement panel examination in case of C1-INH-AAE. Measurement of CAC is recommended to be done parallelly with C1-INH-Ab, so as to detect both free and bound antibodies.


Assuntos
Angioedema , Angioedemas Hereditários , Humanos , Angioedema/diagnóstico , Angioedemas Hereditários/diagnóstico , Proteína Inibidora do Complemento C1 , Autoanticorpos , Ensaio de Imunoadsorção Enzimática
2.
Med Sci Monit ; 29: e938979, 2023 Jan 20.
Artigo em Inglês | MEDLINE | ID: mdl-36659834

RESUMO

BACKGROUND Bone marrow stem cells have been shown to be a promising therapeutic strategy for autoimmune diseases. This study aimed to assess the safety and efficacy of autologous hematopoietic stem cell (ABMSC) transplantation without immunoablation used to suppress the autoimmune reaction in 6 children with newly diagnosed autoimmune diabetes mellitus. We monitored the levels of islet cell antibodies (ICA), antibodies against islet antigen-related tyrosine phosphatase 2 (IA2), glutamic acid-decarboxylase (GAD) antibodies, and anti-insulin antibodies (AIA). MATERIAL AND METHODS Between 2018 and 2022, 6 children (age 6-10 years, average 8 years) recently diagnosed with type 1 diabetes mellitus with the presence of ICA, IA2, GAD, AIA and ketoacidosis, were treated with an ABMSC stimulated with Filgrastim, granulocyte colony-stimulating factor (G-CSF), 10 ug/kg/day for 4 days. Bone marrow was harvested on day 5, collected by puncture and identified as mononuclear cells >180×106/kg, CD34+ >0.22%, and transplanted by intravenous (i.v.) infusion. Patients were monitored with ICA, IA2, GAD, AIA, C-peptide, blood glucose, and glycosylated hemoglobin A1c (HbA1C) 6 months after the procedure. RESULTS At 6-month follow-up, we observed a negative value of the ICA, which was previously positive (P<0.001). The IA2 (p=0.037) and GAD (P=0.377) antibodies decreased slowly but were significantly lower. AIA remained high. A decrease in blood glucose and HbA1C levels was observed (P<0.001). No complications occurred during follow-up. CONCLUSIONS Autologous hematopoietic stem cell transplantation without immunoablation was safe and effective in significantly decreasing the production and effect of autoantibodies against ICA, GAD, and IA2, as well as decreasing blood sugar levels and HbA1c.


Assuntos
Diabetes Mellitus Tipo 1 , Transplante de Células-Tronco Hematopoéticas , Ilhotas Pancreáticas , Humanos , Criança , Autoanticorpos , Glicemia , Glutamato Descarboxilase
3.
Chest ; 163(1): e1-e5, 2023 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-36628678

RESUMO

Melanoma differentiation-associated gene 5 (MDA5) positive dermatomyositis is a rare systemic autoimmune disease that is associated with life-threatening rapidly progressive interstitial lung disease. We report the case of a 19-year-old male patient with a life-threatening disease course caused by rapidly progressive interstitial lung disease that caused respiratory failure despite intensive immunosuppression with multiple agents (steroids, IV immunoglobulins, tofacitinib, cyclophosphamide, mycophenolate mofetil, ciclosporin and rituximab). Rescue therapy with daratumumab, an anti-CD38-antibody, was initiated. Significant pulmonary improvement was noticed after 4 weekly injections of 1,800 mg. After 6 months of follow up, stable disease remission with significant pulmonary improvement and persistent depletion of CD38+ plasma cells and MDA5-antibody titers were seen. This is the first report of the successful use of daratumumab in dermatomyositis. It highlights the potential of CD38 targeted therapies for severe antibody-mediated autoimmune diseases such as dermatomyositis.


Assuntos
Dermatomiosite , Doenças Pulmonares Intersticiais , Humanos , Masculino , Adulto Jovem , Autoanticorpos , Ciclofosfamida , Dermatomiosite/tratamento farmacológico , Dermatomiosite/complicações , Helicase IFIH1 Induzida por Interferon , Doenças Pulmonares Intersticiais/etiologia
4.
Clin Appl Thromb Hemost ; 29: 10760296221151165, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-36653966

RESUMO

INTRODUCTION: Immunosuppressive therapy (IST) for acquired hemophilia A (AHA) results in remission within days to months in 60% to 80% of patients. However, little is known regarding the predictors of response. AIM: This study aimed to identify the factors that influence response to treatment. METHODS: The data of 42 patients with AHA from three hospitals were retrospectively analyzed. RESULTS: All 42 AHA patients received IST; complete treatment data were available for 34 patients. The response rate was 60% among the 5/34 (14.7%) patients who received steroids alone, 70.8% among the 24/34 (70.6%) patients who received steroids plus cyclophosphamide, and 80% among the 5/34 (14.7%) patients who received steroids plus cyclophosphamide and rituximab. Overall, 29/34 (85.3%) patients achieved CR; 4/34 (13.8%) of them relapsed after a median time of 410 (21-1279) days. Adverse events occurred in 14/34 (41.2%) patients: 13/34 (38.2%) had infections and 1/34 (2.9%) developed pancytopenia. In univariate and multivariate Cox regression analyses, FVIII inhibitor titer ≥20 BU/mL was the only significant prognostic factor affecting time to CR. No variable had significant effect on OS. CONCLUSION: FVIII inhibitory antibody titer ≥20 BU/mL appears to be an important predictor of time to complete response in patients with acquired hemophilia A treated with immunosuppressive therapy.


Assuntos
Hemofilia A , Humanos , Hemofilia A/terapia , Imunossupressores/uso terapêutico , Estudos Retrospectivos , Fator VIII/uso terapêutico , Ciclofosfamida/uso terapêutico , Esteroides/uso terapêutico , Autoanticorpos
5.
J Neurol Sci ; 445: 120540, 2023 Feb 15.
Artigo em Inglês | MEDLINE | ID: mdl-36608627

RESUMO

BACKGROUND: Antibodies against glutamic acid decarboxylase (GAD-abs) at high serum levels are associated with diverse autoimmune neurological syndromes (AINS), including cerebellar ataxia, epilepsy, limbic encephalitis and stiff-person syndrome. The impact of low serum GAD-ab levels in patients with suspected AINS remains controversial. Specific intrathecal GAD-ab synthesis may serve as a marker for GAD-ab-associated nervous system autoimmunity. We present characteristics of a multicentric patient cohort with suspected AINS associated with GAD antibodies (SAINS-GAD+) and explore the relevance of serum GAD-ab levels and intrathecal GAD-ab synthesis. METHODS: All patients with SAINS-GAD+ included in the registry of the German Network for Research on Autoimmune Encephalitis (GENERATE) from 2011 to 2019 were analyzed. High serum GAD-ab levels were defined as RIA>2000 U/mL, ELISA>1000 U/mL, or as a positive staining pattern on cell-based assays. RESULTS: One-hundred-one patients were analyzed. In descending order they presented with epilepsy/limbic encephalitis (39%), cerebellar ataxia (28%), stiff person syndrome (22%), and overlap syndrome (12%). Immunotherapy was administered in 89% of cases with improvements in 46%. 35% of SAINS-GAD+ patients had low GAD-ab serum levels. Notably, unmatched oligoclonal bands in CSF but not in serum were more frequent in patients with low GAD-ab serum levels. GAD-ab-levels (high/low) and intrathecal GAD-ab synthesis (present or not) did not impact clinical characteristics and outcome. CONCLUSIONS: Overall, immunotherapy in SAINS-GAD+ was moderately effective. Serum GAD-ab levels and the absence or presence of intrathecal GAD-ab synthesis did not predict clinical characteristics or outcomes in SAINS-GAD+. The detection of unmatched oligoclonal bands might outweigh low GAD-ab serum levels.


Assuntos
Ataxia Cerebelar , Encefalite Límbica , Rigidez Muscular Espasmódica , Humanos , Ataxia Cerebelar/tratamento farmacológico , Glutamato Descarboxilase , Autoanticorpos , Bandas Oligoclonais , Encefalite Límbica/terapia , Rigidez Muscular Espasmódica/terapia
7.
Med. clín (Ed. impr.) ; 160(1): 10-16, enero 2023. tab, graf
Artigo em Inglês | IBECS | ID: ibc-213902

RESUMO

Objectives: Idiopathic inflammatory myopathies (IIMs) are systemic, heterogeneous diseases, which mainly affect skeletal muscle. Myositis with cancer is often referred to as cancer-associated myositis (CAM), which is associated with poor prognosis. This study aimed to determine the cancer associated myositis-specific autoantibodies (MSAs) and to elucidate their associations with clinical features in Chinese patients with IIMs.MethodsThis retrospective study enrolled 312 patients with IIMs who were treated at Tianjin Medical University General Hospital, China, from January 2015 to December 2020. Clinical data were collected. Serum MSAs, including anti-Mi-2, anti-TIF1-γ, anti-NXP2, anti-SAE, anti-MDA5, anti-SRP, anti-Jo-1, anti-PL-7, anti-PL-12, anti-OJ, anti-EJ and anti-HMGCR antibodies were detected. Cancer-associated MSAs, their phenotypic and survival features were estimated through SPSS 20.0.ResultsThe results revealed that anti-TIF1-γ antibody and anti-SAE antibody were cancer-associated autoantibodies with odds ratios (95% CI) of 8.70 (3.35–22.64) and 22.31 (4.32–115.05), respectively. Skin lesions, proximal weakness, dysphagia and dysarthria were observed more frequently in patients carrying anti-TIF1-γ antibody. By contrast, patients with anti-TIF1-γ antibody had a lower frequencies of fever, arthritis/arthralgia and interstitial lung disease (ILD). Anti-TIF1-γ antibody positive CAM comprised about half of CAM entities and had the characteristic of close temporal association with cancer detection/recurrence. Female-dominant, common reproductive system tumors were other clinical features of this subset. Besides, patients with anti-TIF1-γ antibody positive had significantly lower survival rates than the anti-TIF1-γ antibody negative group. (AU)


Objetivo: La miopatía inflamatoria idiopática (IIM, por sus siglas en inglés) es una enfermedad sistémica y heterogénea que afecta principalmente al músculo esquelético. La miositis asociada al cáncer se denomina a menudo miositis relacionada con el cáncer, y está relacionada con un mal pronóstico. El objetivo de este estudio fue identificar autoanticuerpos específicos de miositis relacionados con el cáncer en pacientes chinos, y dilucidar su correlación con las características clínicas.MétodosEl estudio retrospectivo incluyó a 312 pacientes con IIM tratados en el hospital general de la Universidad Médica de Tianjin, China, de enero de 2015 a diciembre de 2020. Recoger datos clínicos. Se detectaron autoanticuerpos específicos de miositis sérica, incluyendo anti-Mi-2, anti-TIF1-γ, anti-NXP2, anti-SAE, anti-MDA5, anti-SRP, anti-Jo-1, anti-PL-7, anti-PL-12, anti-OJ, anti-EJ, anti-HMGCR. Los autoanticuerpos relacionados con el cáncer, sus fenotipos y sus características de supervivencia fueron evaluados por SPSS® 20.0.ResultadosLos resultados mostraron que el anticuerpo anti-TIF1-γ y el anticuerpo anti-SAE eran autoanticuerpos relacionados con el cáncer con una relación de predominio (IC 95%) de 8,70 (3,35–22,64) y 22,31 (4,32–115,05), respectivamente. La frecuencia de lesiones cutáneas, debilidad proximal, disfagia y disartria fue mayor en los pacientes portadores de anticuerpos anti-TIF1-γ. En comparación, la incidencia de fiebre, artritis/artralgia y enfermedad pulmonar intersticial (ILD) en pacientes con anticuerpos anti-TIF1-γ fue menor. La miositis relacionada con el cáncer con anticuerpos anti-TIF1-γ positivos representa aproximadamente la mitad de la miositis relacionada con el cáncer y tiene características temporales estrechamente relacionadas con la detección/recidiva del cáncer. (AU)


Assuntos
Humanos , Autoanticorpos , Miosite , Neoplasias/complicações , China/epidemiologia , Estudos Retrospectivos
8.
Med. clín (Ed. impr.) ; 160(1): 30-38, enero 2023. ilus, tab
Artigo em Espanhol | IBECS | ID: ibc-213906

RESUMO

Las anemias hemolíticas autoinmunes (AHAI) son trastornos hematológicos adquiridos ocasionados por una destrucción periférica de eritrocitos incrementada, mediada por autoanticuerpos dirigidos frente a antígenos eritrocitarios. Se clasifican según etiología en primarias y secundarias, y según el tipo de anticuerpo detectado y temperatura de reacción en AHAI por anticuerpos calientes (AHAI-C) y AHAI por anticuerpos fríos (AHAI-F).El pilar del manejo en AHAI-C continúa siendo el tratamiento con glucocorticoides, y la adición precoz de rituximab ha demostrado buenos resultados en los últimos estudios. Las AHAI-F primarias se tratan principalmente con rituximab, solo o combinado con quimioterapia.En fase de desarrollo avanzado encontramos nuevos fármacos como los inhibidores de Syk, Ig anti-FcRn e inhibidores del complemento, que permitirán ampliar el arsenal terapéutico, especialmente en casos refractarios o recidivantes. (AU)


Autoimmune haemolytic anaemias (AIHA) are acquired haematological disorders caused by increased peripheral erythrocyte destruction mediated by autoantibodies against erythrocyte antigens. They classified according to aetiology into primary and secondary, and according to the type of antibody and reaction temperature into AIHA due to warm antibodies (w-AIHA) and AIHA due to cold antibodies (c-AIHA).The mainstay of management in w-AIHA remains glucocorticoid therapy, and the early addition of rituximab has shown good results in recent studies. Primary c-AIHA is mainly treated with rituximab, alone or in combination with chemotherapy.New drugs such as Syk inhibitors, anti-FcRn Ig and complement inhibitors are in advanced development and will expand the therapeutic arsenal, especially in refractory or relapsed cases. (AU)


Assuntos
Humanos , Anemia Hemolítica Autoimune/tratamento farmacológico , Anemia Hemolítica Autoimune/terapia , Rituximab/uso terapêutico , Autoanticorpos , Temperatura
9.
Platelets ; 34(1): 2161498, 2023 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-36597279

RESUMO

The pathogenesis of thrombocytopenia in chronic hepatitis C (CHC) conceivably involves autoimmunity; however, the dynamics of autoantibodies and other autoimmune mechanisms remain unclear. In this study, we examined the changes in the frequency of anti-glycoprotein (GP) IIb/IIIa antibody-producing B cells and the levels of plasma B-cell-activating factor (BAFF), a proliferation-inducing ligand (APRIL), and interleukin (IL)-21 following treatment of CHC with direct-acting antiviral agents (DAA). We recruited 28 patients with CHC who underwent treatment with DAA for 8-12 weeks and subsequently tested negative for serum hepatitis C virus RNA. Thirty healthy controls were recruited for comparison. Platelet counts increased significantly (p = .016), and the frequency of anti-GPIIb/IIIa antibody-producing B cells decreased significantly (p = .002) in CHC patients with thrombocytopenia at the end of treatment (EOT) than before DAA treatment (baseline). However, these changes were not observed in CHC patients without thrombocytopenia. Plasma BAFF levels in CHC patients with thrombocytopenia significantly decreased from baseline to EOT (p = .002). Anti-GPIIb/IIIa antibody-producing B cells were positively correlated with plasma BAFF levels in these patients (r = 0.669, p = .039). These results suggest that DAA treatment suppresses the autoimmune response against platelets and improves thrombocytopenia.


What is the context? Production of antiplatelet antibodies is one of the mechanisms underlying thrombocytopenia in patients with chronic hepatitis C.Antiplatelet antibodies against platelet membrane glycoprotein (GP) IIb/IIIa are commonly detected in hepatitis C virus-associated immune thrombocytopenia.Hepatitis C virus elimination by direct-acting antiviral agents (DAA) improves thrombocytopenia in patients with hepatitis C; however, the dynamics of autoantibodies and other autoimmune mechanisms remain unclear.What is new? In this study, we determined whether DAA treatment can alter the autoimmune response against platelets and improve platelet count.The frequency of anti-GPIIb/IIIa antibody-producing B cells decreased significantly from the baseline following DAA treatment in chronic hepatitis C patients with thrombocytopenia.DAA treatment reduced the levels of B-cell-activating factor, a cytokine associated with autoantibody production.What is the impact? The study provides evidence that DAA treatment diminishes the autoimmune response to GPIIb/IIIa and, therefore, improves platelet counts in chronic hepatitis C patients with thrombocytopenia.


Assuntos
Anemia , Hepatite C Crônica , Trombocitopenia , Humanos , Antivirais/farmacologia , Antivirais/uso terapêutico , Hepatite C Crônica/complicações , Hepatite C Crônica/tratamento farmacológico , Trombocitopenia/tratamento farmacológico , Trombocitopenia/etiologia , Complexo Glicoproteico GPIIb-IIIa de Plaquetas , Plaquetas , Autoanticorpos
10.
BMC Neurol ; 23(1): 24, 2023 Jan 17.
Artigo em Inglês | MEDLINE | ID: mdl-36647033

RESUMO

BACKGROUND: Autonomic dysfunctions including bladder dysfunction, gastrointestinal dysfunction and orthostasis are common symptoms of autoimmune glial fibrillary acidic protein astrocytopathy (A-GFAP-A); however, cardiac autonomic dysfunction and abnormal circadian rhythm of blood pressure, which can lead to poor prognosis and even sudden cardiac death, has never been reported in A-GFAP-A patient. CASE PRESENTATION: A 68-year-old male Chinese patient presented to our hospital with headache, fever, progressive disturbance of consciousness, dysuria, and limb weakness. Abnormal heart rate variability and non-dipper circadian rhythm of blood pressure gradually developed during hospitalization, which is rare in A-GFAP-A. He had positive GFAP IgG in cerebrospinal fluid (CSF). Enhanced brian MRI showed uneven enhancement and T2 hyperintense lesions of medulla oblongata; Cervical spine MRI showed T2 hyperintense lesions in medulla oblongata and upper margin of the T2 vertebral body. A contrast-enhanced thoracic spine MRI showed uneven enhancement and T2 hyperintense lesions of T1 to T6 vertebral segments. After treatment with intravenous immunoglobulin and corticosteroids, the patient's symptoms, including autonomic dysfunction, alleviated dramatically. Finally, his heart rate variability and blood pressure variability became normal. CONCLUSIONS: Our case broadens the spectrum of expected symptoms in A-GFAP- A syndromes as it presented with heart rate variability and blood pressure variability.


Assuntos
Imunoglobulinas Intravenosas , Medula Espinal , Masculino , Humanos , Idoso , Pressão Sanguínea , Proteína Glial Fibrilar Ácida , Frequência Cardíaca , Medula Espinal/metabolismo , Autoanticorpos
11.
Kidney Int ; 103(2): 251-253, 2023 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-36681452

RESUMO

The major form of membranous nephropathy is characterized by autoantibodies to phospholipase A2 receptor 1 (PLA2R1). The study by Tomas et al. describes the first animal model where human PLA2R1 is ectopically expressed in mouse podocytes. Intriguingly, the transgenic mice spontaneously develop anti-human PLA2R1 antibodies and membranous nephropathy-like features, including immune deposits and nephrotic syndrome. The model raises questions about the spontaneous production of anti-human PLA2R1 antibodies and the additional steps to establish a bona fide animal model of membranous nephropathy.


Assuntos
Glomerulonefrite Membranosa , Síndrome Nefrótica , Podócitos , Humanos , Animais , Camundongos , Glomerulonefrite Membranosa/genética , Receptores da Fosfolipase A2/genética , Modelos Animais , Autoanticorpos
14.
Indian J Pathol Microbiol ; 66(1): 24-30, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-36656206

RESUMO

Background: IgA anti-tissue transglutaminase-2 antibody (anti-TG2Ab) deposits in intestinal and extraintestinal organs have been used to link the respective pathological changes in these organs with celiac disease (CeD). Aims: To know if parts of intestine other than the duodenum, such as esophagus, stomach, and colon, have any pathology related to potential CeD or have mucosal IgA anti-TG2 Ab deposits. Settings and Design: A prospective case-control study conducted from April 2018 to December 2019. Materials and Methods: Nine patients with potential CeD and 27 age- and gender-matched patients with irritable bowel syndrome were recruited as cases and controls, respectively. Mucosal biopsies were collected from esophagus, stomach, duodenum, and rectosigmoid regions, histological changes were evaluated, and IgA anti-TG2 Ab deposits were analyzed in these regions by two-color immunohistochemical staining. Statistics: Data were analyzed using statistical software Stata 14.0. Results: No distinct difference in mucosal lymphocytosis were identified between biopsies of patients with potential CeD and controls at the following sites: esophagus (11.1% vs 0%, P = 0.079), stomach (14.3% vs 7.7%, P = 0.590), and rectum (20% vs 0%, P = 0.067). Co-localized IgA anti-TG2Ab deposits were observed more in potential CeD than in controls at esophagus 22.2% (2/9) vs 0%, P = 0.012; stomach 66.7% (6/9) vs 11.5% (3/26), P < 0.001; and duodenum 66.7% (6/9) vs 0%, P < 0.001 but not at rectum 0% (0/4) vs 0% (0/25). Conclusion: Although histological changes are not distinct, a subset of subjects with potential CeD has pan-intestinal involvement other than in the duodenum.


Assuntos
Doença Celíaca , Humanos , Estudos de Casos e Controles , Transglutaminases , Mucosa Intestinal/patologia , Intestino Delgado/patologia , Biópsia , Imunoglobulina A , Autoanticorpos
15.
Nat Commun ; 14(1): 319, 2023 Jan 19.
Artigo em Inglês | MEDLINE | ID: mdl-36658110

RESUMO

The immune mechanisms that mediate synovitis and joint destruction in rheumatoid arthritis (RA) remain poorly defined. Although increased levels of CD8+ T cells have been described in RA, their function in pathogenesis remains unclear. Here we perform single cell transcriptome and T cell receptor (TCR) sequencing of CD8+ T cells derived from anti-citrullinated protein antibodies (ACPA)+ RA blood. We identify GZMB+CD8+ subpopulations containing large clonal lineage expansions that express cytotoxic and tissue homing transcriptional programs, while a GZMK+CD8+ memory subpopulation comprises smaller clonal expansions that express effector T cell transcriptional programs. We demonstrate RA citrullinated autoantigens presented by MHC class I activate RA blood-derived GZMB+CD8+ T cells to expand, express cytotoxic mediators, and mediate killing of target cells. We also demonstrate that these clonally expanded GZMB+CD8+ cells are present in RA synovium. These findings suggest that cytotoxic CD8+ T cells targeting citrullinated antigens contribute to synovitis and joint tissue destruction in ACPA+ RA.


Assuntos
Artrite Reumatoide , Sinovite , Humanos , Linfócitos T CD8-Positivos/metabolismo , Membrana Sinovial/metabolismo , Receptores de Antígenos de Linfócitos T , Autoantígenos , Autoanticorpos
16.
J Med Case Rep ; 17(1): 21, 2023 Jan 22.
Artigo em Inglês | MEDLINE | ID: mdl-36681831

RESUMO

BACKGROUND: Neuromyelitis optica is a relapsing-remitting disease characterized by a recurrent attack of optic neuritis and transverse myelitis; sometimes associated with acute brainstem syndrome. Systemic lupus erythematosus is an autoimmune multisystem disorder in which ocular involvement such as acute ischemic optic neuropathy is a rare manifestation. However, neuromyelitis optica can be associated with systemic lupus erythematosus. CASE PRESENTATION: A 24-year-old Bangladeshi woman was admitted to the hospital with complaints of sudden, progressive, painless vision loss in both eyes, and progressive weakness in both lower limbs for 48 hours. She also gave a history of arthralgia, a photosensitive skin rash, intermittent fever, oral ulcerations, and alopecia for the last 2 months. On examination, the fundus was suggestive of bilateral acute ischemic neuropathy, and examinations of the lower limb revealed spastic paraparesis with sensory abnormality. Laboratory investigations revealed the presence of positive anti-aquaporin 4 antibody, strongly positive antinuclear antibody, and anti-ds DNA with the longitudinally extensive lesion on magnetic resonance imaging of the spinal cord. She was treated with methylprednisolone, hydroxychloroquine, and mycophenolate, and was discharged with improvement of her paraparesis. However, her vision did not improve substantially. CONCLUSION: The importance of this report is to shed some light on the occurrence of two devastating complications that is, bilateral acute ischemic optic neuropathy in systemic lupus erythematosus complicated by neuromyelitis optica, as well as evidence of rare presentations for systemic lupus erythematosus and treatment modalities of ischemic optic neuropathy with systemic lupus erythematosus.


Assuntos
Doenças Autoimunes , Lúpus Eritematoso Sistêmico , Neuromielite Óptica , Neuropatia Óptica Isquêmica , Feminino , Humanos , Adulto Jovem , Adulto , Neuromielite Óptica/complicações , Neuromielite Óptica/tratamento farmacológico , Neuropatia Óptica Isquêmica/etiologia , Neuropatia Óptica Isquêmica/complicações , Autoanticorpos , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Metilprednisolona/uso terapêutico
17.
PLoS One ; 18(1): e0280906, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-36693083

RESUMO

BACKGROUND: Hereditary hypothyroidism represents a concern for dog breeders; thus, surveillance programs have been established for several dog breeds. METHODS: Thyroid profiles (total thyroxine (TT4), thyrotropin (thyroid stimulating hormone (TSH)), and thyroglobulin autoantibodies (TgAA)) collected as part of a breed surveillance program in Eurasians (2009-2017) were retrospectively analyzed. The study included data from 1,501 Eurasians from a German breeding club. Classification was exclusively based on laboratory data. Hypothyroidism was defined as a combined decrease in TT4 and increase in TSH in serum and was classified as TgAA-positive and TgAA-negative hypothyroidism. Thyroglobulin autoantibodies (TgAA) independent of the concentrations of TT4 and TSH were determined. The overall prevalence of hypothyroidism, TgAA-positive hypothyroidism, TgAA-negative hypothyroidism and TgAA-positivity was assessed when the dogs entered the program. Follow-up laboratory data was available for 324 dogs without hypothyroidism on initial examination. RESULTS: The initial screening was performed at a median age of 18 months (interquartile range (IQR): 15-29). The overall prevalence of hypothyroidism was 3.9% (n = 58; 95% CI: 2.9-4.8%) and the prevalence of a positive TgAA status was 7.9% (n = 118; 95% CI: 6.6-9.3%). The prevalence of TgAA-positive and TgAA-negative hypothyroidism was 1.7% (n = 26; 95% CI: 1.1-2.4%) and 2.1% (n = 32; 95% CI: 1.4-2.9%), respectively. 22.0% of dogs with positive TgAA status (26/118) were already hypothyroid on initial examination. Overall, 42.5% (17/40) of TgAA-positive dogs on initial examination developed hypothyroidism on follow-up. CONCLUSION: The results of this study demonstrate that the Eurasian dog breed exhibits a relevant risk for hypothyroidism and presence of TgAA. The predictive value of TgAA for hypothyroidism or developing hypothyroidism was high in this breed. Further investigations with longitudinal studies in individual dogs are warranted.


Assuntos
Doenças do Cão , Hipotireoidismo , Animais , Cães , Tireoglobulina , Autoanticorpos , Estudos Retrospectivos , Hipotireoidismo/epidemiologia , Hipotireoidismo/veterinária , Tiroxina , Tireotropina
20.
Zhongguo Dang Dai Er Ke Za Zhi ; 25(1): 67-72, 2023 Jan 15.
Artigo em Chinês | MEDLINE | ID: mdl-36655666

RESUMO

OBJECTIVES: To study the clinical features of children with autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A). METHODS: A retrospective analysis was performed on the medical data of 34 children with GFAP-A who attended the Department of Neurology, Children's Hospital of Chongqing Medical University, from January 2020 to February 2022. The medical data included clinical manifestations, cerebrospinal fluid features, imaging examination results, treatment, and prognosis. RESULTS: The median age of onset was 8.4 (range 1.9-14.9) years for the 34 children with GFAP-A. The main clinical manifestations included headache (50%, 17/34), fever (47%, 16/34), visual impairment (47%, 16/34), and disturbance of consciousness (44%, 15/34). Abnormal cerebrospinal fluid results were observed in 19 children (56%, 19/34), among whom 8 children had positive autoantibody. The children with overlap syndrome had significantly higher recurrence rate and rate of use of immunosuppressant than those without overlap syndrome (P<0.05). About 77% (24/31) of the children had good response to immunotherapy, and only 1 child had poor prognosis. CONCLUSIONS: Children with GFAP-A often have non-specific clinical symptoms and show good response to immunotherapy. Children with overlap syndrome have a high recurrence rate, and early application of immunosuppressants may help to prevent recurrence and alleviate symptoms.


Assuntos
Astrócitos , Doenças Autoimunes , Proteína Glial Fibrilar Ácida , Adolescente , Criança , Pré-Escolar , Humanos , Lactente , Astrócitos/metabolismo , Autoanticorpos/metabolismo , Proteína Glial Fibrilar Ácida/líquido cefalorraquidiano , Proteína Glial Fibrilar Ácida/metabolismo , Prognóstico , Estudos Retrospectivos , Doenças Autoimunes/diagnóstico , Doenças Autoimunes/metabolismo
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