Recommendations for diagnosis and treatment of systemic lupus erythematosus / 中华内科杂志

Systemic lupus erythematosus (SLE) is a systemic autoimmune disease with complicated pathogenesis and diverse clinical manifestations. The current recommendations of the Chinese Rheumatology Association are based on a comprehensive investigation of evidence based medicine, domestic and international guidelines for SLE, and experts' proposals, and aim to provide a more scientific and authoritative reference for the diagnosis and management of SLE. The recommendations focus on four aspects; clinical manifestations, laboratory evaluation, diagnosis and disease assessment, and disease treatment and monitoring. The goal of the recommendations is to standardize the diagnosis and treatment of SLE in China so as to improve the prognosis of SLE patients.

Associations between vitamin D levels and systemic lupus erythematosus risk:a Mendelian randomized study / 中华预防医学杂志

Objective: To explore the causal effects of the serum Vitamin D levels on the risk of systemic lupus erythematosus (SLE). Methods: A two-sample Mendelian randomization (MR) study was performed to infer the causality. Three Genome-wide association studies (GWAS) for circulating Vitamin D levels, including 25-hydroxyvitamin D [25(OH)D], 25-hydroxyvitamin D3 [25(OH)D3] and C3-epimer of 25-hydroxyvitamin D3 [C3-epi-25(OH)D3] published in 2020, and one GWAS for SLE published in 2015 were utilized to analyze the causal effects of the serum Vitamin D levels on the risk of SLE. MR analyses were conducted using the inverse-variance weighted method (IVW), weighted median, MR-Egger methods, MR-pleiotropy residual sum and outlier (MR-PRESSO) method. Results: 34, 29 and 6 SNPs were respectively selected as instrumental variables to analyze the causal association of total 25 (OH) D level, 25 (OH) D3 level and C3-epi-25 (OH) D3 level with the risk of SLE. The MR results showed that each standard deviation decrease in the level of 25(OH)D3 would result in 14.2% higher risk of SLE (OR, 0.858; 95%CI, 0.753-0.978; P=0.022). The levels of 25(OH)D and C3-epi-25(OH)D3 had null associations with risk of SLE (OR, 0.849; 95%CI, 0.653-1.104; P=0.222; OR, 0.904; 95%CI, 0.695-1.176; P=0.452). Conclusion: This study have identified a causal effect of 25(OH)D3 on increased risk of SLE. These findings highlighted the significance of active monitoring and prevention of SLE in population of low Vitamin D levels.

Systemic Lupus Erythematosus Combined with Chorea: Report of One Case and Literature Review / 中国医学科学院学报

Systemic lupus erythematosus combined with chorea is relatively rare in China,and there are no unified diagnostic criteria or specific ancillary tests.Therefore,it is confirmed by exclusionary clinical diagnosis.To improve the understanding of this disease among rheumatologists,we report the clinical data of a patient with systemic lupus erythematosus combined with chorea admitted to the Department of Rheumatology and Immunology in the First Affiliated Hospital of Jinan University in January 2022.Furthermore,we review the relevant literature in the past 10 years and summarize the clinical features of these cases.

Risk factors of childhood systemic lupus erythematosus with thyroid dysfunction / 中华儿科杂志

Objective: To investigate the risk factors of childhood systemic lupus erythematosus (SLE) with thyroid dysfunction and to explore the relationship between thyroid hormone and kidney injury of lupus nephritis (LN). Methods: In this retrospective study, 253 patients who were diagnosed with childhood SLE and hospitalized in the First Affiliated Hospital of Zhengzhou University from January 2019 to January 2021 were enrolled in the case group, and 70 healthy children were the control cases. The patients in the case group were divided into the normal thyroid group and the thyroid dysfunction group. Independent t-test, χ2 test, and Mann-Whitney U test were used for comparison between the groups, Logistic regression analysis was used for multivariate analysis, and Spearman correlation. Results: A total of 253 patients, there were 44 males and 209 females in the case group, and the age of onset was 14 (12, 16) years; a total of 70 patients, 24 males and 46 females were in the control group, and the age of onset was 13 (10, 13) years. The incidence of thyroid dysfunction in the case group was higher than that in the control group (48.2% (122/253) vs. 8.6% (6/70), χ²=36.03, P<0.05). Of the 131 patients, there were 17 males and 114 females in the normal thyroid group, and the age of onset was 14 (12, 16) years. Of the 122 patients in the thyroid dysfunction group, 28 males and 94 females were in the thyroid dysfunction group, and the age of onset was 14 (12, 16) years. Of the 122 had thyroid dysfunction, including 51 cases (41.8%) with euthyroid sick syndrome, 25 cases (20.5%) with subclinical hypothyroidism, 18 cases (14.8%) patients with sub-hyperthyroidism, 12 cases (9.8%) with hypothyroidism, 10 cases (8.2%) with Hashimoto's thyroiditis, 4 cases (3.3%) with hyperthyroidism, and 2 cases (1.6%) with Graves disease. Compared to patients with normal thyroid function, the serum level of triglyceride, total cholesterol, urine white blood cell, urine red blood cell, 24 h urine protein, D-dimer, and fibrinogen, ferritin and systemic lupus erythematosus disease activity Index-2000 (SLEDAI-2K) score were higher in patients with thyroid dysfunction (Z=3.07, 3.07, 2.48, 3.16, 2.40, 3.99, 2.68, 2.55, 2.80, all P<0.05), while the serum level of free thyroxine and C3 were lower in thyroid disfunction patients (10.6 (9.1, 12.7) vs. 11.3 (10.0, 12.9) pmol/L, and 0.46 (0.27, 0.74) vs. 0.57 (0.37, 0.82) g/L, Z=2.18, 2.42, both P<0.05). The higher level of triglyceride and D-dimer were the independent risk factors for childhood SLE with thyroid dysfunction (OR=1.40 and 1.35, 95%CI 1.03-1.89 and 1.00-1.81, respectively, both P<0.05). There were 161 patients with LN in the case group, all of which were conducted with renal biopsies, including 11 cases (6.8%) with types Ⅰ LN, 11 cases (6.8%) with typesⅡLN, 31 cases (19.3%) with types Ⅲ LN, 92 cases (57.1%) with types Ⅳ LN, and 16 cases (9.9%) with types Ⅴ LN. There were significant differences in the level of free triiodothyronine and thyroid stimulating hormone among different types of kidney pathology (both P<0.05); compared with types I LN, the serum level of free triiodothyronine was lower in types Ⅳ LN (3.4 (2.8, 3.9) vs. 4.3 (3.7, 5.5) pmol/L, Z=3.75, P<0.05). The serum level of free triiodothyronine was negatively correlated with the acute activity index score of lupus nephritis (r=-0.228, P<0.05), while the serum level of thyroid stimulating hormone was positively correlated with the renal pathological acute activity index score of lupus nephritis (r=0.257, P<0.05). Conclusions: There is a high incidence of thyroid dysfunction in childhood SLE patients. The higher SLEDAI and more severe renal damage were found in SLE patients with thyroid dysfunction compared to these with normal thyroid functions. The risk factors of childhood SLE with thyroid dysfunction are the higher level of triglyceride and D-dimer. The serum level of thyroid hormone is possibly related to the kidney injury of LN.

Systemic lupus erythematosus with acquired hemophilia A: A case report and literature review / 中南大学学报(医学版)

Systemic lupus erythematosus (SLE) complicated with acquired hemophilia A (AHA) is a rare condition with frequently delayed diagnosis and a high mortality rate, so it is necessary to strengthen the understanding of this disease. In this study, the characteristics and treatment in 1 case of SLE complicated by AHA is reported and analyzed, and a literature review is conducted. The patient was a 29-year-old young female with a 10-year history of SLE, the main clinical manifestation was severe abdominal bleeding. Laboratory tests revealed that the activated partial thromboplastin time (APTT) was notably prolonged (118.20 s), and the coagulation factor VIII activity (FVIII꞉C) was extremely decreased (0.20%) with high-titer of factor VIII (FVIII) inhibitor (31.2 BU/mL). After treating with high-dose glucocorticoid, immunoglobulin, cyclophosphamide, rituximab, blood transfusion, and intravenous infusion of human coagulation FVIII, the coagulation function and coagulation FVIII꞉C were improved, and FVIII inhibitor was negative without serious adverse reactions. During the next 5-year follow-up, the patient's condition was stable and no bleeding occurred. In the case of coagulation dysfunction in SLE, especially with isolated APTT prolongation, AHA should be screened. When the therapeutic effects of glucocorticoid combined with immunosuppressants are not desirable, rituximab could be introduced.

Apendicitis aguda en una gestante lúpica / Acute appendicitis in pregnant lupus

El lupus eritematoso sistémico es una enfermedad autoinmune que se caracteriza por un proceso inflamatorio crónico y el aumento de la producción de autoanticuerpos como mecanismos patogénicos. Se presenta con mayor frecuencia en pacientes femeninas y en edad fértil. La gestación en pacientes con esta enfermedad se considera como una condición de extrema precaución, ya que existe influencia de la gestación en la actividad clínica del lupus y del lupus en la evolución de la gestación. Las complicaciones quirúrgicas, como es el caso de una apendicitis aguda, aportan mayor riesgo al binomio madre-feto. El objetivo del presente trabajo es comunicar la experiencia de tratamiento de una paciente de 31 años de edad, con diagnóstico de lupus eritematoso sistémico y a quien a las 35,6 semanas de gestación se le presentó un cuadro de apendicitis aguda que no solo provocó la actividad de la enfermedad, sino que causó la interrupción de la gestación. La paciente y el recién nacido presentaron una evolución favorable sin complicaciones posteriores.

Systemic lupus erythematosus is an autoimmune disease that includes the presence of a chronic inflammatory process and increased production of autoantibodies as etiopathogenic mechanisms. As a disease, it occurs more frequently in female patients and those of childbearing age. Pregnancy in patients with this disease is considered an element of extreme caution since there is an influence of pregnancy on the clinical activity of lupus and lupus on the evolution of pregnancy. The presence of surgical complications, as is the case of acute appendicitis, brings greater risk to the mother-fetus binomial. The objective of this report is to communicate the treatment experience of a 31-year-old patient, diagnosed with systemic lupus erythematosus and who at 35.6 weeks of gestation presented acute appendicitis that not only causes disease activity, but it generates the need to interrupt the pregnancy. The patient and the newborn had a favorable evolution, with no subsequent complications.

Síndrome de Guillain-Barré como forma de presentación de lupus eritematoso sistémico en paciente masculino / Guillain Barré Syndrome as a form of presentation of systemic lupus erythematosus in a male patient

El lupus eritematoso sistémico es una enfermedad reumática, autoinmune, inflamatoria, sistémica y crónica que se caracteriza por afectar principalmente pacientes femeninas en edad fértil. Su mecanismo patogénico se centra en la presencia de un proceso inflamatorio crónico que es el responsable de las manifestaciones clínicas y complicaciones de la enfermedad. Las manifestaciones clínicas se subdividen en articulares y extraarticulares. Entre estas últimas la afectación neurológica cobra especial atención tanto por su frecuencia de presentación elevada como por la gravedad de estas. El síndrome de Guillain-Barré es una enfermedad autoinmune, que puede aparecer como complicación del lupus eritematoso sistémico y se caracteriza por parálisis motora y otros síntomas y signos neurológicos. El objetivo del presente trabajo es reportar el caso de un paciente masculino, de 27 años de edad, que comienza con manifestaciones neuropáticas y se llega a diagnosticar un síndrome de Guillain-Barré que constituyó la forma de presentación del lupus eritematoso sistémico. Por ser una forma de presentación poco común, se considera importante su reporte para conocimiento de la comunidad médica(AU)

Systemic lupus erythematosus is a rheumatic, autoimmune, inflammatory, systemic and chronic disease that is characterized by mainly affecting female patients of childbearing age. Its etiopathogenic mechanism focuses on the presence of a chronic inflammatory process that is responsible for the clinical manifestations and complications of the disease. Clinical manifestations are subdivided into articular and extra-articular. Within the latter, neurological involvement receives special attention both due to its high frequency of presentation and its severity. Guillain-Barré syndrome is an autoimmune disease that can appear as a complication of lupus erythematosus and is characterized by motor paralysis and other neurological signs and symptoms. The objective of this paper is to report the case of a 27-year-old male patient who began with neuropathic manifestations and was diagnosed with Guillain-Barré syndrome, which was the presentation of systemic lupus erythematosus. Because it is an uncommon form of presentation, its report is considered important for the knowledge of the medical community(AU)

Miocarditis lúpica: reporte de un caso / Lupus myocarditis: a case report / Miocardite lúpica: relato de um caso

El término miocarditis hace referencia a una inflamación del miocardio, que puede tener diversas causas (infecciones, tóxicos, enfermedades autoinmunes). Su diagnóstico es desafiante debido al gran espectro de presentaciones clínicas que puede adoptar, muchas veces imitando patologías más prevalentes como el infarto agudo de miocardio. La miocarditis asociada a enfermedades autoinmunes es poco frecuente, y la importancia de reconocerla radica en que el diagnóstico e inicio temprano del tratamiento son cruciales para mejorar su pronóstico. Presentamos aquí un caso clínico de una perimiocarditis lúpica.

Myocarditis refers to an inflammation of the myocardium, which can have various causes (infections, toxic substances, autoimmune diseases). Its diagnosis is challenging due to the wide spectrum of clinical presentations, often mimicking more prevalent pathologies such as acute myocardial infarction. Myocarditis associated with autoimmune diseases is rare, and the importance of recognizing is that early diagnosis and initiation of treatment are crucial to improve its prognosis. We present here a clinical case of lupus perimyocarditis.

O termo miocardite refere-se a uma inflamação do miocárdio, que pode ter várias causas (infecções, substâncias tóxicas, doenças autoimunes). Seu diagnóstico é desafiador devido ao amplo espectro de apresentações clínicas que pode ter, muitas vezes mimetizando patologias mais prevalentes como o infarto agudo do miocárdio. A miocardite associada a doenças autoimunes é rara, e a importância de reconhecê-la reside no fato de que o diagnóstico precoce e o início do tratamento são cruciais para melhorar seu prognóstico. Apresentamos aqui um caso clínico de perimiocardite lúpica.

Amicrobial pustulosis of the folds: report of two cases / Pustulosis amicrobiana de los pliegues. Informe de dos casos

ABSTRACT Amicrobial Pustulosis of the Folds is a relapsing, chronic and rare neutrophilic dermatosis, characterized by papulopustular, eczematous and aseptic lesions on skin folds. This disorder usually occurs predominantly in females (30 years of age average) with a history of an autoimmune disorder, especially systemic lupus erythematosus. There is no standard therapy, but systemic corticosteroids, alone or in combination with other immunosuppressive drugs, are usually the first-line therapy. We report two females aged 37 and 20 years with the disease but without associated autoimmune diseases. They were successfully treated with non-steroidal treatments.

La pustulosis amicrobiana de los pliegues es una dermatosis neutrofílica crónica, recurrente y poco común. Se caracteriza por lesiones pápulo-pustulosas, eczematosas y asépticas de los pliegues cutáneos. Este cuadro se presenta predominantemente en mujeres de alrededor de 30 años con enfermedades autoinmunes, especialmente lupus eritematoso sistémico. No existe un tratamiento estándar pero los corticoides solos o con inmunosupresores se usan de primera línea. Informamos dos mujeres de 27 y 20 años sin patología autoinmune, con la enfermedad. Ellas fueron tratadas exitosamente sin usar esteroides.

The Changes of Peripheral Blood FⅫa-AT, TSP-1, LA Ratio in Patients with Systemic Lupus Erythematosus and the Clinical Value of Combined Diagnosis of Thrombotic Events / 中国实验血液学杂志

OBJECTIVE@#To explore the changes of Ⅻ antithrombin (FⅫa-AT), thrombospondin-1 (TSP-1), and lupus anticoagulant (LA) ratio in the peripheral blood factor of patients with systemic lupus erythematosus (SLE) and the clinical value of combined diagnosis of thrombotic events.@*METHODS@#A total of 133 SLE patients treated in Xingtai People's Hospital were selected and divided into simple SLE group (105 cases) and SLE complicated with thrombosis group (28 cases) according to whether thrombotic events occurred, and 102 cases of healthy people in the same period were selected as control. The clinical data of the 3 groups, the level of peripheral blood FⅫa-AT, TSP-1, and LA ratio were compared, the relationship between each peripheral blood index and SLE disease activity index (SLEDAI) score were analyzed. The influencing factors of thrombotic events in SLE patients were analyzed, and the value of each peripheral blood index in the diagnosis of SLE complicated with thrombotic events were evaluated.@*RESULTS@#The proportion of the patients with age ≥60 year, hypertension, and smoking history in SLE complicated with thrombosis group was higher than those in simple SLE group and control group (P＜0.05). The SLEDAI score, peripheral blood FⅫa-AT, TSP-1, LA ratio levels of the patients in SLE complicated with thrombosis group were significantly higher than those in simple SLE group and control group, and the simple SLE group was significantly higher than the control group (P＜0.05). FⅫa-AT, TSP-1, LA ratio in peripheral blood of SLE patients were positively correlated with SLEDAI score (r=0.663, 0.578 and 0.625). Age, blood pressure, smoking history, peripheral blood FⅫa-AT, TSP-1, LA ratio were the important influencing factors of thrombotic events in SLE patients (P＜0.05). The AUC diagnosed by the FⅫa-AT, TSP-1, and LA ratio in peripheral blood was 0.881, the 95% CI was 0.813-0.931, the sensitivity was 82.14%, and the specificity was 91.43%, which was superior to each index alone (P＜0.05).@*CONCLUSION@#Peripheral blood FⅫa-AT, TSP-1, LA ratio level changes in SLE patients are significantly related to disease activity, and the combined diagnosis of thrombotic events is more reliable.

Retinopatia hipertensiva maligna secundária a lúpus eritematoso sistêmico em adolescente / Malignant hypertensive retinopathy secondary to systemic lupus erythematous in adolescent

RESUMO O lúpus eritematoso sistêmico é uma doença que pode apresentar comprometimento oftalmológico geralmente benigno, sendo as alterações mais encontradas a síndrome do olho seco e a catarata. Nos pacientes com a doença estável, o dano oftalmológico parece estar relacionado ao tratamento sistêmico a longo prazo, o que enfatiza a importância do exame oftalmológico completo de rotina. Porém, quando a doença está em franca atividade e, em especial, quando há o envolvimento renal, deve-se iniciar o tratamento precoce com corticoterapia sistêmica e com medidas de suporte, para se evitarem repercussões mais complexas, como as crises hipertensivas que podem levar ao óbito.

ABSTRACT Systemic lupus erythematosus may present ophthalmological involvement, usually benign, and the most common changes are dry eye syndrome and cataract. In patients with stable disease, ophthalmologic damage appears to be related to long-term systemic treatment, emphasizing the importance of routine complete ophthalmologic examination. However, in full-blown disease, especially when there is renal involvement, early treatment should start with systemic steroid therapy and supportive measures, to avoid major repercussions, such as hypertensive crises that may lead to death.

Nefritis como debut lúpico en el embarazo: reporte de dos casos y revisión de la literatura / Nephritis as a lupic debut in pregnancy: report of two cases and review of the literature

ANTECEDENTES: El lupus eritematoso sistémico es una enfermedad autoinmunitaria multisistémica que afecta principalmente a mujeres en edad fértil. La nefritis lúpica es la manifestación clínica más frecuente durante la gestación y constituye un factor de riesgo para la pérdida del embarazo, en especial en pacientes con insuficiencia renal. Además, presenta mayor riesgo de pérdida fetal, restricción del crecimiento intrauterino e hipertensión. CASOS CLÍNICOS: El primer caso se trata de una gestante de 28 + 2 semanas con daño renal grave y anticuerpos anti-DNA en título elevado. En el segundo caso destaca el debut con sintomatología de dificultad respiratoria y edemas como manifestaciones clínicas de nefropatía lúpica tipo V a las 23 semanas de gestación. En ambas pacientes destaca la prematuridad como complicación perinatal, así como el crecimiento intrauterino retardado en el primer caso. Por último, se describen los resultados clínico-analíticos tras el inicio terapéutico específico en ambos casos. CONCLUSIONES: El diagnóstico diferencial del debut de nefritis lúpica durante la gestación continúa siendo un desafío, a pesar de los avances en cuanto a marcadores angiogénicos. La valoración clínica continúa siendo la piedra angular de este proceso diagnóstico y de sus implicaciones en cuanto a complicaciones del embarazo actuales y futuras.

BACKGROUND: Systemic lupus erythematosus is a multisystem autoimmune disease that mainly affects women of childbearing age. Lupus nephritis represents the most frequent clinical manifestation in pregnancy, constituting a risk factor for pregnancy loss, especially in patients with kidney damage. It also has a higher risk of fetal loss, intrauterine growth restriction, and gestational hypertension. CLINICAL CASES: The first case is a 28 + 2-week pregnant woman with severe kidney damage and high-titles anti-DNA antibodies. In the second case, we highlight the debut with symptoms of respiratory distress and edema as clinical manifestations of type V lupus nephropathy in a 23-week gestation. In both cases, prematurity stands out as a perinatal complication, as well as delayed intrauterine growth in the former. Finally, the clinical-analytical results are described, after the specific therapeutic initiation in both cases. CONCLUSIONS: The differential diagnosis of the onset of lupus nephritis during pregnancy continues to be a challenge, despite the advances in angiogenic markers; clinical assessment continues to be the cornerstone of this diagnostic process and its implications for current and future pregnancy complications.

Non-lupus full-house nephropathy: a case series / Nefropatia "full-house" não relacionada ao lúpus: uma série de casos

Abstract Systemic lupus erythematosus (SLE) is a chronic multisystem autoimmune inflammatory disease. However, some patients may exhibit a histological pattern of kidney injury, with characteristics indistinguishable from lupus nephritis, but without presenting any extrarenal symptoms or serologies suggestive of SLE. Such involvement has recently been called non-lupus full-house nephropathy. The objective is to report a series of clinical cases referred to the Laboratory of the Federal University of Maranhão that received the diagnosis of "full-house" nephropathy unrelated to lupus, upon immunofluorescence and to discuss its evolution and outcomes. Non-lupus full-house nephropathy represents a diagnostic and therapeutic challenge, because it is a new entity, which still needs further studies and may be the initial manifestation of SLE, isolated manifestation of SLE or a new pathology unrelated to SLE.

Resumo O lúpus eritematoso sistêmico (LES) é uma doença inflamatória crônica autoimune multissistêmica. Alguns pacientes, contudo, podem exibir um padrão histológico de lesão renal, com características indistinguíveis da nefrite lúpica, porém sem apresentar quaisquer sintomas extrarrenais ou sorologias sugestivas de LES. Tal acometimento tem sido recentemente denominado nefropatia "full-house" não relacionada ao lúpus. O objetivo é relatar uma série de casos clínicos encaminhados ao Laboratório da Universidade Federal do Maranhão que receberam o diagnóstico de nefropatia "full-house" não relacionada ao lúpus à imunofluorescência e discutir sua evolução e desfechos. A nefropatia "full-house" não relacionada ao lúpus representa um desafio diagnóstico e terapêutico por ser uma entidade nova, que ainda necessita de maiores estudos e pode ser a manifestação inicial do LES, manifestação isolada do LES ou uma patologia nova não relacionada ao LES.

Oftalmoplejía internuclear bilateral (WEBINO) en un paciente pediátrico con Lupus Eritematoso Sistémico / Wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) in a pediatric patient with Systemic Lupus Erythematosus

Resumen El síndrome de WEBINO (wall-eyed bilateral internuclear ophthalmoplegia), se presenta por una lesión del tegmento pontino (incluye área pontina paramediana, fascículo longitudinal medial y núcleo del abducens). Presenta limitación bilateral en la aducción y exotropía en la posición de la mirada primaria, nistagmo del ojo que abduce e incapacidad para la convergencia. Reporte de caso: Presentamos el caso de una paciente de 14 años con antecedente de Lupus Eritematoso Sistémico que debutó con diplopía horizontal de inicio súbito. El diagnóstico de WEBINO fue clínico y asociado con hallazgos de lesión isquémico pontomesencefálica en Resonancia Nuclear Magnética y angioresonancia cerebral. Se administró tratamiento con Metilprednisolona y presentó resolución gradual de los síntomas, sin embargo una semana después falleció por criptococosis sistémica. Conclusiones: Hacer el diagnostico de WEBINO se hace desafiante por su rareza y por la precisión de su localización neuroanatómica. Se debe realizar una exploración detallada para definir la causa probable y establecer el tratamiento oportuno que favorezca el pronóstico neurológico.

Background: Wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) is presented by a lesion of the pontine tegment (includes paramedian pontine area, medial longitudinal fascicle and nuclei of the abducens). It presents bilateral limitation in adduction and exotropia in the position of the primary gaze, abducting eye nystagmus and inability to converge. Case report: We present the case of a 14-year-old patient with a history of Systemic Lupus Erythematosus who debuted with sudden onset horizontal diplopia. WEBINO's diagnosis was clinical and associated with findings of ponto-mesencephalic ischemic injury in magnetic resonance imaging and magnetic resonance angiography. Treatment with Methylprednisolone was administered and she presented gradual resolution of the symptoms, however, one week later she died of systemic cryptococcosis. Conclusions: Making the WEBINO diagnosis is challenging due to its rarity and the precision of its neuroanatomical location. A detailed examination should be performed to define the probable cause and establish the appropriate treatment that favors the neurological prognosis.

Enterite mesentérica como complicação rara do lúpus eritematoso sistêmico / Mesenteric enteritis as a rare complication of systemic lupus erythematosus

A dor abdominal no paciente com lúpus eritematoso sistêmico tem amplo espectro clínico, variando desde condições inespecí- ficas, como diarreia e vômitos, até eventos de importante morbi- mortalidade, como o abdome agudo inflamatório e/ou perfura- tivo. A seguir, descreve-se um caso de paciente do sexo feminino, de 23 anos, internada por dor abdominal associada a vômitos e à diarreia crônica e progressiva. Foi diagnosticada com lúpus eritematoso sistêmico há 2 anos. Durante a internação, evoluiu com quadro de abdome agudo, e foi realizada tomografia compu- tadorizada de abdome, revelando importante edema de parede intestinal difuso. Isso, somado a alterações clínico-laboratoriais, permitiu o diagnóstico de enterite lúpica. Foi realizado tratamen- to conservador, com corticoterapia e terapia de suporte com correção de distúrbios eletrolíticos severos, sendo iniciado ciclo- fosfamida, com resolução dos sintomas gastrintestinais.

Abdominal pain in patients with systemic lupus erythematosus has a broad clinical spectrum, ranging from nonspecific symp- toms, such as diarrhea and vomiting, to events of significant morbidity and mortality, such as acute inflammatory and/or per- forating abdomen. This article describes a case of a 23-year-old female patient hospitalized for abdominal pain, associated with vomiting and progressive chronic diarrhea. She was diagnosed with systemic lupus erythematosus 2 years ago. During hospita- lization, the patient progressed with acute abdomen, and an ab- dominal computed tomography scan was performed, revealing major diffuse intestinal wall edema. This, added to clinical and laboratories alterations, allowed the diagnosis of lupus enteritis. A conservative treatment with corticotherapy and supportive therapy with correction of severe electrolyte disturbances were initiated, as well as the prescription of cyclophosphamide, with resolution of gastrointestinal symptoms.

Manejo oportuno de púrpura trombocitopénica trombótica en paciente con lupus eritematoso sistémico: reporte de caso / Timely management of thrombotic thrombocytopenic purpura in a patient with systemic lupus erythematosus: case report

Introducción: La púrpura trombocitopénica trombótica puede presentarse en menos del 2 por ciento de los pacientes con lupus eritematoso sistémico. Esta asociación implica un aumento de la mortalidad y un periodo de remisión más prolongado. Objetivo: Se presenta el caso de paciente peruana que desarrolló esta asociación y presentó complicaciones relacionadas con shock séptico. Caso clínico: Paciente femenina, con antecedente de púrpura trombocitopénica inmunológica y lupus eritematoso sistémico, acudió a emergencia por presentar palidez cutánea generalizada, petequias en miembros inferiores y hematuria. Posteriormente, su estado de salud se complicó con un shock séptico y deterioro del nivel de conciencia. Por todo esto, es referida a un hospital de mayor complejidad y hace su ingreso a la unidad de cuidados intensivos. La clínica y los exámenes de laboratorio revelaron hallazgos compatibles con púrpura trombocitopénica trombótica (anemia grave, plaquetopenia, esquistositosis) y lupus eritematoso sistémico activo grave. Antes de ser referida, recibió pulsos de metilprednisona y prednisona. Ya en unidad de cuidados intensivos, se cambió a soporte ventilatorio y tratamiento antibiótico. Con el diagnóstico presuntivo de púrpura trombocitopénica trombótica, asociada a lupus eritematoso sistémico activo grave, se inició tratamiento oportuno con plasmaféresis, corticoterapia y ciclofosfamida. La paciente recuperó los niveles plaquetarios y el nivel óptimo de conciencia. Actualmente acude a controles. Conclusiones: La púrpura trombocitopénica trombótica es una emergencia hematológica con alta mortalidad en ausencia de tratamiento. Su reconocimiento oportuno, sin dosificación de la proteína ADAMTS13, en esta asociación poco frecuente con lupus eritematoso sistémico es importante en el buen pronóstico del paciente(AU)

Introduction: Thrombotic thrombocytopenic purpura may occur in less than 2 percent of patients with systemic lupus erythematosus. This association implies an increase in mortality and a longer remission period. Objective: We present the case of a Peruvian woman who developed this association, and complicating herself with septic shock. Clinical case: A female patient, with a history of immunological thrombocytopenic purpura and systemic lupus erythematosus, comes to the emergency room due to generalized skin pallor, lower limb petechiae and hematuria. Subsequently, her state of health gets complicated with a septic shock and deterioration of the level of consciousness. For all of this, she was referred to a hospital of greater complexity and makes admission to an intensive care unit. Clinical and laboratory tests revealed findings compatible with thrombotic thrombocytopenic purpura (severe anemia, platelet disease, schistositosis) and severe active systemic lupus erythematosus. Before being referred, she received pulses of methylprednisone and prednisone. When already in the intensive care unit, it was changed to ventilatory support andantibiotic treatment. With the presumptive diagnosis of thrombotic thrombocytopenic purpura, associated with severe active systemic lupus erythematosus, a timely treatment was initiated with plasmapheresis, corticosteroids and cyclophosphamide. The patient recovered platelet levels and optimal level of consciousness. She is currently going to controls. Conclusions: Thrombotic thrombocytopenic purpura is a hematological emergency with high mortality in the absence of treatment. Its timely recognition, without dosing of ADAMTS13 protein, in this rare association with systemic lupus erythematosus is important in the good prognosis of the patient(AU)

Disección coronaria espontánea asociada a lupus eritematoso sistémico / Spontaneous coronary dissection associated with systemic lupus erythematosus

Resumen A 29 year old female with a past medical history of systemic lupus erythematosus, diagnosed 15 years earlier, presents with lupus nephritis, currently on peritoneal dialysis. She had myopericarditis in 2012 and is currently on immunosuppressants. The patient began with exertional dyspnea and angina 2 weeks before admission. An echocardiogram was performed, reporting severe mitral and tricuspid insufficiency. Afterwards, the patient presented with resting angina associated with an adrenergic and vagal response. Initially, rheumatology ruled out autoimmune activity caused by lupus. We performed a coronary angiogram based on clinical presentation, EKG changes and biomarkers, finding a trivascular coronary artery disease classified as a Markis I coronary artery ectasia and a coronary dissection of the ramus intermedius and the circumflex, posterior to the first obtuse marginal artery. Cardiothoracic surgery considered intervention with a coronary bridge posterior to the dissection of the intermedius ramus artery, marginal obtuse and posterolateral artery, as well as a mitral valve replacement and a tricuspid valve repair. Coronary dissection is more common in women (70%), clinical presentation varies from unstable angina to sudden death. In lupus nephritis, it is an uncommon form of extra renal vasculitis. Treatment depends on the number of arteries affected, as well as the haemodynamic state of the patient. It is imperative to individualize treatment options.

Abstract Se presenta el caso de una paciente de 29 años con antecedente de lupus eritematoso sistémico diagnosticado 15 años antes, que desarrolló nefropatía lúpica actualmente en diálisis peritoneal, cuadro de miopericarditis en 2012 y bajo tratamiento inmunosupresor. Inició con deterioro de su clase funcional por disnea y angina 2 semanas previas al ingreso. Se le realizó ecocardiograma, el cual reportó insuficiencias mitral y tricuspídea graves. Posteriormente presentó angina en reposo asociada a descarga adrenérgica y vagal. A su ingreso se descarta actividad lúpica por reumatología. Por presentación clínica, cambios en electrocardiograma y biomarcadores, se realizó cateterismo cardiaco, que reportó enfermedad coronaria trivascular con ectasia coronaria Markis I y disección coronaria de ramo intermedio y circunfleja posterior a la primera marginal obtusa. Se consideró por el servicio de cirugía cardiotorácica realizar intervención con puente coronario posterior a disección del ramo intermedio, marginal obtusa y posterolateral, así como cambio valvular mitral y plastia tricuspídea. La disección coronaria espontánea es más frecuente en las mujeres (70%); puede presentarse como angina inestable y hasta como muerte súbita. La asociación con lupus eritematoso sistémico es poco frecuente, con una incidencia del 0.42%. En la nefropatía lúpica es una manifestación poco frecuente de vasculitis extrarrenal. El tratamiento de elección depende del número de vasos afectados y del estado hemodinámico, por lo que es necesario individualizarlo para cada paciente.

Consideraciones anestésicas en lupus eritematoso sistémico / Systemic lupus erythematosus: implications for anesthesia

INTRODUCTION: Sistemyc lupus erythematosus (SLE) is an autoinmune disease associated to severe organ damage and mortality. SLE patients have a higher surgical risk. The objective of this article is to review the systemic manifestations of SLE and how they can challenge the anesthetic management, so a safer and more tailored anesthesia can be provided. METHODS: A search was made on Pubmed, ProQuest, and EMBASE databases obtaining a total of 2,028 articles. The titles of articles found were reviewed, of which 88 merited greater review. The complete text of each article of this group was reviewed, references that were of greater interest were also reviewed. Finally, a total of 29 works to be included in this review were selected. RESULTS: Musculoskeletal, cardiac, renal and laringeal involvement are the most frequent and important complications to anesthetic management. It´s clinical manifestations must be assess in the preoperative evaluation as well as potential pharmacological interactions with regular medications that the patient might be taking. The need of antibiotic profilaxis and steroidal supplementation has to be addressed. The post operative management should be focused on assessment of frecuent complications as cardiovascular or thrombotic events. CONCLUSION: SLE is a complex disease that represent a challenge in surgical context. Its complications can affect the airway, ventilatory or hemodinamic management as well as renal function. That is why the multidisciplinary management of these patients and a complete preoperative evaluation are essentials so a risk estratification can be made and anesthesiologists can prepare correctly to handle potential complications.

INTRODUCCIÓN: El lupus eritematoso sistémico es una enfermedad autoinmune, asociada a complicaciones severas y mayor mortalidad. Por las complicaciones de la enfermedad estos pacientes son de alto riesgo quirúrgico. El objetivo de este trabajo es revisar las manifestaciones sistémicas y complicaciones del LES y como éstas pueden afectar el manejo anestésico, de manera de proporcionar una anestesia más segura y acorde a las necesidades de cada paciente. MATERIALES Y MÉTODOS: Se realizó una búsqueda en Pubmed, Embase y ProQuest, encontrándose un total de 2.028 trabajos. Se revisaron los títulos, encontrando 88 de interés para mayor revisión. Se revisaron los textos completos, incluyendo además referencias y sugerencias que parecieron pertinentes. Se seleccionaron, finalmente, 29 trabajos para ser incluidos en esta revisión. RESULTADOS: El compromiso musculoesquelético, cardiaco, renal y laríngeo son los más frecuentes e importantes en el manejo anestésico. Deben buscarse de manera dirigida en la evaluación preoperatoria, además de evaluar posibles interacciones farmacológicas entre anestésicos y medicamentos de uso habitual. Por el uso de corticoides e inmunosupresores, considerar el uso de profilaxis antibiótica y reemplazo esteroidal. El manejo posoperatorio debe enfocarse en la búsqueda de complicaciones más frecuentes como eventos cardiovasculares o trombóticos. DISCUSIÓN: El LES es una enfermedad compleja con desafíos en el contexto quirúrgico. Sus complicaciones afectan el manejo de vía aérea, terapia ventilatoria, manejo hemodinámico y mantención de la función renal. Es por ello que el manejo multidisciplinario y una buena evaluación preoperatoria son fundamentales, de manera de estratificar el riesgo y prepararse oportunamente.