Lupus de inicio tardío en un grupo de pacientes paraguayos

Introducción: Los pacientes con lupus eritematoso sistémico (LES) diagnosticados después de los 50 años presentan una enfermedad menos severa y un curso clínico más leve. El objetivo de este estudio es describir las características clínicas y de laboratorios del LES en pacientes de edad avanzada. Material y Método: Estudio observacional, descriptivo, de corte trasverso, retrospectivo, de pacientes con el diagnóstico de LES, de inicio posterior a los 50 años de edad, que consultaron en el Hospital Nacional, en el periodo comprendido entre diciembre de 2016 y mayo de 2024. Resultados: Se estudiaron 30 pacientes entre 51 y 87 años (edad media: 62,5 años ± 8,5), 16 mujeres (53,3%) y 14 varones 14 (46,6 %). El tiempo de enfermedad previo al diagnóstico fue de 59,4 ± 8.3 (50-80) días. La duración de la enfermedad fue en promedio 5 años ± 5,1 (1-26). Las principales manifestaciones clínicas fueron las artralgias 26 (86,6%), artritis 22 (72,3%), pérdida de peso 10 (33,3%) y fiebre prolongada (30%). Presentaron comorbilidades 19 pacientes (63,3 %), siendo la hipertensión arterial la principal. El anti-DNA fue positivo en 12 pacientes (42,8%), el anti-Ro en 5/25 pacientes (20%), el anti-Sm en 2/26 (7,9%). La eritrosedimentación en la primera hora fue ≥ 20 mm en 17/23 (73,9%). El 100% recibió tratamiento con hidroxicloroquina, mientras que recibieron corticoides 26 (86.6%) pacientes, micofenolato mofetil 7 (24,4%), ciclofosfamida 4 (13,3%). La mortalidad fue del 6,6 %. Conclusión: Los principales hallazgos fueron artralgias y artritis, siendo menos frecuentes los casos graves. La mayoría presentó comorbilidades, siendo la hipertensión arterial la más frecuente. La mortalidad fue del 6,6% de causa cardiovascular.

Introduction: Patients with systemic lupus erythematosus (SLE) diagnosed after the age of 50 have a less severe disease and a milder clinical course. The objective of this study is to describe the clinical and laboratory characteristics of SLE in elderly patients. Material and Method: Observational, descriptive, cross-sectional, retrospective study of patients with the diagnosis of SLE, with onset after 50 years of age, evaluated at the National Hospital, in the period between December 2016 and May of 2024. Results: 30 patients between 51 and 87 years old (mean age: 62.5 years ± 8.5) were studied, 16 women (53.3%) and 14 men (46.6%). The time to diagnosis was 59.4 ± 8.3 (50-80) days. The duration of the disease was on average 5 years ± 5.1 (1-26). The main clinical manifestations were arthralgia in 26 (86.6%), arthritis in 22 (72.3%), weight loss in 10 (33.3%) and prolonged fever (30%). Nineteen patients (63.3%) had comorbidities, the main one being high blood pressure. Anti-dsDNA was positive in 12 patients (42.8%), anti-Ro in 5/25 patients (20%), anti-Sm in 2/26 (7.9%). The erythrocyte sedimentation rate was ≥ 20 mm in 17/23 (73.9%). All patients were treatment with hydroxychloroquine, 26 (86.6%) patients received corticosteroids, 7 (24.4%) mycophenolate mofetil, 4 (13.3%) cyclophosphamide. Mortality was 6.6%. Conclusion: The main findings were arthralgia and arthritis, with severe cases being less frequent. The majority presented comorbidities, with high blood pressure being the most common. Mortality was 6.6% due to cardiovascular causes.

A rare case report on complications in pregnancy with systemic lupus erythematosus in a post-renal transplant patient

To preview the feto-maternal outcome in post-renal transplant pregnant women with systemic lupus erythematosus (SLE). To distinguish preeclampsia from hypertension in renal transplant recipients as diagnosis is not always straightforward and all differentials need a thorough evaluation. Hypertension is a prevalent issue among kidney transplant recipients, with reported incidence rates ranging from 52% to 69%. Additionally, the occurrence of pre-eclampsia in renal transplant recipients falls within the range of 24% to 38%, demonstrating a significantly elevated risk compared to the 4-5% incidence rate seen in the general population. A 29-year-old female para 1 IUFD 1 abortion 1, in a known case of SLE with hypothyroidism with lupus nephritis with post renal transplant status with thrombocytopenia with preeclampsia with day 7 of emergency LSCS done in view of non-progress of labor with intrauterine fetal demise with abruptio placenta referred in view of query SLE flare or severe preeclamptic features with rectus sheath hematoma. Renal transplant restores fertility; thus, pregnancy requires careful planning and affected women should be managed in tertiary care obstetrics centers working in tight multidisciplinary cooperation with transplant physicians.

A mysterious hematochezia in an adolescent boy: atypical presentation of childhood systemic lupus erythematosus

Thrombosis is a well-known entity in presence of antiphospholipid antibody (APLA) associated with systemic lupus erythematosus (SLE) as a hematological complication. Bleeding manifestations instead of thrombosis is hardly found in literature in presence of APLA seromarkers in SLE. Since these can range from minor bleeding like epistaxis to major life-threatening intracranial bleeding, early diagnosis and prompt treatment is essential to manage such condition. We report a 12 years old boy with no significant past history presented with hematochezia and epistaxis along with pallor requiring blood transfusion. Hematological investigations were normal except for elevated PT, aPTT and INR. Common causes of coagulopathy were ruled out. Upon suspecting systemic diseases, the investigations were carried out which revealed ANA 4+ along with high titre of dsDNA, low C3 and C4 complement and positive anti-?2 glycoprotein, anticardiolipin antibody and lupus anticoagulant. Diagnosis of SLE was made according to ACR-EULAR criteria with no renal involvement. Immunological basis was considered for coagulopathy in this child. He was started on oral prednisolone, hydroxychloroquine and methotrexate. He is now under close monitoring of the coagulation profile for titration of steroid dose. We want to create awareness about the uncommon hematological manifestation of SLE presenting as bleeding diathesis instead of thrombosis through this case report and that can be life threatening too if not treated promptly. A high index of suspicion and careful follow-up may help in preventing adverse outcome of the disease.

Establishment and evaluation of nomogram for differential diagnosis of systemic lupus erythematosus based on laboratory indications / 上海交通大学学报（医学版）

Objective·To establish a nomogram for the differential diagnosis of early systemic lupus erythematosus(SLE)and other autoimmune diseases based on laboratory indications,and to evaluate its efficacy.Methods·A total of 535 SLE patients admitted to the First Hospital of Lanzhou University from January 2017 to December 2021 were selected as SLE group,and 535 patients with other autoimmune diseases during the same period were selected as control group.Basic information and laboratory test indicators of the SLE group and control group were collected and compared.The SLE group and control group were randomly assigned to the training set and the validation set at a ratio of 7∶3,respectively.LASSO regression method and multivariate Logistic regression were used to select the main risk factors of SLE.The nomogram for differential diagnosis of early SLE(SLE nomogram)was established according to the selected main risk factors.Bootstrap method was used to conduct internal repeated sampling for 1 000 times to calibrate the nomogram.The receiver operator characteristic curve(ROC curve)and decision curve analysis(DCA)were performed to evaluate the differential diagnosis ability and the value in clinical application of SLE nomogram,respectively.The"DynNom"package of R language was used to convert the nomogram into an electronic calculator,and its consistency with SLE nomogram was verified by data from 3 groups of patients.Results·LASSO regression and multivariate Logistic regression identified six major risk factors for SLE,including antinuclear antibody(ANA),anti-double-stranded DNA(anti-dsDNA)antibody,anti-ribonucleoprotein antibody/anti-Simth antibody(anti-nRNP/Sm),anti-ribosomal P protein(anti-P)antibody,anti-nucleosome antibody(ANuA)and urinary protein(PRO),which were used to construct the SLE nomogram.The calibration curve of the SLE nomogram had standard errors of 0.009 and 0.015 in the training set and validation set,respectively,and its area under the curve(AUC)was 0.889 and 0.869,respectively.The results of DCA showed that when the risk threshold of SLE nomogram was 0.15?0.95,the model achieved more net benefit.The prediction results of the electronic calculator showed that when ANA(titer 1∶100)was positive in SLE patient No.1,the prevalence was 0.166;when both ANA(titer 1∶100)and ANuA(titer 1∶100)were positive in patient No.2,the prevalence was 0.676;when all of PRO,ANA(titer 1∶100),ANuA(titer 1∶100)and anti-P antibody(titer 1∶100)were positive in patient No.3,the prevalence was 0.990,which was consistent with the differential diagnosis results of the SLE nomogram.Conclusion·The established SLE nomogram based on ANA,anti-dsDNA antibody,anti-nRNP/Sm,anti-P antibody,ANuA and PRO and its conversion into an electronic calculator can effectively distinguish early SLE from other autoimmune diseases,and have important clinical application value.

Systemic lupus erythematosus complicated with pure red cell aplasia:a case report and literature review / 上海交通大学学报（医学版）

This article reports a single case of a patient with systemic lupus erythematosus(SLE)combined with pure red cell aplasia(PRCA),and reviews 51 additional cases of patients reported by domestic and overseas papers from 1974 to 2021.These 52(51+1)cases were analyzed to summarize the epidemiological features,clinical features,laboratory inspections,treatments and prognosis of the patients.The results indicated that among all the 52 cases,cases of SLE combined with PRCA were mostly seen in Asian childbearing age women.The median ages of patients diagnosed with SLE and diagnosed with PRCA were 31.5 years and 36.0 years,respectively.The time interval between the initial diagnosis of SLE and subsequent diagnosis of PRCA was significantly longer than the interval for the initial diagnosis of PRCA,suggesting a delayed onset of SLE in these patients(P=0.042).Various clinical features of the 52 patients were reported,including mostly fatigue,joint pains,Raynaud phenomena and rashes,and SLE maybe combined with autoimmune hemolytic anemia(AIHA),thymoma,hypothyroidism and myasthenia gravis(MG).In these reported cases,laboratory indicators showed higher proportions of antinuclear antibody(ANA),anti double stranded DNA antibody(anti-dsDNA antibody),positive urinary protein and low complement levels.Among the 52 patients,51 cases(98.08%)were treated with glucocorticoids,followed by blood transfusion,cyclosporin A,cyclophosphamide and high-dose intravenous immunoglobulin.Of the 50 patients whose prognoses were reported,44 showed improvement,while 3 treatments were not effective and 3 resulted in death.This article aims to enhance the understanding of SLE combined with PRCA among doctors.

Causal ambiguity: deciphering the etiology of secondary thrombotic microangiopathy with systemic lupus erythematosus and vivax malaria

Hemolytic uremic syndrome (HUS) falls under the spectrum of thrombotic microangiopathy (TMA) characterized by microangiopathic hemolytic anemia, thrombocytopenia, and thrombi in small vessels leading to end-organ damage. It's classified into typical HUS (caused by Shiga toxin-producing E. coli), atypical HUS (due to uncontrolled complement activation), and secondary HUS (sHUS) linked with coexisting conditions. We present a compelling case of a 21-year-old female with fever, jaundice, anemia, thrombocytopenia, and oliguric acute kidney injury (AKI), ultimately diagnosed with Plasmodium vivax malaria. Despite adequate antimalarial therapy, the patient's clinical trajectory remained intricate, characterized by sustained hematological abnormalities and renal dysfunction. A comprehensive assessment revealed Coombs-negative hemolytic anemia. Subsequently, a renal biopsy confirmed TMA. Considering the rarity of vivax malaria causing TMA, an autoimmune workup was conducted, suggesting systemic lupus erythematosus (SLE). Systemic autoimmune disease-associated HUS (SAID-HUS) is a rare entity that exhibits diverse clinical presentations, with SLE being best-described etiology in literature. SLE-associated HUS was considered and was managed with steroids and hydroxychloroquine resulting in significant renal and hematological improvement. This report underscores significance of assessing autoimmune factors in case of secondary TMA, while also shedding light on evolving understanding of vivax malaria's potential relationship with TMA.

Complete heart block: an atypical presentation with an atypical diagnosis

Congenital heart block is frequently associated with underlying structural congenital heart disease or connective tissue disorders. Neonatal lupus is considered a form of passively acquired autoimmune disease in which maternal autoantibodies, namely, anti-Ro and anti-La, cross the placenta and injure the previously normal fetal heart. Here, a young female, 30 years of age, who presented with atypical cardiac manifestations, was diagnosed with complete heart block. She was evaluated thoroughly for its etiology, including structural, medication, infective, thyroid and autoimmune panel. Antinuclear antibody (ANA) was positive by immunofluorescence method with 1:160 titre and with fine speckled pattern. Anti-SSA/Ro antibody was positive with a titre of 30.20 units. Anti-Smith antibody was also reported to be positive. On further investigations, her mother also had positive ANA by Immunofluorescence method with a titre of 1:80, with anti dsDNA, anti Ro/SSA, anti La/SSB positivity. The patient was thus diagnosed as a case of lupus causing her to present with complete heart block in adulthood. Patient was managed with TPI followed by PPI and was discharged with no complications. Neonatal lupus can cause heart block of varying degrees that may be noted in utero presenting as congenital heart block. SLE presenting as congenital heart block in young adults is a rare finding. This case report reviews an adult female of SLE presenting to the emergency for the first time with atypical cardiac manifestations and diagnosed as a case of congenital heart block with laboratory investigations revealing lupus as the primary etiology.

Unusual case of cold agglutination induced hemolysis in a child with systemic lupus erythematosus

Autoimmune hemolytic anemia (AIHA) is a common symptom of systemic lupus erythematosus (SLE), but cold agglutinin disease is uncommon. We describe a rare instance of cold agglutinin causing hemolytic anemia in an eight-year-old girl who had a history of maternal antiphospholipid antibody syndrome. The girl had hemolytic anemia, and the Coombs test revealed both warm and cold antibodies with a significant titer of cold agglutinin. Whole exon sequencing revealed a heterozygous mutation in the GPI gene, which was previously described for non-spherocytic hemolytic anemia, but its significance in SLE is unknown. Patient responded to corticosteroids and azathioprine. The purpose of this article is to highlight this extremely rare SLE manifestation and compare it to previously reported cases.

A rare case report of 32 years old female suffering from lupus panniculitis as an initial sign of systemic lupus erythematosus

This case report describes a rare case of lupus panniculitis (LEP) in a 32-year-old Egyptian lady who did not have any history of chronic illness. The patient initially presented with leg pain, erythematous nodules, and back ulcers. In addition to proteinuria and increased erythrocyte sedimentation rate (ESR) levels, laboratory tests revealed atypical ANA titers and positive results for anti-DNA, anti-Scl 70, anti-SSa, and anti-SSb antibodies. There was a decline in C3 and C4 levels. A physical examination revealed well-defined ulcers on her back and subcutaneous lumps in both of her legs. The diagnosis of LEP was confirmed by a nodule biopsy. Systemic lupus erythematosus (SLE) was finally determined to be the patient's diagnosis in accordance with the classification standards of the American College of Rheumatology. Methylprednisolone therapy resulted in the resolution of ulcers and nodules, as well as normalization of CBC and C3 levels and a drop in ANA titer. In order to enhance patient outcomes, this example highlights the necessity of treating LEP as a symptom of SLE and the value of early diagnosis and treatment.

Lupus anticoagulant-hypoprothrombinemia syndrome associated with systemic lupus erythematosus / Síndrome de LAHS asociado a lupus eritematoso sistêmico

Abstract Introduction: thromboembolic phenomena are among the most common hematologic mani festations in patients with systemic lupus erythematosus (SLE) who have lupus anticoagulant, while hemorrhagic events are less frequent and tend to occur with Factor II deficiency. Lupus anticoagulant-hypoprothrombinemia syndrome (LAHS) is a rare disorder and its association with SLE is uncommon, especially in adults. Case presentation: we present the case of a 19-year-old male patient diagnosed with LAHS associated with SLE, with kidney and skin involvement evidenced by lower extremity purpura and hematuria. Treatment was begun early with corticosteroid pulses, cyclophosphamide and mycophenolate mofetil, with an adequate clinical response. Conclusion: understanding the association between LAHS and SLE helps providers suspect this condition in patients with acquired coagulation disorders and recognize it as the initial manifestation of an underlying systemic disease. Early diagnosis and prompt treatment reduce mortality in these patients. (Acta Med Colomb 2022; 48. DOI:https://doi.org/10.36104/amc.2023.2745).

Resumen Introducción: los fenómenos tromboembólicos hacen parte de las manifestaciones hematológicas más comunes de los pacientes con lupus eritematoso sistémico (LES) que presentan anticoagulante lúpico, mientras que los eventos hemorrágicos son menos frecuentes y suelen manifestarse en pre sencia de deficiencia de factor II. El síndrome de LAHS es una afección rara y su asociación con LES es infrecuente, especialmente en etapa adulta. Presentación del caso: se presenta el caso de un paciente masculino de 19 años diagnosticado con síndrome de LAHS asociado a LES con compromiso renal y cutáneo manifestado por púrpuras en extremidades inferiores y hematuria. Se instauró tratamiento con pulsos de corticoides, ciclofosfamida y micofenolato mofetil de forma temprana observándose una adecuada respuesta clínica. Conclusión: conocer la asociación entre el síndrome de LAHS y LES permite sospechar esta entidad en pacientes con trastornos adquiridos de la coagulación y reconocerla como manifestación inicial de una enfermedad sistémica subyacente. El diagnóstico temprano y tratamiento oportuno reduce la mortalidad en estos pacientes. (Acta Med Colomb 2022; 48. DOI:https://doi.org/10.36104/amc.2023.2745).

Unusual presentation and rare association of pediatric lupus

Systemic lupus erythematosus (SLE) is a chronic multisystem autoimmune inflammatory disorder with diverse clinical manifestations which can lead to significant morbidity and mortality. Children and adolescents represent 15% to 20% of all patients with SLE. Childhood onset SLE is a rare disease with a prevalence of 3.5 to 8.8 per one lakh children. Localized or generalized lymphadenopathy may be an uncommon presenting feature of SLE. None of the classification criteria for SLE include lymphadenopathy as a criterion including the latest 2019 American college of rheumatology (ACR) / European league against rheumatism (EULAR) criteria. Nine-year-old female child presented with history of swelling on both sides of neck associated with fever and easy fatiguability for 2 months. Clinical examination revealed significant bilateral cervical and axillary lymph nodes with hepatomegaly. Excision biopsy of lymph node was suggestive of Kikuchi Fujimoto disease (KFD). As rare association of KFD with SLE is well described in literature, despite negative clinical features and laboratory findings of SLE, including an initial negative ANA IF test, we did a repeat ANA IF, complement (C3) level and dsDNA testing, all of which turned out to be positive, confirming the diagnosis of SLE. The child was started on hydroxychloroquine and oral steroids with dramatic response. During steroid tapering she developed a flare needing the addition of azathioprine. Currently she is in remission and is under follow up. Although rare, lupus lymphadenopathy may be a presenting feature of SLE. This can even antecede the diagnosis of SLE by many years, when the presence of auto antibodies or low complement levels are not detected and are associated with higher disease activity. This emphasizes the importance of high index of suspicion of SLE in cases of lymphadenopathy, enabling early diagnosis and management which is essential for preventing morbidity and mortality.

Posterior segment involvement in systemic lupus erythematosus – A series from South India

Purpose: To report the spectrum of posterior segment manifestations and visual outcomes in a large series of patients with systemic lupus erythematosus (SLE). Methods: Retrospective study at a tertiary referral eye center in south India between 2016 and 2022. Results: Charts of 109 patients diagnosed to have SLE were retrieved from our medical database. Only nine cases of SLE (8.25%) had posterior segment involvement. The male: female ratio was 1:8. The mean age was 28 years. Unilaterality was the most common presentation in eight cases (88.89%). Lupus nephritis was the most common systemic presentation in five cases (55.56%). Antiphospholipid antibodies (APLA) positivity was seen in two cases (22.22%). Ocular manifestations included microangiopathy (cotton wool spots) in one case, occlusive retinal vasculitis with cotton wool spots in four cases (five eyes), optic disc edema with combined venous and arterial occlusion (one case), central retinal vein occlusion with cotton wool spots and hemorrhages (one case), macular edema (four cases), posterior scleritis with optic disc edema and exudative retinal detachment in the posterior pole (one case), and tubercular choroidal granuloma (one case). Treatment included systemic steroids, hydroxychloroquine sulfate (HCQS), and immunosuppression in all cases, blood thinners in two cases, and laser photocoagulation in four cases. HCQS?related retinal toxicity was not seen in any of the 109 cases. Ocular manifestation was the initial presentation of SLE in one case. Visual outcome was poor in three cases. Conclusion: Presence of posterior segment findings in cases with SLE may suggest a severe systemic disease. Early detection and aggressive treatment result in better visual outcomes. Ophthalmologists could play a vital role in guiding systemic therapy.

Association between sleep and obesity in adults / 预防医学

Objective@#To examine the associations of sleep with overweight/obesity and central obesity in adults, so as to provide insights into improving sleep quality and preventing obesity in adults.@*Methods@#Demographics, height, body weight, waist circumstance and sleep status were collected from the Hubei Provincial Surveillance Program for Adult Chronic Diseases and Their Risk Factors in 2020. Subjects' sleep condiction, overweight/obesity and central obesity were descriptively analyzed. The associations of sleep with overweight/obesity and central obesity were examined using a multivariable logistic regression model.@*Results@#A total of 17 789 participants were recruited, with an average age of (56.21±13.05) years, 61.50% women, and mean duration of (7.18±1.56) h/d. There were 7 019 participants with snoring/asphyxia/suffocation (39.46%), 6 108 participants with sleep difficulty (34.34%), 8 064 participants with night waking at least twice (45.33%), 268 participants taking hypnotics (1.51%), and 6 267 participants with early morning awakening and difficulty in sleep again (35.23%), and there were 8 960 participants with overweight/obesity (50.37%) and 6 148 participants with central obesity (34.56%). Multivariable logistic regression analysis showed that sleep duration of <7 h/d (OR=1.081, 95%CI: 1.007-1.159), snoring/asphyxia/suffocation (OR=2.367, 95%CI: 2.222-2.521), and night waking at least twice (OR=1.106, 95%CI: 1.028-1.191) significantly correlated with overweight/obesity, and sleep duration of >8 h/d (OR=0.834, 95%CI: 0.761-0.913), snoring/asphyxia/suffocation (OR=2.153, 95%CI: 2.019-2.297), and night waking at least twice (OR=1.193, 95%CI: 1.105-1.288) were statistically associated with central obesity.@*Conclusion@#Sleep duration, snoring/asphyxia/suffocation and night waking are associated with overweight/obesity and central obesity.

Secondary herpes zoster wound cured by allogeneic platelet-rich plasma: a case report / 中国输血杂志

【Objective】 To investigate the feasibility of allogeneic platelet-rich plasma (PRP) for the treatment of herpes zoster wounds secondary to systemic lupus erythematosus (SLE), especially large ulcer wounds. 【Methods】 The treatment process of a patient with massive herpes zoster wounds in perineum and hip accompanied by extensive soft tissue necrosis secondary to SLE was retrospectively analyzed. The clinical efficacy of allogeneic PRP was explored combined with treatment key points and literature review. 【Results】 The patient′s wound bed was prepared until the wound was fresh, then treated externally with allogeneic PRP 3 times a week. The wound was healed completely after 42 days. 【Conclusion】 In the case of autologous PRP unavailable or unsuitable, allogeneic PRP is a safe alternative, which can effectively promote tissue regeneration, and this patient achieved curative effect in a short period of time.

Research progress of tradition Chinese medicine treatment mechanism of systemic lupus erythematosus based on biomarkers / 药学实践与服务

Systemic lupus erythematosus (SLE) is an autoimmune disease with multiple organ involvement. There are still many limitations and individual differences in the treatment based on glucocorticoids and immunosuppressants. In recent years, more and more studies have shown that the combination of traditional Chinese medicine in the treatment of SLE has the advantages of good efficacy, low adverse reactions, and high safety. However, the exact regulatory mechanism and combined traditional Chinese medicine in the treatment of SLE are still unclear. This paper reviews the research on the mechanism of traditional Chinese medicine in the treatment of SLE from metabonomic, immune cells, lymphocyte factors and apoptosis, etc, provides ideas for exploring the mechanism of traditional Chinese medicine in the treatment of SLE with modern methods.

Clinical efficacy of cognitive and behavioral therapy assisted by five-tone therapy for insomnia in sub-healthy people / 中国基层医药

Objective:To investigate the clinical efficacy of cognitive and behavioral therapy assisted by five-tone therapy for insomnia in sub-healthy people based on digital polysomnography observation.Methods:The clinical data of 70 sub-healthy people with insomnia who received treatment in Wenzhou Hospital of Traditional Chinese Medicine, Zhejiang University of Traditional Chinese Medicine from June 2019 to June 2021 were retrospectively analyzed. These patients were divided into a control group and an observation group ( n = 35 per group) according to different treatment methods. The control group was treated with cognitive and behavioral therapy, while the observation group was treated with cognitive and behavioral therapy assisted by five-tone therapy. Clinical efficacy, syndrome score, sleep quality, quality of life, and digital polysomnography index levels were compared between the two groups. Results:After 4 weeks of treatment, the total effective rate in the observation group was 97% (29/35), which was significantly higher than 83% (34/35) in the control group ( χ2 = 8.77, P = 0.012). The syndrome score in the observation group was (3.26 ± 2.12) points, which was significantly lower than (6.00 ± 1.68) points in the control group ( t = 5.99, P < 0.001). The Pittsburgh Sleep Quality Index score in the observation group was (3.46 ± 2.36) points, which was significantly lower than (5.60 ± 3.35) points in the control group ( t = 3.09, P = 0.003). The WHOQOL-BREF score in the observation group was (67.00 ± 5.26) points, which was significantly higher than (61.54 ± 4.63) points in the control group ( t = 4.61, P < 0.001). Total sleep time, sleep efficiency, and sleep latency in the observation group were superior to those in the control group ( t = 4.56, 5.57, 3.96, all P < 0.05). Conclusion:Cognitive and behavioral therapy assisted by five-tone therapy can markedly reduce insomnia severity and improve the quality of life in sub-healthy people with insomnia. The evaluation of insomnia status based on digital polysomnography indicators can effectively objectively evaluate the causes of insomnia, which is of great significance for guiding clinical treatment.

A Teenage Boy with Systemic Lupus Erythematosus Complicated with Acquired von Willebrand Syndrome: A Rare Case and Challenging in Making Diagnosis

@#In systemic lupus erythematosus (SLE), haematological abnormalities are frequent, although they are an uncommon cause of acquired von Willebrand syndrome (AVWS). AVWS is a rare condition that can cause a bleeding disorder. We presented a case of AVWS in the early diagnosis of SLE. One month before admission, the patient had a history of recurrent epistaxis. He presented to the hospital with symptomatic anaemia and was noted to have severe anaemia with iron deficiency. During hospitalisation, recurrent epistaxis recurred and was found to have prolonged activated partial thromboplastin time (aPTT), presence of lupus anticoagulant (LA), and lower von Willebrand factor (VWF), and factor 8 (VIII) levels. Simultaneously, he was diagnosed with SLE based on Systemic Lupus International Collaborating Clinics (SLICC) criteria. He underwent blood transfusions and was treated with immunosuppressive drugs such as steroids, mycophenolate mofetil, and an anti-fibrinolytic agent; he subsequently stopped bleeding and showed clinical improvement.

Blood levels of brain-derived neurotrophic factor (BDNF) in systemic lupus erythematous (SLE): a systematic review and meta-analysis

Abstract Objectives BDNF has been implicated in the pathophysiology of systemic lupus erythematosus (SLE), especially its neuropsychiatric symptoms. The purpose of this study was to investigate the profile of blood BDNF levels in patients with SLE. Methods We searched PubMed, EMBASE, and the Cochrane Library for papers that compared BDNF levels in SLE patients and healthy controls (HCs). The Newcastle-Ottawa scale was used to assess the quality of the included publications, and statistical analyses were carried out using R 4.0.4. Results The final analysis included eight studies totaling 323 healthy controls and 658 SLE patients. Meta-analysis did not show statistically significant differences in blood BDNF concentrations in SLE patients compared to HCs (SMD 0.08, 95% CI [− 1.15; 1.32], P value = 0.89). After removing outliers, there was no significant change in the results: SMD -0.3868 (95% CI [− 1.17; 0.39], P value = 0.33. Univariate meta-regression analysis revealed that sample size, number of males, NOS score, and mean age of the SLE participants accounted for the heterogeneity of the studies (R2 were 26.89%, 16.53%, 18.8%, and 49.96%, respectively). Conclusion In conclusion, our meta-analysis found no significant association between blood BDNF levels and SLE. The potential role and relevance of BDNF in SLE need to be further examined in higher quality studies.

Impact of baseline fluorescent antinuclear antibody positivity on the clinical outcome of patients with primary autoimmune hemolytic anemia

Abstract Introduction Autoimmune haemolytic anaemia (AIHA) is an autoimmune disorder that can present in primary or secondary forms. The literature looking at impact of baseline fluorescent antinuclear antibody (FANA) positivity on outcomes of AIHA patients is infrequent. Objective To study the impact of baseline FANA positivity in patients with primary AIHA. Method A prospective cohort study involving 29 consecutive primary AIHA patients presenting to the Haematology department from 2013 to 2015 was analysed. After recording baseline investigations including fluorescent ANA, all patients were treated as per the standard therapeutic protocols. Clinical remission, disease free survival, relapse, mortality were compared between the FANA positive and FANA Negative AIHA groups. Results Baseline FANA positivity was found in 17 patients (58.62%). Both the groups were comparable in terms of age, sex, Hemoglobin, LDH at presentation, number of lines of treatment needed and duration of follow up. Evan's syndrome was seen in six of FANA positive patients which was statistically significant (0 v/s 6, p= 0.023). FANA positive patients had significantly higher rates of relapse per patient month follow up (1.22 v/s 3.57, p= 0.023) and lower rates of complete response (83.33% v/s 35.29%, p= 0.0118) and relapse free survival at five years. Morbidity and mortality were numerically higher in FANA positive patients. Conclusion Baseline FANA positivity among AIHA patients was found to be associated with lower complete response rates and higher relapse rates with possible higher rates of morbidity. Presence of FANA will give us prognostic value and help us in deciding the treatment options.

Risk factors of systemic lupus erythematosus: an overview of systematic reviews and Mendelian randomization studies

Abstract Background The etiology of systemic lupus erythematosus is complex and incurable. A large number of systematic reviews have studied the risk factors of it. Mendelian randomization is an analytical method that uses genetic data as tool variables to evaluate the causal relationship between exposure and outcome. Objective To review the systematic reviews and Mendelian randomization studies that focused on the risk factors of systemic lupus erythematosus and shed light on the development of treatments for its prevention and intervention. Methods From inception to January 2022, we systematically searched MEDLINE (via PubMed) and Embase for related systematic reviews and Mendelian randomization studies. Extract relevant main data for studies that meet inclusion criteria. The quality of systematic reviews was assessed by using Assessment of Multiple Systematic Reviews 2 (AMSTAR-2). Finally, the risk factors are scored comprehensively according to the results' quantity, quality, and consistency. Results Our study involved 64 systematic reviews and 12 Mendelian randomization studies. The results of systematic reviews showed that diseases (endometriosis, atopic dermatitis, allergic rhinitis), lifestyle (smoking, drinking, vaccination), and gene polymorphism influenced the incidence of systemic lupus erythematosus. The results of Mendelian randomization studies identified the role of disease (periodontitis, celiac disease), trace elements (selenium, iron), cytokines (growth differentiation factor 15), and gut microbiome in the pathogenesis of systemic lupus erythematosus. Conclusion We should pay attention to preventing and treating systemic lupus erythematosus in patients with endometriosis, celiac disease, and periodontitis. Take appropriate dietary supplements to increase serum iron and selenium levels to reduce the risk of systemic lupus erythematosus. There should be no excessive intervention in lifestyles such as smoking and drinking.