Doença autoimune de múltiplos órgãos tipicamente associada a vasculopatia e produção de autoanticorpos. A maioria dos pacientes apresenta anticorpos antinucleares (ANA). A presença de anticorpos anti-dsDNA ou anti-Smith é altamente específica.
Introdução
O que você precisa saber de cara
O lúpus eritematoso sistêmico (LES) é uma doença crônica que causa inflamação nos tecidos conjuntivos, como a cartilagem e o revestimento dos vasos sanguíneos, que fornecem força e flexibilidade às estruturas em todo o corpo. Os sinais e sintomas do LES variam entre as pessoas com a condição e podem envolver muitos órgãos e sistemas, incluindo a pele, as articulações, os rins, os pulmões, o sistema nervoso central e o sistema formador de sangue (hematopoiético). O LES é uma das muitas condições chamadas distúrbios autoimunes que ocorrem quando o sistema imunológico ataca os próprios tecidos e órgãos do corpo.
O LES pode se manifestar inicialmente como fadiga extrema (cansaço), uma sensação vaga de desconforto ou doença (mal‑estar), febre, perda de apetite e perda de peso. A maioria das pessoas com a condição também apresenta dor nas articulações, tipicamente afetando as mesmas articulações em ambos os lados do corpo, além de dor e fraqueza muscular. Problemas de pele são comuns no LES. Uma característica típica é uma erupção cutânea plana e vermelha que se estende pelas bochechas e pela ponte do nariz, chamada de “erupção em borboleta” devido ao seu formato. A erupção, que geralmente não dói nem coça, costuma aparecer ou tornar‑se mais pronunciada quando exposta à luz solar. Outros problemas de pele que podem ocorrer no LES incluem depósitos de cálcio sob a pele (calcinosis), vasos sanguíneos danificados (vasculite) na pele e pequenos pontos vermelhos chamados petéquias. As petéquias são causadas por uma escassez de células envolvidas na coagulação (plaquetas), o que leva a sangramento sob a pele. As pessoas com a condição também podem apresentar queda de cabelo (alopecia) e úlceras abertas (ulcerações) no revestimento úmido (mucosas) da boca, do nariz ou, menos frequentemente, dos genitais.
Cerca de um terço das pessoas com LES desenvolvem doença renal (nefrite). Problemas cardíacos também podem ocorrer no LES, incluindo inflamação da membrana em forma de saco ao redor do coração (pericardite) e anomalias nas válvulas cardíacas, que controlam o fluxo sanguíneo no coração. A doença cardíaca causada pelo acúmulo de gordura nos vasos sanguíneos (aterosclerose), que é muito comum na população geral, é ainda mais frequente em pessoas com LES. A inflamação característica do LES também pode danificar o sistema nervoso e pode resultar em sensação anormal e fraqueza nos membros (neuropatia periférica); convulsões; AVC; e dificuldade em processar, aprender e lembrar informações (comprometimento cognitivo). Ansiedade e depressão também são comuns no LES.
As pessoas com LES têm episódios nos quais a condição piora (exacerbações) e outros momentos em que melhora (remissões). No geral, o LES piora gradualmente ao longo do tempo, e o dano aos principais órgãos do corpo pode ser ameaçador à vida.
Fonte: MedlinePlus Genetics (NLM/NIH), traduzidoDoença autoimune de múltiplos órgãos tipicamente associada a vasculopatia e produção de autoanticorpos. A maioria dos pacientes apresenta anticorpos antinucleares (ANA). A presença de anticorpos anti-dsDNA ou anti-Smith é altamente específica.
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 71 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 155 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Genes associados
36 genes identificados com associação a esta condição. Padrão de herança: Not applicable.
Curadoria gene-doença
fontes oficiaisSystemic lupus erythematosus 6
A chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys and serosal membranes. It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system. The disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow.
Ehlers-Danlos syndrome, periodontal type, 1
A form of Ehlers-Danlos syndrome, a connective tissue disorder characterized by hyperextensible skin, atrophic cutaneous scars due to tissue fragility and joint hyperlaxity. EDSPD1 is characterized by the association of typical features of Ehlers-Danlos syndrome with gingival recession and severe early-onset periodontal disease, leading to premature loss of permanent teeth. EDSPD1 inheritance is autosomal dominant.
C1q deficiency 1
An autosomal recessive disorder caused by impaired activation of the complement classical pathway. It generally leads to severe immune complex disease characterized by recurrent skin lesions, chronic infections, an increased risk of systemic lupus erythematosus, and glomerulonephritis.
Nephrolithiasis, calcium oxalate, 1
A form of nephrolithiasis, a condition in which urinary supersaturation leads to stone formation in the urinary system. Patients manifest acute renal colic with severe pain originating in the flank. Patients with small, non-obstructing stones or those with staghorn calculi may be asymptomatic. The majority of renal calculi contain calcium. CAON1 is characterized by calcium oxalate kidney stones.
Systemic lupus erythematosus 16
A rare autosomal recessive form of systemic lupus erythematosus with childhood onset, characterized by high frequency of anti-neutrophil cytoplasmic antibodies and lupus nephritis. Systemic lupus erythematosus is a chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys and serosal membranes. The disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow.
Autoinflammatory syndrome, familial, Behcet-like 1
An autosomal dominant, autoinflammatory disorder with early onset, characterized by ulceration of mucosal surfaces, particularly in the oral and genital areas. Additional variable features include skin rash, uveitis, and polyarthritis.
Systemic lupus erythematosus
A chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys and serosal membranes. It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system. The disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow.
Aicardi-Goutieres syndrome 1
A form of Aicardi-Goutieres syndrome, a genetically heterogeneous disease characterized by cerebral atrophy, leukoencephalopathy, intracranial calcifications, chronic cerebrospinal fluid (CSF) lymphocytosis, increased CSF alpha-interferon, and negative serologic investigations for common prenatal infection. Clinical features as thrombocytopenia, hepatosplenomegaly and elevated hepatic transaminases along with intermittent fever may erroneously suggest an infective process. Severe neurological dysfunctions manifest in infancy as progressive microcephaly, spasticity, dystonic posturing and profound psychomotor retardation. Death often occurs in early childhood.
Immunodeficiency 74, COVID19-related, X-linked
An X-linked recessive immunologic disorder characterized by impaired type I and type II interferon responses due to defective TLR7 signaling. Individuals with TLR7 deficiency develop severe respiratory insufficiency in response to infection with SARS-CoV-2 coronavirus. Death from respiratory failure may occur.
Systemic lupus erythematosus 11
A chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys and serosal membranes. It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system. The disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow.
Systemic lupus erythematosus
A chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys and serosal membranes. It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system. The disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow.
Angelman syndrome
A neurodevelopmental disorder characterized by severe motor and intellectual retardation, ataxia, frequent jerky limb movements and flapping of the arms and hands, hypotonia, seizures, absence of speech, frequent smiling and episodes of paroxysmal laughter, open-mouthed expression revealing the tongue.
Systemic lupus erythematosus
A chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys and serosal membranes. It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system. The disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow.
Systemic lupus erythematosus
A chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys and serosal membranes. It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system. The disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow.
Systemic lupus erythematosus
A chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys and serosal membranes. It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system. The disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow.
Systemic lupus erythematosus
A chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys and serosal membranes. It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system. The disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow.
Autoimmune disease, multisystem, infantile-onset, 4
An autosomal recessive immunologic disorder characterized by lymphoproliferative autoimmunity and onset of various autoimmune diseases in early childhood. Death from autoimmune pneumonitis may occur.
Multiple myeloma
A malignant tumor of plasma cells usually arising in the bone marrow and characterized by diffuse involvement of the skeletal system, hyperglobulinemia, Bence-Jones proteinuria and anemia. Complications of multiple myeloma are bone pain, hypercalcemia, renal failure and spinal cord compression. The aberrant antibodies that are produced lead to impaired humoral immunity and patients have a high prevalence of infection. Amyloidosis may develop in some patients. Multiple myeloma is part of a spectrum of diseases ranging from monoclonal gammopathy of unknown significance (MGUS) to plasma cell leukemia.
Maturity-onset diabetes of the young 11
A form of diabetes that is characterized by an autosomal dominant mode of inheritance, onset in childhood or early adulthood (usually before 25 years of age), a primary defect in insulin secretion and frequent insulin-independence at the beginning of the disease.
Complement component 4A deficiency
A rare defect of the complement classical pathway associated with the development of autoimmune disorders, mainly systemic lupus with or without associated glomerulonephritis.
Systemic lupus erythematosus
A chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys and serosal membranes. It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system. The disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow.
Inflammatory bowel disease 14
A chronic, relapsing inflammation of the gastrointestinal tract with a complex etiology. It is subdivided into Crohn disease and ulcerative colitis phenotypes. Crohn disease may affect any part of the gastrointestinal tract from the mouth to the anus, but most frequently it involves the terminal ileum and colon. Bowel inflammation is transmural and discontinuous; it may contain granulomas or be associated with intestinal or perianal fistulas. In contrast, in ulcerative colitis, the inflammation is continuous and limited to rectal and colonic mucosal layers; fistulas and granulomas are not observed. Both diseases include extraintestinal inflammation of the skin, eyes, or joints.
Systemic lupus erythematosus 9
A chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys and serosal membranes. It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system. The disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow.
Medicamentos aprovados (FDA)
1 medicamento encontrado nos registros da FDA americana.
Variantes genéticas (ClinVar)
129 variantes patogênicas registradas no ClinVar.
Classificação de variantes (ClinVar)
Distribuição de 204 variantes classificadas pelo ClinVar.
Vias biológicas (Reactome)
102 vias biológicas associadas aos genes desta condição.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
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Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Lúpus eritematoso sistêmico
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Publicações mais relevantes
Mostrando amostra de 200 publicações de um total de 29.664
Magnetic Resonance Imaging-Negative Lupus Myelitis: A Narrative Review.
Magnetic resonance imaging (MRI)-negative lupus myelitis (MNLM) is a rare neurological manifestation of systemic lupus erythematosus (SLE). Diagnosing MNLM without prior systemic involvement remains challenging. This review aims to analyze all published cases and compare MNLM patients with (Group A) and without prior SLE diagnosis (Group B), emphasizing on demographic and clinical profiles, treatment, and outcomes. A PubMed literature review on MNLM was conducted. Demographic profile, clinical presentations, and management were analyzed. MNLM patients were categorized into Group A and B and compared using univariate and multivariate comparison methods, where a P value <0.05 was considered significant. In total, 41 cases were analyzed. The cohort was predominantly female (8:1 ratio, mean age 31.22 ± 11.91 years, range 15-59 years). Thoracic cord involvement was the most common (55%), with myelitis presenting as the first manifestation in 49% of patients. Clinical presentations were predominantly subacute (71%) with partial tract involvement in 63%. Group A demonstrated significantly higher cervical cord localization (P = 0.019) compared to Group B's thoracic involvement (P = 0.041). Antiphospholipid syndrome (APS) antibody-positive patients showed 3.2-fold higher odds for partial recovery (P = 0.16). Logistic regression analysis identified Anti Smith/ Ribonucleoprotein/ Ribosomal (Sm/RNP/Rib) antibodies as independent risk factors for poor complete recovery (odds ratio (OR) = 0.61), whereas cervical cord localization independently improved odds for complete recovery (OR = 3.2). MNLM presents with variable clinical manifestations suggesting selective tract involvement. Group A exhibits higher cervical involvement, while Group B shows predominantly thoracic involvement. APS antibodies favor partial recovery, cervical localization predicts complete recovery, and Sm/RNP/Rib antibodies are independent predictors of poor recovery outcomes.
Long-term outcomes of hydroxychloroquine dose reduction in Puerto Ricans with systemic lupus erythematosus: 6-year follow-up.
To reduce the risk of hydroxychloroquine (HCQ)-induced retinopathy, the American Academy of Ophthalmology recommends a maximum HCQ dose of ≤5.0 mg/kg/day. Previously, we reported that reducing HCQ to this threshold did not affect clinical outcomes in Puerto Rican patients with systemic lupus erythematosus (SLE) after 2 years. To evaluate longer term effects, we extended follow-up to 6 years post-HCQ dose adjustment. We conducted a retrospective study on a cohort of Puerto Rican SLE patients whose HCQ doses were adjusted to ≤5.0 mg/kg/day. Disease activity (per-Systemic Lupus Erythematosus Disease Activity Index), lupus exacerbations, hospitalisations, disease damage (per SLICC/ACR Damage Index) and pharmacologic treatments were assessed. Outcomes during the 4-year extension were compared with preadjustment and initial 2-year postadjustment periods. A control group of SLE patients who did not require HCQ dose adjustment was also included. Among the 304 patients included in the study, 60 required HCQ dose adjustment, whereas 244 patients did not require modification of their HCQ regimen and served as controls. During the 4-year extension period, a small but statistically significant increase in disease damage was observed in patients who underwent HCQ dose adjustment compared with earlier follow-up periods. Tacrolimus exposure significantly increased, whereas other immunosuppressive therapies remained stable. No increase in disease activity, lupus exacerbations or hospitalisations was observed. Clinical features were comparable between patients who underwent HCQ dose adjustment and those who did not, although patients in the adjusted-dose group had lower weight-based daily HCQ dosing and were less likely to develop HCQ-induced retinopathy. In this cohort, reducing HCQ to ≤5.0 mg/kg/day was associated with a slight increase in long-term disease damage. No adverse outcomes on disease activity, exacerbations or hospitalisations were observed, supporting the long-term safety of HCQ dose adjustment in Puerto Rican SLE patients.
'Getting comfortable with the uncomfortable': barriers, facilitators and influences on reproductive health discussions from Hispanic women with systemic lupus erythematosus in the USA - a qualitative study.
Systemic lupus erythematosus (SLE) primarily affects women of reproductive age, necessitating complex conversations surrounding pregnancy and contraception use. Hispanic women in the USA bear a disproportionate and severe burden of SLE, facing language and cultural barriers to care, and higher rates of unplanned pregnancy and under-representation in reproductive health research. This study explores patient-reported barriers and facilitators to reproductive health conversations as well as their attitudes and perceptions on how these conversations can be improved. We conducted 30 semistructured interviews in both English and Spanish with Hispanic women aged 18-45 with SLE. Participants were recruited from Los Angeles General Medical Center and Lupus LA. Eligible participants were heterosexually active or considering pregnancy within 3 years. Interviews were analysed using grounded theory to identify themes related to barriers and facilitators in clinical reproductive health interactions. Three major themes emerged: (1) barriers to reproductive health conversations were often driven by competing clinical priorities as well as discomfort discussing sensitive topics; (2) facilitators to reproductive health conversations included the presence of genuine clinician-patient rapport, well-controlled disease activity and patient proactiveness; (3) areas of improvement included both a more encompassing and thorough data-driven approach to reproductive health discussions throughout disease course. Findings reported herein underscore the importance of early, individualised reproductive health counselling for women with SLE. Enhanced clinician communication with sensitive topics, refined patient education and frequent interdisciplinary collaboration can help address the emotional, medical and informational needs of Hispanic women with SLE navigating reproductive decisions.
Clinical, laboratory, and histopathological characteristics of pediatric lupus nephritis: a retrospective study in a national referral center in Mexico.
Pediatric lupus nephritis (LN) remains a major cause of morbidity and mortality, yet data from Latin American populations are limited. This study aimed to describe the clinical, laboratory, and histopathological characteristics of pediatric LN and identify prognostic factors associated with renal replacement therapy (RRT). We conducted a retrospective cross-sectional study including patients <18 years of age with LN diagnosed between 2020 and 2024 at a national referral center in Mexico. Demographic, clinical, immunological, histopathological, and therapeutic variables at diagnosis were analyzed. Multivariable logistic regression was performed to identify predictors of RRT. Eighty patients were included (83% female; mean age 15.1 ± 2.8 years). Median proteinuria was 41 mg/m²/h; hematuria and leukocyturia were present in 46% and 26% of patients, respectively. All patients were ANA positive, with frequent hypocomplementemia and elevated anti-double-stranded DNA titers. Among biopsied patients, class IV was the most common histological subtype (60%). Proliferative forms were associated with reduced glomerular filtration rate (<90 mL/min/1.73 m²; p = 0.012) and higher activity index scores (p = 0.04), while chronicity indices were low. Fifteen patients (18.8%) required RRT, and mortality was 6.25%. In multivariable analysis, hypoalbuminemia (<2.5 g/dL) was independently associated with RRT (OR 6.04; 95% CI 1.33-27.50; p = 0.020). This study represents one of the largest pediatric LN cohorts reported from Mexico. Proliferative forms were associated with greater inflammatory activity and impaired renal function at diagnosis. Hypoalbuminemia emerged as a simple and accessible biomarker for early risk stratification of severe renal outcomes.
Advances in endoplasmic reticulum stress response in immune-associated skin diseases: Mini-review.
The skin protects the body from external allergens and pathogens. Disruption of the physiological state of the skin is a hallmark of many immune-associated skin disorders and is well-characterized in disease states such as psoriasis, vitiligo, and systemic lupus erythematosus (SLE). The endoplasmic reticulum (ER) is a crucial organelle within the cell responsible for functions such as protein synthesis, modification and folding. Endoplasmic reticulum stress (ERS) is characterized as an intracellular condition of stress that typically arises from aberrant protein synthesis or environmental changes. Various stimuli such as ischemia, hypoxia, and oxidative stress can trigger ERS, resulting in the misfolding of newly synthesized proteins and the activation of the unfolded protein response (UPR). Emerging evidence indicates that UPR signaling modules have fundamental roles in multiple physiological processes beyond the homeostatic control of protein folding including the pathogenesis of several immune-related skin disorders. We summarize the potential role of ER stress and the UPR in immune-associated skin diseases. This review aims to explore the mechanisms underlying ERS response in these immune-associated skin diseases, offering valuable insights for further research. Understanding the molecular mechanism of UPR activation and ER stress may represent a novel target for drug discovery and innovative therapeutic strategies in the context of immune-associated skin diseases.
Publicações recentes
Efficacy and Safety of Mizoribine in the Treatment of Lupus Nephritis: A Systematic Review and Meta-Analysis.
Unusual Presentation of Pustular Psoriasis on the Face: A Case Report.
Contraception in Women With Medical Comorbidities: Clinical Risk Stratification and Individualized Therapeutic Strategies.
📖 RevisãoEffusive-constrictive pericarditis in a patient with late-onset systemic lupus erythematosus.
🥉 Relato de casoSerum RNASE1 as a biomarker for disease activity and lupus nephritis in systemic lupus erythematosus.
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Two-Sample Bidirectional Mendelian Randomization Analysis of the Causal Relationship Between Immune Cell Phenotypes and Systemic Lupus Erythematosus.
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CASE (Philadelphia, Pa.)Integrative Use of Belimumab and Individualized Traditional Chinese Medicine in Refractory Pediatric Lupus Nephritis: A Case Report.
Journal of inflammation researchPrevalence and Risk Factors of Systemic Lupus Erythematosus in Patients With Chronic Cutaneous Lupus: A Bicentric Study Employing Three Classification Criteria.
International journal of dermatologyDiagnostic classification of dermatomyositis with and without electrodiagnostic study: real-world clinical practice.
Singapore medical journalMagnetic Resonance Imaging-Negative Lupus Myelitis: A Narrative Review.
Annals of Indian Academy of NeurologyLong-term outcomes of hydroxychloroquine dose reduction in Puerto Ricans with systemic lupus erythematosus: 6-year follow-up.
Lupus science & medicine'Getting comfortable with the uncomfortable': barriers, facilitators and influences on reproductive health discussions from Hispanic women with systemic lupus erythematosus in the USA - a qualitative study.
Lupus science & medicineTapering strategies for immunosuppressive, corticosteroid, and biologic therapy in lupus nephritis: a national survey of rheumatology and nephrology practices in Saudi Arabia.
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Amino acidsFrequency and clinical associations of Epstein-Barr virus molecular detection in systemic lupus erythematosus: A follow-up study.
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Antibodies (Basel, Switzerland)Therapeutic Potential of Stem Cells From Human Exfoliated Deciduous Teeth and Their Derivatives in Immune-Mediated Inflammatory Diseases: Mechanisms and Perspectives.
Advanced biologyThe research progress on the role of glucose-6-phosphate dehydrogenase in immune regulation.
PeerJEpstein-Barr Virus Transformed B Cells From Systemic Lupus Erythematosus and Multiple Sclerosis Patients Differ in EBV Lytic and Latency Marker Expression.
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Expert reviews in molecular medicineEfficacy of telitacicept in a refractory systemic lupus erythematosus patient with lupus nephritis and subsequent pregnancy: a case-based review.
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Journal of inflammation researchLupus Enteritis as an Initial Manifestation in a Previously Undiagnosed Case of Systemic Lupus Erythematosus: A Case Report.
The Indian journal of radiology & imagingLUNELORD: A descriptive, prospective study on the demographics, disease characteristics and health-related quality of life of patients with LUpus NEphritis and long-term ORgan damage in rheumatology clinics in the Arabian Gulf.
LupusSLAMF1 at the crossroads of immunity and disease: Biology, pathology, and therapeutic opportunities.
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Pediatric rheumatology online journalAdvances in endoplasmic reticulum stress response in immune-associated skin diseases: Mini-review.
Tissue & cellThe Relationship Between Climate Change and Rheumatic Disease: A Review.
International archives of allergy and immunologyGenetic association of Angiopoietin-2 rs12674822 and rs3739390 polymorphisms with systemic lupus erythematosus risk: a preliminary single-center study in Egyptian patients.
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Current rheumatology reviewsThe Auditory Manifestations of Autoimmune Rheumatologic Diseases: A Review of Current Evidence.
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Current molecular medicineSerum epitope-specific anti-recoverin autoantibodies as biomarkers for lupus retinopathy.
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Journal of microbiology, immunology, and infection = Wei mian yu gan ran za zhiDivergent comorbidity profiles in neuromyelitis optica spectrum disorder and multiple sclerosis: A large single-centre cohort from Turkey.
Multiple sclerosis and related disordersmicroRNA-155: a versatile noncoding RNA.
Molecular biology reportsB cell receptor signaling in autoimmune rheumatic diseases: regulatory mechanisms and therapeutic targeting.
Frontiers in immunologyBelimumab-induced remission in refractory lupus mesenteric vasculitis: a case report with 49-month follow-up.
Frontiers in immunologyUnveiling biomarkers of telitacicept's efficacy in SLE treatment through proteomics and metabolomics.
Frontiers in immunologyImmunopathogenesis of accelerated atherosclerosis in systemic lupus erythematosus: from innate and adaptive dysregulation to clinical implications.
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Clinical rheumatologyRisk factor of osteoporosis in systemic lupus erythematosus patients: a cross-sectional study in a tertiary hospital in Indonesia.
Lupus science & medicine[Perspectives and strategic approaches on unmet therapeutic needs in systemic lupus erythematosus].
Zhonghua yi xue za zhiAnifrolumab in systemic lupus erythematosus: real-world evidence from a Spanish multicentre cohort of 206 patients and literature review.
RMD openAnti-CD19 CAR T cell therapy for refractory SLE-ITP.
Med (New York, N.Y.)GBP2 promotes podocyte pyroptosis and contributes to the pathogenesis of pediatric lupus nephritis.
PloS oneGut and oral microbiota characterized in systemic lupus erythematosus patients from India: A pilot study.
LupusMultidisciplinary advances in oral health management in patients with systemic lupus erythematosus: a comprehensive review.
Quintessence international (Berlin, Germany : 1985)A Case of Disseminated Trichophytosis With Vascular Invasion and Multiple Ulcers: Case Report and Literature Review.
The Journal of dermatologyScavenging of Isolevuglandins Attenuates Neutrophil Migration and Neutrophil Extracellular Trap Formation in Systemic Lupus Erythematosus.
ACR open rheumatologyCellular therapies for rheumatic disease.
Current opinion in rheumatologyImpact of Cervical Radiculopathy on the Risk of Cubital Tunnel Syndrome Following Distal Humerus Fracture: A Retrospective Cohort Analysis of 165 Million Patients.
Hand (New York, N.Y.)Characterizing the effects of genetic liability to autoimmune conditions on pregnancy outcomes using Mendelian randomization.
BMC medicinePrasugrel inhibits TLR7-driven autoimmunity in systemic lupus erythematosus by acetylating cGAS.
Nature communicationsNeutrophil transcriptomics in SLE reveals intrinsic disease signatures, shared ex vivo adaptation, and transcriptional reset after CAR T-cell therapy.
Annals of the rheumatic diseasesTwelve-month follow-up after telitacicept withdrawal in patients with SLE: a single-centre study.
Lupus science & medicineOutcomes of Transcatheter Aortic Valve Replacement in Patients with Rheumatologic Diseases: A Large-Scale Propensity-Matched Analysis.
The American journal of the medical sciencesRapid response of pyoderma gangrenosum associated with systemic lupus erythematosus to anifrolumab.
Medicina clinicaComorbidities in Discoid Lupus: A Systematic Review and Meta-analysis of Prevalence and Association.
Dermatology and therapyBone microarchitecture impairment in juvenile systemic lupus erythematosus patients: A comparative study.
LupusCytomegalovirus Infection Mimics Manifestations of Underlying Diseases in Patients With Autoimmune Disorders: A Case Report and Literature Review.
Immunity, inflammation and diseaseHydroxychloroquine withdrawal triggers pregnancy-associated pulmonary arterial hypertension in systemic lupus erythematosus: a case report and exploration of the Complement-EndMT axis.
Frontiers in pharmacologyVitamin D3 as an immunomodulatory agent: molecular mechanisms, clinical translation, and precision therapeutic strategies.
Frontiers in immunologyNovel TLR7 gain-of-function variant and review of the associated disease spectrum.
Journal of human immunityAcute Myocardial Infarction Secondary to Triple Vessel Coronary Artery Disease in a 31-year-old Female with Systemic Lupus Erythematosus: Case Report and Review of Literature.
Acta medica PhilippinaElevated Peripheral CD161+ Treg Cells and Their Clinical Significance in Behçet Syndrome.
ACR open rheumatologyHLA - C*03:02:02 and DPA1*01:03:01 protect against discoid rash in Thai Systemic Lupus Erythematosus patients.
Scientific reportsComparative 1H NMR Lipoprotein Profiling of Rheumatoid Arthritis and Systemic Lupus Erythematosus Patients Highlights Disease-Specific Lipoprotein Dysregulation.
Journal of proteome researchThe crosstalk between iron metabolism and immune tolerance in autoimmunity.
Inflammation research : official journal of the European Histamine Research Society ... [et al.]Systemic Lupus Erythematosus and Epstein-Barr Virus: Recent Discoveries and Open Questions.
Clinical chemistryCentral memory T cells: stem-like biology, pathogenic roles in autoimmunity, and therapeutic applications.
Clinical and experimental rheumatologyClinical and immunologic status of a child conceived following maternal administration of CD19 CAR T-cells for systemic lupus erythematosus.
Clinical and experimental rheumatologySystemic lupus erythematosus: one year in review 2026.
Clinical and experimental rheumatologySLE-diseaseome: a comprehensive meta-collection of systemic lupus erythematosus relevant functional pathways.
Bioinformatics advancesLong-term follow-up in a patient with Purtscher-like retinopathy associated with systemic lupus erythematosus.
Korean journal of ophthalmology : KJOIncreased soluble CD200 in serum of SLE patients does not reflect disease activity or affect CD200R1 function in T cells.
Arthritis research & therapyHargraves Remembered: Bone Marrow L.E. Cells as a Clue to Active Systemic Lupus Erythematosus.
Mayo Clinic proceedingsThe impact of anti-SSA/Ro antibodies on pregnancy outcomes in patients with systemic lupus erythematosus.
Rheumatology (Oxford, England)Severe systemic lupus erythematosus with thrombotic microangiopathy: rescue therapy with pegcetacoplan.
Pediatric nephrology (Berlin, Germany)Recent Advances in Multi-Omics of Systemic Lupus Erythematosus.
Current rheumatology reviewsCardiogenic shock treated effectively by Berlin Heart implantation in a young patient with systemic lupus erythematosus.
Kardiologia polskaAdvances in the study of low-density neutrophils in rheumatic diseases.
Frontiers in immunologyShared immune dysregulation in systemic lupus erythematosus and colorectal cancer: a multi-omics guided discovery of DNASE1L3-centric efferocytosis deficiency.
Frontiers in immunologyBone marrow mesenchymal stromal cells metabolic reprogramming in systemic lupus erythematosus: remodeling of bone marrow microenvironment and regulation of immune cell fate.
Frontiers in immunologyPericarditis in Patients with Autoimmune Disease: Insights into Prevalence and Optimal Management.
Methodist DeBakey cardiovascular journal[Clinical Manifestations of Childhood Systemic Lupus Erythematosus-associated Acute Pancreatitis and Evaluation of the Efficacy of Plasma Exchange Combined With Glucocorticoids].
Sichuan da xue xue bao. Yi xue ban = Journal of Sichuan University. Medical science editionPhenotypic profiling of pristane-induced mimicking human systemic lupus erythematosus in Macaca fascicularis.
Animal models and experimental medicine[Systemic lupus erythematosus in a child complicated with Fabry disease].
Zhonghua er ke za zhi = Chinese journal of pediatricsB-cell monitoring predicts infection risk in childhood systemic lupus erythematosus patients on belimumab.
Rheumatology (Oxford, England)IL-33 is Required for Behavioral Deficits in a Murine Model of Systemic Lupus Erythematosus Through Regulation of Microglia Glycolysis.
CNS & neurological disorders drug targetsBullous Systemic Lupus Erythematosus in a Southeast Asian Cohort: High Systemic Association Driving Systemic Therapy.
Asian Pacific journal of allergy and immunologyDelayed Recognition of Systemic Lupus Erythematosus Presenting With Visceral Muscle Dysmotility and Chronic Mucus-Bloody Enteropathy: A Case Report.
The American journal of case reportsOptic nerve head microcirculation on optical coherence tomography angiography is reduced in systemic lupus erythematosus and relates to nailfold videocapillaroscopy phenotypes: a cross-sectional case-control study.
Clinical rheumatologyCo-occurrence of systemic lupus erythematosus and myasthenia gravis: Insights from a cross-sectional cohort.
Clinical neurology and neurosurgeryUnique brain mechanisms in early events in SLE with diffuse neuropsychiatric disease (NPSLE): Implications for the management.
LupusKnockdown of LncRNA NIFK-AS1 inhibits the activation of CD4 + T cells in systemic lupus erythematosus by regulating the let-7f-5p/STAT3 axis.
Immunologic researchEvaluation of cardiovascular morbidities in children and adolescents with lupus nephritis from south India: A cross-sectional analytical study.
LupusOne-Step Multiplex PCR Reveals Selective Activation of Immunostimulatory Human Endogenous Retroviruses and Epigenetic Imbalance in Systemic Lupus Erythematosus.
International journal of molecular sciencesAdvances in Diagnosing and Managing Primary Systemic Vasculitides: A Transforming Landscape.
Diagnostics (Basel, Switzerland)Glycomic Profiles of IgG, C3 and Alpha-1-Acid Glycoprotein (AGP) Before and One Year After Treatment for Active Lupus Nephritis.
CellsProfiling miRNA in Systemic Lupus Erythematosus Patients Adhering to a Mediterranean Diet: An Interventional Pilot Study.
Journal of clinical medicineRecurrence and Outcomes of Lupus Nephritis After Renal Transplantation: Analysis of Nine Cases and Review of the Literature.
Journal of clinical medicineAcute rheumatic fever or rheumatoid arthritis? The role of hydroxychloroquine: a case report.
Journal of medical case reportsCorrelation between anti-retinal antibodies and lupus retinopathy in systemic lupus erythematosus.
Scientific reportsHyposplenism in systemic lupus erythematosus: biological and radiological characteristics and its association with increased risk of infection.
RMD openLeft Ventricular Mass Index as an independent predictor of major adverse cardiovascular and cerebrovascular events in patients with SLE.
Lupus science & medicineP2 purinergic receptors in systemic lupus erythematosus: from experimental findings to therapeutic perspectives.
Current opinion in immunology'Water Target Sign' on CT Scan in Systemic Lupus Erythematosus: Author's Reply.
Indian journal of pediatricsClinical characteristics and diagnostic indicators of childhood-onset systemic lupus erythematosus complicated with macrophage activation syndrome.
Clinical rheumatologyShrinking lung syndrome in systemic lupus erythematosus: Insights from a case series and a systematic review of the past decade.
LupusNon-Coding Regulatory Variants in Autoimmune Disease: Biological Mechanisms, Immune Context, and Integrative Multi-Omics Interpretation.
BiologyEfficacy of Low-dose Aspirin in Preventing Hypertensive Disorders of Pregnancy in Women with Systemic Lupus Erythematosus: A Single-Center Retrospective Cohort Study.
Modern rheumatologyPredictive value of interferon regulatory factor 5 nuclear translocation in B cells for disease activity and flare in systemic lupus erythematosus: a prospective observational cohort study.
Modern rheumatologySymptomatic Spontaneous Intracranial Hypotension Caused by a Cerebrospinal Fluid Leak at the C1-C2 Level of the Spine Sealed With an Epidural Blood Patch-Case Series and Review of Literature.
Pain practice : the official journal of World Institute of PainPalmar Cutaneous Erythematous Macules: A Case Report of an Atypical Presentation of Chilblain Lupus Erythematosus.
CureusThe emerging role of histone acetylation in rheumatic diseases: unraveling mechanisms and therapeutic prospects.
Frontiers in immunologyBidirectional Mendelian Randomization Analysis Reveals Causal Associations Between Autoimmune Diseases and Colorectal Cancer.
World journal of oncologyDevelopment of a PERMA-Based Intervention Program to Address Fear of Disease Progression in Systemic Lupus Erythematosus: A Delphi Consensus Study.
Journal of multidisciplinary healthcareHematopoietic Stem Cell Transplantation in Rheumatic Diseases.
International journal of rheumatic diseasesInverse association between hydroxychloroquine use and depressive symptoms in systemic lupus erythematosus: a single-center cross-sectional study.
BMC rheumatologyMonogenic lupus with SLC7A7 mutations: a retrospective study from a Chinese center.
Orphanet journal of rare diseasesSerum IL-2 levels are associated with disease activity and related to dyslipidaemia and the immunological profile in systemic lupus erythematosus.
Lupus science & medicineCross-tissue integrative transcriptomic and multimodal analyses suggest shared immune signatures linking lupus nephritis and cutaneous lupus erythematosus.
Frontiers in immunologyThe role of programmed cell death 1 in autoimmune diseases: mechanisms and therapeutic implications.
Frontiers in immunologySexual dimorphism of early GPR183-dependent B cell responses in systemic lupus erythematosus.
iScienceDemographic and conceptual disparities in systemic lupus erythematosus: A comparative analysis from Riyadh, Saudi Arabia.
Journal of family medicine and primary careA novel epigenetic regulation of JAM-A by EZH2-DNMT3A cascade contributes to T cell adhesion via the activation of Rap1a in lupus patients.
Journal of translational autoimmunityAnalysis of the impact of pharmacological treatment on arterial stiffness in patients with connective tissue diseases.
American heart journal plus : cardiology research and practiceSystemic Lupus Erythematosus With IgA Nephropathy: Challenges in Diagnosis and Management.
The American journal of case reportsFrom Treg, FOXP3 to RBPJ: Linking basic science and clinical medicine.
Taiwanese journal of obstetrics & gynecologyPulmonary Involvement in ANCA-associated Vasculitis Overlapping with Long-standing Systemic Lupus Erythematosus.
Internal medicine (Tokyo, Japan)Biomarker-stratified efficacy and safety of biologics in systemic lupus erythematosus: a systematic review and meta-analysis.
Lupus science & medicineAtypical lupus nephritis unmasked after albumin correction: a diagnostic pitfall presenting with cerebral venous sinus thrombosis.
BMJ case reportsRethinking headache in juvenile systemic lupus erythematosus: the need for broader perspectives.
Arquivos de neuro-psiquiatriaPrecision immunotherapies for systemic lupus erythematosus: From pathogenic targets to translational horizons.
Autoimmunity reviewsGlucagon-like peptide-1 receptor agonist and lupus nephritis in systemic lupus erythematosus with type 2 diabetes.
The American journal of medicineThe role of clot waveform analysis and related parameters in the diagnosis and treatment of systemic lupus erythematosus.
Thrombosis researchCD39 Expression in Peripheral CD4+ T Lymphocytes Is Associated With Disease Activity in Patients With Systemic Lupus Erythematosus.
Journal of immunology researchBarriers to therapy adherence in a group of Italian patients with systemic lupus erythematosus.
Clinical and experimental rheumatologyAssociation between autoimmune diseases and the gut microbiome.
International immunologyRemodeling the Inflammatory Microenvironment: Nanomaterial-Based Targeted Strategies for Systemic Lupus Erythematosus and Lupus Nephritis.
Small scienceAtopic Comorbidities Associated With Chronic Spontaneous Urticaria: A Case-Control Analysis of the All of Us Research Program.
JEADV clinical practiceIschemic duodenal injury due to systemic lupus erythematosus: A case report.
World journal of gastroenterologyClinical significance of miR-146a-5p and miR-192-5p expression in relation to disease activity and immunobiochemical markers in systemic lupus erythematosus.
Molecular biology reportsPsychosis in patients with systematic lupus erythematosus: A systematic literature review and meta-analysis.
LupusCognitive Screening and Neuropsychological Evaluation in Children and Young Adults With Childhood-Onset Systemic Lupus Erythematosus: A Survey of Rheumatologists.
ACR open rheumatologyClinical and Immunological Profiles With Treatment Patterns in Systemic Lupus Erythematosus Patients in the Eastern Part of India: A Retrospective, Hospital-Based, Cross-Sectional Study.
CureusNatural killer cells as biomarkers for disease activity, lupus nephritis, and time to remission in treatment-naïve childhood-onset systemic lupus erythematosus: a cohort study.
Pediatric rheumatology online journalRisk Factors for Peritonitis in Patients With Systemic Lupus Erythematosus Undergoing Peritoneal Dialysis.
International journal of rheumatic diseasesExploring the metabolic mysteries: Mechanistic insights into lactylation-mediated regulation of autoimmune diseases.
Autoimmunity reviewsInfluence of frailty syndrome on disease outcomes in systemic lupus erythematosus: a systematic review and narrative synthesis.
Current medical research and opinionModels Integrating Disease Heterogeneity and MPA Exposure Predict Systemic and Renal Efficacy of Mycophenolate in Pediatric Lupus Nephritis.
PharmacotherapyValidation of the Systemic Lupus Erythematosus Disease Activity Score (SLE-DAS) in a Chinese Cohort: Comparison with SLEDAI-2-K.
Rheumatology and therapyMemory-biased CD8αα⁺ T cells show reduced abundance and phenotypic remodeling in SLE.
Immunologic researchIntegrating Patient-Reported Quality Measures in SLE: Development of the American College of Rheumatology Implementation Guide.
Arthritis care & researchDeveloping and Evaluating a Laboratory-Based Frailty Index (FI-Lab) for the Prediction of Long-Term Health Outcomes in Systemic Lupus Erythematosus.
Arthritis care & researchClinical Significance of Therapeutic Drug Level Monitoring for Mycophenolate in Patients with Extra-Renal Systemic Lupus Erythematosus - A Systematic Review & Meta-analysis.
Arthritis care & researchUnveiling Endotypes in Systemic Lupus Erythematosus Through Multi-Omic Analysis: Insights into Cardiovascular and Renal Complications.
Arthritis & rheumatology (Hoboken, N.J.)Serum Endocan in Systemic Lupus Erythematosus: Implication for Endothelial Dysfunction and Cardiovascular Risk.
International journal of rheumatic diseasesThe Th17/Treg axis: a key to understanding and treating autoimmune disorders.
Open life sciencesApolipoprotein L1 High-Risk Genotypes are Associated With Lupus Nephritis Incidence.
Kidney international reportsExosomal non-coding RNAs in autoimmune diseases: molecular mechanisms and potential applications.
Frontiers in immunologyNon-Conventional and Emerging Autoantibodies in Sjögren's Disease.
Journal of inflammation researchCardiac Complications in Systemic Lupus Erythematosus: A Systematic Review of Diagnostic and Prognostic Gaps.
CureusPurulent Pericarditis With Cardiac Tamponade Triggered by Left-Sided Infective Endocarditis in Active Systemic Lupus Erythematosus: A Case Report.
CureusRisk factors for depression in systemic lupus erythematosus: a systematic review and meta-analysis.
Frontiers in medicineFrom Hemolysis to Lupus: A Case of Evans Syndrome Revealing Systemic Autoimmunity.
Clinical case reportsBioinformatics Analysis Reveals ALDH5A1: A Novel Molecular Brake on Th17 Cell Pathogenicity in Systemic Lupus Erythematosus.
FASEB journal : official publication of the Federation of American Societies for Experimental BiologyVagus nerve stimulation in autoimmune diseases: Mechanisms, therapeutic potential, and clinical applications.
Autoimmunity reviewsErythroderma-Like Manifestations and High Fever in Palisaded Neutrophilic and Granulomatous Dermatitis Without Any Underlying Systemic Immunological Disease.
The Journal of dermatologyShenQi DiHuang Decoction (SQDHD) Ameliorates Neuroinflammation and Neuropsychiatric Manifestations in Pristane Induced Lupus Mice via Blocking JAK1-STAT3 Pathway.
CNS neuroscience & therapeuticsEndothelial Dysfunction: Insights into Systemic Lupus Erythematosus-associated Cardiovascular Disease and Neuropsychiatric Manifestations.
Journal of cardiovascular translational researchCAR-T therapy for autoimmune rheumatic diseases: navigating clinical frontiers between breakthroughs and uncertainties.
Clinical and experimental medicineClinical Characteristics of 30 Cases of Childhood Haemolytic Uremic Syndrome in a Single Centre.
Nephrology (Carlton, Vic.)Association of Mitochondrial DNA Genetic Variants With Depression and Anxiety in Chinese Patients With Systemic Lupus Erythematosus.
International journal of rheumatic diseasesProgression from at-risk state to clinical and severe systemic lupus erythematosus involves molecular dysregulations potentially reversible by biologics: implications for early diagnosis and treatment.
Annals of the rheumatic diseasesClinical utility and performance of anti-C1q antibodies for SLE: comparative analysis of three different assays.
Lupus science & medicineLGALS3BP promotes M1 polarization of macrophages and interacts with LGALS3, damaging endothelial function and exacerbating pulmonary arterial hypertension in systemic lupus erythematosus.
Pathology, research and practiceNeutralizing autoantibodies against interferon alpha in systemic lupus erythematosus: Prevalence, age of onset, and clinical associations.
Lupus'Water Target Sign' on CT Scan in Systemic Lupus Erythematosus: Correspondence.
Indian journal of pediatricsAssociações
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Doença com base genética
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Doenças relacionadas
Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico
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Este é o documento que organiza diagnóstico, tratamento e dispensação de Lúpus eritematoso sistêmico no SUS. O que a farmácia entrega é o que está no PCDT vigente — não o que foi aprovado fora do Brasil.
É uma doença autoimune multissistêmica crônica na qual o sistema imunológico ataca tecidos saudáveis do corpo, gerando inflamações vasculares e lesões em múltiplos órgãos. A maioria das pessoas acometidas apresenta autoanticorpos circulantes característicos, como anticorpos antinucleares (ANA), anti-dsDNA e anti-Smith.
Referências
Fontes citadas no texto, publicações do grafo e bases de dados usadas neste verbete
10 publicações do grafo RarasNet (PubMed) · 8 bases de dados. Títulos, periódicos e PMIDs vêm direto da fonte, sem intermediação de IA.
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Citar este verbete
Raras. (s.d.). Lúpus eritematoso sistêmico. Em Raras — Enciclopédia de Doenças Raras do Brasil. https://raras.org/doenca/lupus-eritematoso-sistemico
Formato APA. Conteúdo sob CC BY 4.0 — reuso livre com atribuição.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Lúpus eritematoso sistêmico
📋 Origem dos dados
Esta página agrega dados de fontes públicas e oficiais. Dados sobre cobertura no SUS (PCDT, CEAF) são verificados ativamente por agente proativo (ver badge no infobox). Demais dados têm atribuição de fonte + data da última sincronização — clique para abrir o original.
- Doença rara (ontologia)
- fonte: Orphanet
- Identificador unificado
- fonte: MONDO
- Genética mendeliana
- fonte: OMIM
- Codificação WHO/SUS
- fonte: WHO ICD-10 / DATASUS
- CID-11 (futuro)
- fonte: WHO ICD-11
- NIH/GARD
- fonte: GARD (NIH)
- Indexação biomédica
- fonte: MeSH (NLM)
- Dado público estruturado
- fonte: Wikidata
- Medicamentos aprovados FDA
- fonte: FDA OpenFDA
- Reposicionamento
- fonte: Drug Repurposing Hub
- Ensaios clínicos
- fonte: ClinicalTrials.gov