Lupus eritematoso sistémico: ¿es una sola enfermedad?

Rivas-Larrauri, Francisco; Yamazaki‐Nakashimada, Marco Antonio

doi:10.1016/j.reuma.2016.01.005

Cited by 31 publications

(7 citation statements)

References 90 publications

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“…Genetic analysis to assess monogenic lupus (whole exome or whole genome sequencing) is very important to improve the knowledge of the genetic basis and increase options for new drug targets and biomarker development [ 77 ], and therefore should be considered for cSLE patients with early onset of lupus manifestations (mainly in infants and toddlers with cSLE) [ 78 ]. Patients with the gene defect have a specific disease presentation and progression [ 79 ], such as recurrent infection and prominent cutaneous manifestation, requiring personalized management.…”

Section: Pathogenesismentioning

confidence: 99%

An Update on the Management of Childhood-Onset Systemic Lupus Erythematosus

et al. 2021

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Childhood-onset systemic lupus erythematosus (cSLE) is a prototype of a multisystemic, inflammatory, heterogeneous autoimmune condition. This disease is characterized by simultaneous or sequential organ and system involvement, with unpredictable flare and high levels of morbidity and mortality. Racial/ethnic background, socioeconomic status, cost of medications, difficulty accessing health care, and poor adherence seem to impact lupus outcomes and treatment response. In this article, the management of cSLE patients is updated. Regarding pathogenesis, a number of potential targets for drugs have been studied. However, most treatments in pediatric patients are off-label drugs with recommendations based on inadequately powered studies, therapeutic consensus guidelines, or case series. Management practices for cSLE patients include evaluations of disease activity and cumulative damage scores, routine non-live vaccinations, physical activity, and addressing mental health issues. Antimalarials and glucocorticoids are still the most common drugs used to treat cSLE, and hydroxychloroquine is recommended for nearly all cSLE patients. Disease-modifying antirheumatic drugs (DMARDs) should be standardized for each patient, based on disease flare and cSLE severity. Mycophenolate mofetil or intravenous cyclophosphamide is suggested as induction therapy for lupus nephritis classes III and IV. Calcineurin inhibitors (cyclosporine, tacrolimus, voclosporin) appear to be another good option for cSLE patients with lupus nephritis. Regarding B-cell-targeting biologic agents, rituximab may be used for refractory lupus nephritis patients in combination with another DMARD, and belimumab was recently approved by the US Food and Drug Administration for cSLE treatment in children aged > 5 years. New therapies targeting CD20, such as atacicept and telitacicept, seem to be promising drugs for SLE patients. Anti-interferon therapies (sifalimumab and anifrolumab) have shown beneficial results in phase II randomized control trials in adult SLE patients, as have some Janus kinase inhibitors, and these could be alternative treatments for pediatric patients with severe interferon-mediated inflammatory disease in the future. In addition, strict control of proteinuria and blood pressure is required in cSLE, especially with angiotensin-converting enzyme inhibitor and angiotensin receptor blocker use.

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Section: Pathogenesismentioning

confidence: 99%

An Update on the Management of Childhood-Onset Systemic Lupus Erythematosus

et al. 2021

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“…However, there were no reported cases of SLE with the only or the initial symptom of hypothyroidism, the present study reported on a case to highlight the diversity of the disease. SLE is a multifactorial autoimmune disease with a complex pathogenesis and clinical manifestations ( 28 ). The present case and previous studies suggest that hypothyroidism is more likely to occur in patients with SLE in whom there is a higher incidence of anti-thyroid antibodies ( 7-26 ); the reason for this remains elusive.…”

Section: Discussionmentioning

confidence: 99%

Systemic lupus erythematosus with hypothyroidism as the initial clinical manifestation: A case report

Zhang

Liu

et al. 2020

Exp Ther Med

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Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease and hypothyroidism is an organ-specific autoimmune disease. The two diseases may occur successively or simultaneously. The majority of previous studies observed that thyroid disease was more frequent in patients with SLE than in the general population, particularly those who had a higher incidence of anti-thyroid antibodies. However, there are no reported cases of SLE with hypothyroidism as the initial clinical manifestation, to the best of our knowledge. The present study reported on a case of SLE with this unusual initial clinical manifestation and reviewed the literature to estimate the prevalence of clinical hypothyroidism in patients with SLE (range, 3.0-21.4%). The case of the present study had no obvious facial erythema, photosensitivity or recurrent oral ulcers, and only had hypothyroidism as the initial clinical symptom, but the laboratory examination supported the diagnosis of SLE. The present study suggested that in the clinical diagnosis, attention should be paid to screening for connective tissue diseases when diagnosing hypothyroidism, and the importance of thyroid dysfunction should also be recognized in the treatment of SLE.

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“…Systemic lupus erythematosus (SLE) is a multisystemic disease with diverse pathogenesis and clinical presentations with considerable comorbidities and significant drug side effects (1)(2)(3). Understanding the root cause of SLE is still incomplete.…”

Section: Introductionmentioning

confidence: 99%

Association Between Hypoalbuminemia, Degree of Proteinuria, and Lupus Nephritis Class: A Single-Center Cross-sectional Biopsy Study

et al. 2021

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Background: The current study intended to determine whether serum albumin level and urine protein/creatinine rate (uPCR) are appropriate predictors of severe lupus nephritis in childhood-onset SLE. Objectives: Following a cross-sectional single-center design, 85 LN children referred to the National Children Hospital, Ha Noi, Viet Nam, from 6/2019 to 6/2020 were recruited. Renal biopsy was performed for all participants. Methods: Following a cross-sectional single-center design, 85 LN children referred to the National Children Hospital, Ha Noi, Viet Nam, from 6/2019 to 6/2020 were recruited. Renal biopsy was performed for all participants. Results: The mean SLEDAI score of all patients was 14.69. The proportion of patients with high and very high SLEDAI was 61.2 and 17.6%, respectively. The mean concentration of serum albumin was 28.55 g/L, and the proportion of decreased albumin concentration was 55.3%. The median uPCR was 446.6 mg/mmol in which 76.5% of values were ≥ 200 mg/mmol. Pathological morphology of LN class from I to VI was observed in 0%, 17.6%, 37.6 %, 37.6%, 7.1%, and 0%, respectively. Serum albumin level and uPCR presented the predictive value for severe and active LN (class IV and V); (AUC: 0.725 P < 0.001 for both). Conclusions: Serum albumin and uPCR were appropriate predictors for severe and active LN in Vietnamese children.

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Lupus eritematoso sistémico: ¿es una sola enfermedad?

Cited by 31 publications

References 90 publications

An Update on the Management of Childhood-Onset Systemic Lupus Erythematosus

An Update on the Management of Childhood-Onset Systemic Lupus Erythematosus

Systemic lupus erythematosus with hypothyroidism as the initial clinical manifestation: A case report

Association Between Hypoalbuminemia, Degree of Proteinuria, and Lupus Nephritis Class: A Single-Center Cross-sectional Biopsy Study

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