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1.
Ter Arkh ; 96(5): 453-458, 2024 Jun 03.
Article in Russian | MEDLINE | ID: mdl-38829805

ABSTRACT

AIM: To evaluate the levels of MPO-DNA complex in patients with systemic lupus erythematosus (SLE) and its association with the presence of lupus nephritis (LN). MATERIALS AND METHODS: The study included 77 patients with SLE, of whom 30 had SLE without anti phospholipid syndrome (APS), 47 had SLE with APS, and 20 were healthy individuals serving as the control group. The MPO-DNA complex in the serum was investigated using ELISA. RESULTS: The levels of MPO-DNA complex in serum were significantly higher in patients with SLE compared to healthy controls (p=0.001). Among the patients with SLE, 30 (39%) had elevated levels of MPO-DNA complex. The presence of elevated MPO-DNA complex was significantly associated with the presence of a history of LN (p=0.009). Moreover, among the patients included in the study, 20 had active LN, and patients with elevated MPO-DNA complex levels were more likely to have active LN than patients without elevated MPO-DNA complex concentrations [12 (40%) of 30 vs 8 (17%) of 47, χ2=5.029; p=0.034]. An association was found between elevated levels of MPO-DNA complex and the presence of proteinuria, hematuria, cellular hematic/granular casts and aseptic leukocyturia. A direct correlation of MPO-DNA complex with SLEDAI-R was found in patients with active LN (rs=0.497; p=0.026). CONCLUSION: Elevated levels of MPO-DNA complex were detected in 39% of patients with SLE. These patients had a higher prevalence of LN in their medical history and at the time of inclusion in the study. The correlation between MPO-DNA complex levels and the activity of LN according to SLEDAI-R indicates the potential role of MPO-DNA complex as a biomarker for assessing the activity of renal damage in SLE.


Subject(s)
DNA , Lupus Nephritis , Peroxidase , Humans , Lupus Nephritis/blood , Lupus Nephritis/epidemiology , Lupus Nephritis/diagnosis , Lupus Nephritis/complications , Female , Adult , Male , Peroxidase/blood , Extracellular Traps/metabolism , Middle Aged , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/blood , Lupus Erythematosus, Systemic/epidemiology , Biomarkers/blood
2.
Lupus ; 33(8): 886-891, 2024 Jul.
Article in English | MEDLINE | ID: mdl-38719778

ABSTRACT

In rare instances, patients with SLE may exhibit atypical clinical manifestations, such as Hypocomplementemic Urticarial Vasculitis, which can pose diagnostic challenges. Here, we present a case report of a 28-year-old female with a history of SLE with lupus nephritis clase IV who developed HUV-like symptoms, ultimately leading to a diagnosis of C1q Vasculitis. This case underscores the importance of considering C1q Vasculitis in SLE patients presenting with HUV-like features and highlights Rituximab as a promising therapeutic option for managing this rare condition.


Subject(s)
Complement C1q , Lupus Erythematosus, Systemic , Rituximab , Urticaria , Vasculitis , Humans , Female , Adult , Complement C1q/deficiency , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/diagnosis , Vasculitis/diagnosis , Vasculitis/drug therapy , Urticaria/diagnosis , Rituximab/therapeutic use , Lupus Nephritis/diagnosis , Lupus Nephritis/complications , Lupus Nephritis/drug therapy , Diagnosis, Differential
3.
Lupus Sci Med ; 11(1)2024 Apr 09.
Article in English | MEDLINE | ID: mdl-38599669

ABSTRACT

OBJECTIVE: Circadian rhythm disruption (CRD) has been associated with inflammation and immune disorders, but its role in SLE progression is unclear. We aimed to investigate the impact of circadian rhythms on immune function and inflammation and their contribution to SLE progression to lupus nephritis (LN). METHODS: This study retrospectively analysed the clinical characteristics and transcriptional profiles of 373 samples using bioinformatics and machine-learning methods. A flare risk score (FRS) was established to predict overall disease progression for patients with lupus. Mendelian randomisation was used to analyse the causal relationship between CRD and SLE progression. RESULTS: Abnormalities in the circadian pathway were detected in patients with SLE, and lower enrichment levels suggested a disease state (normalised enrichment score=0.6714, p=0.0062). The disruption of circadian rhythms was found to be closely linked to lupus flares, with the FRS showing a strong ability to predict disease progression (area under the curve (AUC) of 5-year prediction: 0.76). The accuracy of disease prediction was improved by using a prognostic nomogram based on FRS (AUC=0.77). Additionally, Mendelian randomisation analysis revealed an inverse causal relationship between CRD and SLE (OR 0.6284 (95% CI 0.3630 to 1.0881), p=0.0485) and a positive causal relationship with glomerular disorders (OR 0.0337 (95% CI 1.634e-3 to 6.934e-1), p=0.0280). CONCLUSION: Our study reveals that genetic characteristics arising from CRD can serve as biomarkers for predicting the exacerbation of SLE. This highlights the crucial impact of CRD on the progression of lupus.


Subject(s)
Lupus Erythematosus, Systemic , Lupus Nephritis , Humans , Disease Progression , Inflammation , Lupus Erythematosus, Systemic/complications , Lupus Nephritis/complications , Retrospective Studies , Mendelian Randomization Analysis
4.
Medicine (Baltimore) ; 103(14): e37821, 2024 Apr 05.
Article in English | MEDLINE | ID: mdl-38579022

ABSTRACT

Systemic lupus erythematosus mainly affects young women, and approximately half of systemic lupus erythematosus patients develop lupus nephritis (LN). However, data on the types and remission rates of LN in Saudi Arabia are limited. Therefore, we aimed to highlight the LN remission rates in our population. A retrospective record review was conducted between January 2007 and December 2020 in a tertiary center in the western region of Saudi Arabia to determine the remission rates among patients with biopsy-proven LN who met the EULAR\ACR 2019 classification criteria. We identified 59 patients with biopsy-proven LN, mostly in young women. The common histopathological pattern was Class IV LN in 26 patients (44%). Three induction protocols were identified, along with systemic steroids: the high-dose cyclophosphamide protocol in 21 patients (35.6%), low-dose protocol in 4 patients (6.8%), and mycophenolate mofetil (MMF) in 41 patients (69.5%). Partial response, defined as the reduction of the 24-hour proteinuria by 25% at 3 months and 50% at 6 months, was achieved in 18 patients (33.3%) at 3 months and decreased to 13 patients (24.1%) at 6 months. Complete clinical response, defined as 24-hour urinary protein between 500 and 700 mg at 12 months, was achieved in 44 patients (81.5%). Complete remission was higher among patients with Class IV LN (64.4%). The achievement of partial clinical response at 3 months was significantly lower among patients with hypertension (P = .041). This study presented the LN remission rates in a single center in Saudi Arabia. Similar to previous studies, Class IV LN were the most common histopathological finding in this study. Complete remission at 12 months was achieved in 44 (81%) patients. Delayed remission is associated with hypertension at the time of LN diagnosis.


Subject(s)
Hypertension , Lupus Erythematosus, Systemic , Lupus Nephritis , Humans , Female , Lupus Nephritis/complications , Lupus Nephritis/drug therapy , Lupus Nephritis/diagnosis , Immunosuppressive Agents/therapeutic use , Retrospective Studies , Saudi Arabia/epidemiology , Treatment Outcome , Cyclophosphamide/therapeutic use , Lupus Erythematosus, Systemic/complications , Mycophenolic Acid/therapeutic use , Hypertension/complications , Pathologic Complete Response , Remission Induction
5.
Lupus Sci Med ; 11(1)2024 Mar 21.
Article in English | MEDLINE | ID: mdl-38519060

ABSTRACT

INTRODUCTION: It remains unclear how the presence of renal involvement will affect the cardiovascular (CV) risk factors and complications in patients with SLE. METHODS: We conducted a systematic review and meta-analysis using PubMed, EMBASE, MEDLINE and Scopus to identify studies published between 1947 and 2022 that evaluate the CV risk factors and complications in patients with SLE with or without lupus nephritis (LN). RESULTS: 58 studies were evaluated, with 22 two-arm studies (n=8675) included in two-arm meta-analysis and 45 studies (n=385 315) included in proportional meta-analysis. Patients with SLE with LN showed significantly higher risk of hypertension (HT) (OR=4.93, 95% CI=3.17 to 7.65, p<0.00001, I2=56%), hyperlipidaemia (OR=11.03, 95% CI=4.20 to 28.95, p<0.00001, I2=0%) and diabetes mellitus (DM) (OR=1.88, 95% CI=1.09 to 3.25, p=0.02, I2=32%) compared with those without LN. Patients with LN showed numerically higher prevalence of myocardial infarction (OR=1.35, 95% CI=0.53 to 3.45, p=0.52, I2=78%) and cerebrovascular accident (OR=1.64, 95% CI=0.79 to 3.39, p=0.27, I2=23%) than general patients with SLE. The incidence rates of CV mortality are also increased in patients with SLE with LN compared with those without LN (11.7/1000 patient-years vs 3.6/1000 patient-years). CONCLUSION: Patients with SLE with LN show increased risk of CV risk factors including DM, HT and hyperlipidaemia. Early identification and optimal control of these CV risk factors may reduce the risk of CV disease and other non-CV complications. PROSPERO REGISTRATION NUMBER: CRD42022314682.


Subject(s)
Cardiovascular Diseases , Hyperlipidemias , Hypertension , Lupus Erythematosus, Systemic , Lupus Nephritis , Humans , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/epidemiology , Cardiovascular Diseases/etiology , Cardiovascular Diseases/complications , Risk Factors , Lupus Nephritis/complications , Lupus Nephritis/epidemiology , Heart Disease Risk Factors , Hypertension/complications , Hypertension/epidemiology , Hyperlipidemias/complications , Hyperlipidemias/epidemiology
6.
Curr Opin Rheumatol ; 36(3): 163-168, 2024 05 01.
Article in English | MEDLINE | ID: mdl-38517337

ABSTRACT

PURPOSE OF REVIEW: Lupus nephritis is a common complication of systemic lupus erythematosus and is associated with significant morbidity and mortality. The utility of sodium-glucose cotransporter 2 (SGLT2) inhibitors in the management of lupus nephritis is currently uncertain. Here, we summarize the rationale for their use among patient with lupus nephritis. RECENT FINDINGS: SGLT2 inhibitors were initially developed as antihyperglycemic agents. They have since been shown to have additional, profound effects to slow the progression of chronic kidney disease and lessen the long-term risks of cardiovascular disease in large clinic trials of patients with chronic kidney disease, with and without diabetes, as well as in patients with and without proteinuria. Patients with recent exposure to immunosuppression were excluded from these trials due to concern for risk of infection. In the few, small trials of patients with lupus nephritis, SGLT2 inhibitors were found to be well tolerated. They have been shown to reduce proteinuria and to have modest beneficial effects on blood pressure and BMI among patients with lupus nephritis. They have not been shown to influence disease activity. SUMMARY: SGLT2 inhibitors may have a role in mitigating the chronic renal and cardiovascular effects of lupus nephritis. They should be introduced after kidney function has been stabilized with appropriate immunosuppression, in conjunction with angiotensin-converting enzyme inhibitors or angiotensin receptor blockers. They currently have no role in active disease.


Subject(s)
Lupus Nephritis , Renal Insufficiency, Chronic , Sodium-Glucose Transporter 2 Inhibitors , Humans , Glucose/metabolism , Lupus Nephritis/drug therapy , Lupus Nephritis/complications , Proteinuria/drug therapy , Proteinuria/etiology , Renal Insufficiency, Chronic/complications , Sodium/metabolism , Sodium-Glucose Transporter 2 Inhibitors/therapeutic use
7.
Exp Clin Transplant ; 22(Suppl 1): 336-337, 2024 Jan.
Article in English | MEDLINE | ID: mdl-38385421

ABSTRACT

Posterior reversible encephalopathy syndrome is an emergency medical condition with varied causes presenting as reversible subcortical vasogenic brain edema caused by endothelial injury, resulting from changes in blood pressure or direct effects of cytokines on endothelium. Posterior reversible encephalopathy syndrome is manifested by neurologic symptoms. Common causes include hypertensive emergency, renal disease, preeclampsia, eclampsia, and immunosuppressive drugs. In this case report, a 17-year-old female patient on hemodialysis as a result of lupus nephritis who had previously undergone deceased donor organ transplant and was on triple immunosuppression presented with neurological symptoms of posterior reversible encephalopathy syndrome in the early posttransplant period. She was normotensive, and tacrolimus level was in desired level. She improved after cessation of tacrolimus from immunosuppression with complete resolution of radiological lesions. Posterior reversible encephalopathy syndrome can occur in solid-organ transplant recipients who are on tacrolimus as a part of immunosuppression.


Subject(s)
Kidney Transplantation , Lupus Nephritis , Posterior Leukoencephalopathy Syndrome , Pregnancy , Female , Humans , Adolescent , Tacrolimus/adverse effects , Posterior Leukoencephalopathy Syndrome/chemically induced , Posterior Leukoencephalopathy Syndrome/diagnosis , Kidney Transplantation/adverse effects , Immunosuppressive Agents/adverse effects , Lupus Nephritis/diagnosis , Lupus Nephritis/drug therapy , Lupus Nephritis/complications
8.
Clin Rheumatol ; 43(4): 1319-1326, 2024 Apr.
Article in English | MEDLINE | ID: mdl-38409491

ABSTRACT

BACKGROUND: Type 2 systemic lupus erythematosus (SLE) symptoms, including fatigue, fibromyalgia, and brain fog, contribute to poor health-related quality of life (HRQoL) in patients with lupus. To test the hypothesis that Type 1 (classical inflammatory lupus) activity is associated with Type 2 SLE activity, we characterized the features of Type 2 SLE in patients with a range of lupus nephritis (LN) activity. METHODS: This was a cross-sectional study of SLE patients [American College of Rheumatology (ACR) 1997 or Systemic Lupus International Collaborating Clinics (SLICC) 2012 classification criteria] from June 2018 to March 2020. Patients completed the Systemic Lupus Activity Questionnaire (SLAQ) and the Polysymptomatic Distress Scale. Patients were divided into groups based on their renal status. Active nephritis was defined using the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) lupus nephritis parameter. Differences across groups were analyzed by Fisher's exact test and ANOVA. RESULTS: In this cohort of 244 patients (93% female, mean age 43 years, 58% Black), 10% had active nephritis, 35% had historical nephritis, and 55% never had nephritis (non-nephritis). Active nephritis and non-nephritis patients had a similar burden of Type 2 SLE symptoms, despite a difference in Type 1 SLE activity. Patients with active nephritis had higher Type 2 PGA (Physician Global Assessment) scores and reported more Type 2 SLE symptoms than inactive nephritis patients. Patients with inactive nephritis had the lowest Type 2 SLE activity. CONCLUSIONS: While Type 2 SLE symptoms are common in SLE, our findings suggest that patients with active nephritis experience significant Type 2 SLE symptoms that may be ameliorated as nephritis improves. We also observed that non-nephritis patients had a similar burden of Type 2 SLE symptoms as patients with active nephritis, despite having on average lower Type 1 SLE activity. Therefore, the etiology of Type 2 SLE symptoms is likely multifactorial and may be driven by inflammatory and non-inflammatory biopsychosocial factors. Key Points • Patients with active nephritis experienced significant Type 2 symptoms that may be ameliorated as nephritis improves. • Non-nephritis patients had a similar burden of Type 2 SLE symptoms as patients with active nephritis, despite having on average lower Type 1 SLE activity. • Because etiology of Type 2 SLE symptoms is likely multifactorial and may be driven by inflammatory and non-inflammatory biopsychosocial factors.


Subject(s)
Lupus Erythematosus, Systemic , Lupus Nephritis , Humans , Female , United States , Adult , Male , Lupus Nephritis/complications , Lupus Nephritis/diagnosis , Quality of Life , Cross-Sectional Studies , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/diagnosis , Surveys and Questionnaires
9.
Reumatol. clín. (Barc.) ; 20(1): 20-23, Ene. 2024. tab, ilus
Article in Spanish | IBECS | ID: ibc-228930

ABSTRACT

Antecedentes y objetivo: La interrupción del embarazo en pacientes con enfermedades reumatológicas es controvertida y pocas veces se realiza un análisis bioético. En este estudio analizamos el caso de una paciente embarazada con nefritis lúpica sin respuesta a tratamiento a quien se le planteó la interrupción del embarazo. Métodos: Se aplicó el modelo integral, incluyendo distintos sistemas normativos para el análisis del dilema. Resultados: Desde el utilitarismo es justificable la interrupción del embarazo, buscando el mayor beneficio para la mayoría de las partes. La deontología justifica tanto continuar como interrumpir el embarazo, centrando la importancia en la acción misma y en la autonomía. Para la ética de la virtud, la importancia de las decisiones recae en la persona que realiza la acción buscando el florecimiento: la interrupción del embarazo sería justificable. Discusión y conclusiones: La interrupción del embarazo sería una solución adecuada según el modelo integral. El análisis bioético de casos paradigmáticos es fundamental para asegurar el mejor actuar posible en casos similares en reumatología.(AU)


Background and objective: Termination of pregnancy in patients with rheumatic diseases is controversial and a bioethical analysis is rarely performed. In this study we analysed the case of a pregnant patient with lupus nephritis unresponsive to treatment, for whom termination of pregnancy is considered. Methods: The integrative model was applied combining different normative ethical theories. Results: From a utilitarian perspective, termination of pregnancy is justifiable, seeking the greatest benefit for the greatest number of stakeholders. Deontology justifies both terminating and continuing the pregnancy, focusing on the action itself and on autonomy. In virtue ethics the importance of decisions rests with the person who performs the action seeking flourishing; termination of pregnancy would be justifiable. Discussion and conclusions: Interruption of pregnancy is a justifiable solution following the integrative model. Bioethical analysis of paradigmatic cases is essential to ensure the best possible action and as a precedent for future similar situations in rheumatology.(AU)


Subject(s)
Humans , Female , Adult , Abortion, Spontaneous , Lupus Nephritis/complications , Pregnancy Complications , Pregnant Women , Bioethics , Rheumatology , Rheumatic Diseases , Bioethical Issues , Inpatients , Physical Examination , Lupus Erythematosus, Systemic
10.
Paediatr Int Child Health ; 44(1): 42-47, 2024 05.
Article in English | MEDLINE | ID: mdl-38184810

ABSTRACT

BACKGROUND: Enteritis is one of the rare systemic manifestations in childhood-onset systemic lupus erythematosus and its diagnosis is very challenging. This is a rare case of an adolescent girl with recurrent non-specific gastro-intestinal symptoms which were later diagnosed to be owing to lupus enteritis, the only presenting manifestation of an active flare. CASE REPORT: A 15-year-old girl was admitted with recurrent episodes of abdominal pain, vomiting and loose stools. She had diffuse abdominal tenderness. Abdominal ultrasonography demonstrated moderate ascites. A contrast-enhanced abdominal computerised tomography scan revealed thickening of the small bowel wall. On colonoscopy, there were rectal erosions, and microscopic examination of the biopsy specimens demonstrated mild inflammation. Non-specific enteritis was diagnosed and she was given antibiotics and supportive care. She was re-admitted 6months later with abdominal pain. An abdominal contrast-enhanced computerised tomography scan revealed thickening of the bowel wall and the target sign and comb sign in the small intestine. The anti-nuclear antibody was positive. Renal biopsy demonstrated grade 2 lupus nephritis. Lupus enteritis was diagnosed and the case satisfied the 2019 EULAR-ACR criteria and SLICC criteria. She was treated with methylprednisolone, cyclophosphamide and hydroxychloroquine. She improved with treatment and has remained asymptomatic during follow-up. CONCLUSION: This case emphasises the need for healthcare providers to be alert to the possibility of lupus enteritis. It also highlights the importance of close follow-up of cases who have non-specific gastro-intestinal symptoms. Lupus enteritis should be considered in the differential diagnosis of recurrent non-specific gastro-intestinal symptoms in children, especially adolescents, to ensure timely diagnosis and treatment.Abbreviations: ACR American College of Rheumatology; ANA anti-nuclear antibody; CRP: C-reactive protein; CT: computerised tomography; CECT: contrast-enhanced computerised tomography; EULAR: European League Against Rheumatism; GI: gastro-intestinal; LE: lupus enteritis; SLE systemic lupus erythematosis; SLICC: Systemic Lupus International Collaborating Clinics; SLEDAI: SLE disease activity index.


Subject(s)
Enteritis , Lupus Erythematosus, Systemic , Lupus Nephritis , Adolescent , Female , Humans , Abdominal Pain/complications , Cyclophosphamide , Enteritis/diagnosis , Enteritis/etiology , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/diagnosis , Lupus Erythematosus, Systemic/pathology , Lupus Nephritis/complications
11.
Ren Fail ; 46(1): 2296000, 2024 Dec.
Article in English | MEDLINE | ID: mdl-38178546

ABSTRACT

To explore the effect of lupus nephritis (LN) on graft survival in renal transplant patients. Literature search was conducted in PubMed, EMBASE and Scopus database for randomized controlled trials (RCTs), cohort, and case-control studies. The target population of interest was adult patients (aged >18 years) with end-stage renal disease (ESRD) and no history of previous renal transplants. Primary outcomes of interest were graft survival and patient survival. Pooled effect estimates were calculated using random-effects models and reported as hazard ratio (HR) with 95% confidence intervals (CI). A total of 15 studies were included. Compared to patients with ESRD due to other causes, patients with LN undergoing kidney transplant had lower patient survival rate (HR 1.15, 95% CI: 1.01, 1.31; N = 15, I2=34.3%) and worse graft survival (HR 1.06, 95% CI: 1.01, 1.11; N = 16, I2=0.0%), especially when studies with deceased donor were pooled together. Studies with a larger sample size (>200) showed that LN was strongly associated with lower graft and patient survival rates. Elevated risk of mortality in LN patients was detected in case-control studies, but not RCTs. On the other hand, RCTs, but not case-control studies, showed an increased risk of poor graft survival in LN patients. The findings suggest that the presence of LN might have a negative impact on both the graft survival and the overall patient survival of post-transplant ESRD patients. Further studies that account for factors such as study methodology, donor characteristics, and sample size are needed to reach definitive conclusions. Renal transplant patients with LN should undergo regular follow-up examinations.


Subject(s)
Kidney Failure, Chronic , Kidney Transplantation , Lupus Nephritis , Adult , Humans , Case-Control Studies , Graft Survival , Kidney Failure, Chronic/complications , Kidney Transplantation/mortality , Lupus Nephritis/complications , Lupus Nephritis/surgery , Lupus Nephritis/epidemiology , Retrospective Studies
12.
Pediatr Rheumatol Online J ; 22(1): 17, 2024 Jan 18.
Article in English | MEDLINE | ID: mdl-38238724

ABSTRACT

BACKGROUND: Childhood systemic lupus erythematosus (cSLE) has been considered as a polygenic autoimmune disease; however, a monogenic lupus-like phenotype is emerging with the recent recognition of several related novel high-penetrance genetic variants. RASopathies, a group of disorders caused by mutations in the RAS/MAPK pathway, have been recently described as a cause of monogenic lupus. CASE PRESENTATION: We present a 13-year-old boy with Noonan-like syndrome with loose anagen hair who developed a monogenic lupus. The renal biopsy confirmed a class III lupus nephritis and identified the presence of zebra bodies. CONCLUSIONS: RASopathies represent a cause of monogenic lupus. We report a new case of monogenic lupus in a child with Noonan-like syndrome with loose anagen hair. Lupus nephritis which has never been described in this context, may be part of the presentation. The presence of zebra bodies in SLE or RASopathies in unclear, but no other known conditions (Fabry disease or drugs) were identified as the cause of zebra bodies in our patient.


Subject(s)
Loose Anagen Hair Syndrome , Lupus Erythematosus, Systemic , Lupus Nephritis , Noonan Syndrome , Adolescent , Humans , Male , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/diagnosis , Lupus Erythematosus, Systemic/genetics , Lupus Nephritis/diagnosis , Lupus Nephritis/genetics , Lupus Nephritis/complications , Noonan Syndrome/complications , Noonan Syndrome/diagnosis , Noonan Syndrome/genetics
13.
J Clin Rheumatol ; 30(2): e58-e62, 2024 Mar 01.
Article in English | MEDLINE | ID: mdl-37798827

ABSTRACT

OBJECTIVE: To determine the association between disease activity and choroidal thickness in patients with systemic lupus erythematosus (SLE). METHODS: We conducted a cohort study of 24 SLE patients and 13 healthy controls recruited at Washington University School of Medicine between June 2019 and November 2021. SLE disease activity was assessed using the SLE Disease Activity Index-2000 Responder Index-50 (S2K RI-50). Patients were divided into four groups: high disease activity/no lupus nephritis (HDA/no LN; S2K RI-50 > 4), HDA/active LN (HDA/active LN; S2K RI-50 > 4), low disease activity/inactive LN (LDA/inactive LN; S2K RI-50 ≤ 4), and LDA/no LN (LDA/no LN; S2K RI-50 ≤ 4). LDA/no LN patients were age-, sex-, and race-matched to healthy controls and patients in other SLE groups. Choroidal thickness of the right eye was measured blinded to disease activity on a horizontal section through the fovea on optical coherence tomography images taken within a week of disease assessment. RESULTS: Patients with HDA had choroidal thickening compared with matched patients with LDA. After controlling for multiplicity, choroidal thinning remained statistically significant at 1000 µm nasal to the fovea (308 ± 68 vs 228 ± 64 µm, p = 0.001). Choroidal thickness was not different between LDA/no LN and LDA/inactive LN or healthy controls. CONCLUSION: HDA in patient with SLE is associated with increased choroidal thickness whereas comorbid inactive LN did not affect choroidal thickness. Additional studies in a larger longitudinal cohort are needed to study whether choroidal thickness may be used as a noninvasive, adjunctive measure for disease activity in SLE.


Subject(s)
Lupus Erythematosus, Systemic , Lupus Nephritis , Humans , Cohort Studies , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/diagnosis , Lupus Nephritis/diagnosis , Lupus Nephritis/complications , Tomography, Optical Coherence , Biomarkers
14.
Clin Exp Rheumatol ; 42(1): 30-38, 2024 01.
Article in English | MEDLINE | ID: mdl-38019149

ABSTRACT

OBJECTIVES: Increased serum uric acid (SUA) levels are well known to be concomitant of cardiovascular and kidney diseases, and have been proposed to be implicated in the development of arteriolar damage. The aim of the present study was to assess the association between SUA levels, renal damage and its implication for outcome in patients with lupus nephritis (LN). METHODS: This retrospective study included 194 cases with biopsy proven LN at the Affiliated Hospital of Qingdao University between January 2013 and June 2021. We reviewed clinical, laboratory and histologic data of patients and analysed the correlation between SUA levels, renal damage and the primary outcome (death or ESRD). Biopsy-proven arteriolar damage was defined by the presence of arteriolar hyalinosis and/or intimal thickening. RESULTS: Compared to LN patients without hyperuricemia, LN patients with hyperuricaemia presented with higher BP, hyperlipidaemia, lower eGFR, lower haemoglobin, lower serum albumin, worse renal arteriolar damage and proteinuria, and also higher SLEDAI score, activity index and chronicity index (p<0.05). At logistic regression analysis, SUA was independently related to the presence of arteriolar damage. For each 100 µmol/L increase in SUA levels the risk for arteriolar damage raised by 53.8% (hazard ratio [HR] =1.538; 95% CI: 1.147-2.063; p=0.004) after adjustment for haemoglobin, serum creatinine and erythrocyte sedimentation rate. Cox regression analysis showed that female (HR=3.180; 95% CI: 1.216-8.313; p=0.018), white blood cell count (HR=1.111; 95% CI: 1.027-1.202; p=0.009), SUA (HR=1.100; 95% CI: 1.023-1.253; p=0.035), serum creatinine (HR=1.800; 95% CI: 1.348-2.404; p<0.001), and renal arteriolar damage (HR=3.117; 95% CI: 1.022-9.511; p=0.046) was significantly associated with development of ESRD or death in patients with LN after adjustment for several potential confounding factors. Furthermore, for each 100 µmol/L increase in SUA levels, the risk of ESRD or death increased by 10%. CONCLUSIONS: SUA levels are directly associated with renal arteriolar damage and poor prognosis in LN patients. Hyperuricaemia is an important predictor for poor prognosis in patients with LN.


Subject(s)
Hyperuricemia , Kidney Failure, Chronic , Lupus Nephritis , Humans , Female , Lupus Nephritis/complications , Lupus Nephritis/diagnosis , Lupus Nephritis/pathology , Uric Acid , Hyperuricemia/complications , Hyperuricemia/diagnosis , Retrospective Studies , Creatinine , Kidney/pathology , Hemoglobins , Risk Factors
15.
Lupus ; 33(1): 48-57, 2024 Jan.
Article in English | MEDLINE | ID: mdl-38019182

ABSTRACT

OBJECTIVE: For the majority of patients with lupus nephritis-related end-stage kidney disease (LN-ESKD), kidney transplant is associated with better outcomes than dialysis. Access to kidney transplant requires an initial referral to a transplant center and medical evaluation prior to waitlisting. The study's objective was to examine access to these early steps in the kidney transplant process among patients with LN-ESKD. METHODS: Adults who began treatment for ESKD in the Southeast, Northeast, New York, or Ohio River Valley U.S. regions from 1/1/2012 to 12/31/2019, followed through 6/30/2021, were identified from the United States Renal Data System. Referral and evaluation start data were collected from 28 of 48 transplant centers across these regions. The exposure was primary cause of ESKD (LN-ESKD vs other-ESKD). The outcomes were referral and evaluation start at a transplant center. Cox models quantified the association between LN-ESKD (vs other-ESKD) and referral and evaluation start. RESULTS: Among 192,318 patients initiating treatment for ESKD, 0.4% had LN-ESKD. Over half (58%) of LN-ESKD patients were referred before study end, and among those referred, 66% started the evaluation. In adjusted analyses, patients with LN-ESKD were referred (HR: 1.09, 95% CI: 0.99, 1.19) and started the transplant evaluation (HR: 1.13, 95% CI: 1.00, 1.28) at a higher rate than patients with other-ESKD. Among referred patients with LN-ESKD, the median time from ESKD start to referral was 2.9 months (IQR: <1 to 11.7 months), which is similar to patients with other-ESKD (median 2.6 months, IQR: <1 to 8.8 months). CONCLUSIONS: Among incident patients with ESKD, having a primary diagnosis of LN-ESKD versus other-ESKD is associated with higher rates of early transplant access outcomes. Despite this, patients with LN-ESKD (vs other-ESKD) are less likely to be preemptively referred (i.e., referred prior to ESKD start) for kidney transplant. While providers may no longer be delaying the early steps in the kidney transplantation process among this patient population, there is still room for improvement in the rates of preemptive referral. Access to kidney transplant referral prior to ESKD could result in increased transplant rates and better transplant outcomes for patients with LN-ESKD.


Subject(s)
Kidney Failure, Chronic , Kidney Transplantation , Lupus Erythematosus, Systemic , Lupus Nephritis , Adult , Humans , United States , Kidney Transplantation/adverse effects , Lupus Nephritis/complications , Lupus Nephritis/surgery , Lupus Nephritis/diagnosis , Lupus Erythematosus, Systemic/complications , Kidney Failure, Chronic/etiology , Kidney Failure, Chronic/surgery , Kidney Failure, Chronic/epidemiology , Referral and Consultation , Kidney
16.
Clin J Am Soc Nephrol ; 19(3): 309-318, 2024 03 01.
Article in English | MEDLINE | ID: mdl-38110196

ABSTRACT

BACKGROUND: In a phase 3 study of adults with active lupus nephritis, addition of voclosporin to mycophenolate mofetil (MMF) and low-dose glucocorticoids led to significant improvements in the proportion of participants achieving complete and partial renal response as well as sustained reduction in proteinuria. This analysis examined the efficacy and safety of voclosporin in a subgroup of the phase 3 study with proliferative lupus nephritis and high levels of proteinuria. METHODS: Participants were randomized to oral voclosporin (23.7 mg twice daily) or placebo for 12 months; all participants received MMF and low-dose glucocorticoids. This analysis includes participants with class III or IV (±class V) lupus nephritis and baseline urine protein-creatinine ratio (UPCR) ≥3 g/g. Efficacy end points included complete renal response (UPCR ≤0.5 g/g with stable eGFR, low-dose glucocorticoids, and no rescue medication), partial renal response (≥50% reduction from baseline UPCR), and UPCR over time. Safety outcomes were also assessed. RESULTS: A total of 148 participants were in the voclosporin ( n =76) and control ( n =72) arms. At 12 months, 34% and 11% of participants in the voclosporin and control arms, respectively, achieved a complete renal response (odds ratio, 4.43; 95% confidence interval [CI], 1.78 to >9.99; P = 0.001). A partial renal response was achieved by 65% of the voclosporin arm and 51% of the control arm at 12 months (odds ratio, 1.60; 95% CI, 0.8 to 3.20; P = 0.18). More voclosporin- than control-treated participants achieved UPCR ≤0.5 g/g (51% versus 26%), and voclosporin-treated participants met this end point significantly earlier (hazard ratio, 2.07; 95% CI, 1.19 to 3.60; P = 0.01). The incidence of adverse events was similar between the arms; mean eGFR values remained stable and within normal range in both arms. CONCLUSIONS: Addition of voclosporin to MMF and low-dose glucocorticoids resulted in a significantly higher proportion of participants with proliferative lupus nephritis achieving complete and partial renal responses as well as earlier reductions in proteinuria, with no evidence of worsening kidney function.


Subject(s)
Lupus Nephritis , Adult , Humans , Lupus Nephritis/drug therapy , Lupus Nephritis/complications , Immunosuppressive Agents/adverse effects , Cyclosporine/adverse effects , Mycophenolic Acid/adverse effects , Proteinuria/drug therapy , Proteinuria/etiology , Glucocorticoids/adverse effects , Treatment Outcome
17.
Indian J Pathol Microbiol ; 66(4): 751-757, 2023.
Article in English | MEDLINE | ID: mdl-38084527

ABSTRACT

Background: Lupus nephritis (LN) is the assemblage of glomerular, tubulointerstitial and vascular changes. Despite the fact that glomerular changes are overemphasized in pathological classification and scoring system, but the existence of vascular damage negatively impact the clinical course. Aims and Objective: This study was conducted to determine the clinicopathological spectrum of renal vascular lesions in lupus nephritis. Materials and Methods: Renal microvascular lesions in biopsy proven lupus nephritis were classified into 5 major categories-thrombotic microangiopathy, true vasculitis; lupus vasculopathy, uncomplicated vascular immune deposits, and arterial. Clinical details, laboratory parameters and histopathological variables were compared among all groups. Summary of chronic changes was also assessed. Results: Biopsies from 56 patients revealed thrombotic microangiopathy (2), lupus vasculopathy (3), uncomplicated vascular immune deposit (6), PAN type vasculitis (1) and arterial sclerosis (13). No renal vascular lesions were found in 35.18% of patients. At the time of biopsy, arterial sclerosis or lupus vasculopathy patients were older Nephritis subtype. Activity indices were higher in lupus vasculopathy group whereas patients with arteriosclerosis showed highest chronicity index. Conclusions: Renal vascular lesions are common in systemic lupus erythematosus patients with nephritis and may be associated with aggressive clinical course.


Subject(s)
Lupus Nephritis , Thrombotic Microangiopathies , Vasculitis , Humans , Lupus Nephritis/complications , Tertiary Care Centers , Sclerosis/pathology , Kidney/pathology , Thrombotic Microangiopathies/complications , Thrombotic Microangiopathies/pathology , Vasculitis/pathology , Disease Progression , Biopsy
18.
Saudi J Kidney Dis Transpl ; 34(2): 154-160, 2023 Mar 01.
Article in English | MEDLINE | ID: mdl-38146725

ABSTRACT

According to the current guidelines, renal biopsies are performed in systemic lupus erythematosus (SLE) patients for proteinuria of 0.5 g/24 h or higher. Renal pathology may be present in patients with lower-level proteinuria (<0.5 g/24 h). We aimed to review the renal histopathology in SLE patients, with lower levels of proteinuria. In this retrospective study, we retrieved SLE patients' data, including 24-h urinary protein excretion and renal histopathology results. We compared various parameters in different lupus nephritis (LN) classes and in different levels of proteinuria (urinary protein <0.5 g, 0.5 to <1 g, and ≥1 g per 24 h). Out of 476 patients, 274 (57.6%) had proteinuria of <0.5 g, 44 (9.2%) had 0.5 to <1 g, and 158 (33.2%) had ≥1 g per 24 h. SLE patients with proteinuria of <0.5 g/24 h were found to have LN, including the proliferative classes. Of the 299 LN cases confirmed by a renal biopsy, low-level proteinuria (<0.5 g) was found in 39.8% of all LN patients, in 50% of patients with Class III LN, 33.3% of those with Class IV LN, 31.4% of those with Class V LN, and 41.4% of those with other LN classes (II/V, III/V, and IV/V). Overall, 35.9% (87/242) of patients with the proliferative LN classes (III, IV, V, II/V, III/V and IV/V) had low-level proteinuria of <0.5 g/24 h. SLE patients with low-level proteinuria had significant renal pathology. Our study suggests there is a need to perform renal biopsies at lower levels of proteinuria.


Subject(s)
Lupus Erythematosus, Systemic , Lupus Nephritis , Humans , Retrospective Studies , Kidney/pathology , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/diagnosis , Lupus Erythematosus, Systemic/pathology , Lupus Nephritis/complications , Lupus Nephritis/diagnosis , Lupus Nephritis/pathology , Proteinuria/etiology , Proteinuria/pathology
19.
J Investig Med High Impact Case Rep ; 11: 23247096231215705, 2023.
Article in English | MEDLINE | ID: mdl-38097351

ABSTRACT

Calciphylaxis is a rare and severe disease characterized by calcification, fibrosis, and thrombosis of small blood vessels. Although it primarily affects patients with end-stage renal disease (ESRD) on dialysis, limited cases have been reported of calciphylaxis in patients with acute kidney injury (AKI) and lupus. This case report describes the occurrence of calciphylaxis in a 35-year-old female recently diagnosed with lupus nephritis class IV and AKI requiring dialysis.


Subject(s)
Acute Kidney Injury , Calciphylaxis , Kidney Failure, Chronic , Lupus Nephritis , Female , Humans , Adult , Lupus Nephritis/complications , Lupus Nephritis/diagnosis , Calciphylaxis/diagnosis , Calciphylaxis/etiology , Kidney Failure, Chronic/complications , Kidney Failure, Chronic/therapy , Renal Dialysis/adverse effects , Acute Kidney Injury/etiology
20.
Semin Arthritis Rheum ; 63: 152308, 2023 12.
Article in English | MEDLINE | ID: mdl-37976812

ABSTRACT

OBJECTIVE: To compare the risk of end-stage renal disease (ESRD) between patients with early-onset lupus nephritis (EOLN) and those with delayed-onset LN (DOLN). METHODS: This retrospective study of incident cases of systemic lupus erythematosus (SLE) used nationwide Korean claims databases and data from 2008 through 2018. We divided LN patients into two groups: the EOLN group (with LN onset within 12 months of SLE diagnoses) and the DOLN group (with LN onset later than 12 months after SLE diagnoses). Patients were observed from the date of LN diagnosis to the development of ESRD, death, or the last follow-up. Cox proportional hazards modeling was used to predict hazard ratios (HRs) for progression to ESRD with death as a competing risk. RESULTS: We identified 3779 incident SLE patients who developed LN during follow-up: 60 % (n = 2281) had EOLN, and 40 % (n = 1489) had DOLN. Sixty-nine patients with EOLN (3.0 %) and 29 patients with DOLN (1.9 %) progressed to ESRD. After adjusting for confounders, the ESRD risk associated with EOLN was comparable to the risk associated with DOLN (HR 1.10, 95 % confidence interval [CI] 0.57 to 2.11). In the subgroup of patients on aggressive immunosuppressive therapy (670 with EOLN and 179 with DOLN), the ESRD risk was higher in the DOLN group (HR 2.6, 95 % CI 1.11 to 6.10). CONCLUSION: The risk of ESRD was comparable between patients with EOLN and DOLN. However, among patients on aggressive immunosuppressive therapy, compared with EOLN, DOLN was associated with a higher risk of progression to ESRD.


Subject(s)
Kidney Failure, Chronic , Lupus Erythematosus, Systemic , Lupus Nephritis , Humans , Lupus Nephritis/complications , Lupus Nephritis/epidemiology , Lupus Nephritis/diagnosis , Retrospective Studies , Cohort Studies , Kidney Failure, Chronic/epidemiology , Kidney Failure, Chronic/etiology , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/epidemiology , Lupus Erythematosus, Systemic/diagnosis
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