Monophasic Disease Course in Systemic Lupus Erythematosus

Objective.Disease course in systemic lupus erythematosus (SLE) is primarily relapsing-remitting. Long quiescent and chronically active patterns are less frequent. We recently described an atypical “monophasic” course in a small number of patients. The aim of the present study was to assess the prevalence and characteristics of such patients in a defined SLE cohort.Methods.The inception patients of the University of Toronto Lupus Clinic (enrolled within 18 mos of diagnosis) were investigated. No time interval > 18 months was allowed between consecutive visits. A monophasic course was defined as Systemic Lupus Erythematosus Disease Activity Index 2000 = 0 (serology excluded), achieved within 5 years since enrollment and maintained for ≥ 10 years. Descriptive statistics were used.Results.Of 267 inception patients, 27 (10.1%) achieved prolonged clinical remission (≥ 10 yrs) and 20 (7.5%) sustained remission for the entire followup (18 yrs on average). Twelve patients were receiving no maintenance treatment 10 years after achieving remission. Clinical manifestations at diagnosis (apart from skin and musculoskeletal involvement) included 25% in each of central nervous system involvement and lupus nephritis (LN). Half the patients were serologically active. Ten years after achieving remission, two-thirds of the patients had discontinued glucocorticosteroids; the remaining were treated with 5 mg/day on average. Seven patients relapsed after 10 years, 4 with arthritis, 2 LN, and 1 catastrophic antiphospholipid syndrome.Conclusion.A monophasic disease course was observed in 7.5% in this inception cohort. Patients sustained remission for 18 years on average, eventually without medications. Further study of such patients may provide unique pathophysiologic insights for SLE.

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THE CLINICAL, LABORATORY MANIFESTATIONS AND HISTOPATHOLOGY IN NEPHROTIC PATIENTS WITH LUPUS NEPHRITIS

Journal of Medicine and Pharmacy ◽

10.34071/jmp.2018.2.9 ◽

2018 ◽

pp. 52-58

Author(s):

Le Thuan Nguyen ◽

Bui Bao Hoang

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Nephritis ◽

Lupus Erythematosus ◽

Clinical Laboratory ◽

Activity Index ◽

Clinical Manifestations ◽

Systemic Lupus ◽

Cross Sectional ◽

Organ Systems ◽

Tubulointerstitial Lesions

Introduction: Systemic lupus erythematosus (SLE) is an autoimmune disease involving multiple organ systems. The kidney appears to be the most commonly affected organ, especially nephrotic is a serious kidney injury. The clinical, laboratory manifestations and histopathology are very useful for diagnosis, provide the means of predicting prognosis and guiding therapy in nephrotic patients with lupus nephritis. Methods: Descriptive cross-sectional study of nephrotic patients with lupus treated in the Department of Nephrology Trung Vuong Hospital and Cho Ray Hospital between May/2014 and May/2017. Renal histopathological lesions were classified according to International Society of Nephrology/Renal Pathology Society - ISN/RPS ’s 2003. The clinical, laboratory manifestations and histopathological features were described. Results: Of 32 LN with nephritic range proteinuria cases studied, 93.7% were women. The 3 most common clinical manifestations were edema (93.8%), hypertension (96.8%) and pallor (68.9%), musculoskeletal manifestions (46.9%), malar rash (40.6%). There was significant rise in laboratory and immunological manifestions with hematuria (78.1%), Hb < 12g/dL (93.5%), increased Cholesterol (100%), and Triglycerid (87.5%), Creatinine > 1.4 mg/dL (87.5%), increased BUN 71.9%, ANA (+) 93.8%, Anti Ds DNA(+) 96.9%, low C3: 96.9%, low C4: 84.4%. The most various and severe features were noted in class IV with active tubulointerstitial lesions and high activity index. Conclusion: Lupus nephritis with nephrotic range proteinuria has the more severity of histopathological feature and the more severity of the more systemic organ involvements and laboratory disorders were noted. Key words: Systemic lupus, erythematosus (SLE) lupus nepphritis, clinical

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Elevated levels of IL-24 in systemic lupus erythematosus patients

Lupus ◽

10.1177/0961203319845476 ◽

2019 ◽

Vol 28 (6) ◽

pp. 748-754 ◽

Cited By ~ 1

Author(s):

R C Li ◽

J Guo ◽

L C Su ◽

A F Huang

Keyword(s):

Systemic Lupus Erythematosus ◽

Disease Activity ◽

Lupus Erythematosus ◽

Inflammatory Cytokine ◽

Activity Index ◽

Clinical Manifestations ◽

Mrna Levels ◽

Healthy Controls ◽

Systemic Lupus ◽

Good Ability

Objective This study aimed to assess IL-24 levels and their association with clinical manifestations in patients with systemic lupus erythematosus (SLE). Methods There were 75 patients with SLE and 58 healthy controls recruited in this study. Serum levels of IL-24 were measured by enzyme-linked immunosorbent assays, and mRNA levels of IL-24 were tested by quantitative real-time polymerase chain reaction . The area under the curve of the receiver operating characteristic (ROC) curve was used for diagnostic ability of the inflammatory cytokine. Results Serum IL-24 levels were significantly higher in SLE patients than that in healthy controls. SLE patients with nephritis had higher IL-24 levels than those without nephritis. Active SLE patients showed higher expression of IL-24 as compared to less active disease patients. The mRNA levels of IL-24 were much higher in SLE patients. Correlation analysis showed significant correlation between serum IL-24 levels and SLE disease activity index. In addition, ROC analysis may suggest good ability of serum IL-24 in differentiating SLE. Conclusion The inflammatory cytokine correlated with SLE disease activity, and may be involved in this disease pathogenesis.

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From TTP to Glomerulonephritis: A Lifetime of Lupus

Case Reports in Medicine ◽

10.1155/2021/6654748 ◽

2021 ◽

Vol 2021 ◽

pp. 1-5

Author(s):

Fadi Kharouf ◽

Sigal Shahar ◽

Yoav Hershkovitz ◽

Alaa Shaheen ◽

Areej Bayatra ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

Deep Vein Thrombosis ◽

Lupus Erythematosus ◽

Clinical Manifestations ◽

Disease Course ◽

Systemic Lupus ◽

Thrombocytopenic Purpura ◽

Vein Thrombosis ◽

Deep Vein ◽

Excellent Clinical Outcome

We report the case of a 56-year-old male patient, who over two decades, sequentially presented with a combination of clinical manifestations. These included thrombotic thrombocytopenic purpura (TTP), right leg deep vein thrombosis (DVT), and eventually constitutional symptoms, arthralgia, diffuse lymphadenopathy, pancytopenia, skin rash, pericarditis, and glomerulonephritis. Serologic tests and renal pathology uncovered a diagnosis of systemic lupus erythematosus (SLE), and immunosuppressive therapy was initiated. Soon after, the patient developed striking cytomegalovirus (CMV) viremia, requiring prolonged antiviral therapy and reduction of immunosuppression. Finally, an acute embolic stroke complicated the disease course. Prompt interventions allowed an excellent clinical outcome.

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Active human herpesvirus infections in adults with systemic lupus erythematosus and correlation with the SLEDAI score

Advances in Rheumatology ◽

10.1186/s42358-020-00144-6 ◽

2020 ◽

Vol 60 (1) ◽

Cited By ~ 1

Author(s):

Alex Domingos Reis ◽

Cristiane Mudinutti ◽

Murilo de Freitas Peigo ◽

Lucas Lopes Leon ◽

Lilian Tereza Lavras Costallat ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

Disease Activity ◽

Lupus Erythematosus ◽

Clinical Symptoms ◽

Activity Index ◽

Clinical Manifestations ◽

Human Herpesvirus ◽

Nucleic Acid Amplification ◽

Systemic Lupus ◽

Hcmv Disease

Abstract Background Human herpesviruses (HHVs) are responsible for a significant number of clinical manifestations in systemic lupus erythematous (SLE) patients. The aim of this study was to determine the frequency of active HHV infections in SLE patients and correlating them with disease activity. Methods Serum samples were collected from 71 SLE patients and their DNAs were extracted and analyzed to detect HHV-DNA viruses using the nucleic acid amplification technique. Results Fifteen out of the 71 (21.1%) patients tested positive for the HHV-DNA virus. Of them, 11/15 HHV-DNA-positive patients (73.3%) had SLE activity index (SLEDAI – Systemic Lupus Erythematosus Disease Activity Index) ≥8 (p = 0.0001). Active HCMV infection was the mostly frequently observed infection, occurring in 6/15 patients (40%). The frequencies of other active viral infections were 22% for HSV-1, 16.7% for HHV-7, and 5.5% for HSV-2. Viral coinfection (two or more viruses detected in the same sample) occurred in three patients (16.7%). Active HHV infections in SLE patients are more frequent in those with active SLE (≥8), who is at high risk of HHV reactivation and HCMV disease. Conclusion Viral surveillance is important to identify active HHV infections that can cause clinical symptoms and other complication in SLE patients.

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New EULAR/ACR 2019 SLE Classification Criteria: defining ominosity in SLE

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-218670 ◽

2021 ◽

pp. annrheumdis-2020-218670

Author(s):

Laura P Whittall Garcia ◽

Dafna D Gladman ◽

Murray Urowitz ◽

Zahi Touma ◽

Jiandong Su ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

Disease Severity ◽

Lupus Erythematosus ◽

Validation Cohort ◽

Activity Index ◽

Immunosuppressive Treatment ◽

Classification Criteria ◽

Disease Course ◽

Characteristic Analysis ◽

Systemic Lupus

ObjectiveTo determine the ominosity of the European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) Systemic Lupus Erythematosus Classification Criteria by determining its predictive role for disease severity in the first 5 years following diagnosis.Methods867 patients with systemic lupus erythematosus (SLE) from the Toronto Lupus Clinic were included (all first 12 months after SLE diagnosis). The EULAR/ACR criteria score was calculated based on baseline information. To determine disease severity in the first 5 years after diagnosis, adjusted mean SLE Disease Activity Index 2000 (AMS), flares, remission and immunosuppressive treatment were used as outcomes. The Systemic Lupus International Collaborating Clinics (SLICC) registry comprised the validation cohort.ResultsBased on receiver operating characteristic analysis, a EULAR/ACR score of 20 was used as a threshold to compare outcomes between groups. In the first 5 years of disease course, patients with a score of ≥20 had higher AMS scores (p<0.001) and were more likely to ever experience a flare (p<0.001). These patients had lower probabilities of achieving remission and higher requirements for immunosuppressives. Results were confirmed in the SLICC validation cohort. Patients with a score of ≥20 had higher AMS during the first 5 years of disease (5.4 vs 3.1% and ≥20 vs <20 respectively, p≤0.001). The score correlated with AMS (r=0.43, p≤0.001) in the same time frame.ConclusionA EULAR/ACR score of ≥20 is an indicator of ominosity in SLE. Patients with a score of ≥20 were characterised by a more active disease course throughout the first 5 years. These criteria provide prognostic information regarding disease severity in the first 5 years following diagnosis.

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Disease course patterns in systemic lupus erythematosus

Lupus ◽

10.1177/0961203318817132 ◽

2018 ◽

Vol 28 (1) ◽

pp. 114-122 ◽

Cited By ~ 7

Author(s):

K. Tselios ◽

D.D. Gladman ◽

Z. Touma ◽

J. Su ◽

N. Anderson ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

Disease Activity ◽

Lupus Erythematosus ◽

Activity Index ◽

Disease Activity Index ◽

Disease Course ◽

Systemic Lupus Erythematosus Disease ◽

Systemic Lupus ◽

Relapsing Remitting ◽

Active Patients

Background Disease activity in systemic lupus erythematosus follows three different courses: long quiescent, relapsing remitting and persistently active. However, the patterns of disease course since diagnosis are not known. This study aimed to assess the prevalence and characteristics of such patterns over 10 years. Patients and methods The inception cohort of the Toronto Lupus Clinic (≥10 year follow up, between visit interval ≤18 months) was investigated. Prolonged remission was defined as a clinical Systemic Lupus Erythematosus Disease Activity Index 2000 = 0 achieved within 5 years of enrolment and maintained for ≥10 years. The relapsing-remitting pattern was defined based on ≥2 remission periods (clinical Systemic Lupus Erythematosus Disease Activity Index 2000 = 0 for two consecutive visits). Patients with no remission were categorized as persistently active. Groups were compared for baseline characteristics, cumulative damage, flare rate, mortality and certain co-morbidities. Results Of 267 patients, 27 (10.1%) achieved prolonged remission, 180 (67.4%) relapsing-remitting and 25 (9.4%) persistently active. In total, 35 (13.1%) had only one remission period (hybrid). At enrollment, there were no differences regarding clinical and immunological variables. At 10 years, persistently active patients had accumulated significantly more damage than the prolonged remission and relapsing-remitting patients. Being of Black race and higher adjusted mean Systemic Lupus Erythematosus Disease Activity Index 2000 over the first 2 years were associated with a more severe disease course. Relapsing-remitting and persistently active patients had an increased flare rate and accrued more osteoporosis, osteonecrosis and cardiovascular events. Conclusions Approximately 70% of systemic lupus erythematosus patients followed a relapsing-remitting course, whereas 10% displayed prolonged remission and another 10% a persistently active course. Early response to treatment was associated with a less severe course and better prognosis.

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Increased Proportion of CD226+ B Cells Is Associated With the Disease Activity and Prognosis of Systemic Lupus Erythematosus

Frontiers in Immunology ◽

10.3389/fimmu.2021.713225 ◽

2021 ◽

Vol 12 ◽

Author(s):

Miki Nakano ◽

Masahiro Ayano ◽

Kazuo Kushimoto ◽

Shotaro Kawano ◽

Kazuhiko Higashioka ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

B Cells ◽

Disease Activity ◽

Lupus Erythematosus ◽

Activity Index ◽

Renal Involvement ◽

Laboratory Data ◽

Clinical Manifestations ◽

Systemic Lupus ◽

Dsdna Antibody

BackgroundCD226, an activating receptor expressed on the surface of natural killer (NK) cells and T cells, is also seen on B cells and CD226 polymorphism is associated with systemic lupus erythematosus (SLE). Because the specific roles of CD226+ B cells in SLE are still unknown, we investigated the association of CD226+ B cells with SLE.MethodsWe measured CD226 expression on B cells and its subsets using flow cytometry in 48 SLE patients and 24 healthy controls (HCs). We assessed the relationships between CD226+ B cells and SLE Disease Activity Index 2000 (SLEDAI-2K), clinical manifestations, laboratory data, and prognosis after 12 months.ResultsThe proportions of CD226+ cells in whole B cells and all its subsets were significantly higher in SLE patients than HCs. In SLE patients, the proportions of CD226+ B cells and CD226+ switched-memory (SM) B cells were significantly correlated with SLEDAI-2K scores and anti-dsDNA antibody titers, and negatively correlated with serum complement levels. Moreover, basal percentages of CD226+ B cells and CD226+ SM B cells were low in patients who were in Lupus Low Disease Activity State after 12 months. In patients with renal involvement, the proportion of CD226+ B cells increased. Additionally, the proportion of CD226+ B cells was higher in patients who were not in complete renal remission after 12 months.ConclusionsIncreased proportion of CD226+ B cells was associated with disease activity and prognosis of SLE. CD226+ B cells may be a useful biomarker for the management of SLE.

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Antibodies to extractable nuclear antigens (ENAS) in systemic lupus erythematosus patients: correlations with clinical manifestations and disease activity

Reumatismo ◽

10.4081/reumatismo.2018.1027 ◽

2018 ◽

Vol 70 (2) ◽

pp. 85 ◽

Cited By ~ 2

Author(s):

Y. Emad ◽

T. Gheita ◽

H. Darweesh ◽

P. Klooster ◽

R. Gamal ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

Disease Activity ◽

Lupus Erythematosus ◽

Disease Duration ◽

Activity Index ◽

Age At Onset ◽

Clinical Manifestations ◽

Clinical Aspects ◽

Systemic Lupus ◽

Nuclear Antigens

The aim was to explore possible correlations of antibodies to extractable nuclear antigens (ENA) with clinical manifestations and disease activity indices in systemic lupus erythematosus (SLE) patients. A total of 70 consecutive SLE patients (64 females) were included. Disease activity was assessed by SLE activity index (SLEDAI), and British Isles Lupus Assessment Group (BILAG). Anti-Ro/SSA correlated positively with, headache (r=0.24, p=0.04), blurring of vision (r=0.25, p=0.03) and SLEDAI (r=0.25, p=0.04) and negatively with C3 (r=–0.35, p=0.003). Anti-Ro/SSA correlated with anti La/SSB antibodies (r=0.69, p<0.001), but not with anti-DNA, anti-RNP and anti-Sm antibodies. Anti-La/SSB antibodies correlated with headache (r=0.26, p=0.03), SLEDAI (r=0.25, p=0.03) and negatively with C3 (r=–0.34, p=0.004). Anti-La/SSB did not correlate with anti-RNP or anti-Sm antibodies. Anti-Sm antibodies correlated with disease duration (r=0.34, p=0.003), 24 hours urinary proteins (r=0.31, p=0.008), SLEDAI (r=0.31, p=0.009), BILAG renal score (r=0.29, p=0.02) and negatively with age at onset (r=–0.27, p=0.02), WBCs (r=–0.29, p=0.014) and C4 (r=–0.25, p=0.049). In multivariate analyses, anti-Ro/SSA antibodies remained associated with headache, blurring of vision and C3 and anti-La/SSB antibodies remained associated with C3 and with headache. Anti-Sm antibodies were independently associated with disease duration and total SLEDAI scores, while anti-RNP antibodies remained significantly associated with BILAG mucocutaneous scores only. Antibodies to ENAs are associated with clinical aspects of SLE and may play a role in the assessment of disease activity. Insight into these ENAs may lead to new approaches to diagnostic testing, accurate evaluation of disease activity and lead to target approach for SLE.

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Prednisolone dose during treatment of tuberculosis might be a risk factor for mortality in patients with systemic lupus erythematosus: a hospital-based cohort study

Lupus ◽

10.1177/0961203319882759 ◽

2019 ◽

Vol 28 (14) ◽

pp. 1699-1704 ◽

Cited By ~ 2

Author(s):

C F Cheng ◽

Y M Huang ◽

C H Lu ◽

S C Hsieh ◽

K J Li

Keyword(s):

Systemic Lupus Erythematosus ◽

Risk Factor ◽

Lupus Erythematosus ◽

Independent Risk Factor ◽

Cox Regression ◽

Activity Index ◽

Central Nervous System Involvement ◽

Systemic Lupus ◽

Prednisolone Dose ◽

Tb Treatment

Patients with systemic lupus erythematosus (SLE) are at high risk of tuberculosis (TB) because of their immunocompromised status and the use of immunosuppressive drugs. In endemic regions, TB complicates the diagnosis and treatment of SLE, but the risk factors of mortality in these patients have not been investigated. In this study, we reviewed medical records during 2006–2016. Patients who fulfilled the 1997 American College of Rheumatology SLE criteria and presented with definite TB were enrolled. The primary outcome was mortality during TB treatment. There were 5388 SLE patients screened, and 30 patients were enrolled. Seven patients died during follow-up. Compared with the survival group, patients in the mortality group had significantly more central nervous system involvement of TB, higher Systemic Lupus Erythematosus Disease Activity Index-2000 scores and more cyclophosphamide use before TB, and higher prednisolone dose before and during TB treatment. Cox regression showed that prednisolone dose during TB treatment was an independent risk factor for mortality (per 10 mg/day increase, hazard ratio (HR) 1.61, p = .019). For SLE patients, prednisolone dose during TB treatment is an independent risk factor for mortality. Keeping prednisolone dose at less than 25 mg per day during TB treatment might be a reasonable strategy in these patients.

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The problem of fatigue in patients with systemic lupus erythematosus according to the data on a Russian RENAISSANCE cohort

Modern Rheumatology Journal ◽

10.14412/1996-7012-2020-4-23-30 ◽

2020 ◽

Vol 14 (4) ◽

pp. 23-30

Author(s):

E. A. Aseeva ◽

S. K. Solovyev ◽

N. Yu. Nikishina ◽

G. M. Koilubaeva ◽

T. A. Lisitsyna ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

Disease Activity ◽

Lupus Erythematosus ◽

Activity Index ◽

Damage Index ◽

Clinical Manifestations ◽

Common Symptom ◽

Systemic Lupus ◽

Wide Range ◽

Antibody Levels

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with a wide range of clinical manifestations. Numerous observations and surveys of patients have shown that the most common symptom of SLE is fatigue complaints in 51 to 90% of patients.Objective: to determine the significance of fatigue in the general health status of RENAISSANCE cohort patients with SLE who were hospitalized in the Clinic, V.A. Nasonova Research Institute of Rheumatology.Patients and methods. The investigation included SLE patients aged 18 years and older who met the 2012 SLICC criteria. The standard examination accepted in the management of patients with SLE was made. Disease activity was determined by SLEDAI-2K; irreversible lesions in various organs were identified using the SLICC damage index. The SF-36 and the LupusQoL questionnaires were used to assess health-related quality of life (HRQOL) and the FACIT-Fatigue scale was applied to measure fatigue.Results and discussion. The investigation enrolled 328 patients, mainly women (91%); the mean age was 34.4±11.5 years; the duration of the disease was 106.3±97.9 months. In this group, moderate and high disease activities (SLEDAI-2K scores of 6–10 and 11–19, respectively) were observed at approximately the same frequency. At the time of inclusion, more than half (56.5%) of the patients already had various irreversible organ lesions. At Visit 1, the FACIT-Fatigue scale showed that fatigue was present in 148 (45%) of the 328 patients. According to the presence of fatigue, the patients were divided into two groups. Group 1 included 148 patients with fatigue; Group 2 consisted of 180 patients without fatigue. The Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) and anti-DNA antibody levels were significantly higher in the fatigue group (p=0.01 and p=0.02, respectively); the patients also had decreased HRQOL according to 7 LupusQol domains (p<0.001). The patients with fatigue were significantly more likely to receive intravenous glucocorticoids and rituximab. At 12 months after the start of treatment, the patients with fatigue were found to have a statistically significant reduction in disease activity, as well as normalization of anti-DNA antibody levels, improvements in HRQOL according to the LupusQol domains, and less severity of fatigue according to the FACIT-Fatigue scale.Conclusion. Fatigue was detected in almost half (45–53%) of SLE patients. It is associated with a higher disease activity by SLEDAI-2K and with a high anti-DNA antibody level. The patients with fatigue are observed to have an obvious worsening of HRQOL according to all LupusQol domains.

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