scholarly journals Clinical impact of hydroxychloroquine dose adjustment according to the American Academy of Ophthalmology guidelines in systemic lupus erythematosus

2020 ◽  
Vol 7 (1) ◽  
pp. e000395 ◽  
Author(s):  
Ileana Vázquez-Otero ◽  
Nicolle Medina-Cintrón ◽  
Mariangelí Arroyo-Ávila ◽  
Lorena González-Sepúlveda ◽  
Luis M Vilá

ObjectiveThe American Academy of Ophthalmology recommends a maximum hydroxychloroquine (HCQ) dose of ≤5.0 mg/kg/day to reduce the risk of HCQ-induced retinopathy. To determine if this dose adjustment would have an impact on the clinical course of SLE, we compared outcome measures in a cohort of patients with SLE before and after adjusting HCQ dose.MethodsSixty Puerto Ricans with SLE (per 1997 American College of Rheumatology criteria) treated with HCQ who were changed to HCQ ≤5.0 mg/kg/day were studied. Visits were ascertained every 6 months for 2 years before and 2 years after HCQ dose adjustment (baseline visit). Disease activity (per Systemic Lupus Erythematosus Disease Activity Index (SLEDAI)), SLE exacerbations, emergency room visits, hospitalisations, disease damage (per Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index), corticosteroids exposure, prednisone dose and immunosuppressive drugs exposure were determined before and after HCQ dose change.ResultsAt baseline visit, the mean age was 43.8±15.1 years. All patients were women. The mean disease duration was 13.8±9.1 years. After HCQ dose adjustment, patients required a lower prednisone dose when compared with visits before HCQ dose reduction. No significant differences were observed for mean SLEDAI scores, lupus exacerbations, emergency room visits, hospitalisations, disease damage and exposure to immunosuppressive drugs before and after HCQ dose adjustment.ConclusionsThis study suggests that adjustment of daily HCQ dose to ≤5.0 mg/kg/day of actual body weight does not have a significant impact on the short-term and mid-term outcomes in this group of patients with SLE.

2014 ◽  
Vol 41 (5) ◽  
pp. 875-880 ◽  
Author(s):  
Murray B. Urowitz ◽  
Dafna D. Gladman ◽  
Dominique Ibañez ◽  
Jorge Sanchez-Guerrero ◽  
Juanita Romero-Diaz ◽  
...  

Objective.To determine the frequency of each American College of Rheumatology (ACR) criterion met at time of enrollment, and the increase in each of the criteria over 5 years.Methods.In 2000 the Systemic Lupus International Collaborating Clinics (SLICC) recruited an international inception cohort of patients with systemic lupus erythematosus (SLE; ≥ 4 ACR criteria) who were followed at yearly intervals according to a standard protocol. Descriptive statistics were used to assess the total and cumulative number of ACR criteria met at each visit. Regression models were done to compare the increase of individual and cumulative criteria as a function of race/ethnicity group, and sex.ResultsIn all, 768 patients have been followed for a minimum of 5 years. Overall, 59.1% of the patients had an increase in the number of ACR criteria they met over the 5-year period. The mean number of ACR criteria met at enrollment was 5.04 ± 1.13 and at year 5 was 6.03 ± 1.42. At enrollment, nonwhite patients had a higher number of ACR criteria (5.19 ± 1.23) than white patients. The total number of criteria increased in both white and nonwhite ethnicities, but increased more among whites. Males had a slightly lower number of criteria at enrollment compared to females and males accrued fewer criteria at 5 years.Conclusion.In this international inception cohort of SLE patients with at least 4 ACR criteria at entry, there was an accumulation of ACR criteria over the following 5 years. The distribution of criteria both at inception and over 5 years is affected by sex and ethnicity.


Author(s):  
T. P. Makarova ◽  
A. V. Sukalo ◽  
I. A. Kazyro ◽  
Yu. S. Melnikova ◽  
N. N. Firsova ◽  
...  

Systemic lupus erythematosus is an autoimmune disease characterized by a pronounced polymorphism of clinical manifestations. Lupus nephritis is the most severe manifestation of the disease. The article presents a retrospective analysis of the cases of systemic lupus erythematosus and assessment of the clinical manifestations of the disease and variants of lupus nephritis in children in the Republics of Belarus and Tatarstan. The authors analyzed 60 cases of systemic lupus erythematosus, lupus nephritis. All patients had at least 4 of the 11 diagnostic criteria proposed by the American College of rheumatology (ACR, 1997), and 35 patients had a morphologically verified nephritis. It was found that the disease in children developed very actively with fast multi-organ involvement and it required aggressive therapy with several immunosuppressive drugs. During follow-up, the percentage of patients with renal damage increased, so renal function should be controlled in all patients with systemic lupus erythematosus, especially with early onset. Lupus nephritis is combined with extrarenal manifestations and it is difficult to diagnose when it begins with kidney damage. The overall survival rate of children with systemic lupus erythematosus is closely related to the severity of renal manifestations. Lupus nephritis is a serious problem that requires early aggressive intervention and continuous maintenance therapy.


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 646.1-646
Author(s):  
O. Gioti ◽  
K. Chavatza ◽  
G. Belevonis ◽  
S. Koutsoviti ◽  
C. Katsimpari ◽  
...  

Background:The current goal of treatment in SLE is remission or low disease activity (LDA) and prevention of flares, achieved with the lowest possible dose of glucocorticoids. Nevertheless, in current clinical practice a significant number of patients still has active disease.1,2Objectives:To assess the current disease activity state of SLE patients during their most recent visit in two centers (Department of Rheumatology in “Asklepieio” Hospital and Rheumatology Unit in “Attikon” Hospital, both in Athens, Greece).Methods:Cross-sectional study of patients with a diagnosis of SLE for at least one year. Patients were divided into four groups: 1) Remission off-therapy: SLE Disease Activity Index (SLEDAI)=0 without prednisone or immunosuppressive drugs (IS), 2) Remission on-therapy: SLEDAI=0, prednisone dose ≤5mg/day and/or IS (conventional and biologic, maintenance phase), 3) LDA: SLEDAI ≤4, prednisone dose ≤7.5mg/day and/or IS (maintenance phase), 4) Active disease: SLEDAI >4 and/or prednisone dose >7.5mg/day and/or IS (induction phase).2 Hydroxychloroquine was allowed in all groups.Results:205 patients were included [95.1% female, mean (SD) age 48.4 (14.9) years and median disease duration (IQR) 6.2 (12.6) years]. A history of lupus nephritis and neuropsychiatric SLE was present in 16.6% and 17.1% of our patients, respectively, and 39% of patients had SLICC/ACR damage index (SDI) > 0. At last visit, remission off-therapy and remission on-therapy was present in 8.3% (n=17) and 15.1% (n=31) of our patients, respectively. Seventy-five patients (36.6%) had LDA, whereas 82 patients (40%) had active disease. More than 85% (86.3%) of patients were in treatment with hydroxychloroquine and 64.4% were receiving immunosuppressive drugs. Regarding glucocorticoids, 50.2% (n=103) were treated with prednisone dose ≤7.5mg/day and over 40% (42.4%, n=87) did not receive prednisone at all. A SLEDAI score 0 and 1-4 was achieved in 24.4% and 42.9% of patients, respectively, but only 3.9% had a SLEDAI > 8, indicative of high disease activity.Conclusion:Although the majority of our patients were treated with hydroxychloroquine and glucocorticoids in acceptable levels of daily dose, four out of ten patients in our practice have active disease during their last visit. Achieving treatment goals in SLE patients remains a challenge for future novel therapies.References:[1]Fanouriakis A, Kostopoulou M, Alunno A, et al. 2019 update of the EULAR recommendations for the management of systemic lupus erythematosus. Ann Rheum Dis 2019; 78: 736–745.[2]Ugarte-Gil MF, Wojdyla D, Pons-Estel GJ, et al. Remission and Low Disease Activity Status (LDAS) protect lupus patients from damage occurrence: data from a multiethnic, multinational Latin American Lupus Cohort (GLADEL). Ann Rheum Dis 2017; 76: 2071–2074.Disclosure of Interests:None declared


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1175.1-1175
Author(s):  
R. Hamdy Abdellatif Mohammed ◽  
H. Lotfy Fayed ◽  
N. Emara

Background:Systemic lupus erythematosus (SLE) is a complex autoimmune disorder with considerably high mortality.Objectives:To investigate the survival rates 5, 10, 15 and 20 years and the impact of disease related morbidity and mortality among Egyptians patients afflicted by SLE.Methods:This is a single center observational study performed in one of the leading medical school governmental hospital for teaching and training in the north African region and middle east sectors Kasr Alainy School of Medicine- Cairo University. Medical records of adult SLE patients ≥ 16 years (classified according to ACR 1997 SLE classification criteria set forth by Hochberg, 1997) who received longitudinal clinical care during the time period from 1999 to 2019 were included. Data analysis: causes of mortality, damage score and survival were determined from the time of SLE diagnosis to the last contact or date of death.Results:Records of two hundred and two SLE patients were included, 91.1% were females and 8.9% patients were males (ratio is 10:1). The mean age at diagnosis 26.71 ± 7.93 years with a mean follow up between mean: 6.6 ± 4.58 years, 34.15% had damage in at least one of the organ systems by SLICC/ACR-DI in the first 6 months. Considering an outcome label of dead or alive at the end of follow up period, results showed a total of 52 mortalities, 88.5% were females, the mean age at death onset was 30.9±8.8 years. Results identified the following death causalities in the studied SLE patients in order of frequency: Septic shock and disseminated intravascular coagulation in 11.5 %, acute respiratory distress syndrome ARDS in 11.5 %, congestive heart failure in 9.6%, thrombotic microangiopathy 5.7%, cerebritis, acute renal failure 5.7%, intracranial hemorrhage 5.7%, hypertensive encephalopathy in 5.7%. Alveolar hemorrhage, infection, intraoperative deaths each contributed to deaths in 3.8%. Hypovolemic shock, acute liver failure, brain edema, thrombotic thrombocytopenic purpura, end stage kidney disease, pulmonary renal syndrome, suicide and acute hydrocephalus contributed to fatalities in 1.9%. The cause of death was unclearly identified in 26.9%. Results of the Kaplan Meier survival curve in the studied SLE cohort showed an overall cumulative probability of survival at 5, 10, 15 and 20 years after SLE diagnosis was 82.9%, 68.8%,51.4% and 20.4%, respectively. Multivariate regression analysis revealed psychosis, chronic kidney disease and heart failure were independent predictors of survival (HR= 4.3 times, 3.58 times and 3 times respectively, p < 0.001), while the use of hydroxychloroquine and AZA showed a protective effect.Figure 1.Kaplan-Meier estimated survival function, starting at date of SLE diagnosis.Conclusion:The cumulative probability of survival at 5, 10, 15 and 20 years after SLE diagnosis was 82.9%, 68.8%,51.4% and 20.4%, respectively. The presence of renal manifestations, neuropsychiatric lupus and heart failure were independent predictors of poor survival in our cohort. The use of hydroxychloroquine and AZA were protective.Corresponding author: Reem Hamdy Abdellatif Mohammed (Reem H A Mohammed), e-mail: [email protected],. https://orcid.org/0000-0003-4994-7687, Scopus Author ID: 35280107100.References:[1]Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. 1997 Sep;40(9):1725. doi: 10.1002/art.1780400928. PMID: 9324032.[2]Kasitanon N, Magder LS, Petri M. Predictors of survival in systemic lupus erythematosus. Medicine (Baltimore) 2006;85:147–56.[3]Gladman D, Ginzler E, Goldsmith C, et al. The development and initial validation of the Systemic Lupus International Collaborating Clinics/American College of Rheumatology damage index for systemic lupus erythematosus. Arthritis Rheum 1996;39:363–9.Disclosure of Interests:None declared


Lupus ◽  
2021 ◽  
pp. 096120332110345
Author(s):  
Stefan Vordenbäumen ◽  
Alexander Sokolowski ◽  
Anna Rosenbaum ◽  
Claudia Gebhard ◽  
Johanna Raithel ◽  
...  

Objective Hypomethylation of CD40-ligand (CD40L) in T-cells is associated with increased disease activity in systemic lupus erythematosus (SLE). We therefore investigated possible associations of dietary methyl donors and products with CD40L methylation status in SLE. Methods Food frequency questionnaires were employed to calculate methyl donor micronutrients in 61 female SLE patients (age 45.7 ± 12.0 years, disease duration 16.2 ± 8.4 years) and compared to methylation levels of previously identified key DNA methylation sites (CpG17 and CpG22) within CD40L promotor of T-cells using quantitative DNA methylation analysis on the EpiTYPER mass spectrometry platform. Disease activity was assessed by SLE Disease Activity Index (SLEDAI). Linear regression modelling was used. P values were adjusted according to Benjamini & Hochberg. Results Amongst the micronutrients assessed (g per day), methionine and cysteine were associated with methylation of CpG17 (β = 5.0 (95%CI: 0.6-9.4), p = 0.04; and β = 2.4 (0.6-4.1), p = 0.02, respectively). Methionine, choline, and cysteine were additionally associated with the mean methylation of the entire CD40L (β = 9.5 (1.0-18.0), p = 0.04; β = 1.6 (0.4-3.0), p = 0.04; and β = 4.3 (0.9-7.7), p = 0.02, respectively). Associations of the SLEDAI with hypomethylation were confirmed for CpG17 (β=-32.6 (-60.6 to -4.6), p = 0.04) and CpG22 (β=-38.3 (-61.2 to -15.4), p = 0.004), but not the mean methylation of CD40L. Dietary products with the highest impact on methylation included meat, ice cream, white bread, and cooked potatoes. Conclusions Dietary methyl donors may influence DNA methylation levels and thereby disease activity in SLE.


Lupus ◽  
2021 ◽  
pp. 096120332110050
Author(s):  
Rory C Monahan ◽  
Liesbeth JJ Beaart-van de Voorde ◽  
Jeroen Eikenboom ◽  
Rolf Fronczek ◽  
Margreet Kloppenburg ◽  
...  

Introduction We aimed to investigate risk factors for fatigue in patients with systemic lupus erythematosus (SLE) and neuropsychiatric symptoms in order to identify potential interventional strategies. Methods Patients visiting the neuropsychiatric SLE (NPSLE) clinic of the Leiden University Medical Center between 2007–2019 were included. In a multidisciplinary consensus meeting, SLE patients were classified as having neuropsychiatric symptoms of inflammatory origin (inflammatory phenotype) or other origin (non-inflammatory phenotype). Fatigue was assessed with the SF-36 vitality domain (VT) since 2007 and the multidimensional fatigue inventory (MFI) and visual analogue scale (VAS) since 2011. Patients with a score on the SF-36 VT ≥1 standard deviation (SD) away from the mean of age-related controls of the general population were classified as fatigued; patients ≥2 SD away were classified as extremely fatigued. Disease activity was measured using the SLE disease activity index-2000. The influence of the presence of an inflammatory phenotype, disease activity and symptoms of depression and anxiety as measured by the hospital anxiety and depression scale (HADS) was analyzed using multiple regression analyses corrected for age, sex and education. Results 348 out of 371 eligible patients filled in questionnaires and were included in this study . The majority was female (87%) and the mean age was 43 ± 14 years. 72 patients (21%) had neuropsychiatric symptoms of an inflammatory origin. Fatigue was present in 78% of all patients and extreme fatigue was present in 50% of patients with an inflammatory phenotype vs 46% in the non-inflammatory phenotype. Fatigue was similar in patients with an inflammatory phenotype compared to patients with a non-inflammatory phenotype on the SF-36 VT (β: 0.8 (95% CI −4.8; 6.1) and there was less fatigue in patients with an inflammatory phenotype on the MFI and VAS (β: −3.7 (95% CI: −6.9; −0.5) and β: −1.0 (95% CI −1.6; −0.3)). There was no association between disease activity and fatigue, but symptoms of anxiety and depression (HADS) associated strongly with all fatigue measurements. Conclusion This study suggests that intervention strategies to target fatigue in (NP)SLE patients may need to focus on symptoms of anxiety and depression rather than immunosuppressive treatment.


Author(s):  
Fahima Hossain ◽  
Mohammad Delwer Hossain Hawlader ◽  
Dipak Kumar Mitra ◽  
Mohammad Hayatun Nabi ◽  
Md. Mujibur Rahman

Abstract Background Neuropsychiatric systemic lupus erythematosus (NPSLE) is well known for its varying presentations and poor outcomes, but little is evident about its distribution and characteristics among the Bangladeshi population. This study aimed to assess the pattern and prevalence of neuropsychiatric symptoms in female systemic lupus erythematosus (SLE) patients of Bangladesh. A retrospective study was conducted at a tertiary care hospital in Dhaka, Bangladesh, between January and December 2018. One hundred female SLE patients were included in the study purposively. Data were collected on sociodemographic and clinical characteristics of diagnosed SLE cases visiting the SLE clinic and indoor medicine department. Neuropsychiatric (NP) syndromes were defined according to the widely accepted American College of Rheumatology (ACR) nomenclature and case definitions. Results A total of 244 NP events were identified in fifty-five patients. Headache was the most frequent symptom (55%), followed by cognitive dysfunction (50%), anxiety (49%), psychosis (43%), seizure (23%), depression (17%), and cerebrovascular disease (ischemic type, 7%). The NP manifestations were more prevalent among urban residents (58.2%), younger patients (41.8%), and patients with graduate-level education (34.5%). Besides, young age at diagnosis (p = 0.038), Raynaud’s phenomenon (p = 0.015), other organ involvement (p < 0.001), and time of NPSLE development (p < 0.001) were found to be significantly associated with the development of these manifestations. Conclusion NP damage is prevalent among Bangladeshi female SLE patients (55%) with headache and cognitive dysfunction being the most common symptoms. Routine screening for neuropsychiatric symptoms among suspected SLE cases and further evaluation with a larger population are warranted.


Healthcare ◽  
2021 ◽  
Vol 9 (6) ◽  
pp. 726
Author(s):  
Fulvia Ceccarelli ◽  
Venusia Covelli ◽  
Giulio Olivieri ◽  
Francesco Natalucci ◽  
Fabrizio Conti

Background: The COVID-19 pandemic contributes to the burden of living with different diseases, including Systemic Lupus Erythematosus (SLE). We described, from a narrative point of view, the experiences and perspectives of Italian SLE adults during the COVID-19 emergency, by distinguishing the illness experience before and after the lockdown. Methods: Fifteen patients were invited to participate. Illness narratives were collected between 22 and 29 March 2020 using a written modality to capture patients’ perspectives before and after the COVID-19 lockdown. We performed a two-fold analysis of collected data by distinguishing three narrative types and a qualitative analysis of content to identify the relevant themes and sub-themes reported. Results: Eight narratives included in the final analysis (mean length 436.9 words) have been written by eight females (mean age 43.3 ± 9.9 years, mean disease duration 13.1 ± 7.4 years). Six patients provided a quest narrative, one a chaos and the remaining one a restitution narrative. By text content analysis, we identified specific themes, temporally distinct before and after the lockdown. Before COVID-19, all the patients referred to a good control of disease, however the unexpected arrival of the COVID-19 emergency broke a balance, and patients perceived the loss of health status control, with anxiety and stress. Conclusions: We provided unique insight into the experiences of people with SLE at the time of COVID-19, underlining the perspective of patients in relation to the pandemic.


Lupus ◽  
2021 ◽  
pp. 096120332110310
Author(s):  
Mehmet Ersin ◽  
Mehmet Demirel ◽  
Mehmet Ekinci ◽  
Lezgin Mert ◽  
Çiğdem Çetin ◽  
...  

Objective Osteonecrosis (ON), also known as avascular necrosis, is characterized by the collapse of the architectural bone structure secondary to the death of the bone marrow and trabecular bone. Osteonecrosis may accompany many conditions, especially rheumatic diseases. Among rheumatic diseases, osteonecrosis is most commonly associated with systemic lupus erythematosus (SLE). We assessed prevalence and distribution pattern of symptomatic ON in patients with SLE and compare the natural courses of hip and knee ON. Methods 912 SLE patients admitted between 1981 and 2012 were reviewed. SLE patients with symptomatic ON were retrospectively identified both from the existing SLE/APS database. The prevalence of symptomatic ON was calculated; with ON, the joint involvement pattern was determined by examining the distribution of the joints involved, and then the data about the hip and knee joints were entered in the Kaplan-Meier analysis. Kaplan-Meier methods were used to calculate 5- and 10-year rates of ON-related hip (the hip group) and knee survival (the knee group). Results Symptomatic ON developed in various joints in 97 of 912 patients with SLE, and the overall prevalence of ON was detected as 10.6%. The mean age at the time of SLE and ON diagnoses were 27.9 ± 9.9 (14–53) and 34.2 ± 11.3 (16–62) years, respectively. The mean duration from diagnosis of SLE to the first development of ON was 70.7± 60.2 (range = 0–216) months. The most common site for symptomatic ON was the hips (68%, n=66), followed by the knees (38%, n = 37). According to Kaplan-Meier analysis, hip and knee joint survival rates associated with 5-year ON were 51% and 88%, and 10-year survival rates were 43% and 84%, respectively. Conclusion We observed that the prevalence of symptomatic ON in patients with SLE was 10.6%. With the estimated 10-year survival rates of 40% versus 84% for the hip and knee joints, respectively, hip involvement may demonstrate a more aggressive course to end-stage osteoarthritis than the knee involvement.


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