scholarly journals Utilization of Rituximab for Refractory Rowell Syndrome

2021 ◽  
Vol 2021 ◽  
pp. 1-6
Author(s):  
Sukhraj Singh ◽  
Sandra Sheffield ◽  
Nisha Chowdhury ◽  
Swetha Nuthulaganti ◽  
Zareen Vaghaiwalla ◽  
...  

Rowell syndrome describes the occurrence of erythema multiforme-like lesions in patients with cutaneous lesions of lupus erythematosus. The clinical picture of atypical erythema multiforme-like lesions, presence of chilblains, speckled ANA pattern, anti-Ro/SSA, or anti-La/SSB antibodies, and absence of infectious or pharmacologic triggers in a patient with systemic lupus erythematosus are some of the classic clinical and serologic features. Histopathologic and serologic findings can help differentiate this process from erythema multiforme. We present a case of young woman with systemic lupus erythematosus, end-stage renal disease due to lupus nephritis, and a remote history of Steven–Johnson syndrome due to sulfa allergy who presented to the hospital with a recurrent, progressive, targetoid erythematous rash involving more than 60% of her body surface area. Our patient had several hospitalizations in the recent past for this erythematous rash and had failed oral therapy with prednisone 1 mg/kg and hydroxychloroquine. In view of the minimal improvement and increasing severity and patient exhibiting early features of mast cell activation syndrome, the patient was treated with pulse intravenous glucocorticoids followed by rituximab with an excellent response. We highlight a unique case report of progressive Rowell syndrome refractory to standard of care with an excellent response to rituximab.

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 235.2-235
Author(s):  
J. Y. E. Lee ◽  
A. Mendel ◽  
I. Malhamé ◽  
S. Bernatsky ◽  
E. Vinet

Background:Pregnant women with systemic lupus erythematosus (SLE) are at high risk of preeclampsia, leading to substantial maternal and fetal morbidity. Aspirin reduces preeclampsia risk but recent studies suggest aspirin is used only in a minority of SLE pregnancies. There is an urgent need to improve preeclampsia counselling and management in this vulnerable population.Objectives:We are conducting the PREPARE (PREeclamPsia knowledge & Aspirin adheRence in lupus prEgnancies) trial, a randomized controlled trial (RCT) evaluating an educational tool on preeclampsia knowledge and aspirin adherence among pregnant women with SLE. We present preliminary analyses of the effect of this tool on preeclampsia knowledge.Methods:Consecutive pregnant SLE women are recruited until the 16th gestational week at 5Canadian Systemic Lupus International Collaborating Clinics centres (i.e. Montreal, Halifax, Quebec, Winnipeg, and Calgary) since 05/2018. Subjects are randomly assigned to receive either the specifically-designed educational tool (intervention group) or standard of care (control group). At baseline (i.e. first trimester) and second trimester visits, the participants complete self-administered preeclampsia knowledge questionnaires (scored out of 30 by the research team blinded to the intervention). We restricted the current analysis to participants enrolled in Montreal (accounting for nearly half of the total planned sample size). We performed a univariate linear regression analysis to assess the effect of the educational tool on preeclampsia knowledge (i.e. mean score difference between the two groups from baseline to second trimester visit).Results:Thirty-three pregnant SLE women were included in the study, among which 16 were exposed to the intervention and 17 were unexposed. Baseline characteristics were well balanced between the two groups with similar mean maternal age between intervention group (32.2 years, standard deviation, SD, 4.6) and control group (34.1 years, SD 4.2) and identical proportion of subjects with post-secondary education (i.e. 80%). The difference in mean preeclampsia knowledge scores between second trimester and baseline visits in the intervention group was 4.4 points (95% CI -0.1, 9.0) and in the control group was 1.5 points (95% CI -2.7, 5.7). The mean difference in knowledge scores (from baseline to second trimester) for those receiving the educational tool was 2.7 points higher (95% CI -1.5, 6.9) than those receiving standard of care.Conclusion:Approximately midway into the PREPARE trial, we observed a trend for improvement in preeclampsia knowledge from the baseline to the second trimester visit in pregnant women with SLE who received a specifically-designed educational tool compared to the control group, although the CIs included the null. Our RCT is well-poised to provide a new evidence-based approach to improve preeclampsia knowledge in pregnant women with SLE, which could help to optimize aspirin use and outcomes in this vulnerable population.References:[1]Schramm AM, Clowse ME. Aspirin for prevention of preeclampsia in lupus pregnancy. Autoimmune Dis. 2014;2014:920467. doi:10.1155/2014/920467[2]Bujold E, Roberge S, Lacasse Y, et al. Prevention of preeclampsia and intrauterine growth restriction with aspirin started in early pregnancy: a meta-analysis. Obstet Gynecol. 2010;116(2 Pt 1):402-414. doi:10.1097/AOG.0b013e3181e9322a[3]Andreoli L, Bertsias GK, Agmon-Levin N, et al. EULAR recommendations for women’s health and the management of family planning, assisted reproduction, pregnancy and menopause in patients with systemic lupus erythematosus and/or antiphospholipid syndrome. Ann Rheum Dis. 2017 Mar;76(3):476–85. doi: 10.1136/annrheumdis-2016-209770.[4]Mendel A, Bernatsky SB, Hanly JG, et al. Low aspirin use and high prevalence of preeclampsia risk factors among pregnant women in a multinational SLE inception cohort. Ann Rheum Dis. 2019;78(7):1010-1012. doi:10.1136/annrheumdis-2018-214434Disclosure of Interests:None declared.


Lupus ◽  
2021 ◽  
pp. 096120332199010
Author(s):  
Vineeta Shobha ◽  
Anu Mohan ◽  
AV Malini ◽  
Puneet Chopra ◽  
Preethi Karunanithi ◽  
...  

Objective Despite the significant advancement in the understanding of the pathophysiology of systemic lupus erythematosus (SLE) variable clinical response to newer therapies remain a major concern, especially for patients with lupus nephritis and neuropsychiatric systemic lupus erythematosus (NPSLE). We performed this study with an objective to comprehensively characterize Indian SLE patients with renal and neuropsychiatric manifestation with respect to their gene signature, cytokine profile and immune cell phenotypes. Methods We characterized 68 Indian SLE subjects with diverse clinical profiles and disease activity and tried to identify differentially expressed genes and enriched pathways. To understand the temporal profile, same patients were followed at 6 and 12-months intervals. Additionally, auto-antibody profile, levels of various chemokines, cytokines and the proportion of different immune cells and their activation status were captured in these subjects. Results Multiple IFN-related pathways were enriched with significant increase in IFN-I gene signature in SLE patients as compared to normal healthy volunteers (NHV). We identified two transcriptionally distinct clusters within the same cohort of SLE patients with differential immune cell activation status, auto-antibody as well as plasma chemokines and cytokines profile. Conclusions Identification of two distinct clusters of patients based on IFN-I signature provided new insights into the heterogeneity of underlying disease pathogenesis of Indian SLE cohort. Importantly, patient within those clusters retain their distinct expression dynamics of IFN-I signature over the time course of one year despite change in disease activity. This study will guide clinicians and researchers while designing future clinical trials on Indian SLE cohort.


2009 ◽  
Vol 15 (8) ◽  
Author(s):  
Arnold Lee ◽  
Priya Batra ◽  
Vika Furer ◽  
Wang Cheung ◽  
Nadia Wang ◽  
...  

2003 ◽  
Vol 7 (3) ◽  
pp. 232-235
Author(s):  
Audrey Dupéré ◽  
Yves Poulin

Background: Acquired partial lipodystrophy is a rare disorder. An association with systemic lupus erythematosus has been reported. In these cases, an immunologic basis is suggested by the presence of C3 nephritic factor and hypocomplementemia. Objective: The following report presents the case of a woman who developed a rapid loss of facial subcutaneous fat a few months after complete spontaneous resolution of cutaneous lesions of lupus. Conclusion: Absence of C3NeF in this case suggests that other immunological factors may be involved in the pathogenesis.


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