Cytomegalovirus-induced cutaneous ulcer mimicking vasculitis in a patient with systemic lupus erythematous: A case report and review of the literature

Lupus ◽  
2020 ◽  
Vol 30 (1) ◽  
pp. 149-154
Author(s):  
Zahra Hajihashemi ◽  
Farahnaz Bidari-zerehpoosh ◽  
Khatere Zahedi ◽  
Behnaz Eslami ◽  
Nikoo Mozafari
Keyword(s):  
Lupus Erythematosus ◽  
Correct Diagnosis ◽  
Cmv Infection ◽  
Systemic Lupus ◽  
Cytoplasmic Inclusions ◽  
Delay In Diagnosis ◽  
Reactivation Treatment ◽  
Cutaneous Ulcer ◽  
Chronic Autoimmune Disease ◽  
Cmv Reactivation

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease which can be complicated with cytomegalovirus (CMV) infection during its course. CMV reactivation can mimic an SLE flare and lead to delay in diagnosis. Here, we reported a previously diagnosed SLE patient who presented with fever, leukopenia, and cutaneous ulcers. Initially, this was diagnosed as an SLE flare and the patient was treated with higher doses of corticosteroids but no improvement was observed. Both nuclear and cytoplasmic inclusions inside the endothelial cells in the skin biopsy as well as positive immunohistochemistry (IHC) staining for CMV antigen were clues to the correct diagnosis of CMV reactivation. Treatment with ganciclovir resulted in clinical resolution. In this report, a very rare clinical form of CMV infection manifesting as cutaneous necrotizing vasculitis on the lower extremity is described and the literature regarding this case is reviewed.

scholarly journals OP0102 GENE SCREENING OF PRIMARY IMMUNODEFICIENCY DISEASES IN PATIENTS WITH JUVENILE SYSTEMIC LUPUS ERYTHEMATOSUS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 66.2-66
Author(s):  
J. LI ◽  
W. Wang ◽  
C. Y. Wang ◽  
J. Y. Pan ◽  
H. Song
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Primary Immunodeficiency ◽  
Lupus Erythematosus ◽  
Gene Sequencing ◽  
Primary Immunodeficiency Disease ◽  
Type I ◽  
Cmv Infection ◽  
Systemic Lupus ◽  
Genetic Tests ◽  
Immunodeficiency Disease

Background:Systemic lupus erythematosus (SLE) is one of the most common auto-immune diseases in childhood. Primary immunodeficiency disease (PID) patients may present or combine with autoimmune diseases.Objectives:This study aimed to perform gene sequencing via high-throughput sequencing technology in a series of Chinese pediatric SLE patients, and investigate the concomitant situation of PIDs and SLE. Gene sequencing results may help clarify the pathogenesis of SLE.Methods:This was a retrospective case series of SLE children who referred to the Peking Union Medical College Hospital between 01/2016 and 09/2019. Genetic tests were performed in patients who met the inclusion criteria. We then collected demographic, clinical, and treatment information of all involved patients. Descriptive statistics were used.Results:Seventy-one patients were finally included (eighteen boys and fifty-three girls). The median age at the time of disease onset was 9.5 (range, 3-15) years. It is notable that five patients experienced their first attack before the age of five. Twenty-seven patients showed a persistent increase in ESR during treatment, while thirteen cases presented with repeated CMV infection, thirty-four cases with persistent low complement levels, seven with basal ganglia calcification showed in skull CT or MRI, four with special type of rash (i.e., frostbite-like rash, discoid erythema, reticular erythema), two with obvious hepatosplenomegaly, and one case with type I diabetes. Gene sequencing results showed that about ten patients combine with primary immunodeficiency disease, including Aicardi-Goutières Syndrome (AGS) (n=4), Spondyloenchondro-dysplasia with immune dysregulation (SPENCDI) (n=1), STING-associated vasculopathy with onset in infancy (SAVI) (n=1), lysinuric protein intolerance (LPI) (n=1), Ras-associated autoimmune leukoproliferative disorder (RALD) (n=2).Conclusion:SLE patients who present atypical or refractory manifestations should attach importance to the existence of primary immunodeficiency disease. Genetic tests are recommended for patients with early-onset SLE, especially those with recurrent frostbite-like rash or persistent CMV infection since childhood.References:[1]T Tarr, B Dérfalvi, N Győri, et al. Similarities and differences between pediatric and adult patients with systemic lupus erythematosus[J]. Lupus. 2015, 24: 796–803.[2]Gupta S, Louis A G. Tolerance and Autoimmunity in Primary Immunodeficiency Disease: a Comprehensive Review[J]. Clinical Reviews in Allergy & Immunology, 2013, 45(2):162-169.Disclosure of Interests:None declared

Frequency of Histopathological patterns of Lupus Nephritis according to classification by WHO among Pakistani patients

2021 ◽  
Vol 15 (9) ◽  
pp. 2343-2344
Author(s):  
Aijaz Z. Khan Chachar ◽  
Miqdad Haider ◽  
Naveed A. Lashari ◽  
M. Mueed Yasin ◽  
Hafiz B. A. Kalhoro ◽  
...  
Keyword(s):  
Lupus Nephritis ◽  
Renal Biopsy ◽  
Lupus Erythematosus ◽  
Who Classification ◽  
Correct Diagnosis ◽  
Class Ii ◽  
Class Iii ◽  
Childbearing Age ◽  
Systemic Lupus ◽  
Cross Sectional

Background: Systemic lupus erythematosus (SLE) is an autoimmune disorder, multisystemic in nature more common in females of childbearing age. There are certain risk factors which predispose to this disease. It affects various organs, kidney is among them. Almost 60% patients having SLE ultimately leads to kidney dysfunction at some stage of the life. Aim: To find out pattern of histopathological findings of lupus nephritis as per WHO classification on kidney biopsy in Pakistan. Methodology: This cross-sectional study was completed in department of Medicine, Fatima Memorial Hospital, Lahore, from March 2016 to May, 2018. Total sample size was 165 patients. Only patients who fulfilled the 2012 SLICC (Systemic Lupus International Collaborating Clinics) criteria were included in the study. SPSS version 25.0 was used data analysis. Results: Age of the patients was between 31-50 years i.e. 114(69.09%), mean and SD was 43.96±4.84 years, females were more commonly affected by calculating 99(59.70%). Patterns of lupus nephritis as per WHO classification and renal biopsy were noted which shows 18(10.91%) had Class I, 53(32.12%) Class II, 43(26.07%) Class III, 35(21.20%) Class IV, 10(6.06%) Class V and 6(3.64%) had Class VI. Conclusion: Class II and Class III Lupus Nephritis are the most common modalities found in patients of SLE. Every patient with Lupus Nephritis should undergo a Renal Biopsy for correct diagnosis of the class of this disease and further management accordingly. Keywords: Lupus Nephritis, SLE, renal biopsy

scholarly journals Dendritic cells’ characteristics in patients with treated systemic lupus erythematosus

2020 ◽  
Author(s):  
Anna Wardowska ◽  
Żaneta Smoleńska ◽  
Katarzyna A. Lisowska ◽  
Zbigniew Zdrojewski ◽  
Michał Pikuła
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Dendritic Cells ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Systemic Lupus ◽  
Complement Components ◽  
Expression Of Genes ◽  
Luminex Technology ◽  
Transcriptional Changes ◽  
Chronic Autoimmune Disease

The systemic lupus erythematosus (SLE) is a chronic autoimmune disease related to a loss of immune tolerance against autoantigens that leads to tissue inflammation and organ dysfunction. Constant stimulation of dendritic cells (DC) with autoantigens is hypothesized to increase the B cells’ activity which are involved in production of autoantibodies that play an essential role in the SLE development. We focused our study on detecting alterations in DCs at the cellular and molecular levels in patients with treated SLE, depending on the disease activity and treatment. In order to phenotype subpopulations of DCs, multicolor flow cytometry was used. Transcriptional changes were identified with quantitative PCR, while soluble cytokine receptors were assessed with the Luminex technology. We show that SLE patients display a higher percentage of activated myeloid DCs (mDCs) when compared to healthy people. Both, the mDCs and plasmacytoid DCs (pDCs) of SLE patients were characterized by changes in expression of genes associated with their maturation, functioning and signalling, which was especially reflected by low expression of regulatory factor ID2 and increased expression of IRF5. pDCs of SLE patients also showed increased expression of IRF1. There were also significant changes in the expression of APRIL, MBD2, and E2-2 in mDCs that significantly correlated with some serum components, i.e. anti-dsDNA antibodies or complement components. However, we did not find any significant differences depending on the disease activity. While the majority of available studies focuses mainly on the role of pDCs in the disease development, our results show significant disturbances in the functioning of mDCs in SLE patients, thus confirming mDCs’ importance in SLE pathogenesis.

scholarly journals CYTOMEGALOVIRUS AND VIRUS EPSTEIN- BARR INFECTION IN PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS AND ITS DEPENDENCE ON GENDER AND AGE OF PATIENTS

World Science ◽  
2020 ◽  
Author(s):  
O. Abrahamovych ◽  
U. Abrahamovych ◽  
S. Guta ◽  
M. Farmaha ◽  
L. Kobak
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Epstein Barr Virus ◽  
Epstein Barr Virus Infection ◽  
Cmv Infection ◽  
Systemic Lupus ◽  
Barr Virus ◽  
Ebv Infection ◽  
Epstein Barr ◽  
Barr Virus Infection

Introduction. Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by various manifestations and clinical course, many aspects of the etiology and pathogenesis of which remain unclear. Recently, the interest of researchers in studying the role of cytomegalovirus (CMV) and Epstein - Barr virus (EBV) has been growing in the occurrence and course of a number of human diseases due to their ability to affect almost all organs and systems of the body, causing the formation of latent, active or chronic infection, which can often cause temporary disability, disability or even death, however, for the patients with SLE, despite the possibility of approaching the difficult problem of diagnosis and treatment of this disease, this issue is given insufficient attention, as evidenced by isolated studies.The aim of the study. Detect cytomegalovirus and Epstein - Barr infection in patients with systemic lupus erythematosus and its dependence on gender and age of patients. Materials and methods of research. The study involved 120 patients (15 men (12.50%) and 105 women (87.50%) aged 18 to 69 years with SLE, who were in the rheumatology department of the Communal Non-Commercial Enterprise of the Lviv Regional Council "Lviv Regional Clinical Hospital" in 2014-2019. To diagnose CMV and EBV infection by enzyme-linked immunosorbent assay, antibodies of IgM and IgG to viruses were detected in blood serum, and viruses were detected by polymerase chain reaction. According to the results of virus detection, formed groups of the patients, namely: patients with active CMV infection, active EBV, active CMV and EBV, without active CMV and EBV. All patients with SLE included in the study were subsequently stratified by age according to the classification of the World Health Organization (2015), according to which the following age limits were determined: young age, middle-aged, elderly, senile. Statistical analysis was performed on a personal computer in MS Excel and Statistica 6.0 using descriptive statistics. The frequency of cases of active CMV and EBV infection was calculated mathematically by the binomial coefficient of I. Newton. Research results and their discussion. We found in the vast majority of patients with SLE (117 patients, 97.50%) increase in the titer of specific antibodies to CMV. Only in 3 patients (2.50%) the titer of antibodies to this virus was within normal limits. Analyzing the frequency of EBV infection in patients with SLE, we recorded an increase in the titer of specific antibodies to the virus in 119 patients (99.17%). Among the examined patients with SLE in all (100.00%) found an increase in the titer of antibodies to CMV and / or EBV, of which 97.50% - infected with CMV and 97.17% - infected with EBV. The active phase of CMV and / or EBV infection was detected in 54.17%, of which 23.33% - active CMV infection, 17.50% - active EBV infection and 12.50% - a combination of active CMV and EBV infection simultaneously, which indicates a high frequency of CMV and EBV infection in patients with SLE and reflects the urgency of the problem of diagnosing herpesvirus infection in them. We found that activeCMV, EBV infections and their combinations are present only in women (64 patients, which is 60.96% of the total number of women with SLE), of which 28 patients (26.67%) there was only active CMV infection, in 21 patients (20.00%) - only active EBV infection and in 15 patients (14.29%) – combination of active CMV and EBV infection. 41 women (39.05%) and all (100.00%) men were not found to have active CMV and EBV infection, which indicates that men at the time of the survey were significantly more likely to have this infection in the integration phase. The most frequently active EBV infection was detected in patients with SLE of young age (17 cases, 24.64%), and in middle-aged patients 3 cases (6.52%) were recorded, which indicates a significant (p <0.05) difference in the frequency of cases of active EBV infection in patients of both groups. Only 1 case (20.00%) of active EBV infection was detected in elderly patients. Conclusions. All patients with systemic lupus erythematosus are infected - 97.50% with cytomegalovirus and 97.17% with Epstein-Barr virus infection, that was confirmed by the increased titer of antibodies to them. Among the mentioned patients 53.33% of them had the active phase of infection (23.33% - cytomegalovirus infection in the replication phase, 17.50% - the Epstein- Barr virus infection in the replication phase and 12.50% - their combination). The prevalence of active viral infection in patients with systemic lupus erythematosus depends on gender (active cytomegalovirus, active Epstein-Barr virus infection and their combination are significantly more common in women) and age - they are probably more common in young patients.  

scholarly journals A 4-year old presenting with fever and achiness

2021 ◽  
Vol 9 ◽  
pp. 2050313X2110564
Author(s):  
Caroline Plott ◽  
Thomas Gracie ◽  
Courtney Alvis ◽  
Amanda Schlefman ◽  
Robert Dudas
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Correct Diagnosis ◽  
Laboratory Studies ◽  
Systemic Lupus ◽  
Class V ◽  
Pediatric Systemic Lupus Erythematosus ◽  
System Disease ◽  
Membranous Lupus Nephritis ◽  
Onset Systemic Lupus Erythematosus

In children under the age of 5 who have abnormalities in history, physical examination, and laboratory studies indicating multi-system disease, uncovering the correct diagnosis is challenging. Here, we report the course of a 4-year-old girl who presented with a change in behavior, fever, arthralgia, arthritis, and hematuria following three recent hospitalizations for pneumonia and impetigo. Serologic findings were suggestive of a rheumatologic etiology and a renal biopsy was consistent with Membranous Lupus Nephritis Class V which helped secure the diagnosis of pediatric systemic lupus erythematosus. We review the clinical features and diagnostic criteria of early-onset systemic lupus erythematosus and discuss diagnostic considerations and prognosis.

scholarly journals Recent advances in the management of systemic lupus erythematosus

F1000Research ◽  
2018 ◽  
Vol 7 ◽  
pp. 970 ◽  
Author(s):  
Savino Sciascia ◽  
Massimo Radin ◽  
Dario Roccatello ◽  
Giovanni Sanna ◽  
Maria Laura Bertolaccini
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Side Effects ◽  
Autoimmune Disease ◽  
Lupus Erythematosus ◽  
Clinical Manifestations ◽  
Therapeutic Strategies ◽  
Systemic Lupus ◽  
Systemic Involvement ◽  
Phase Ii And Iii ◽  
Chronic Autoimmune Disease

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease presenting highly heterogeneous clinical manifestations and multi-systemic involvement. Patients are susceptible to relapse­ and remission, thus making management challenging. Moreover, a considerable number of side effects may occur with conventional therapies; therefore, there is clearly a need for new therapeutic strategies. Since the pathogenesis of SLE is highly complex, it is far from being fully understood. However, greater understanding of the pathways and of the cellular and molecular mediators involved in SLE is being achieved. Emerging evidence has allowed the development of new biological therapeutic options targeting crucial molecular mediators involved in the pathogenesis of SLE. This literature review analyzes the availability of biological and target-directed treatments, phase II and III trials, and new therapies that are being developed for the treatment of SLE.

scholarly journals Ocular Complications in Cutaneous Lupus Erythematosus: A Systematic Review with a Meta-Analysis of Reported Cases

2015 ◽  
Vol 2015 ◽  
pp. 1-8 ◽  
Author(s):  
L. Arrico ◽  
A. Abbouda ◽  
I. Abicca ◽  
R. Malagola
Keyword(s):  
Lupus Erythematosus ◽  
Meta Analysis ◽  
Correct Diagnosis ◽  
Cutaneous Lupus Erythematosus ◽  
Discoid Lupus Erythematosus ◽  
Systemic Lupus ◽  
Ocular Complications ◽  
Ocular Symptoms ◽  
Inflammatory Syndrome ◽  
Cutaneous Lupus

Ocular complications associated with cutaneous lupus erythematosus (CLE) are less studied compared with those ones associated with systemic lupus erythematosus (SLE). The main ocular sites involved in patients affected by discoid lupus erythematosus (DLE) are eyelids followed by orbit and periorbit, the least being cornea. The most common complications are blepharitis usually affecting the lower lid and associated with some type of lid lesion such as plaque or erythematosus patches and madarosis. Few cases with LE profundus (LEP) and ocular complications are reported, but they are associated with orbital inflammatory syndrome and severe complications. The main treatment prescribed is hydroxychloroquine with a dose of 200 mg twice a day for 6 to 8 weeks. Corticosteroids are also used. Intervals between the correct diagnosis and the beginning of the ocular symptoms are commonly delayed. Ophthalmologist should be aware of the ocular manifestation of this autoimmune disease.

scholarly journals AUTOIMMUNE MYELOFIBROSIS ASSOCIATED WITH SYSTEMIC LUPUS ERYTHEMATOSUS: EXCEPTIONALLY RARE OR UNDERRECOGNIZED?

2021 ◽  
Vol 2 (2) ◽  
pp. 96-100
Author(s):  
Döndü Üsküdar Cansu ◽  
Cengiz Korkmaz
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Bone Marrow ◽  
Lupus Erythematosus ◽  
Case Reports ◽  
Classification Criteria ◽  
Systemic Lupus ◽  
Organ Systems ◽  
Hematological Abnormalities ◽  
Chronic Autoimmune Disease ◽  
Rare Symptom

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease which may involve several organs or organ systems. SLE may also have hematological as well as joint, kidney, and central nervous system involvements. Hematological abnormalities such as anemia, leukopenia, thrombocytopenia, and autoimmune hemolytic anemia are the best-known and most common hematological findings detected in SLE patients which are included in the classification criteria for SLE.  Autoimmune myelofibrosis (AIMF) refers to bone marrow (BM) fibrosis (myelofibrosis) that develops in an autoimmune setting. Myelofibrosis is not among the SLE classification criteria and it is also not well-known. Current reports in the literature on SLE-associated AIMF are mostly restricted to case reports or reviews of such case reports. The occurrence of BM fibrosis in SLE patients has been explored merely in few studies which concluded that myelofibrosis is a rare symptom of SLE. Herein, we propose the hypothesis that SLE-associated AIMF is not rare and, on the contrary, it can indeed be more frequent than what is known or expected.

scholarly journals Vesiculobollous disease with Cutaneous Systemic Lupus Erythematosus Treated by Rituxima; a case report

2021 ◽  
Author(s):  
Wigdan Mohammed Niamat Alla ◽  
Burhan Mohamed Ali Ahmed ◽  
Mohammed Elmujtba Adam Essa ◽  
Atif Elhadi Abdalla Babker ◽  
Elnour Mohammed Elagib
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Case Report ◽  
Autoimmune Disease ◽  
Lupus Erythematosus ◽  
Systemic Lupus ◽  
Chronic Autoimmune Disease

SLE is a chronic autoimmune disease characterized by multisystem inflammation, Vesiculobullous (VB) are different groups of oral disorders characterized by the formation of bullae. The aim of this report is to describe a case of a vesiculobullous disease in SLE.

scholarly journals Childhood onset SLE- case report

2021 ◽  
Vol 11 (3) ◽  
pp. 32-33
Author(s):  
Awal Alhusain ◽  
Hasan Alhashim ◽  
Wafa Almuraidif ◽  
Mariam Alghazal ◽  
Awal Alhusain ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Native Americans ◽  
Lupus Erythematosus ◽  
Age Of Onset ◽  
Significant Morbidity ◽  
Childhood Onset ◽  
Systemic Lupus ◽  
The Times ◽  
Chronic Autoimmune Disease ◽  
Children Under 5

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that can affect any organ and may result in significant morbidity and mortality. Childhood-onset c-SLE is a rare disease with an incidence of 0.3-0.9/100,000 children-years and a prevalence of 3.3-8.8/100,000 children.1 Asians, African American, Hispanic and Native Americans have higher frequency of the disease.2,3 Similar to adult SLE, c-SLE affects mainly females with around 80% of the affected children. According to the literature, the median age of onset is 11-12 years and it is rare in children under 5 years of age. The manifestations of c-SLE are variable and most of the times are atypical compared to adult SLE which make the diagnosis of cSLE a challenge to the treating physician.4 This report shows a series of presentation in pediatric patient who finally was diagnosed with SLE.

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