CMV Infection Presenting as a Cavitary Lung Lesion in a Patient with Systemic Lupus Erythematosus Receiving Immunosuppressive Therapy

Abstract Background Systemic lupus erythematosus (SLE) patients with intense immunosuppressive therapy (IT) are at higher risk for cytomegalovirus (CMV) reactivation and may develop the end-organ disease. However, the real epidemiology, associated risk factors, pathogenesis, and clinical outcomes have not been fully elucidated. Objectives To investigate the associated risk factors, possible predictors in the aspect of immunology of CMV infection and to study epidemiology, and clinical outcomes prospectively in active SLE patients within 3 months after intense IT. Methods A prospective cohort study of active SLE patients required intense IT from November 2017 to March 2019 was conducted. We collected patients’ demographics, potential risk factors, onset and presentations of CMV infection after intense IT, data on IT, cytokine panels, and flow cytometry at weeks 0, 2, 4, 8, and 12 after enrollment. Intense IT was defined as an induction therapy of active SLE disease with either the National Institute of Health or Euro-Lupus Nephritis Trial protocol regimens. Results A total of 24 patients have enrolled with a median age of 32 years old and 22/24 patients were female. Renal involvement was the most common and found in 79.2% of the patients. Median SLE disease activity index at enrollment was 14 (25%-75% interquartile range (IQR) = 8–19). At week 0, no CMV infection was documented, 91.7% of the patients had positive CMV IgG, and the median absolute lymphocyte count was 938 cells/mm3. At week 12, the median cumulative corticosteroid dose was 0.74 mg/kg/day (25%-75%IQR = 0.34–1.20) and the prevalence of CMV infection was 12.5%. Elevated interleukin-23 and tumor necrotic factor-α levels were associated with protective effect (hazard ratio (HR) 0.12, 95% confidence interval (CI) 0.02–0.58, p = 0.009 and HR 0.55, 95% CI 0.31–0.99, p = 0.049, respectively). Neurologic involvement was the independent factor that increased the risk of CMV infection (HR 0.26; 95% CI 0.08–0.79, p = 0.018). No mortality was detected. Conclusion CMV infection is common when IT is used, but only some SLE patients with intense IT develop CMV infection. Certain characteristics of the patients may assist predict future CMV infection following IT. However, further study on a larger scale is encouraged. Disclosures All authors: No reported disclosures.

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A Prospective Study of Cytomegalovirus-Specific Cell-mediated Immune Monitoring and Cytomegalovirus Infection in Patients with Active Systemic Lupus Erythematosus Receiving Immunosuppressants

Open Forum Infectious Diseases ◽

10.1093/ofid/ofab248 ◽

2021 ◽

Author(s):

Jackrapong Bruminhent ◽

Suphanan Autto ◽

Porpon Rotjanapan ◽

Pintip Ngamjanyaporn ◽

Asalaysa Bushyakanist ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

Autoimmune Diseases ◽

Immunosuppressive Therapy ◽

Lupus Erythematosus ◽

Active Systemic Lupus Erythematosus ◽

Enzyme Linked Immunosorbent Assay ◽

Cell Mediated Immunity ◽

Specific Cell ◽

Cmv Infection ◽

Systemic Lupus

Abstract Introduction The effects of cytomegalovirus (CMV)-specific cell-mediated immunity (CMI) on CMV infection in patients with autoimmune diseases receiving immunosuppressants have not been explored. Methods Patients with active systemic lupus erythematosus (SLE) were preemptively monitored for clinically significant CMV infection (CsCMVI, defined as plasma CMV DNA loads > 3 log10 IU/mL). CMV-specific CMI was assessed using an enzyme-linked immunosorbent assay (QuantiFERON-CMV®, QF) before as well as 1 and 3 months after intense immunosuppressive therapy. Results The study included 55 patients with active SLE; patients had a mean age of 34 years (standard deviation [SD] 13 years), a median SLEDAI-2K score of 14 (SD 8), and 93% were female. Most patients had renal involvement (67%), received methylprednisolone (93%), and were CMV seropositive (95%). Thirteen (23.6%) patients developed CsCMVI. Among patients with active SLE who were QF-negative (QF–) and QF-positive (QF+) before receiving immunosuppressive therapy, 28.6% and 25% developed CsCMVI, respectively (p=0.69). However, 1 month post-immunosuppression, more QF– than QF+ patients developed CsCMVI (44.4% vs. 11.8%, p=0.03; adjusted hazard ratio 4.97 [95% confidence interval 1.07–23.10], p=0.04). Conclusions Patients with active SLE and low CMV-specific T cell responses could develop CMV infection after receiving immunosuppressants. Further studies should focus on CMV-specific CMI among patients with autoimmune diseases.

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

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.4199 ◽

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

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Pulmonary Arterial Hypertension Responsive to Immunosuppressive Therapy in Systemic Lupus Erythematosus

Lupus ◽

10.1177/096120339300200606 ◽

1993 ◽

Vol 2 (6) ◽

pp. 367-369 ◽

Cited By ~ 26

Author(s):

Sergio Morelli ◽

Marcello Giordano ◽

Paolo De Marzio ◽

Roberta Priori ◽

Alessandro Sgreccia ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Immunosuppressive Therapy ◽

Lupus Erythematosus ◽

Systemic Lupus ◽

Pulmonary Arterial

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A Rare Neurological Complication - Posterior Reversible Encephalopathy Syndrome in a Patient with Systemic Lupus Erythematosus and Class IV Lupus Nephritis

Internal Medicine ◽

10.2478/inmed-2018-0027 ◽

2018 ◽

Vol 15 (4) ◽

pp. 27-34

Author(s):

Anna Mirela Stroie ◽

Mircea Nicolae Penescu

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Nephritis ◽

Immunosuppressive Therapy ◽

Posterior Reversible Encephalopathy Syndrome ◽

Lupus Erythematosus ◽

Neurological Complication ◽

Systemic Lupus ◽

Posterior Reversible Encephalopathy ◽

Reversible Encephalopathy ◽

Class Iv

AbstractPosterior reversible encephalopathy syndrome is a rare manifestation of systemic lupus erythematosus, characterized by altered mental status, headache, convulsions, visual field impairment and posterior and reversible alterations on imaging scans(1,2). The clinical picture develops over a few hours, presenting with rapidly progressive neurological symptoms(3). It was first described in 1996. It is more frequent in patients with acute kidney injury or chronic kidney disease, thus in lupus patients with kidney disorders. It is associated with hypertension, other autoimmune diseases beside lupus, immunosuppressive therapies, especially antibody-based immunosuppressive therapy, and organ transplantation. It is clinically reversible within one week and imaging changes resolve within 2-4 weeks. It is treatable and has a good prognosis. We present the case of a young woman of 27 years, diagnosed with systemic lupus erythematosus who developed convulsive seizures, headache, visual impairment, being under immunosuppressive therapy with azathioprine. The kidney biopsy revealed class IV lupus nephritis and partial remission of the nephrotic syndrome. The other manifestations of SLE in this patient were cutaneous, immunological, articular and haematological. The patient had a good short, medium and long-term prognosis at 30 days and also at 6 months.

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

World Science ◽

10.31435/rsglobal_ws/31102020/7225 ◽

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.

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