scholarly journals Sysyemic Lupus Erythematosus with Multiple Organ Damage

2020 ◽  
Vol 17 (5) ◽  
pp. 63-73
Author(s):  
Andreea Alexandra Nicola ◽  
Mădălina Dună ◽  
Ioana Miler ◽  
Nicoleta Petre ◽  
Denisa Predeţeanu
Keyword(s):  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Clinical Manifestations ◽  
Organ Damage ◽  
Multiple Organ ◽  
Alveolar Haemorrhage ◽  
Interdisciplinary Management ◽  
Childbearing Age ◽  
Pulmonary Infarction ◽  
Pulmonary Manifestations

Abstract Systemic lupus erythematosus (SLE) is a heterogeneous rheumatic disease with various clinical manifestations and a multifactorial pathogenesis. Although the etiology of SLE is unknown, certain risk factors have been identified as promoters of an imbalance in the immune system with antibody formation and tissue damage secondary to the deposition of immune complexes. Women of childbearing age are predominantly affected, regardless of ethnicity. SLE affects the kidneys in about 50% of patients, lupus nephritis (LN) being a major risk factor for overall morbidity and mortality. The lungs are commonly involved later in the course of the disease, pulmonary involvement in SLE being extremely varied. It includes along with pleuritis which is the most common manifestation, acute lupus pneumonitis, pulmonary vasculitis, pulmonary embolism, diffuse alveolar haemorrhage; opportunistic pulmonary infections or drug toxicity could be other pulmonary manifestations. We present the case of a young woman with SLE complicated with lupus nephritis class V and pulmonary infarction due to thromobosis of a pulmonary artery. It is a case of SLE with multiple organ damage and life-threatening complications that require interdisciplinary management and appropriate treatment.

scholarly journals ETIOPATHOGENETIC ASPECTS OF LIVER INJURY IN PATIENTS WITH COVID-19

2020 ◽  
Vol 76 (4) ◽  
pp. 9-15
Author(s):  
V.I. Petrov ◽  
◽  
A.V. Ponomareva ◽  
I.V. Ivakhnenko ◽  
O.V. Razvalyaeva ◽  
...  
Keyword(s):  
Liver Damage ◽  
Liver Diseases ◽  
Inflammatory Process ◽  
Clinical Manifestations ◽  
Organ Damage ◽  
Multiple Organ ◽  
Pulmonary Manifestations ◽  
Coronavirus Infection ◽  
Factors Of Influence ◽  
Extra Pulmonary

Infection caused by the new coronavirus SARS-CoV-2 initially associated with respiratory damage and the development of respiratory symptoms. However, it was subsequently shown that COVID-19 can be considered as a systemic inflammatory process with multiple organ damage. One of the most frequent extra pulmonary manifestations of SARS-CoV-2 infection is liver damage. Several factors of influence on the liver in COVID-19 are currently being considered: viral immunological damage, hypoxia, systemic inflammatory process, drug toxicity and progression of existing liver diseases. This review is devoted to the analysis of available data on the mechanisms and clinical manifestations of liver damage in a new coronavirus infection.

scholarly journals Case Report: Kidney Transplantation in a Patient With Acquired Agammaglobulinemia and SLE. Issues and Challenges

2021 ◽  
Vol 8 ◽  
Author(s):  
Paraskevi Pavlakou ◽  
Marios Papasotiriou ◽  
Theodoros Ntrinias ◽  
Alexandra Kourakli ◽  
Adamantia Bratsiakou ◽  
...  
Keyword(s):  
Kidney Transplantation ◽  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Immunosuppressive Agents ◽  
Young Patients ◽  
Clinical Manifestations ◽  
Organ Damage ◽  
Cumulative Impact ◽  
Cadaveric Kidney ◽  
End Stage

Lupus nephritis in the context of Systemic Lupus Erythematosus (SLE) is characterized by an unpredicted course with remissions and flare-ups. Among others, it remains a significant cause of end-stage kidney disease (ESKD) in relatively young patients. Therapeutic regimens with newer immunosuppressive agents have been introduced in order to control SLE clinical manifestations more efficiently and limit organ damage induced by immune complex formation and sustained inflammation. Treatment is usually long-term, and the cumulative impact of immunosuppression is expressed through the increased frequency of infections and neoplasms. However, if the observed immunity dysregulation is secondary and pharmaceutically induced or there is a pre-existing, primary immunodeficiency that shares common pathogenetic pathways with SLE's autoimmunity is not always clear. Herein, we present the case of a 39-year-old woman, that reached ESKD due to lupus nephritis. After an upper respiratory cytomegalovirus (CMV) infection and concomitant CMV reactivations the investigation revealed significant immunodeficiency. Not long after the initiation of intravenous immunoglobulin (IVIG) administration, patient received a cadaveric kidney transplant. IVIG was continued along with standard immunosuppression so that both recurrent infections and allograft rejection are avoided. Patient is closely monitored, and her post-transplant course is remarkably satisfying so far. ESKD patients with immunodeficiency syndromes should not be excluded by definition from kidney transplantation.

Pediatric lupus in Asia

Lupus ◽  
2010 ◽  
Vol 19 (12) ◽  
pp. 1414-1418 ◽  
Author(s):  
JL Huang ◽  
KW Yeh ◽  
TC Yao ◽  
YL Huang ◽  
HT Chung ◽  
...  
Keyword(s):  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Disease Onset ◽  
Clinical Manifestations ◽  
Organ Damage ◽  
Infectious Complications ◽  
Asian Patients ◽  
Poor Outcomes ◽  
Diffuse Proliferative Glomerulonephritis ◽  
Pediatric Onset

Of all patients with systemic lupus erythematosus (SLE), 15—20% are diagnosed during childhood, with disease onset prior to the age of 16 years. Because disease expression in SLE is influenced by environment factors and differs between racial and ethnic groups. The aims of this review were to describe prevalence, clinical manifestations, common infectious complications, and outcome of pediatric-onset SLE in Asia. The prevalence of pediatric-onset SLE was 6.3—19.3 per 100,000 in Asia. The ratio of female to male was 4.7—6.2. The mean age at diagnosis of pediatric-onset SLE was 8.6—13.5 years. The most common clinical features of pediatric-onset SLE in Asia were cutaneous rashes, arthritis, hematological involvement and nephritis. The occurrence of nephritis varies from 29% to 81%. The most common histopathology of lupus nephritis was diffuse proliferative glomerulonephritis (WHO Class-IV) which occurred in 39.4—54% of case of lupus nephritis. Pediatric-onset SLE patients with infections have poor outcomes than uninfected patients. Gram-negative bacilli are the most common microorganisms responsible for bacteremia in Asian patients with SLE. Recurrent major infections predict poorer disease outcome and associated organ damage in pediatric-onset SLE. Improving the survival of SLE patients was reported in Asia in recent decades. The survival was 92% at the age of 5 years, 86% at 10 years and 79% at 15 years in children with SLE in Taiwan in 2008.

scholarly journals Anti-TLR7 Antibody Protects Against Lupus Nephritis in NZBWF1 Mice by Targeting B Cells and Patrolling Monocytes

2021 ◽  
Vol 12 ◽  
Author(s):  
Yusuke Murakami ◽  
Ryutaro Fukui ◽  
Reika Tanaka ◽  
Yuji Motoi ◽  
Atsuo Kanno ◽  
...  
Keyword(s):  
B Cells ◽  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Organ Damage ◽  
Multiple Organ ◽  
Innate Immune ◽  
Toll Like Receptor ◽  
Cellular Mechanisms ◽  
Autoantibody Production ◽  
Patrolling Monocytes

Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by autoantibody production and multiple organ damage. Toll-like receptor 7 (TLR7), an innate immune RNA sensor expressed in monocytes/macrophages, dendritic cells (DCs), and B cells, promotes disease progression. However, little is known about the cellular mechanisms through which TLR7 drives lupus nephritis. Here, we show that the anti-mouse TLR7 mAb, but not anti-TLR9 mAb, protected lupus-prone NZBWF1 mice from nephritis. The anti-TLR7 mAb reduced IgG deposition in glomeruli by inhibiting the production of autoantibodies to the RNA-associated antigens. We found a disease-associated increase in Ly6Clow patrolling monocytes that expressed high levels of TLR7 and had upregulated expression of lupus-associated IL-10, CD115, CD31, and TNFSF15 in NZBWF1 mice. Anti-TLR7 mAb abolished this lupus-associated increase in patrolling monocytes in the circulation, spleen, and glomeruli. These results suggested that TLR7 drives autoantibody production and lupus-associated monocytosis in NZBWF1 mice and, that anti-TLR7 mAb is a promising therapeutic tool targeting B cells and monocytes/macrophages.

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

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

scholarly journals AB0750 FUNCTIONAL CONDITION OF KIDNEYS IN THE LONG-TERM COURSE OF SLE IN ADOLESCENTS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1403.2-1403
Author(s):  
L. Bohmat ◽  
N. Shevchenko ◽  
I. Bessonova
Keyword(s):  
Renal Function ◽  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Disease Duration ◽  
Renal Involvement ◽  
Clinical Manifestations ◽  
Clinical Analysis ◽  
Average Dose ◽  
One Year

Background:Lupus nephritis is the most severe and adverse systemic lupus erythematosus (SLE) syndrome. According to modern recommendations, the clinical manifestations of active nephritis should be taken under medical control in 6 months after the start of the disease’s treatment1.Objectives:The aim of this study was to examine the functional status of the kidneys in children with SLE in the course of the disease for more than one year.Methods:The analysis included case histories of 43 patients with SLE, mostly females (41), aged 7 to 18 years (mean age 14.4 years) with disease duration of 4.75 ± 0.58 years of whom 22 were less than three years, 21 - more than three years. All children received corticosteroid therapy, at the time of the examination the average dose was 13.85 ± 1.86 mg per day in terms of prednisolone. The second component of therapy was azathioprine (average dose 97.61 ± 2.11 mg). All children received hydroxychloroquine (5 mg/kg per day).To determine the functional state of the kidneys a clinical analysis of urine, a study of the scope of specific gravity of urine during the day (Zymnytsky test), the content of creatinine and urea in serum to determine the glomerular filtration rate (GFR), the level of microalbuminuria per day were evaluated.Results:Renal involvement in the developed SLE occurred in 73.08% of patients. Among them, therapy during the first 6 months was considered quite effective in 58.06% of patients. It was found that in children with disease duration from one to three years proteinuria was registered in 68.18%, a decrease in GFR in 4.45% and hyperfiltration in 9.09%. In the group of children with duration of SLE more than three years revealed deeper changes in renal function; there was proteinuria in 90.47%, the frequency of GFR decreased was in 19.04%, a decrease of renal concentration function was in 14.28% of cases.Indicators of renal function in children with SLE depending on the duration of the disease (M ± m)IndicatorDuration of the diseasefrom 1 year to 3 years n = 22over 3 yearsn = 21Creatinine, mmol/l0,080 ± 0,0140,090 ± 0,018Мочевина, mmol/l5,66 ± 1,425,63 ± 1,61GFR, ml/min117,05 ± 19,68100,20 ± 18,98 *Microalbuminuria, mg/day24,41 ± 13,1334,73 ± 24,76Density min1,007 ± 0,0051,006 ± 0,005Density max1,024 ± 0,0051,019 ± 0,005 ***р<0,03;**р<0,01 the probability of differences when comparing between groupsConclusion:Long-term follow-up of children with SLE over one year reveals a prolongation of renal dysfunction, which worsens after three years, and is the basis for the development of irreversible renal impairment.References:[1]European evidence-based recommendations for the diagnosis and treatment of childhood-onset lupus nephritis: the SHARE initiative /Noortje Groot, Nienke de Graeff, Stephen D Marks et all. //Ann Rheum Dis. 2017 Dec;76(12):1965-1973.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 Atypical hemolytic uremic syndrome secondary to lupus nephritis, responsive to eculizumab

2016 ◽  
Vol 8 (3) ◽  
Author(s):  
Alexander G. Raufi ◽  
Shruti Scott ◽  
Omar Darwish ◽  
Kevin Harley ◽  
Kanwarpal Kahlon ◽  
...  
Keyword(s):  
Lupus Nephritis ◽  
Hemolytic Uremic Syndrome ◽  
Lupus Erythematosus ◽  
Multidisciplinary Approach ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Organ Damage ◽  
Atypical Hus ◽  
First Line Therapy ◽  
Autoimmune Attack ◽  
Uremic Syndrome

Among the spectrum of disease manifestations associated with systemic lupus erythematosus, lupus nephritis is particularly concerning due to the potential for renal failure. This autoimmune attack may not, however, be limited to the kidney and is increasingly being recognized as a trigger for atypical Hemolytic Uremic Syndrome (aHUS). Atypical HUS falls under the spectrum of the thrombotic microangiopathies (TMAs) – a group of disorders characterized by microangiopathic hemolytic anemia, thrombocytopenia, and end organ damage. Although plasma exchange is considered first-line therapy for thrombotic thrombocytopenic purpura – a TMA classically associated with autoimmune depletion of ADAMTS-13 – aHUS demonstrates less reliable responsiveness to this modality. Instead, use of the late complement inhibitor Eculizumab has emerged as an effective modality for the management of such patients. Diagnosis of aHUS, however, is largely clinically based, relying heavily upon a multidisciplinary approach. Herein we present the case of a patient with atypical HUS successfully treated with Eculizumab in the setting of Class IV-G (A) lupus nephritis and hypocomplementemia.

scholarly journals Predictors of mortality in children with lupus nephritis

2014 ◽  
Vol 54 (6) ◽  
pp. 338
Author(s):  
Lukman Oktadianto ◽  
Risky Vitria Prasetyo ◽  
Ninik Asmaningsih Soemyarso ◽  
Mohammad Sjaifullah Noer
Keyword(s):  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Renal Involvement ◽  
Hypertensive Crisis ◽  
Clinical Signs ◽  
Clinical Manifestations ◽  
Predictors Of Mortality ◽  
Cox Proportional Hazard ◽  
Kaplan Meier ◽  
Mean Time

Background Renal involvement during the clinical course ofsystemic lupus erythematosus (SLE) is generally considered to bethe most important factor influencing disease prognosis in termsof morbidity and mortality. Various factors have been reported toinfluence the prognosis of lupus nephritis (LN).Objective To analyze clinical signs and laboratory parameters thatmight serve as predictors associated with mortality in pediatricLN.Methods Retrospectively, medical records of children with LNat Soetomo Hospital from 1998 to 2011 were studied. Diagnosisof SLE was based on Revised American Rheumatism Associationcritera, while patients with clinical manifestations of hypertension,abnormal urinalysis, and serum creatinin > 1 mg/dL wereconsidered as lupus nephritis. Cox proportional hazard modelingwas used to assess for associations of clinical signs and laboratoryparameters with mortality. Kaplan-Meier survival analysis wasused to assess the cumulative survival from the time of diagnosisto the outcome.Results There were 57 children with LN of whom 43 (75%) weregirls. The female-to-male ratio was 3:1. Subjects’ mean age was 10.6(SD 6.87) years. The mean time of observation was 51 (SD 74.54)months and 23 (40%) children died. Age, gender, hypertension,hematuria, proteinuria, and anemia were not significant aspredictors for mortality. However, hypertensive crisis (HR=2.79;95%CI 1.16 to 6.75; P=0.02) and initial glomerular filtration rate(GFR) of <75 mL/min/1.73m2 (HR=3.01; 95%CI 1.23 to 7.34;P=0.01) were significant predictors of mortality in children with LN.The mean survival time of LN with hypertensive crisis and initialGFR <75 mL/min/1.73m2 was 36.9 (SD 12.17) months.Conclusion Hypertensive crisis and GFR <75 mL/min/1.73m2 aresignificant predictors of mortality in children with LN.

scholarly journals Leukoencephalopathy and cerebral edema as the presenting manifestations of SLE in an ANA-negative adolescent female: A case report and review of literature.

2020 ◽  
Author(s):  
Alexandra Theisen ◽  
Paroma Bose ◽  
Christina Knight ◽  
Melissa Oliver
Keyword(s):  
Cerebral Edema ◽  
Lupus Erythematosus ◽  
Primary Care Physicians ◽  
Neuropsychiatric Symptoms ◽  
Clinical Manifestations ◽  
Central Nervous System Involvement ◽  
Multiple Organ ◽  
Small Subset ◽  
Adolescent Female ◽  
Organ Systems

Abstract Background: Systemic lupus erythematosus (SLE) is an autoimmune disease with various clinical manifestations involving multiple organ systems. Neuropsychiatric manifestations of SLE has been associated with increased morbidity and mortality, thus it is important to recognize and diagnosis the disease entity and treat early. When neuropsychiatric symptoms are involved, typically there are many other systemic features to aid in the diagnosis of SLE. Many autoantibodies have been discovered and are used to help diagnose SLE. The antibody present in most cases of pediatric SLE, as well as in many other rheumatic diseases, is the nonspecific antinuclear antibody (ANA), making it a commonly used screening tool by primary care physicians when evaluating a patient with a possible rheumatic disorder. However, a small subset of SLE patients, 1-5%, present with a negative ANA, and it is important to keep SLE on the differential diagnosis in specific instances when a thorough infectious and neurological workup has been completed and proven to be inconclusive. Case Presentation: This case involves a Hispanic adolescent female with a negative ANA who presented with diffuse cerebral edema secondary to leukoencephalopathy due to SLE with central nervous system involvement. She had an extensive workup while inpatient involving metabolism, infectious disease, rheumatology, and neurology prior to obtaining the diagnosis of SLE. She was treated with both cyclophosphamide and rituximab and showed improvement. Conclusions: A review of the literature revealed 8 cases with SLE presenting with or developing diffuse cerebral edema and/or leukoencephalopathy. Our patient’s case differs in that she was also ANA negative despite other autoantibody positivity. While she did have low complements and transient leukopenia, she did not present with other signs of organ involvement, which made the diagnosis of SLE with neuropsychiatric involvement quite challenging. We discuss the importance of keeping SLE on the differential despite a negative ANA in complex cases without any other cause and to consider initial screening with not only the ANA but also dsDNA and complements to avoid missed diagnoses.

Sign in / Sign up

Export Citation Format

Share Document