CLINICAL OBSERVATION OF UNTREATED SYSTEMIC LUPUS ERYTHEMATOSUS WITH INFECTIOUS COMPLICATIONS AND FATAL OUTCOME

2020 ◽  
Vol 6 (3) ◽  
pp. 33-36
Author(s):  
B. Barieva
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Fatal Outcome ◽  
Clinical Situation ◽  
Infectious Complications ◽  
Multiple Organ ◽  
Rapid Progression ◽  
Systemic Lupus ◽  
Diagnostic Difficulties ◽  
Omental Bursa

Among rheumatic diseases, a special place is occupied by systemic lupus erythematosus (SLE), this is due to its variety of clinical nonspecific manifestations, which leads to diagnostic difficulties in the early stages.This article presents a clinical case of severe SLE, previously not diagnosed and untreated, with the rapid progression of multiple organ failure (damage to the lungs, cardiovascular system, kidneys), which led to death. Previously, the patient was treated in the oncology and surgical departments with an abscess of the omental bursa and steatopancreonecrosis, later she was hospitalized in the hematology department with three-growth pancytopenia and hemolytic anemia, and from there transferred to the rheumatology department. This clinical situation demonstrates the importance of timely diagnosis, diagnosis and treatment of SLE.

scholarly journals AB0818 ADVERSE EVENTS UNDER B-CELL DIRECTED THERAPIES IN A LARGE SINGLE-CENTER COHORT OF PATIENTS WITH RHEUMATOID ARTHRITIS, SYSTEMIC LUPUS ERYTHEMATOSUS, ANCA-ASSOCIATED VASCULITIS AND RENAL DISEASES

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1433.1-1433
Author(s):  
J. G. Rademacher ◽  
V. Korendovych ◽  
P. Korsten
Keyword(s):  
Rheumatoid Arthritis ◽  
Systemic Lupus Erythematosus ◽  
Adverse Events ◽  
B Cell ◽  
Clinical Data ◽  
Lupus Erythematosus ◽  
Infectious Complications ◽  
Single Center ◽  
Systemic Lupus ◽  
Anca Associated Vasculitis

Background:The anti-CD20 antibody rituximab (RTX) is approved for the treatment of rheumatoid arthritis (RA) and ANCA-associated vasculitis (AAV). In addition, RTX is used in a wide range of autoimmune diseases. Belimumab (BEL) is an anti-BAFF antibody approved for the treatment of non-renal systemic lupus erythematosus (SLE) in Europe. These agents are generally well-tolerated but severe adverse events (AEs) can occur. The frequency of and factors associated with AEs are currently unknown.Objectives:To identify adverse events with the use of B-cell directed therapies in a large population of RA, AAV, and SLE.Methods:This is a single-center retrospective cohort study using routine clinical data over a ten-year period (2010-2020). We recorded epidemiological and clinical data of patients receiving either BEL or RTX. Data included age, gender, type of disease, number and efficacy of infusions, patient-years and concomitant treatment. Patient records were screened for AEs, such as infections, anaphylaxis, occurrence of malignant disease, laboratory abnormalities and immunoglobulin (Ig) deficiency. Between group comparisons were performed.Results:Database screening yielded 445 patients treated with RTX and 23 with BEL. After exclusion of patients with incomplete data, 425 RTX and 23 BEL patients were analyzed.Our preliminary analysis of a sample of 60 of these 448 patients (184 patient-years) resulted in 43 patients (72%) with RA, 8 patients with AAV (13%), 5 patients with a renal disease, and 4 patients with mixed connective tissue disease, as well 23 SLE patients. 46 (77%) were female. In RA, a median of 13 treatments of 1000 mg were administered, corresponding to 3.37 patient-years per patient. Primary non-response occurred in 2 patients, secondary non-response in 13 patients. For AAV, a median of 8.4 treatments were given (3.3 patient-years), no treatment failure was detected. SLE patients received a median of 15 treatments.15 patients had infectious complications during treatment, 11 needed treatment. Herpes zoster infection occurred in 3 patients with RA. Three of the 8 patients with AAV had an infection requiring treatment. In SLE patients, only 2 developed infectious complications, and no Ig-deficiency occurred.Lymphopenia was the most common laboratory abnormality detected in 25 patients with RTX, 19 of whom had RA. Ig deficiency was common in RA, affecting 30% of patients. Deficiency of IgM and IgG was recognized in 5 patients each; 1 patient had low levels IgA.Neither the maintenance prednisolone dosage nor Ig deficiency were associated with risk for infection. However, lymphopenia appeared to be associated with risk for infection.Conclusion:Our preliminary data observe a 184 patient-year period. RTX and BEL were generally associated with few AEs. RA patients frequently had laboratory abnormalities (lymphopenia, Ig-deficiency) which did not necessarily translate to clinical events. Infections were more common in AAV, BEL was the best tolerated B-cell directed agent. Overall, our data are reassuring, but we suggest a more careful vigilance in AAV patients.Disclosure of Interests:Jan-Gerd Rademacher: None declared, Viktor Korendovych: None declared, PETER KORSTEN Speakers bureau: Abbvie, Sanofi Aventis, GSK, Chugai, Boehringer-Ingelheim, Novartis, Consultant of: Lilly, Gilead, Boehringer-Ingelheim, Novartis, GSK, Grant/research support from: GSK

Lupus Eritematosus Sistemik pada Pria

2021 ◽  
Vol 3 (2) ◽  
pp. 37-42
Author(s):  
Harry Andrean ◽  
Raveinal Raveinal
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Clinical Course ◽  
Multiple Organ ◽  
Systemic Lupus ◽  
Malar Rash ◽  
Mouth Ulcer ◽  
Pulse Dose ◽  
Aggressive Clinical Course ◽  
Complex Autoimmune Disease

Introduction: Systemic lupus erythematosus (SLE) is a complex autoimmune disease characterized by presence of nucleus autoantibody and affected multiple organ. Systemic lupus erythematosus is more common in women than men with ratio 2:1 to 15:1. Men with SLE often have a more aggressive clinical course, lead to a poorer prognosis compared with women with SLE. Case Report: A man, 29 years old came to hospital with main complain joint pain increased since 1 week ago, accompanied with red spot on face, trunk, hands, foot, and back, hair loss, swollen leg, mouth ulcer, and fatique. Malar rash and discoid rash were identified from physical examination. From laboratorium, ANA profile was positive for RNP/Sm, Sm, dsDNA, and histone. Skin biopsy showed a lupus discoid. Conclusion: The patient was treated with pulse-dose methylprednisolone for 3 days and showed a good response clinically.

An update on diet and nutritional factors in systemic lupus erythematosus management

2017 ◽  
Vol 30 (1) ◽  
pp. 118-137 ◽  
Author(s):  
Marina Aparicio-Soto ◽  
Marina Sánchez-Hidalgo ◽  
Catalina Alarcón-de-la-Lastra
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Diet Therapy ◽  
Multiple Organ ◽  
Dietary Factors ◽  
Clinical Experiences ◽  
Systemic Lupus ◽  
Nutritional Factors ◽  
Biological Heterogeneity

AbstractSystemic lupus erythematosus (SLE) is a chronic inflammatory and autoimmune disease characterised by multiple organ involvement and a large number of complications. SLE management remains complicated owing to the biological heterogeneity between patients and the lack of safe and specific targeted therapies. There is evidence that dietary factors can contribute to the geoepidemiology of autoimmune diseases such as SLE. Thus, diet therapy could be a promising approach in SLE owing to both its potential prophylactic effects, without the side effects of classical pharmacology, and its contribution to reducing co-morbidities and improving quality of life in patients with SLE. However, the question arises as to whether nutrients could ameliorate or exacerbate SLE and how they could modulate inflammation and immune function at a molecular level. The present review summarises preclinical and clinical experiences to provide the reader with an update of the positive and negative aspects of macro- and micronutrients and other nutritional factors, including dietary phenols, on SLE, focusing on the mechanisms of action involved.

scholarly journals Cytokine Networks in Systemic Lupus Erythematosus

2010 ◽  
Vol 2010 ◽  
pp. 1-5 ◽  
Author(s):  
Hooi-Ming Lee ◽  
Hidehiko Sugino ◽  
Norihiro Nishimoto
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Cytokine Production ◽  
Organ Damage ◽  
Multiple Organ ◽  
Serum Cytokine ◽  
Systemic Lupus ◽  
Pathological Conditions ◽  
Cytokine Levels ◽  
Cytokine Networks

Systemic lupus erythematosus (SLE) is an autoimmune disease more prominent in women and characterized by multiple organ damage. Imbalance in cytokine production and cytokine levels correlates with SLE progression, making the understanding of SLE cytokine networks very important for SLE treatment strategy and drug development. In this article, we review cytokine networks that may be involved in the pathogenesis of SLE by briefly describing abnormal cytokine production and serum cytokine levels in SLE patients. We also focus on the pathological roles of cytokines and their interactions in immunoregulatory networks and suggest how their disturbances may implicate in pathological conditions in SLE. Finally, we further discuss the influence of estrogen on these cytokine networks.

Systemic Lupus Erythematosus and Peritoneal Dialysis: Outcomes and Infectious Complications

2001 ◽  
Vol 21 (2) ◽  
pp. 143-148 ◽  
Author(s):  
Jenq-Wen Huang ◽  
Kuan-Yu Hung ◽  
Chung-Jen Yen ◽  
Kwan-Dun Wu ◽  
Tun-Jun Tsai
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Infectious Complications ◽  
Control Group ◽  
Systemic Lupus ◽  
Exit Site ◽  
Age And Gender ◽  
Exit Site Infection ◽  
Technique Survival ◽  
And Gender

Objective Systemic lupus erythematosus (SLE) is the most common secondary glomerulonephritis resulting in end-stage renal disease (ESRD) among young adults in Taiwan. Studies of the infectious complications and outcomes among such SLE patients undergoing peritoneal dialysis (PD) are limited. Design A retrospective age- and gender-matched case control study. Setting A university teaching hospital. Patients There were 23 SLE patients with ESRD receiving PD for more than 3 months during the past 15 years. Another 46 age- and gender-matched non-SLE nondiabetic patients receiving PD were selected as the control group in this study. Intervention All patients underwent PD as renal replacement therapy and were regularly followed up at this hospital. Main Outcome Measures Technique survival and incidences of exit-site infection (ESI) and peritonitis in these patients. Results The SLE patients had a lower predialysis serum albumin than the control group (3.16 ± 0.50 g/dL vs 3.52 ± 0.50 g/dL, p < 0.01). The incidences of exit-site infection (ESI) and peritonitis were higher for SLE patients than for control patients ( p < 0.01 and p < 0.001, respectively). Kaplan–Meier survival analysis indicated that SLE patients had shorter time intervals to first infectious complications, and poorer technique survival. Infection was the major cause of dropout and mortality in the SLE patients. The SLE patients had a reduced chance of receiving a renal transplant. The use of steroids by SLE patients was associated with a higher incidence of peritonitis ( p = 0.04), but association with ESI was insignificant. In a Cox regression model, the underlying SLE was the only risk factor for technique failure and time interval to first infectious complication. Conclusion SLE patients undergoing PD are more susceptible to infection than age- and gender-matched non-SLE nondiabetic patients and have poorer technique survival. Systemic lupus erythematosus itself may further compromise the immunity of uremic patients.

scholarly journals Autosomal chronic granulomatous disease and systemic lupus erythematosus with fatal outcome

Rheumatology ◽  
1998 ◽  
Vol 37 (1) ◽  
pp. 109b-111b ◽  
Author(s):  
J. C. Cobeta-Garcia ◽  
J. A. Domingo-Morera ◽  
I. Monteagudo-Saez ◽  
F. J. Lopez-Longo
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Chronic Granulomatous Disease ◽  
Lupus Erythematosus ◽  
Fatal Outcome ◽  
Granulomatous Disease ◽  
Systemic Lupus

scholarly journals Multiple Thromboses in a Patient with Systemic Lupus Erythematosus after Splenectomy

2012 ◽  
Vol 2012 ◽  
pp. 1-3
Author(s):  
Deng-Ho Yang
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Vena Cava ◽  
Fetal Loss ◽  
Pulse Therapy ◽  
Multiple Organ ◽  
Renal Infarction ◽  
Systemic Lupus ◽  
Adequate Treatment ◽  
History Of

Antiphospholipid syndrome is a disorder presenting with arterial or venous thrombus and a history of fetal loss. Early diagnosis and adequate treatment is important to prevent multiple organ failures. Here, we described a woman with a two-year history of systemic lupus erythematosus with severe nephrotic syndrome, manifested multiple thrombi over the portal vein and the inferior vena cava, combined with acute renal infarction. The patient underwent splenectomy 10 months ago. Initially, she received anticoagulant treatment and low-dose glucocorticoid, but multiple organ failure progressed. After emergency plasma exchange followed by glucocorticoid pulse therapy, the patient recovered.

An autosomal recessive DNASE1L3-related autoimmune disease with unusual clinical presentation mimicking systemic lupus erythematosus

Lupus ◽  
2016 ◽  
Vol 26 (7) ◽  
pp. 768-772 ◽  
Author(s):  
A Carbonella ◽  
G Mancano ◽  
E Gremese ◽  
F S Alkuraya ◽  
N Patel ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Autoimmune Disease ◽  
Lupus Erythematosus ◽  
Autosomal Recessive ◽  
Multiple Organ ◽  
Systemic Lupus ◽  
Risk Variants ◽  
Separate Condition ◽  
Hypocomplementemic Urticarial Vasculitis Syndrome ◽  
Three Sisters

We describe the third family in the world, after Arabian and Turkish ones, displaying an autosomal recessive autoimmune disease (AID), mimicking systemic lupus erythematosus (SLE), with unusual manifestations due to a homozygous frame-shift variant in DNASE1L3. SLE is a complex AID characterized by multiple organ involvement. Genetic risk variants identified account for only 15% of SLE heritability. Rare Mendelian forms have been reported, including DNASE1L3-related SLE. Through specific genetic tests we identified a homozygous 2 bp-deletion c.289_290delAC (NM_004944.2) in DNASE1L3, predicting frameshift and premature truncation (p.Thr97Ilefs*2). The same mutation was previously reported in three sisters, born from consanguineous parents and affected with hypocomplementemic urticarial vasculitis syndrome (HUVS). As approximately 50% of individuals affected with HUVS develop SLE, it is still unclear whether it is a SLE sub-phenotype or a separate condition.

scholarly journals Progressive Multifocal Leukoencephalopathy and Systemic Lupus Erythematosus: Focus on Etiology

2016 ◽  
Vol 8 (1) ◽  
pp. 59-65 ◽  
Author(s):  
Shala Ghaderi Berntsson ◽  
Evangelos Katsarogiannis ◽  
Filipa Lourenço ◽  
Maria Francisca Moraes-Fontes
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Progressive Multifocal Leukoencephalopathy ◽  
Lupus Erythematosus ◽  
Jc Virus ◽  
Brain Mri ◽  
Fatal Outcome ◽  
Disease Onset ◽  
Unmet Need ◽  
Human Polyomavirus ◽  
Systemic Lupus

Progressive multifocal leukoencephalopathy (PML) caused by reactivation of the JC virus (JCV), a human polyomavirus, occurs in autoimmune disorders, most frequently in systemic lupus erythematosus (SLE). We describe a HIV-negative 34-year-old female with SLE who had been treated with immunosuppressant therapy (IST; steroids and azathioprine) since 2004. In 2011, she developed decreased sensation and weakness of the right hand, followed by vertigo and gait instability. The diagnosis of PML was made on the basis of brain MRI findings (posterior fossa lesions) and JCV isolation from the cerebrospinal fluid (700 copies/ml). IST was immediately discontinued. Cidofovir, mirtazapine, mefloquine and cycles of cytarabine were sequentially added, but there was progressive deterioration with a fatal outcome 1 year after disease onset. This report discusses current therapeutic choices for PML and the importance of early infection screening when SLE patients present with neurological symptoms. In the light of recent reports of PML in SLE patients treated with rituximab or belimumab, we highlight that other IST may just as well be implicated. We conclude that severe lymphopenia was most likely responsible for JCV reactivation in this patient and discuss how effective management of lymphopenia in SLE and PML therapy remains an unmet need.

Sign in / Sign up

Export Citation Format

Share Document