Systemic Lupus
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2021 ◽  
Vol 9 ◽  
Author(s):  
Chun-Chun Gau ◽  
Li-Lun Lin ◽  
Chao-Yi Wu ◽  
Jing-Long Huang

Systemic lupus erythematosus (SLE) is an autoantibody-related disease that affects multiple organs. Stercoral colitis (SC) is a rare type of inflammatory colitis with a high mortality rate. Here, we report the first case of pediatric-onset lupus in a case complicated by stercoral colitis. We also conducted a literature review of patients with SC under 30 years old to provide useful clues for rapid diagnosis at a young age. A 28-year-old female with a history of lupus and neuropsychiatric SLE was admitted with severe abdominal pain. She was found to have stercoral colitis during surgery. Two years later, the patient underwent Hartman's operation due to ischemia of the colon. In addition, 10 patients younger than 30 years old with a diagnosis of SC were analyzed based on clinical presentation, physical examination, laboratory exam, imaging and treatment. All cases had a favorable outcome without mortality. Stercoral colitis is a rare but lethal complication, emphasizing the importance of a multidisciplinary approach. Differential diagnosis should include stercoral colitis for patients with SLE developing unexplained sharp abdominal pain.


PLoS ONE ◽  
2021 ◽  
Vol 16 (10) ◽  
pp. e0259114
Author(s):  
Beatriz Teresita Martín-Márquez ◽  
Minoru Satoh ◽  
Rogelio Hernández-Pando ◽  
Erika Aurora Martínez-García ◽  
Marcelo Heron Petri ◽  
...  

Introduction Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by the production of autoantibodies such as anti-Sm. Studies in patients with SLE and murine models of lupus reveal that the most critical anti-Sm autoantibodies are predominantly direct against D1(83–119), D2, and B´/B epitopes. Objectives The present study aimed to analyze the induction of antigen-specific tolerance after prophylactic immunization with a DNA vaccine encoding the epitopes: D183-119, D2, B´/B, and B´/BCOOH in co-vaccination with IFN-γ or IL-10 in a murine model of lupus induced by pristane. Material and methods To obtain endotoxin-free DNA vaccines, direct cloning techniques using pcDNA were performed: D183-119, D2, B´/B, B´/BCOOH, IFN-γ, or IL-10. Lupus was induced by 0.5 mL of pristane via intraperitoneal in BALB/c female mice. Immunoprecipitation with K562 cells was metabolically labeled with 35S and ELISA to detect serum antibodies or mice IgG1, IgG2a isotypes. ELISA determined IL-10 and IFN-γ from splenocytes supernatants. Proteinuria was assessed monthly, and lupus nephritis was evaluated by immunofluorescence, and electron microscopy. Results The prophylactic co-vaccination with D2/IL-10 reduced the expression of kidney damage observed by electron microscopy, direct immunofluorescence, and H & E, along with reduced level of anti-nRNP/Sm antibodies (P = 0.048). Conclusion The prophylactic co-vaccination of IL-10 with D2 in pristane-induced lupus ameliorates the renal damage maybe by acting as prophylactic DNA tolerizing therapy.


2021 ◽  
Vol 8 (11) ◽  
pp. 1755
Author(s):  
Avtar S. Dhanju ◽  
Pranjali Batra ◽  
Namit Gupta ◽  
Praneet Manekar ◽  
Suraj Bhola ◽  
...  

Systemic lupus erythematosus (SLE) is a multisystem disorder of autoimmune etiology. Renal involvement is frequently seen in SLE. Tubular dysfunction is also seen in SLE. Authors report a case of distal renal tubular acidosis in patient with SLE. 


2021 ◽  
Vol 12 ◽  
Author(s):  
Danlei Zhou ◽  
Michael Rudnicki ◽  
Gilbert T. Chua ◽  
Simon K. Lawrance ◽  
Bi Zhou ◽  
...  

Human complement C4 is one of the most diverse but heritable effectors for humoral immunity. To help understand the roles of C4 in the defense and pathogenesis of autoimmune and inflammatory diseases, we determined the bases of polymorphisms including the frequent genetic deficiency of C4A and/or C4B isotypes. We demonstrated the diversities of C4A and C4B proteins and their gene copy number variations (CNVs) in healthy subjects and patients with autoimmune disease, such as type 1 diabetes, systemic lupus erythematosus (SLE) and encephalitis. We identified subjects with (a) the fastest migrating C4B allotype, B7, or (b) a deficiency of C4B protein caused by genetic mutation in addition to gene copy-number variation. Those variants and mutants were characterized, sequenced and specific techniques for detection developed. Novel findings were made in four case series. First, the amino acid sequence determinant for C4B7 was likely the R729Q variation at the anaphylatoxin-like region. Second, in healthy White subject MS630, a C-nucleotide deletion at codon-755 led to frameshift mutations in his single C4B gene, which was a private mutation. Third, in European family E94 with multiplex lupus-related mortality and low serum C4 levels, the culprit was a recurrent haplotype with HLA-A30, B18 and DR7 that segregated with two defective C4B genes and identical mutations at the donor splice site of intron-28. Fourth, in East-Asian subject E133P with anti-NMDA receptor encephalitis, the C4B gene had a mutation that changed tryptophan-660 to a stop-codon (W660x), which was present in a haplotype with HLA-DRB1*04:06 and B*15:27. The W660x mutation is recurrent among East-Asians with a frequency of 1.5% but not detectable among patients with SLE. A meticulous annotation of C4 sequences revealed clusters of variations proximal to sites for protein processing, activation and inactivation, and binding of interacting molecules.


2021 ◽  
Vol 8 (11) ◽  
pp. 1749
Author(s):  
Niketha Janga ◽  
Jagadeesan M. ◽  
Kavitha M. M. ◽  
Kannan R.

Systemic lupus erythematosus (SLE) generally affects young to middle-aged women, commonly presenting as a triad of fever, rash, and joint pain. Abdominal pain is a common symptom in patients with SLE. The leading causes of abdominal pain in SLE are lupus enteritis, pancreatitis, pseudo-obstruction, acalculous cholecystitis, mesenteric thrombosis, hepatic thrombosis, medications like (NSAIDS, MMF, steroids, HCQ), colon perforation. The incidence of abdominal pain in patients with SLE ranges from 8-40%, and the commonest cause is lupus enteritis. The following case describes a young woman presenting with lupus enteritis as a manifestation of SLE, the importance of early disease recognition, utilities of abdominal computed tomography (CT) in diagnosis, and current treatment protocols for lupus enteritis. 


2021 ◽  
Author(s):  
Gabrielle Sonigo ◽  
Charles Cassius ◽  
Maxime Battistella ◽  
Hélène Le Buanec ◽  
Martine Bagot ◽  
...  

2021 ◽  
Author(s):  
Yumi Tsuchida ◽  
Hirofumi Shoda ◽  
Masahiro Nakano ◽  
Mineto Ota ◽  
Tomohisa Okamura ◽  
...  

The importance of autotaxin, which catalyzes the production of lysophospholipids, has recently been recognized in various diseases including cancer and autoimmune diseases. We herein report our analysis of autotaxin in systemic lupus erythematosus (SLE), utilizing data from ImmuNexUT, a comprehensive database consisting of transcriptome data and expression quantitative trait locus (eQTL) data of immune cells from patients with immune-mediated disorders. Autotaxin was elevated in the serum of SLE patients, and the expression of ENPP2, which encodes autotaxin, is elevated in plasmacytoid dendritic cells (pDCs) of SLE patients compared to healthy controls. In weighted correlation network analysis, ENPP2 belonged to a module that correlated with disease activity. This module was enriched in interferon-associated genes and included genes whose expression is influenced by SNPs associated with SLE, suggesting that it is a key module connecting genetic risk factors of SLE with disease pathogenesis. The increased expression of ENPP2 in pDCs from SLE patients may be due to increased expression of interferon-associated genes and increased binding of STAT3 complexes to the regulatory region of ENPP2. Thus, autotaxin may play a critical role in connecting genetic risk factors of SLE to disease pathogenesis in pDCs.


2021 ◽  
Vol 7 (2) ◽  
Author(s):  
Yousfi Jaouad ◽  
Oumlil Soukaina ◽  
Benjilali Laila ◽  
Essaadouni Lamiaa

2021 ◽  
Vol 76 (4) ◽  
pp. 394-401
Author(s):  
Yuliya D. Kurochkina ◽  
Maxim A. Korolev

Dendritic cells (DCs) are professional antigen presenting cells that can as stimulate immune response as suppress immune inflamma tion. Recently the role of DCs in the pathogenesis of autoimmune diseases and the possibility of their application as diagnostic markers and methods of treatment has been studied more and more. It was shown that subpopulations DCs play different role in pathogenesis various autoimmune diseases. Thus, pathogenesis of rheumatoid arthritis and ankylosing spondylitis is associated with activity of myeloid DCs and their possibility to present arthritogenic peptides to T-cells. While plasmocytoid DCs are more important in pathogenesis systemic lupus erythematosus and systemic sclerosis. The review presents the results of the latest registered clinical trials about applications DCs in different autoimmune diseases as well as current ideas about functional features DCs during autoimmune diseases. The existing data confirm their possible use as well as the safety of DC in treatment.


Lupus ◽  
2021 ◽  
pp. 096120332110519
Author(s):  
Manar A Helmy ◽  
Amal Saad-Hussein ◽  
Heba Allah Abd E Rahman ◽  
Rasha S Shemies ◽  
Mona Elhelaly ◽  
...  

Organochlorines (OCs) are groups of highly toxic pesticides with known immunotoxicity. The present work aimed to study the potential association between serum residues of OCs and the risk of developing systemic lupus erythematosus (SLE) as well as correlating to the clinical-laboratory manifestations in a sample of Egyptian SLE patients. A cross-sectional study was conducted on 132 patients environmentally exposed to OCs. Patients were diagnosed as SLE based on the American College of Rheumatology (ACR) revised criteria. Systemic Lupus Erythematosus Disease Activity Index-2000 (SLEDAI-2K) score was calculated to stratify the disease severity. Blood and urine samples were collected to measure the levels of OCs, serological markers, and urinary protein. The most frequently detected OCs were p,p’-DDE; lindane; and hexachlorobenzene (HCB). The risk of developing SLE was significantly associated with detected p,p’-DDE and HCB (B value 7.704 and 14.33, respectively). Hexachlorobenzene, in addition, was significantly associated with increased SLEDAI-2K score and polycythemia. Lindane was significantly associated with hypocomplementemia, cardiac manifestations of SLE, anemia, and leucopenia. In conclusion, the detected OCs p,p’-DDE and HCB are associated with increased risk of SLE in Egyptian patients and correlates to the manifestations of disease severity.


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