Cln three-requiring 9 is a negative regulator of Th17 pathway-driven inflammation in anti-glomerular basement membrane glomerulonephritis.
Cln three-requiring 9 is a negative regulator of Th17 pathway-driven inflammation in anti-glomerular basement membrane glomerulonephritis. Am J Physiol Renal Physiol. 2016 Jun 15;:ajprenal.00533.2015 Authors: Lee H, Lee JW, Yoo KD, Yoo JY, Lee JP, Kim DK, Chin HJ, Kim YS, Yang SH Abstract T helper 17 (Th17) lymphocytes promote renal inflammation in anti-glomerular basement membrane glomerulonephritis (anti-GBM GN), and signal transducer and activator of transcription 3 (STAT3) mediates activation of Th17 lymphocytes by interleukin 6 (IL-6) and transforming growth factor beta (TGFβ). Cln three requiring 9 (Ctr9), a subunit of RNA polymerase-associated factor complex (PAFc), regulates the transcription of IL-6/STAT3-dependent genes. Here, we investigated the role of Ctr9 in regulating Th17-driven inflammation in anti-GBM GN. In mice, STAT3β or IL-17 knockout ameliorated anti-GBM autoantibody-induced renal injury. This phenomenon was associated with decreases in retinoic acid receptor-related orphan receptor γt (RORγt), IL-17, phosphorylated STAT3, and pro-inflammatory cytokines. Compared with wild-type mice, Ctr9 increased in both STAT3β-/- and IL-17-/- mice injected with anti-GBM IgG, showing a negative correlation with Th17-related transcripts. Small interfering RNA (siRNA)-mediated knockdown of Ctr9 in intrarenal lymphocytes further upregulated Th17-related transcripts, consistent with repression of Th17 differentiation by Ctr...
Conclusion: Type II (immune complex) CGN was most common type followed by type V (immune negative) and type III (pauci-immune) CGN. The crescentic GN had worse prognosis with>80% of patients progressed to ESRD within 3 month of time from onset of illness. Early diagnosis and treatment is associated with favourable outcome. PMID: 31793263 [PubMed - in process]
Conclusion: CKD-MBD is common among patients on hemodialysis in our center. Screening for CKD-MBD and appropriate use of phosphate binder and Vitamin D when indicated are highly recommended.
ConclusionFew case reports have described histological findings of proteinase 3-antineutrophil cytoplasmic antibody-positive renal injury complicated with infective endocarditis. We believe that an accumulation of histological findings and treatments is mandatory for establishment of optimal management for proteinase 3-antineutrophil cytoplasmic antibody-positive renal injury complicated with infective endocarditis.
Authors: Fabrizi F, Cerutti R, Silva M Abstract Introduction: The introduction of direct-acting antiviral therapy has generated tremendous interest in transplanting organs from HCV-infected donors, an option which has the potential to lower waiting times for solid organ transplantation (including kidneys). Safe, effective and pangenotypic direct-acting antiviral agents are currently available.Areas covered: We have identified studies from PubMed, EMBASE, and the Cochrane database to review risks and benefits on solid organ transplantation from HCV exposed donors in uninfected recipients.Expert opinion: The transmis...
Conditions: C3 Glomerulopathy; Membranoproliferative Glomerulonephritis; Complement Abnormality; Dense Deposit Disease; C3 Glomerulonephritis Interventions: Drug: Aliskiren; Drug: Enalapril Sponsor: Region Skane Not yet recruiting
ConclusionsANCA-GN and IgG4-RKD overlap syndrome concerned mainly MPO-ANCA positive patients. The IgG subclass analysis of MPO-ANCA showed lower percentage of IgG1 subclass. The association between ANCA-GN and IgG4-RKD is possible and represents a special entity.
This study suggests the role of DQ antibodies in determining the graft survival and to highlight the need of HLA DQ typing as a routine of the diagnostic work-up in a solid organ transplant.
CONCLUSIONS: ANCA-GN and IgG4-RKD overlap syndrome concerned mainly MPO-ANCA positive patients. The IgG subclass analysis of MPO-ANCA showed lower percentage of IgG1 subclass. The association between ANCA-GN and IgG4-RKD is possible and represents a special entity. PMID: 31805271 [PubMed - as supplied by publisher]
The objective of this review is: 1) to describe the pathogenesis, clinical and histological features of the disease; 2) to characterize double-positive anti-GBM/ANCA patients; 3) to highlight the prognostic factors of renal and global survival, and 4) to focus on the treatment of anti-GBM disease. PMID: 31776042 [PubMed - as supplied by publisher]
Purpose of review Although uncommon, systemic vasculitis is one of the most severe extraglandular manifestations of primary Sjögren's syndrome (pSS) accounting for the increased morbidity and mortality of the disease. This review aims to describe major previous and recent reports regarding the clinical presentation, prognosis and treatment of systemic vasculitis associated with pSS. Recent findings Both older and recent pSS cohort studies performed over the past several and recent years, have clearly shown that cryoglobulinaemic vasculitis is the most frequent type of systemic vasculitis accompanying pSS. Antineu...