Rapidly Progressive Glomerulonephritis Secondary to IgA Nephropathy in a Patient with Systemic Lupus Erythematosus

Patel, Amie; Romero-Karam, Lily A; Rojas, Stephanie C. Fuentes; Redfearn, Warren E.; Truong, Luan D.; González, Juan

doi:10.1155/2019/8354823

Cited by 7 publications

(8 citation statements)

References 11 publications

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“…Specifically, both have regional and familial clustering and have hematuria and proteinuria as common clinical manifestations, with renal deposition of IgA as the pathology. The co-existence of IgAN and LN has also been reported in clinical practice (Padyukov et al, 2001;Kobak et al, 2011;Patel et al, 2019). However, the two diseases are mutually independent, with IgAN being more common in young men and LN in young women.…”

Section: Introductionmentioning

confidence: 73%

Weighted Gene Co-expression Network Analysis Reveals Different Immunity but Shared Renal Pathology Between IgA Nephropathy and Lupus Nephritis

et al. 2021

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Both IgA nephropathy (IgAN) and lupus nephritis (LN) are immunity-related diseases with a complex, polygenic, and pleiotropic genetic architecture. However, the mechanism by which the genetic variants impart immunity or renal dysfunction remains to be clarified. In this study, using gene expression datasets as a quantitative readout of peripheral blood mononuclear cell (PBMC)- and kidney-based molecular phenotypes, we analyzed the similarities and differences in the patterns of gene expression perturbations associated with the systematic and kidney immunity in IgAN and LN. Original gene expression datasets for PBMC, glomerulus, and tubule from IgAN and systemic lupus erythematosus (SLE) patients as well as corresponding controls were obtained from the Gene Expression Omnibus (GEO) database. The similarities and differences in the expression patterns were detected according to gene differential expression. Weighted gene co-expression network analysis (WGCNA) was used to cluster and screen the co-expressed gene modules. The disease correlations were then identified by cell-specific and functional enrichment analyses. By combining these results with the genotype data, we identified the differentially expressed genes causatively associated with the disease. There was a significant positive correlation with the kidney expression profile, but no significant correlation with PBMC. Three co-expression gene modules were screened by WGCNA and enrichment analysis. Among them, blue module was enriched for glomerulus and podocyte (P < 0.05) and positively correlated with both diseases (P < 0.05), mainly via immune regulatory pathways. Pink module and purple module were enriched for tubular epithelium and correlated with both diseases (P < 0.05) through predominant cell death and extracellular vesicle pathways, respectively. In genome-wide association study (GWAS) enrichment analysis, blue module was identified as the high-risk gene module that distinguishes LN from SLE and contains PSMB8 and PSMB9, the susceptibility genes for IgAN. In conclusion, IgAN and LN showed different systematic immunity but similarly abnormal immunity in kidney. Immunological pathways may be involved in the glomerulopathy and cell death together with the extracellular vesicle pathway, which may be involved in the tubular injury in both diseases. Blue module may cover the causal susceptibility gene for IgAN and LN.

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Section: Introductionmentioning

confidence: 73%

Weighted Gene Co-expression Network Analysis Reveals Different Immunity but Shared Renal Pathology Between IgA Nephropathy and Lupus Nephritis

et al. 2021

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“…Approximately 0.3% (9 out of 251) ESRD in SLE patients is attributed to nonlupus nephritis [ 20 ]. IgA nephropathy in SLE patients has been previously described in a total of 10 patients from 8 reports [ 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 ]. One patient had 2 sequential kidney biopsies, lupus nephritis class II on the initial biopsy, and IgA nephropathy on the follow-up biopsy after treatment [ 9 ].…”

Section: Discussionmentioning

confidence: 99%

“…One patient had 2 sequential kidney biopsies, lupus nephritis class II on the initial biopsy, and IgA nephropathy on the follow-up biopsy after treatment [ 9 ]. Another IgA nephropathy in SLE patient showed intense global granular glomerular IgA and C3 deposition with absence of C1q staining, however, subendothelial, subepithelial, and intramembranous electron-dense deposits were also present [ 12 ]. The diagnosis of IgA nephropathy were rendered for all the reported cases; however, the pathologic features were not classic for IgA nephropathy at least in some of these cases as mentioned above.…”

Section: Discussionmentioning

confidence: 99%

“…IgA nephropathy has been reported in patients with SLE, although rare [ 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 ]. IgA nephropathy, the most common primary glomerulonephritis globally, is characterized by glomerular deposition of polymeric IgA1 with aberrant glycosylation at the hinge region O glycans (Gd-IgA1) and the autoantibody formation against this region.…”

Section: Introductionmentioning

confidence: 99%

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Diagnostic Utility of Galactose-Deficient Immunoglobulin A1 Immunostaining in the Differentiation of Lupus Nephritis and Immunoglobulin A Nephropathy

Kouri³

et al. 2021

Glomerular Dis

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Background: Renal biopsy plays an important role in the establishment of the diagnosis and the management of patients with lupus nephritis. Immunoglobulin A (IgA) nephropathy rarely has been reported in kidney biopsy of lupus patients. Lupus nephritis and IgA nephropathy can be readily diagnosed on renal biopsy when the classic patterns are present. However, atypical patterns can become a diagnostic challenge. Galactose-deficient IgA1 (Gd-IgA1) is a key element in the pathogenesis of primary IgA nephropathy. Glomerular Gd-IgA1 deposits, detected by immunofluorescent staining of KM-55 (a Gd-IgA1-specific monoclonal antibody), are consistently identified in the mesangium of IgA nephropathy but are significantly less or absent in lupus nephritis accompanied by significant IgA deposition. Case Presentation: Here we report the case of an 11-year-old girl who was recently diagnosed with systemic lupus erythematosus (SLE) and was found to have hematuria and proteinuria. Renal biopsy showed focal mesangial hypercellularity with IgA dominant, “full house” like pattern of mesangial deposition. The biopsy findings present a diagnostic dilemma with the differential diagnosis of IgA nephropathy versus lupus nephritis with atypical immunofluorescence, and IgA nephropathy is favored, in the absence of strong straining of C1q or C3, extraglomerular deposits, tissue antinuclear antibodies, and endothelial tubuloreticular inclusions. However, no detectable glomerular KM-55 staining was seen in the kidney biopsy. Conclusions: We demonstrate the unique diagnostic utility of immunostaining for KM-55 in a challenging kidney biopsy of an SLE patient with features suggestive of IgA nephropathy. The absence of KM-55 staining excludes IgA nephropathy, supporting a diagnosis of lupus nephritis with atypical immunofluorescence in this patient with SLE.

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“…GN syndromes are often recognized by the presence of hematuria and proteinuria [ 3 ]. Furthermore, RPGN presents with severe acute kidney injury in a relatively short period of time, ranging from days to weeks, and if not treated, it frequently progresses to end-stage renal disease [ 4 ].…”

Section: Introductionmentioning

confidence: 99%

Lactobacillus acidophilus Endocarditis Complicated by Pauci-Immune Necrotizing Glomerulonephritis

Chukwurah

Takang

Uche

et al. 2020

Case Reports in Medicine

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Infective endocarditis (IE) is more common in patients with predisposing cardiac lesions and has many potential complications, including stroke and arterial thromboembolisms. Renal manifestations have an estimated prevalence of ∼20%. Rapidly progressive glomerulonephritis (RPGN) is a nephrological emergency manifested by autoimmune-mediated progressive loss of renal function over a relatively short period of time. Here, we report the case of a 60-year-old Caucasian male, who presented with speech impairment and was found to have multiple embolic strokes caused by aortic valve IE. His renal function declined rapidly, and his urine sediment featured hematuria and proteinuria. ANCA titer was negative by immunofluorescence (IF); however, the PR3 antibody was elevated. The renal biopsy revealed pauci-immune focally necrotizing glomerulonephritis with the presence of ∼25% cellular crescents. He was initially treated with plasmapheresis and pulse dose steroids. Hemodialysis was initiated for uremic symptoms. After four weeks of antibiotic therapy and with blood cultures remaining negative, he was treated with rituximab. Two months after discharge, his renal function showed improvement, and hemodialysis was discontinued. This case highlights several complications associated with lactobacillus endocarditis including RPGN.

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Rapidly Progressive Glomerulonephritis Secondary to IgA Nephropathy in a Patient with Systemic Lupus Erythematosus

Cited by 7 publications

References 11 publications

Weighted Gene Co-expression Network Analysis Reveals Different Immunity but Shared Renal Pathology Between IgA Nephropathy and Lupus Nephritis

Weighted Gene Co-expression Network Analysis Reveals Different Immunity but Shared Renal Pathology Between IgA Nephropathy and Lupus Nephritis

Diagnostic Utility of Galactose-Deficient Immunoglobulin A1 Immunostaining in the Differentiation of Lupus Nephritis and Immunoglobulin A Nephropathy

Lactobacillus acidophilus Endocarditis Complicated by Pauci-Immune Necrotizing Glomerulonephritis

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