systemic lupus , glomerulonephritis

INTRODUCTION: Podocyte infolding glomerulopathy (PIG) is a newly recognized rare glomerular injury. The clinical significance and mechanism of this injury pattern remains unclear. METHODS: We conducted a retrospective study of renal biopsies from January 2018 to December 2020 in Kingmed Diagnostics. The renal biopsy features and clinical data were reviewed. Laser scanning microdissection and mass spectrometry (LMD/MS) was conducted to analyze the potential mechanism...

Systemic Lupus Erythematosus is a systemic autoimmune disease characterized by a great heterogenicity in course and clinical manifestations. Although prognosis improved in the last decades of the 20th century, mortality remains higher than in the general population and uncontrolled disease activity and therapy-related adverse effects have been identified as major contributors to damage accrual and poor outcomes. Assessment of disease activity and damage in SLE represents a great challenge even to the expert rheumatologist...

Systemic lupus erythematosus (SLE) is a heterogeneous autoimmune inflammatory disease that damages multiple organs by the production of autoantibodies. Numerous research studies have demonstrated the anti-inflammatory effects of ω-3 polyunsaturated fatty acids (PUFAs). A diet rich in ω-3 PUFAs reduces chronic inflammatory and autoimmune conditions. Herein, we investigated the protective effect of ω-3 PUFAs against autoimmune injury in SLE. In a TMPD-induced mouse model of SLE, supplementation with eicosapentaenoic acid (EPA)-rich (97%) fish oil was found to alleviate systemic autoimmune phenotypes such as ascites, lipogranulomas and serum dsDNA levels...

Antiglomerular basement membrane disease (anti-GBM) is an unusual cause of glomerulonephritis. Patients usually present with rapidly progressive glomerulonephritis with or without pulmonary hemorrhage. The diagnosis is based on linear deposits of IgG along the GBM and the presence of anti-GBM antibodies. However, cases with atypical anti-GBM disease in which an anti-GBM antibody was not detected have been reported. We report a 29-year-old pregnant woman with underlying systemic lupus erythematosus (SLE) who presented with severe glomerulonephritis due to atypical antiglomerular basement membrane disease...

Proteinase 3 (PR3) anti-neutrophil cytoplasmic antibodies (ANCA) and anti-double-stranded DNA (anti-dsDNA) antibodies have been associated with a variety of nephritic diseases, most recognizably granulomatosis with polyangiitis and systemic lupus erythematosus (SLE) glomerulonephritis, respectively. We report the first clinical case of positive PR3 and dsDNA in a patient with renal Immunoglobin light chain (AL) amyloidosis. A 75-year-old man presented to the hospital with chronic fatigue, weight loss, and a recent diagnosis of left ventricular infiltrative cardiomyopathy secondary to AL amyloidosis...

BACKGROUND: Estrogen has been thought to play an essential role in the disease pathogenesis of systemic lupus erythematosus, which is 9-10 times more prevalent in the female population. It has been shown that irregular estrogen/estrogen receptor signaling pathways may contribute to the pathophysiology of various renal diseases. In this study, we compared renal estrogen receptors between lupus nephritis, familial Mediterranean fever-associated renal amyloidosis, ANCA-associated nephritis, and intact kidney to investigate their role in the pathophysiology of renal diseases...

Childhood-onset systemic lupus erythematosus is an inflammatory and autoimmune condition characterized by heterogeneous multisystem involvement and a chronic course with unpredictable flares. Kidney involvement, commonly called lupus nephritis, mainly presents with immune complex-mediated glomerulonephritis and is more frequent and severe in adults. Despite a considerable improvement in long-term renal prognosis, children and adolescents with lupus nephritis still experience significant morbidity and mortality...

INTRODUCTION: Systemic lupus erythematosus (SLE) is an autoimmune disease in which circulating immune complexes can cause different types of glomerulonephritis, according to immune deposits and to the type of glomerular cell injury. Proliferative lesions represent the most severe form of lupus nephritis (LN) and often lead to kidney failure and death. Mucosal-associated invariant T (MAIT) cells are a subset of innate-like T cells that recognize microbial-derived ligands from the riboflavin synthesis pathway...

In this clinical case, we report an atypical and unique presentation of systemic lupus erythematosus (SLE) in a 39-year-old female with nephrotic syndrome. The patient exhibited class IV plus V lupus nephritis and extensive immune complex deposition within the intracapillary and arteriolar regions suggestive of cryoglobulinemic glomerulonephritis, despite no detectable circulating cryoglobulins. Electron microscopy revealed cryoglobulin-like deposit distribution in all glomerular examined compartments, namely subendothelial, intramembranous, subepithelial, and mesangial, apparently extending from the capillary hyaline thrombi...

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease and there is a distinct differentiation of clinical manifestations. Lupus nephritis (LN) is clinically apparent in approximately half of patients. A kidney biopsy is essential to define the kidney injury, exclude other injurious causes, and determine the histopathologic subtypes. Autoantibodies are crucial to the pathogenesis and the deposition of immune complexes in glomeruli is a hallmark of LN. The histopathology of LN is quite varied. Despite pauci-immune LN being an unexpected condition in SLE, it has been observed rarely with the presence of antineutrophil cytoplasmic autoantibodies (ANCA)...

Nucleic acid-specific Toll-like receptors (TLRs) have been implicated in promoting disease pathogenesis in systemic lupus erythematosus (SLE). Whether such TLRs mediate disease onset, progression, or both remains undefined; yet the answer to this question has important therapeutic implications. MyD88 is an essential adaptor that acts downstream of IL-1 family receptors and most TLRs. Both global and B cell-specific Myd88 deficiency ameliorated disease in lupus-prone mice when constitutively deleted. To address whether Myd88 was needed to sustain ongoing disease, we induced B cell-specific deletion of Myd88 after disease onset in MRL...

Various immunity-related adverse events have been reported to be associated with the inhibition of programmed cell death receptor 1. We report a rare case of a relapse of lupus nephritis (LN), involving rapidly progressive glomerulonephritis, which was induced by nivolumab treatment in a patient with oral cancer. The patient had a history of systemic lupus erythematosus and underwent treatment with steroids, rituximab, and plasmapheresis. However, her renal function did not improve, and she died of multiple organ failure...

Kidney disease is a global health and healthcare burden. Glomerulonephritis (Gn), both primary and secondary, is generally characterized by an inflammatory glomerular injury and may lead to end-stage renal disease. Kidney biopsy is fundamental to the diagnosis; however, kidney biopsy presents some concerns that may partly hamper the clinical process. Therefore, more accurate diagnostic tools are needed. Extracellular vesicles (EVs) are membranous vesicles released by cells and found in bodily fluids, including urine...

BACKGROUND: Systemic lupus erythematosus (SLE) is an autoimmune disease that primarily affects young women, has many different manifestations, and the disease severity can wax and wane. OBJECTIVES: This study aims to determine the association between prior bariatric surgery and SLE outcomes. SETTING: A population-based, retrospective study using data from the U.S. Nationwide Inpatient Sample (NIS) database between 2005 and 2018. METHODS: Data of hospitalized patients with SLE and morbid obesity were extracted...

A common criticism of the classification of lupus nephritis is the relative scarcity of information regarding tubular, interstitial, and vascular changes compared to the available information regarding glomerular changes, even though their potential for independent progression is known. This study reviewed the importance of less explored lesions by the current and widely used 2003 classification of lupus nephritis of the International Society of Nephrology/Renal Pathology Society (ISN/RPS), with emphasis on the tubulointerstitial, podocyte, and vascular lesions, increasingly recognised as being important in the pathogenesis and prognosis of the disease...

Membranoproliferative glomerulonephritis (MPGN) represents a heterogeneous group of diseases. The common feature of a membranoproliferative lesion pattern in the kidney biopsy can either be idiopathic/primary or-much more frequently-have a secondary cause. The historical classification into MPGN types I to III has largely been abandoned and replaced in recent years by a pathogenesis-oriented classification. A MPGN with C1q, C3 and/or C4 deposits on light microscopy is referred to as immune complex GN (IC-GN), while a MPGN with dominant C3 deposits is referred to as C3 glomerulopathy (C3G)...

Systemic lupus erythematosus (SLE) is a systemic autoimmune disease, that mainly affects skin, joints and kidneys but can affect any organ in the body. It is characterized by presence of multiple autoantibodies like ANA, antibodies to dsDNA and RNA associated proteins. The major mechanism leading to tissue damage includes immune complex mediated complement activation, interferon alpha release by plasmacytoid dendritic cells, NETosis by neutrophils as well as defects in monocytes leading to poor clearance of cellular debris and direct cellular dysfunction mediated by antibodies...

BACKGROUND: We present clinical, biochemical, and histopathological characteristics and treatment outcomes of biopsy proven childhood lupus nephritis (LN) from a low/middle income setting treated in the current era of increased use of Mycophenolate Mofetil (MMF) and biologics. METHODS: Retrospective observational study of children (1-18 years) with biopsy proven LN treated from 01.01.2010 to 31.01.2020. RESULTS: 60 children met our inclusion criteria (80%, n = 48 were females)...

A 64-year-old Caucasian woman with a history of hypertension and systemic lupus erythematosus (SLE) was referred to a nephrology clinic due to persistent microscopic hematuria and trace proteinuria. Initial tests showed elevated antinuclear antibodies (ANA), anti-double-stranded DNA (anti-dsDNA), and anti-Sjögren's syndrome-related antigen A (anti-SSA) levels, while other markers remained within normal limits. Over the course of a year, her urine protein-creatinine ratio increased, prompting a renal biopsy...

OBJECTIVES: Dysregulation of interferon-alpha (IFN-α) is considered central to the immunological abnormalities observed in SLE. Short-term mortality during high disease activity in lupus is up to 30%. Adenovirus vector-introduced IFN-α into a lupus-prone mouse causes the development of glomerulonephritis and death within weeks. We studied serum IFN-α as a biomarker of in-hospital mortality in patients of SLE with high disease activity. METHODS: Serum IFN-α (ELISA) was measured in patients hospitalised for acute severe lupus in a tertiary care rheumatology unit in India and the levels were compared between survivors and non-survivors...