Severe lupus nephritis in the absence of systemic lupus erythematosus (SLE) is a rare condition with an unclear clinical presentation and outcome.We conducted a historical observational study of 12 adult (age >18 years) patients with biopsy-proven severe lupus nephritis or lupus-like nephritis without SLE immunological markers at diagnosis or during follow-up. Excluded were patients with chroni...

We report a case of renal cell carcinoma diagnosed after a patient was treated successfully with intravenous cyclophosphamide for her active proliferative lupus nephritis (classes III and V). After the intravenous cyclophosphamide regimen, the patient was asymptomatic with persistent microscopic haematuria, and no proteinuria. The renal cell carcinoma was located on the left kidney; incidentall...

An estimated 0.9% to 2.4% of patients with systemic lupus erythematosus (SLE) also have hemophagocytic lymphohistiocytosis (HLH). HLH associated with autoimmune diseases is often refractory to corticosteroid treatment; thus, additional immunosuppressive drugs, such as cyclosporine, cyclophosphamide, or tacrolimus, are required. Here, we describe the case of a 44-year-old Japanese woman who deve...

INTRODUCTION Among various lupus renal vascular changes, thrombotic microangiopathy (TMA) presented with the most severe clinical manifestations and high mortality. The pathogenesis of TMA in systemic lupus erythematosus (SLE) was complicated. The aim of this study was to assess clinical manifestations, laboratory characteristics, pathological features and risk factors for clinical outcomes of ...

BACKGROUND Recently, we identified specific N- and 6-O-sulphated heparan sulphate (HS) domains on activated glomerular endothelial cells. In this study, we evaluated in lupus nephritis the expression of different HS domains on glomerular endothelium and in the glomerular basement membrane (GBM). METHODS The expression of specific glomerular HS domains and the presence of immunoglobulins (Ig) ...

INTRODUCTION Systemic lupus erythematosus is more prevalent among Asians, African Americans, and Hispanics than in Caucasians. Progress in its management over the past few decades has resulted in improved patient survival, although a considerable proportion of patients are still afflicted with long-term illnesses such as chronic renal failure secondary to lupus nephritis. METHODS AND RESULTS ...

We used an ELISA employing extracts of human glomerular basement membrane (GBM) to detect, characterize, and evaluate the clinical significance of glomerular-binding IgG in patients with SLE nephritis. Most patients with SLE nephritis exhibited GBM-binding IgG, although many patients with active nonrenal SLE or symptomatic, drug-induced lupus had similar reactivity, albeit at lower levels. IgG ...

BACKGROUND IgA nephropathy (IgAN) and nephritis in Systemic Lupus Erythematosus (SLE) are two common forms of glomerulonephritis in which genetic findings are of importance for disease development. We have recently reported an association of IgAN with variants of TGFB1. In several autoimmune diseases, particularly in SLE, IRF5, STAT4 genes and TRAF1-C5 locus have been shown to be important cand...

Methods We report a series of 30 children, including 25 girls, mean age of onset is 12 years, with a range of 7-16 years, the average time from diagnosis is 6 months. The mode of presentation of the disease is classical (general signs, skin and joints involvement) in 56% of cases, by lupus nephritis in 26.66 % of patients, or a Macrophage Activation Syndrome “MAS” in 13.33 % of cases and lupus ...

Systemic lupus erythematosus (SLE) nephritis often requires treatment with cyclophosphamide, which carries the risk of major side eVects including infection, ovarian failure and bladder malignancy. Therapeutic strategies that would specifically target lymphocytes are appealing. Following the first report of the use of the purine nucleoside analogue cladribine (2-chloro-2’deoxyadenosine), a sele...