Unmasking Renal Disease in Systemic Lupus Erythematosus: Beyond Lupus Nephritis.

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. The biopsy revealed focal crescent formation in some glomeruli and mild segmental mesangial hypercellularity in others. Although the possibility of antineutrophilic cytoplasmic antibody (ANCA)-associated nephritis with superimposed IgA nephropathy was considered, negative myeloperoxidase and proteinase 3 antibody tests led to a final diagnosis of IgA nephropathy. The patient's treatment included adding prednisone to her existing valsartan prescription for hypertension, which resulted in improved proteinuria. SLE is an autoimmune disease that can cause chronic inflammation and damage to vital organs. Approximately 50% of SLE patients may experience lupus nephritis (LN), underscoring the importance of urinalysis and renal function tests. This case presents a female patient with SLE and IgA nephropathy, a rare association that requires distinction as it affects disease management. IgA nephropathy is the most common cause of idiopathic glomerulonephritis and can lead to end-stage kidney disease in around 40% of cases. A renal biopsy is also crucial for diagnosing IgA nephropathy in patients with or without another autoimmune disease. Focal crescent formation, a histological feature observed in this case, helped exclude several diagnoses, such as lupus nephritis or pauci-immune glomerulonephritis. The primary goal of treating IgA nephropathy is to prevent disease progression. Initial treatment includes controlling blood pressure, reducing proteinuria, and implementing lifestyle modifications. Corticosteroid therapy may be considered if supportive care is insufficient.

Nivolumab-Induced Crescentic Immunoglobulin A Nephropathy With Henoch-Schonlein Purpura Features in a Patient Diagnosed With Hepatocellular Carcinoma.

A 60-year-old Polish male with a history of alcoholism, liver cirrhosis, and hepatocellular carcinoma presented via a referral from his primary medical doctor to the emergency room with respiratory distress, acute kidney injury (AKI), and a purpuric rash on both lower extremities. He had received a total of 16 doses of Nivolumab for hepatocellular carcinoma. He had a baseline serum creatinine of 1.5 and Nivolumab was skipped a month prior to presentation because of a rise in creatinine and the onset of the rash. Labs showed a blood urea nitrogen (BUN) level of 52 mg/dl and creatinine of 3.2 mg/dl. Urinalysis revealed 300 mg proteinuria and 25-50 red blood cells on a high-power field. He was subsequently placed on steroids for vasculitis manifesting as glomerulonephritis and dermatitis. Biopsy specimens of the kidney and skin were taken and showed focally crescentic diffuse proliferative glomerulonephritis with low-grade A IgA deposits and acute tubular necrosis. The skin biopsy revealed leukocytoclastic vasculitis. We hereby describe a case of focally crescentic diffuse proliferative glomerulonephritis with low-grade A IgA deposits and acute tubular necrosis in an individual with Nivolumab-treated hepatocellular carcinoma.

Anti-inflammatory Mechanism of Wuwei Xiaoduyin on IgA Nephropathy Rats Based on NF-κB Inflammatory Signaling Pathway / 中国实验方剂学杂志

Objective:To observe the effect of Wuwei Xiaoduyin on the nuclear factor-<italic>κ</italic>B （NF-<italic>κ</italic>B） signaling pathway in immunoglobulinA nephropathy（IgAN） rats， and to explore its mechanism of action in the treatment of IgA nephropathy. Method:The 40 SD rats were randomly divided into control group， model group， benazepril group （10 mg·kg·d^-1） and Wuwei Xiaoduyin group （2.75 g·kg·d^-1）， with 10 rats in each group. The IgA nephropathy rat model was established by intragastric administration of bovine serum albumin （BSA）， subcutaneous injection of carbon tetrachloride （CCl₄） and tail vein injection of lipopolysaccharide （LPS） for 9 weeks. The rats in each group were given corresponding doses of drugs by gavage， while the rats in the control group and model group were given the same amount of normal saline for successive 28 days. The levels of 24-hour urinary protein （UTP）， serum creatinine （SCr）， blood urea nitrogen （BUN） and serum albumin （ALB） were detected. The contents of tumor necrosis factor-<italic>α</italic> （TNF-<italic>α</italic>）， interleukin-1<italic>β</italic> （IL-1<italic>β</italic>） and interleukin-6 （IL-6） in serum were detected by enzyme-linked immunosorbent assay （ELISA）， the hematoxylineosin staining （HE）， immunofluorescence and transmission electron microscopy were used to observe the renal pathological changes， the expressions of IL-6， I<italic>κ</italic>B kinase <italic>β</italic> （IKK<italic>β</italic>）， phosphorylated I<italic>κ</italic>B kinase <italic>β</italic> （p-IKK<italic>β</italic>）， NF-<italic>κ</italic>B inhibitor protein <italic>α</italic> （I<italic>κ</italic>B<italic>α</italic>）， phosphorylated NF-<italic>κ</italic>B inhibitor protein <italic>α</italic> （p-I<italic>κ</italic>B<italic>α</italic>）， NF-<italic>κ</italic>B p65 and p-NF-<italic>κ</italic>B p65 were detected by immunohistochemistry （IHC）， real-time PCR （RT-PCR） and Western blot， respectively. Result:Compared with the control group， the level of UTP in the model group significantly increased （<italic>P</italic><0.01）， cultured glomerular mesangial cells proliferated， mesangial matrix and electronic dense deposit increased， mesentery thickened. A large amount of IgA was deposited in the glomerular mesangial area and showed irregular particles and mass distribution， the levels of TNF-<italic>α</italic>， IL-1<italic>β</italic>， IL-6 in serum significantly increased （<italic>P</italic><0.01）， the expression levels of IL-6， IKK<italic>β</italic>， p-IKK<italic>β， </italic>NF-<italic>κ</italic>B p65 and p-NF-<italic>κ</italic>B p65 in renal tissue significantly increased （<italic>P</italic><0.01）.Compared with the model group， the levels of UTP in each administration group significantly decreased （<italic>P</italic><0.01）， and the renal tissue injury alleviated， the levels of TNF-<italic>α</italic>， IL-1<italic>β</italic>， IL-6 in serum significantly decreased （<italic>P</italic><0.01）， the expressions of IL-6， IKK<italic>β</italic>， p-IKK<italic>β</italic>， I<italic>κ</italic>B<italic>α</italic>， p-I<italic>κ</italic>B<italic>α</italic>， NF-<italic>κ</italic>B p65 and p-NF-<italic>κ</italic>B p65 in the renal tissue significantly decreased （<italic>P</italic><0.01）. There were no significant differences in serum creatinine， blood urea nitrogen and serum albumin among the groups. Conclusion:Wuwei Xiaoduyin can reduce proteinuria in IgA nephropathy rats， and its mechanism may be related to the inhibition of NF-<italic>κ</italic>B signaling pathway and the over expression of related inflammatory factors.

The Effects of Hyperuricemia on the Prognosis of IgA Nephropathy are More Potent in Females.

Hyperuricemia is a potential risk factor for immunoglobulin A nephropathy (IgAN) progression but its sex-specific effects on IgAN progression remain unclear. This study aimed to determine the effect of serum uric acid on IgAN progression and whether its effect varied according to sex. A total of 4339 patients who diagnosed with IgAN by renal biopsy were retrospectively analyzed. We assessed the association of serum uric acid on IgAN progression using Kaplan-Meier survival analyses and Cox proportional hazards models. The study's primary end point was IgAN progression that was defined as a 50% decline in the estimated glomerular filtration rate or the initiation of dialysis. On average, the serum uric acid levels were higher in the men than in the women. In the fully adjusted Cox proportional hazards model that considered all subjects, the risk of IgAN progression increased by about 25.6% for every 1 mg/dL increase in the baseline uric acid level. The serum uric acid level was an independent risk factor for IgAN progression in both sexes but its effect was more pronounced in the women (hazard ratio [HR], 1.383; confidence interval [CI],1.263 to 1.514; p < 0.001) than in the men (HR, 1.181; CI, 1.097 to 1.272; p < 0.001) (pinteraction < 0.001). A sensitivity analysis involving serum uric acid quartiles generated consistent and robust results. In conclusion, the serum uric acid level was an independent risk factor for IgAN progression and its effect was more pronounced among the women compared with that among the men.

Pulmonary cavitary lesions may be one of the presenting features in Ig A nephropathy.

Immunoglobulin A (Ig A) nephropathy is the most frequent primary glomerulonephritis. Renal limited disease is the most widespread clinical form of the disease. Pulmonary involvement may also be seen concomitantly and capillaritis with pulmonary hemorrhage is the most frequent pulmonary involvement. In this paper, for the first time in literature, we describe an Ig A nephropathy patient with multiple pulmonary cavities as one of the presenting features of the disease. Also, no other etiology for the cavities was found other than Ig A nephropathy. Herein, possible pathogenesis might be capillaritis or deposition of immune complexes. As a result, it should be kept in mind that pulmonary cavity may be the presenting feature of Ig A nephropathy especially with other frequent signs of the disease.

Buerger's Disease Associated with IgA Nephropathy: A Case Report / 대한신장학회잡지

No abstract available.

Coexistence of IgA Nephropathy and Post-infectious Glomerulonephritis / 대한신장학회잡지

A 33-year-old man was admitted with macroscopic hematuria and systemic edema appearing after an acute upper respiratory tract infection. On admission, hypertension, nephrotic syndrome were evident together with a decreased renal function. Renal biopsy showed markedly increased mesangial cells acompanied with increase of endocapillary cells including neutrophils. Immunofluorescence microscopy showed granular deposits of C3 and IgA. Electron Microscopy revealed so-called "hump" on the subepithelial area. These features were consistent with the coexistence of IgA nephropathy (IgAN) and post-infectious glomerulonephritis. It is not clear about the prognosis and the therapeutic regimen in the patient who develop above situation. Although the patient showed still persistent proteinuria, high dose steroid therapy was probably useful for improving the disease.