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Typical renal involvement of antineutrophilic cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is pauci-immune glomerulonephritis that presents clinically as rapidly progressive renal failure (RPRF). Here, we report an unusual presentation of myeloperoxidase (MPO)-specific ANCA with isolated involvement of the tubulointerstitium in the form of peritubular capillaritis as the sole lesion without any involvement of the glomerulus. A 52-year-old woman with no previous comorbidities presented with nonspecific symptoms such as fatigue, dysuria, and nausea for two months.
View Article and Find Full Text PDFAtypical Adult-Onset IgA Vasculitis With Extremely Rare Complications Including Diffuse Alveolar Hemorrhage, Heart Failure, and Stroke.
Cureus
December 2024
Department of Respiratory Medicine, Juntendo University School of Medicine Graduate School of Medicine, Tokyo, JPN.
IgA vasculitis (IgAV) generally occurs in young people and presents with a tetrad of symptoms: purpura, abdominal pain, arthralgia, and nephritis. However, it may have an atypical course without the typical tetrad. Diffuse alveolar hemorrhage (DAH), heart failure, and stroke are known complications of IgAV but are all very rare.
View Article and Find Full Text PDFA Challenging Case of IgG4-Related Kidney Disease Accompanied With Positive Serologic Tests of Lupus Erythematosus.
Nephrology (Carlton)
January 2025
Division of Nephrology, Department of Internal Medicine, Faculty of Medicine, Thammasat University, Pathumthani, Thailand.
The case report presents a male patient in his mid-60s with a history of hypertension, benign prostatic hyperplasia and chronic kidney disease (CKD). He presented with gradually increasing serum creatinine levels and hyperglobulinemia, leading to suspicion of multiple myeloma. However, subsequent testing revealed features consistent with systemic lupus erythematosus (SLE) and IgG4-related kidney disease (IgG4-RKD).
View Article and Find Full Text PDFChronic Low-Level IFN-γ Expression Disrupts Mitochondrial Complex I Activity in Renal Macrophages: An Early Mechanistic Driver of Lupus Nephritis Pathogenesis.
Int J Mol Sci
December 2024
Department of Food Science and Nutrition, Kyungpook National University, Daegu 41566, Republic of Korea.
Article Synopsis
- Mitochondrial dysfunction and macrophage dysregulation are important in autoimmune diseases, but how they connect is not fully understood.
- The study focuses on the role of chronic low-level interferon-gamma (IFN-γ) using a mouse model with lupus-like symptoms, finding that this condition suppresses mitochondrial function, especially in the kidneys.
- It suggests that restoring mitochondrial function could improve macrophage activity and provide new targets for treating autoimmune diseases like lupus nephritis.
Calcium calmodulin kinase IV deficiency in podocytes prevents the development of lupus nephritis.
Clin Immunol
January 2025
Department of Medicine, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA 02215, United States of America.
Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by widespread organ involvement including the kidney. Calcium/calmodulin-dependent protein kinase IV (CaMK4) has been shown to conrol immune cell nad podocyte function. To address the effect of genetic podocyte-specific CaMK4 deficiency on systemic autoimmunity and kidney pathology in lupus-prone mice we generated B6.
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