Immune-mediated systemic diseases in children are a heterogeneous group of mostly rare disorders affecting several organs. These diseases can be autoinflammatory, with a major pathophysiological role for inflammasomes, or autoimmune, often characterized by the presence of autoantibodies produced by B cells and the involvement of autoreactive T cells. The clinical presentation of these diseases is highly variable. In many immune-mediated systemic diseases, renal involvement is an important contributor to morbidity and mortality. Acute kidney inflammation may lead to chronic renal changes resulting in severe chronic kidney disease and ultimately end-stage renal disease.
There are several unmet needs regarding kidney involvement in immune-mediated systemic diseases. First, renal biopsy and histopathologic assessment are still the gold standards for diagnosis and prognostic evaluation of renal involvement. There is a need for easy-applicable, validated non-invasive biomarkers. Second, blood-based and/or urinary biomarkers differentiating disease relapse from transplant rejection are lacking in patients who underwent renal transplantation. Third, the long-term prognosis of chronic kidney disease in children is often unfavorable with a substantial risk of developing end-stage renal disease warranting kidney dialysis and/or transplantation. More targeted treatment options are needed, reducing the burden of broad immunosuppressive and cytotoxic agents and optimizing patient outcomes. Finally, notwithstanding many studies are initiated involving adult subjects, research in children with immune-mediated chronic kidney disease is scarce. There is an unmet need for trials involving this neglected patient population.
The overarching goal of this Research Topic is therefore to provide a comprehensive, contemporary collection of research focusing on chronic kidney disease in children with immune-mediated disorders. Specific objectives are:
1. To explore current potential non-invasive biomarkers to diagnose kidney involvement in these patients and correlate these biomarkers to histopathologic findings and patient outcomes.
2. To describe new targeted treatment options in these patients.
3. To assess the long-term prognosis of patients with chronic immune-mediated kidney disease, taking into account the availability of new treatment options.
4. To provide new guidelines concerning the kidney management of children with systemic autoimmune disease and renal involvement.
5. To describe interesting cases of renal involvement in youth with immune-mediated disease providing novel insights into pathophysiology, treatment options, and/or patient outcome.
Scope of the Research Topic:
• Diseases of interest may include lupus nephritis/systemic lupus erythematosus, ANCA-associated glomerulonephritis/vasculitis, IgA vasculitis/nephropathy, anti-GBM disease, tubulointerstitial nephritis and uveitis, familial Mediterranean fever and renal amyloidosis, other autoinflammatory diseases, C3 glomerulonephritis, membranous proliferative glomerulonephritis, mixed connective tissue disease, juvenile systemic sclerosis, Sjögren's syndrome.
• Specific themes may include non-invasive biomarkers, new therapeutic targeted treatment options, pathophysiologic mechanisms, and management guidelines.
Desired manuscript types: original research, narrative reviews, systematic reviews, case reports, and case series.
Immune-mediated systemic diseases in children are a heterogeneous group of mostly rare disorders affecting several organs. These diseases can be autoinflammatory, with a major pathophysiological role for inflammasomes, or autoimmune, often characterized by the presence of autoantibodies produced by B cells and the involvement of autoreactive T cells. The clinical presentation of these diseases is highly variable. In many immune-mediated systemic diseases, renal involvement is an important contributor to morbidity and mortality. Acute kidney inflammation may lead to chronic renal changes resulting in severe chronic kidney disease and ultimately end-stage renal disease.
There are several unmet needs regarding kidney involvement in immune-mediated systemic diseases. First, renal biopsy and histopathologic assessment are still the gold standards for diagnosis and prognostic evaluation of renal involvement. There is a need for easy-applicable, validated non-invasive biomarkers. Second, blood-based and/or urinary biomarkers differentiating disease relapse from transplant rejection are lacking in patients who underwent renal transplantation. Third, the long-term prognosis of chronic kidney disease in children is often unfavorable with a substantial risk of developing end-stage renal disease warranting kidney dialysis and/or transplantation. More targeted treatment options are needed, reducing the burden of broad immunosuppressive and cytotoxic agents and optimizing patient outcomes. Finally, notwithstanding many studies are initiated involving adult subjects, research in children with immune-mediated chronic kidney disease is scarce. There is an unmet need for trials involving this neglected patient population.
The overarching goal of this Research Topic is therefore to provide a comprehensive, contemporary collection of research focusing on chronic kidney disease in children with immune-mediated disorders. Specific objectives are:
1. To explore current potential non-invasive biomarkers to diagnose kidney involvement in these patients and correlate these biomarkers to histopathologic findings and patient outcomes.
2. To describe new targeted treatment options in these patients.
3. To assess the long-term prognosis of patients with chronic immune-mediated kidney disease, taking into account the availability of new treatment options.
4. To provide new guidelines concerning the kidney management of children with systemic autoimmune disease and renal involvement.
5. To describe interesting cases of renal involvement in youth with immune-mediated disease providing novel insights into pathophysiology, treatment options, and/or patient outcome.
Scope of the Research Topic:
• Diseases of interest may include lupus nephritis/systemic lupus erythematosus, ANCA-associated glomerulonephritis/vasculitis, IgA vasculitis/nephropathy, anti-GBM disease, tubulointerstitial nephritis and uveitis, familial Mediterranean fever and renal amyloidosis, other autoinflammatory diseases, C3 glomerulonephritis, membranous proliferative glomerulonephritis, mixed connective tissue disease, juvenile systemic sclerosis, Sjögren's syndrome.
• Specific themes may include non-invasive biomarkers, new therapeutic targeted treatment options, pathophysiologic mechanisms, and management guidelines.
Desired manuscript types: original research, narrative reviews, systematic reviews, case reports, and case series.
