Anca-associated crescentic glomerulonephritis in a child with isolated renal involvement

Çelakıl, Mehtap; Yücel, Burcu; Ozod, Umay; Bek, Kenan

doi:10.1590/2175-8239-jbn-2018-0062

Cited by 2 publications

(1 citation statement)

References 15 publications

(17 reference statements)

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“…The geographical distribution of the serotypes is partially explained by the ambient ultraviolet radiation levels and latitude but also proposes a heterogeneity in the genetic background (13). The average onset of AAV is 65 years and prevalence peaks in the 70-75-year age group (7,12,14), but it is not limited to adults (15,16). Patients with renal involvement are 10 years older on average compared to those without it (4).…”

Section: Prevalencementioning

confidence: 99%

Diagnostic and Therapeutic Approach in ANCA-Associated Glomerulonephritis: A Review on Management Strategies

2022

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Anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis is a destructive small vessel vasculitis affecting multiple organs. Renal involvement often leads to end-stage renal disease and increases mortality. Prompt diagnosis and initiation of adequate immunosuppressive therapy are critical for the best patient and kidney outcomes. However, considerable heterogeneity in symptoms and severity across the patients frequently hinder the diagnosis and management. The objective of this review is to emphasize the heterogeneity of the ANCA-associated vasculitis, facilitate the recognition and give guidance to the therapeutical possibilities. We present epidemiologic and risk factors, pathogenesis, and provide comprehensive clinical features of the disease. This article also focuses on the currently available therapeutic options and emerging cellular and molecular targets for the management of systemic and especially renal disease. We conducted extensive literature research published on PubMed and Google Scholar. We systematically reviewed, analyzed, and assembled databases, covering a broad spectrum of aspects of the disease. We compared and summarized the recommendations of two recent guidelines on ANCA-associated vasculitis. The incidence of ANCA-associated vasculitis, hence glomerulonephritis shows a steady increase. Familiarity with the presenting symptoms and laboratory abnormalities are necessary for rapid diagnosis. Early initiation of treatment is the key aspect for favorable patient and renal outcomes. A better understanding of the pathogenesis constantly leads to more targeted and therefore more efficient and less toxic treatment.

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Section: Prevalencementioning

confidence: 99%

Diagnostic and Therapeutic Approach in ANCA-Associated Glomerulonephritis: A Review on Management Strategies

2022

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Acute Post-Streptococcal Glomerulonephritis in Children: A Comprehensive Review

Pinheiro

Freitas

Castro

et al. 2022

CMC

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Background: Acute post-streptococcal glomerulonephritis (APSGN) is an immune-complex (ICs) mediated glomerular disease triggered by group A β-hemolytic streptococcus (GAS) or Streptococcus pyogenes infections. APSGN represents a major cause of acquired kidney injury in children. Methods: This non-systematic review summarizes recent evidence on APSGN. We discuss the epidemiology, pathogenesis, clinical and laboratory findings, histopathology, treatment and prognosis of the disease. Results: The median APSGN incidence in children in developing countries is estimated at 24.3/100,000 per year, compared with 6.2/100,000 per year in developed countries. Nephritis-associated plasmin receptor, identified as glyceraldehyde-3-phosphate dehydrogenase, and the cationic cysteine proteinase streptococcal pyrogenic exotoxin B are thought to be two leading streptococcal antigens involved in the pathogenesis of APSGN, which activate the complement system, mainly via the alternative but also the lectin pathway. This process is critical for the generation of inflammation by the ICs deposited in the glomerulus. The classic phenotype is an acute diffuse proliferative glomerulonephritis leading to features of the nephritic syndrome including hematuria, oliguria, hypertension and edema. The histopathology shows that the glomeruli are diffused affected, mostly presenting enlarged glomerular tuffs due to hypercellularity. Proliferative endothelial and mesangial cells and inflammation are also observed. APSGN frequently has spontaneous recovery. There is no specific therapy, but its morbidity and mortality are drastically reduced by the prevention and/or treatment of complications. Conclusion: Despite recent advances, the pathogenesis of APSGN is not fully understood. There is no specific treatment for APSGN. The prognosis is generally good. However some cases may evolve to chronic kidney disease.

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Anca-associated crescentic glomerulonephritis in a child with isolated renal involvement

Cited by 2 publications

References 15 publications

Diagnostic and Therapeutic Approach in ANCA-Associated Glomerulonephritis: A Review on Management Strategies

Diagnostic and Therapeutic Approach in ANCA-Associated Glomerulonephritis: A Review on Management Strategies

Acute Post-Streptococcal Glomerulonephritis in Children: A Comprehensive Review

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