Rapidly progressive glomerulonephritis in children

Abstract

Rapidly progressive glomerulonephritis (RPGN), characterized by a rapid development of nephritis with loss of kidney function in days or weeks, is typically associated histologically, with crescents in most glomeruli; and is a challenging problem, particularly in low resource settings. RPGN is a diagnostic and therapeutic emergency requiring prompt evaluation and treatment to prevent poor outcomes. Histopathologically, RPGN consists of four major categories, anti-glomerular basement membrane (GBM) disease, immune complex mediated, pauci-immune disorders and idiopathic /overlap disorders. Clinical manifestations include gross hematuria, proteinuria, oliguria, hypertension and edema. Diagnostic evaluation, including renal function tests, electrolytes, urinalysis/microscopy and serology including (anti GBM antibody, antineutrophil cytoplasmic antibody (ANCA)) starts simultaneously with management. An urgent renal biopsy is required to allow specific pathologic diagnosis as well as to assess disease activity and chronicity to guide specific treatment. The current guidelines for management of pediatric RPGN are adopted from adult experience and consist of induction and maintenance therapy. Aggressive combination immunosuppression has markedly improved outcomes, however, nephrotic syndrome, severe acute kidney injury requiring dialysis, presence of fibrous crescents and chronicity are predictors of poor renal survival. RPGN associated post infectious glomerulonephritis (PIGN) usually has good prognosis in children without immunosuppression whereas immune-complex-mediated GN and lupus nephritis (LN) are associated with poor prognosis with development of end stage kidney disease (ESKD) in more than 50% and 30% respectively. Given the need for prompt diagnosis and urgent treatment to avoid devastating outcomes, we conducted a review of the latest evidence in RPGN management to help formulate clinical practice guidance for children in our setting.Information sources and search strategy: The search strategy was performed in the digital databases of PubMed, Cochrane Library, google scholar, from their inception dates to December 2020. Three investigators independently performed a systematic search using the following search terms “Rapidly progressive glomerulonephritis” “children” “crescentic glomerulonephritis” “management” at the same time, backtracking search for references of related literature.
 doi: https://doi.org/10.12669/pjms.38.ICON-2022.5774
 How to cite this:Moorani KN, Aziz M, Amanullah F. Rapidly progressive glomerulonephritis in children. Pak J Med Sci. 2022;38(2):417-425. doi: https://doi.org/10.12669/pjms.38.ICON-2022.5774
 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Antiglomerular basement membrane antibody type rapidly progressive glomerulonephritis with seizures: Two cases and literature review

“…The autoimmune development of antibodies towards type IV collagen in the glomerular and alveolar basement membranes leads to patients typically presenting with RPGN and pulmonary hemorrhage 15–17 . Evidence of anti‐GBM antibodies in serum or histologically is required for diagnosis 18 . Treatment mainly involves the use of apheresis and intensive, strong immunosuppression such as cyclophosphamide 19 …”

“…[15][16][17] Evidence of anti-GBM antibodies in serum or histologically is required for diagnosis. 18 Treatment mainly involves the use of apheresis and intensive, strong immunosuppression such as cyclophosphamide. 19 PRES is a neurological condition characterized by headache, seizure, impaired visual acuity or blindness, focal weakness, and occasionally focal neurological deficits corresponding to the affected lesions of the brain.…”

Kecurigaan rapid progressive glomerulonephritis akibat nefropati IgA : Sebuah laporan kasus

“…Keterlibatan lebih dari setengah glomeruli dalam proses nefritis dengan bentukan crescent ini akan menyebabkan kehilangan lebih dari 50% fungsi ginjal dalam beberapa hari atau minggu sehingga terjadi gagal ginjal akut (GGA). 6 Sekitar 10% dari GGA disebabkan oleh glomerulonefritis. Rapid Progressive Glomerulonephritis atau RPGN adalah salah satu jenis glomerulonefritis akut dengan manifestasi penurunan fungsi ginjal yang mendadak, cepat, dan progresif, disertai dengan oliguria, edema, hipertensi, serta urin sedimen aktif.…”

“…10 Mayoritas gambaran histopatalogi RPGN adalah necrotizing crescentic glomerulonephritis (NCGN), yang dapat dibagi menjadi 3 tipe berdasarkan temuan deposit imun pada imunofluoresen miskroskopis, yaitu linear (tipe 1), granuler (tipe 2), dan pauci-immune (tipe 3). 6 Nefropati IgA merupakan salah satu penyebab penumpukan komplek imun tipe granuler. Tipe RPGN yang paling sering ditemukan adalah tipe Pauci-immune Disorder atau tipe 3.…”

Glomerulonephritis pattern based on renal biopsy at the tertiary center of King Abdulaziz University Hospital, Saudi Arabia

“…RPGN is characterized by a rapid decline in renal function that can lead to ESRD. [11] Glomerulonephritis (GN) is a major public health problem worldwide including in the Arab world. GN is one of the main causes of ESRD (End Stage Renal Disease) in spite that the incidence rate of RPGN in the United States is estimated to be 7 cases per million per year.…”