Crescentic glomerulonephritis in children

Abstract

Data on patients with crescentic glomerulonephritis (greater than 50% glomeruli with crescents), referred to the Hospital for Sick Children during the past 13 years, were reviewed. Thirty patients (13 male, 17 female) aged 3.7-15.7 years (mean 9.5) were evaluated. Initial clinical features included: oedema (24/30), hypertension (19/30), gross haematuria (15/30), oliguria (15/30) and a decreased glomerular filtration rate (GFR less than 30 ml/min per 1.73 m2) (22/30). Henoch-Schönlein purpura was present in 9 patients, microscopic polyarteritis in 3, polyarteritis nodosa in 1, Wegener's granulomatosis in 1, systemic lupus erythematosus in 1, post-streptococcal glomerulonephritis in 2, mesangiocapillary glomerulonephritis in 7, anti-glomerular basement membrane glomerulonephritis in 2, and 4 were idiopathic. In 10 patients 50%-79% of glomeruli were affected by crescentic changes (group 1) and in the remaining 20, 80% or more (group 2). The crescents were cellular, fibrocellular or fibrous, and the degree of sclerosis was assessed. Patients in both groups were treated with plasma exchange, corticosteroids, anticoagulants, cyclophosphamide and azathioprine in different combinations. On follow-up, 3 patients were dead, 1 was lost to follow-up, 12 were on dialysis/transplant programmes, 4 had a GFR of less than 30 and 10 a GFR of more than 30 ml/min per 1.73 m2. In our experience, 50% progressed to end-stage renal failure. The interval between disease onset and start of treatment was a prognostic factor for outcome. Fibrous crescents were associated with a worse outcome than fibrocellular crescents (P less than 0.05). Outcome was not, however, related to the percentage of glomeruli affected (P greater than 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)

Clinical Evaluation of Acute Kidney Injury in Children

“…Serologic tests including an ANA, ANCA, anti-GBM titers and complement studies are required to evaluate the etiology of the rapidly progressive glomerulonephritis. Active lesions on biopsy are associated with recovery of renal function and may be reversible with immunosuppressive therapy (74)(75)(76). Since specific therapy will depend upon the pathologic findings, a biopsy should be preformed quite promptly when a child presents with clinical characteristics suggestive of rapidly progressive glomerulonephritis so that specific therapy can be initiated promptly.…”

“…Some glomerulonephritides such as post-infectious glomerulonephritis, membranoproliferative glomerulonephritis, HSP nephritis and lupus nephritis present with a rapidly progressive course in a minority of cases. However, other glomerulonephritis such as anti-neutrophil cytoplasmic antibody (ANCA) positive glomerulonephritis, Goodpasture's syndrome, and idiopathic rapidly progressive glomerulonephritis typically presents with acute kidney injury (74)(75)(76). Serologic tests including an ANA, ANCA, anti-GBM titers and complement studies are required to evaluate the etiology of the rapidly progressive glomerulonephritis.…”

Therapeutic plasma exchange for the treatment of pediatric renal diseases in 2013

“…The renal prognosis is dependent on renal function at the time of presentation, with few patients with a creatinine level of >6.6 mg/dL recovering sufficient renal function to avoid renal replacement therapy despite aggressive treatment with plasma exchange and immunosuppression [65,69]. Anti-GBM disease is rare in children and described in only small case series and case reports [70][71][72]. In these series, patients treated with plasma exchange show a reduction in levels of anti-GBM antibodies, but reported cases of improved renal function are rare [73,74].…”

Small-Vessel Vasculitis

“…In addition, necrotizing glomerulonephritis is very common, and pulmonary capillaritis often occurs [1]. Clinically, MPA can be difficult to distinguish from WG and often presents with rapidly progressive pauci-immune glomerulonephritis [44] in association with perinuclear ANCA (pANCA) positivity [1].…”