The clinical features of anti-neutrophil cytoplasmic antibody-associated systemic vasculitis in Chinese children

Abstract

Anti-neutrophil cytoplasmic antibody (ANCA)-associated systemic vasculitis is reported mainly in adults. Studies in children are limited. The current study retrospectively analyzed the clinical characteristics and pathology of ANCA-associated systemic vasculitis in children in our hospital during the past 7 years. Twenty-four pediatric patients were diagnosed as having ANCA-associated systemic vasculitis, including 19 patients with microscopic polyangiitis (MPA), one with Wegener's granulomatosis (WG), three with propylthiouracil (PTU)-induced ANCA-positive vasculitis and one with anti-glomerular basement membrane (GBM) disease. Of patients with primary ANCA-associated systemic vasculitis (MPA and WG), with an average age of 10.8+/-2.8 (6-14) years, 18 patients (90%) were female and two (10%) were male. Nineteen patients (95%) were p-ANCA/MPO-ANCA positive and one (5%) was c-ANCA/PR3-ANCA positive. The interval between onset and diagnosis was 8.5+/-24.3 (0.2-108) months. The majority of the patients (85%) had multi-organ involvement. All patients had clinical evidence of renal involvement and presented with hematuria and proteinuria. Of 20 patients, 16 (80%) also had acute renal failure, and five patients were dialysis dependent. Nine patients underwent renal biopsy and were diagnosed with necrotizing and crescentic glomerulonephritis. However, six biopsies showed immune complex deposition. All patients received immunosuppressive therapy including prednisone and cyclophosphamide, and ten patients also received intravenous administration of methylprednisone pulse therapy according to their clinical situation and renal pathology. Sixteen patients achieved clinical remission, and four patients presented as treatment failure. Patients were followed up for 12.3+/-5.1 months (median 12 months; range 1 to 91 months). Ten patients maintained their clinical remission, and ten progressed to renal failure requiring dialysis. Our study showed that the clinical features and pathology of primary ANCA-associated systemic vasculitis in children were similar to those of adults, but there were a predominance of female patients and late diagnoses. We suggest that early recognition and prompt aggressive treatment might improve outcome.

Interstitial Lung Disease in Children Older Than 2 Years

“…26 While the presentation is often acute with hypoxemia, cough, dyspnea, alveolar infi ltrates on chest fi lms, and hemoptysis, it can also present insidiously with anemia, cough, and exercise limitation. [26][27][28][29] Hemoptysis may not been seen in young children as they tend to swallow their secretions. As shown in Table 2 , alveolar hemorrhage can be classifi ed into 2 groups, those with and those without pulmonary capillaritis.…”

“…Classically, anti-proteinase 3 antibodies (c-ANCA) are found in Wegener's granulomatosis, while anti-myeloperoxidase antibodies (p-ANCA) are found in microscopic polyangiitis. 29 Goodpasture's disease is associated with antiglomerular basement membrane antibodies. 27 These 3 disorders often involve the kidneys as well and thus are known as pulmonary-renal syndromes.…”

An 11-year-old girl with antineutrophil cytoplasmic antibody-associated glomerulonephritis identified by a school urinary screening program

“…As to prognosis, nine of 31 patients progressed to ESRD; of these nine patients, four required dialysis during the initial phase of therapy and did not recover renal function, three progressed to ESRD with episodes of relapse, and the remaining two progressed to ESRD without active disease or relapse [2]. Other studies have revealed that ANCA-associated GN progresses to renal failure in 20-40 % of patients; thus, it is important to identify patients with ANCA-associated GN and to manage treatment in the early stages of the disease [1,[3][4][5][6]. Our case was identified by SUS and was asymptomatic.…”

“…Although ANCA-associated GN is rare in childhood, there are a number of reviews of the clinical features and outcomes of pediatric ANCA-associated GN [2][3][4][5]. Hattori et al [2] reported that the clinical disease spectrum of ANCA-associated GN is similar in pediatric and adult patients and found that 29 % of patients with ANCAassociated GN progressed to end-stage renal disease (ESRD).…”

“…ANCAs were first reported by Davies in 1982 [1] in patients with pauciimmune necrotizing glomerulonephritis (GN) with crescents and are now regarded as a serologic marker for active pauci-immune necrotizing crescentic GN, either in the renally limited form or associated with systemic vasculitis, such as granulomatosis with polyangiitis, microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis [2][3][4][5][6].…”