Epidemiology and clinical features of childhood-onset anti-neutrophil cytoplasmic antibody–associated vasculitis: a clinicopathological analysis

Hirano, Daishi; Ishikawa, Tomoaki; Inaba, Aya; Mai, Sabine; Shinozaki, Tomohiro; Iijima, Kazumoto; Ito, Shuichi

doi:10.1007/s00467-019-04228-4

Cited by 19 publications

(14 citation statements)

References 44 publications

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“…The incidence rate of child AAV is not high, and epidemiological data are few. The incidence rate of AAV in children is estimated to be 3.41-4.28/10 6 [14]. In a Swedish study, the proportion of MPA, GPA, and EGPA in AAV of children was 5.97, 5.97, and 1.49% [15].…”

Section: Discussionmentioning

confidence: 99%

Relationship between Renal Damage and Serum Complement C3 in Children with Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis

Zhang

Yang

et al. 2021

Nephron

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Background: Anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) disease is a well-known antibody-induced autoimmune disease. The pathogenesis of AAV has not yet been completely clarified, but may be related to heredity, infection, environmental factors, cellular immunity, etc. In recent years, complement in AAV pathogenesis has become the latest research hotspot, and the decrease of serum complement C3 is associated with poor prognosis of anti-neutrophil cytoplasmic antibody-associated glomerulonephritis. In the current study, we investigated the associations between serum complement C3 and kidney injury in AAV children. Methods: Twenty-four children with AAV admitted to our hospital from June 2014 to June 2019 were divided into the low C3 group and the normal C3 group. All the children have undergone renal biopsy. The clinical manifestations, laboratory tests, renal pathology, treatment, and prognosis of the 2 groups were observed. The primary end point was end-stage renal disease (ESRD). Results: It was shown that kidney injury was more obvious in patients with low C3 than in patients with normal C3 serum. The values of ESR, Scr, and UA before treatment in the low C3 group were higher than those in the normal C3 group (p < 0.01); the values of RBC, Hb, PLT, ALB, LDH, and eGFR in the normal C3 group were higher than those in the low C3 group (p < 0.01). The values of urinary protein and NAG enzyme in the low C3 group were higher than those in the normal C3 group (p < 0.01). The area of glomerular abandonment, sclerosis, segmental sclerosis, crescent, cellular crescent, cellular fibrous crescent, fibrous crescent, segmental loop necrosis, and the number of cases with acute renal tubulointerstitial lesions in the low C3 group were bigger than those in the normal C3 group (p < 0.05 and < 0.01). The number of cases with C3 deposition in the low C3 group was higher than that in the normal C3 group (p < 0.05). The number of patients receiving CRRT and PE in the low C3 group was higher than that in the normal C3 group (p < 0.05 and < 0.01). In this study, 3 children entered the stage of ESRD and 1 died in the low C3 group. Conclusion: The kidney injury of AAV children with low complement C3 is serious, and the prognosis is poor. We should pay attention to the influence of decreased complement C3 on the condition and prognosis of AAV children.

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Section: Discussionmentioning

confidence: 99%

Relationship between Renal Damage and Serum Complement C3 in Children with Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis

Zhang

Yang

et al. 2021

Nephron

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“…The estimated prevalence of children with ANCA-associated vasculitis is 3.41-4.28 per million children, with a higher female predominance. 3,4 Therefore, GPA was not suspected for this young patient. It was only when all symptoms were put together did a GPA diagnosis become clear.…”

Section: To the Editormentioning

confidence: 93%

“…In summary, the incidence of childhood-onset GPA is exceedingly rare, occurring in only 0.02 to 0.64 per 100,000 children per year, and is nearly undocumented in the T1DM population. 4 It is not clearly understood whether this patient had a propensity toward autoimmune diseases which increased her likelihood of developing GPA while also having T1DM or if this combination is unrelated and completely coinciden-tal. Her history of present illness, including the preceding months of various symptoms, led ultimately to a predominantly pulmonary picture of dyspnea, hypoxia, and hemoptysis on initial presentation without any renal involvement, unlike typical GPA.…”

Section: To the Editormentioning

confidence: 96%

“…3 Clinically, GPA tends to involve the upper respiratory tract, lower respiratory tract, and the renal system(s); however, limited forms of the disease may occur and be confined to a single organ. 4 GPA in childhood tends to be more severe compared to adult-onset with early, aggressive ear-nose-throat (ENT) involvement (subglottic stenosis, nasal septal perforation). A frequent presenting clinical manifestation of childhood GPA is pulmonary involvement, demonstrated by shortness of breath, chronic cough, or hemoptysis secondary to alveolar hemorrhage.…”

Section: To the Editormentioning

confidence: 99%

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Autoimmune Overload: An atypical presentation of granulomatosis with polyangiitis in an adolescent with type-1 diabetes mellitus

Wardinger

Sussman

Litwa

et al. 2021

Preprint

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We report a case of granulomatosis with polyangiitis (GPA) in a pediatric patient with a history of type 1 diabetes mellitus (T1DM) after a somewhat unusual presentation. Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides are a relatively rare entity in the general population, and are even less common amongst the pediatric population. Our patient’s chronic history of vague and systemic symptoms paired with social and environmental stressors contributed to her convoluted picture and late diagnosis. In addition, her T1DM adds an interesting element to her case, begging the question of whether her propensity for autoimmune conditions played a role in the development of this disease. With this case, we hope to increase clinician level of suspicion and promote early diagnosis and treatment for future pediatric patients.

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“…The estimated prevalence of children with AAV is 3.41-4.28 per million children, with a higher female predominance. 3,4 Therefore, GPA was not suspected for this young patient. It was only when all symptoms were put together did a GPA diagnosis become clear.…”

Section: Diagnosis Of Pediatric Granulomatosis With Polyangiitis (Gpa)mentioning

confidence: 93%

Autoimmune overload: An atypical presentation of granulomatosis with polyangiitis in an adolescent with type 1 diabetes mellitus

Wardinger

Sussman

Irish

et al. 2021

Pediatric Pulmonology

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To the Editor, We present the case of an 11-year-old female with a history of type 1 diabetes, hematochezia, and iron deficiency anemia who was admitted for hypoxia following an endoscopy and colonoscopy. Room air saturations were in the low eighties, and she was started on oxygen by nasal cannula. A chest radiograph (Figure 1A) demonstrated findings consistent with viral airway disease, and on a nasal swab, she was positive for rhino/enterovirus but negative for COVID.

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Epidemiology and clinical features of childhood-onset anti-neutrophil cytoplasmic antibody–associated vasculitis: a clinicopathological analysis

Cited by 19 publications

References 44 publications

Relationship between Renal Damage and Serum Complement C3 in Children with Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis

Relationship between Renal Damage and Serum Complement C3 in Children with Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis

Autoimmune Overload: An atypical presentation of granulomatosis with polyangiitis in an adolescent with type-1 diabetes mellitus

Autoimmune overload: An atypical presentation of granulomatosis with polyangiitis in an adolescent with type 1 diabetes mellitus

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