RESUMEN
BACKGROUND: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis and glomerulonephritis is uncommon in children. We sought to characterize the histological and clinical features of the disease and report on risk factors for adverse outcomes in a pediatric cohort. METHODS: Retrospective single-center cohort of all pediatric (< 20 years) patients diagnosed with ANCA-associated glomerulonephritis (AAGN) by kidney biopsy between 2002 and 2022 at Johns Hopkins University. Histological and clinical features were extracted from the medical record. Clinical, laboratory, and histological findings were analyzed to determine the association with kidney failure (KF) and/or death. RESULTS: A total of 17 patients were identified (GPA n = 7, MPA = 10) with a median age of 15 years (IQR 12-17) at presentation, a slight female predominance (59%), with seven patients reaching the composite outcome of death (n = 1) or kidney failure (n = 6). There was no difference in presenting clinical symptoms or extra-renal manifestations between the two groups. Univariable Cox regression identified several factors associated with an increased hazard of endpoint including the degree of global or segmental sclerosis, interstitial fibrosis and tubular atrophy (IFTA), C3 and C1q staining, presence of subendothelial deposits, and proteinuria. Multivariable regression was not performed due to the small sample size. We saw a trend towards increased utilization of plasma exchange and a decrease in cyclophosphamide utilization in the more recent era. There was no association between treatment modality and outcome. CONCLUSIONS: Pediatric AAGN is a rare disease associated with significant morbidity. We identified glomerulosclerosis and IFTA on histology, and proteinuria on initial presentation as risk factors for KF/death.
RESUMEN
The coronavirus disease 2019 has been demonstrated to be a trigger for multiple immune-mediated diseases, such as antineutrophil cytoplasmic antibody-associated vasculitis. Associated vasculitis consists of rare autoimmune disorders that predominantly affect small vessels, leading to endothelial injury and tissue damage. We present a case of a newly diagnosed microscopic polyangiitis temporally associated with coronavirus disease 2019 infection in a previously healthy woman and a literature review. A 66-year-old female presented to the Emergency Room with fever, edema on her legs, productive cough, dyspnea, and hemoptysis. A chest computerized tomography scan revealed bilateral diffuse alveolar opacities with the appearance of diffuse alveolar hemorrhage. Blood analysis revealed a moderate normocytic, normochromic anemia with a hemoglobin of 6.6 g/dL, platelet count of 347 k/dL, leucocytes of 12,000/dL, a creatinine of 3.91 mg/dL (basal Cr: 0.9 mg/dL), and a Blood Urine Nnitrogen of 78 mg/dL. A urine sediment demonstrated glomerular hematuria, with mixed shapes of red blood cells. She was admitted to the intensive care unit and a bedside bronchoscopy revealed progressive bleeding with a bronchioalveolar lavage positive for diffuse alveolar hemorrhage. Given the critical involvement of the lungs and kidney function, the diagnostic approach revealed a positive p-anti-neutrophil cytoplasmic antibody on immunofluorescence and an anti-MPO (myeloperoxidase) level of 124.6 IU/mL. A renal biopsy demonstrated pauciimmune focal and segmental glomerulosclerosis. A diagnosis of microscopic polyangiitis triggered by severe acute respiratory syndrome coronavirus 2 infection was made, and immediate treatment with pulse-dose steroids and cyclophosphamide was initiated. The patient needed renal replacement therapy and was discharged for follow-up with nephrology and rheumatology services. The diagnostic approach of associated vasculitis can be more challenging in the coronavirus disease era. Atypical features in the pulmonary imaging and a rapid deterioration of the renal function should arise the clinical suspicion of the presence of an added condition to the coronavirus disease infection. Autoimmune conditions such as associated vasculitis should be evaluated even in the absence of previous autoimmune history. Prompt diagnosis and treatments must be prioritized to avoid end-organ definite damage. Further, larger and more collaborative studies are needed to confirm the potential role of coronavirus disease 2019 as a trigger of associated vasculitis.