Diagnostic and treatment features of granulomatosis with polyangiitis in children: comparison of two clinical cases

Antineutrophil cytoplasmic antibody-associated systemic vasculitides (AAV) are a heterogeneous group of multisystem diseases characterized by inflammation and necrosis of the vessel wall of small- and medium-sized vessels. The international community subdivides them into three nosological forms: granulomatosis with polyangiitis (GPA), microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis. The epidemiology of this disease in children has been insufficiently studied. In the Russian Federation, there is no unified registry of patients with this diagnosis; in foreign studies, only a very limited view of the problem is presented due to small sample sizes and difficulties in defining inclusion criteria for the study group. Early diagnosis remains a challenging clinical task.

The article describes two clinical cases of GPA in children and presents methodological approaches to disease diagnosis and treatment. A comparative analysis of GPA development in 17- and 10-year-old female patients was performed. Diagnostic methods for GPA in the absence of ANCA and the need to implement lung biopsy as the “gold standard” in ANCA-negative status are discussed. 

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