Difficulties in diagnosis of granulomatosis with polyangiitis in a child with concomitant pulmonary aspergillosis

Granulomatosis with polyangiitis, formerly known as Wegener's granulomatosis, is an autoimmune antineutrophil cytoplasmic antibodies – associated systemic vasculitis with a progressive course.
Granulomatosis with polyangiitis remains one of the most severe and prognostically unfavorable systemic vasculitides, the frequency of which is increasing among children. This article provides a review of contemporary literature on granulomatosis with polyangiitis, covering issues of etiology, pathogenesis, clinical manifestations, and diagnosis, which currently requires updating and systematization to avoid medical errors. The article also presents a case of generalized granulomatosis with polyangiitis in a 12-year-old child with involvement of the upper respiratory tract, lungs, and kidneys, reflecting the diagnostic challenges of granulomatosis in the presence of concomitant pathology. The clinical example discusses the importance of early comprehensive assessment of organ and system conditions for prescribing adequate timely therapy to prevent irreversible complications in granulomatosis with polyangiitis.

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