Abstract
Immunological mechanisms of appearance and therapeutic treatment strategies were discussed on example of the rare granulomatosis with polyangiitis clinical case in young patient. This vasculitis, formerly known as Wegener’s granulomatosis, is a rare multisystem autoimmune disease with necrotizing granulomatous inflammation and pauci-immune vasculitis in small- and medium-sized blood vessels. Autoimmune diseases affect 5 to 7% of people, are commoner in women of childbearing age, and are frequently encountered in pregnancy. They may remit or improve during pregnancy, butcan flare or present in pregnancy with disastrous consequences. Otorhinolaryngologist is the first physician to contact for the majority of patients with GPA. This diagnosis must always be taken into consideration in patients with recurrent upper respiratory tract infections, otitis, mucosal ulcers and laryngitis. Proper and early diagnosis is crucial for imminent therapy implementation and allows avoiding irreversible organ damage.
Highlights
Granulomatosis with polyangiitis, formerly known as Wegener’s granulomatosis, is Antineutrophil Cytoplasmic Autoantibody (ANCA)associated necrotizing vasculitis, with pathophysiological basis consisted from three components: necrotizing granulomatous inflamemation, granulomatous vasculitis of small and medium-sized vessels, including arteries, arterioles, capillaries and venules; and kidney disease
Because biopsy based diagnosis required for granulomatosis with polyangiitis (GPA) remains controversial, tissue diagnosis is recommended if a biopsy site is available, provided that the patient understands the risks of the procedure due to many of severe side-effects of specific for vasculitis therapy
During last hospitalization in Kharkiv region hospital in December 2016 several councils of physicians were made with differential diagnostics between antineutrophylic cytoplasmic antibodies (ANCA) associated vasculitis, Goodpasture syndrome and sepsis, but these diagnoses weren’t confirmed despite ANCA positive analysis (1,05 U/l (N-till 1U/l))
Summary
Granulomatosis with polyangiitis, formerly known as Wegener’s granulomatosis, is Antineutrophil Cytoplasmic Autoantibody (ANCA)associated necrotizing vasculitis, with pathophysiological basis consisted from three components: necrotizing granulomatous inflamemation (most commonly in the upper or lower respiratory tract), granulomatous vasculitis of small and medium-sized vessels, including arteries, arterioles, capillaries and venules; and kidney disease The earliest complaints, in over 90 % of patients with GPA, which are the most common reasons for seeking medical attention, are usually related to upper respiratory tract problems including sinus pain, purulent nasal discharge, epistaxis, nasal ulceration, and serous otitis media. Renal involvement is clinically evident in only 11–20 % of cases at presentation, glomerulonephritis eventually develops in 77–85 % of patients, usually within the first two years of disease onset [4]. The diagnosis of GPA has been made following the criteria of granulomatous involvement of upper and lowers respiratory tract, glomerulonephritis and varying degrees of systemic vasculitis. Fauci and colleagues [5] created effective definitive diagnostic criteria for GPA included clinical evidence of disease in at least two of three areas (upper airways, lung and kidney), and results that show disease in at least one and preferably two of these organ systems. CANCA directed against PR3 is most specific for GPA [6]
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