Granulomatosis with polyangiitis (GPA) – formerly called Wegener’s granulomatosis – (thesaurus synonyms: Allergic angiitis and granulomatosis; Glomerular disease in Wegener’s granulomatosis; Glomerular disorder in Wegener’s granulomatosis; Glomerulonephritis in Wegener’s granulomatosis; Granuloma gangraenescens; granulomatous polyangiitis; granulomatosis Wegener; Klinger-Wegener-Churg syndrome; pulmonary granulomatosis; McBride-Stewart syndrome [granuloma gangraenescens]; Wegener’s disease; necrotizing respiratory granulomatosis; rhinogenic granulomatosis; giant cell granuloarteritis; giant cell granuloarteritis Wegener-Klinger-Churg; Wegener granulomatosis; Wegener-Klinger-Churg syndrome; Wegener-Klinger-Churg syndrome with pulmonary involvement; Wegener disease; Wegener disease with pulmonary involvement; Wegener granulomatosis (or in other order Wegener-Klinger-Churg syndrome); Wegener syndrome; ICD-10-GM M31. 3: Wegener’s granulomatosis) refers to a necrotizing (tissue dying) vasculitis (vascular inflammation) of the small to medium-sized vessels (small vessel vasculitides), which is associated with granuloma formation (nodule formation) in the upper respiratory tract (nose, sinuses, middle ear, oropharynx) as well as the lower respiratory tract (lungs). The inflammation of the blood vessels is triggered immunoreactively.
Granulomatosis with polyangiitis belongs to the group of ANCA-associated vasculitides (AAV). ANCA stands for anti-neutrophil cytoplasmic antibodies. ANCA-associated vasculitides are systemic diseases, i.e. they can affect almost all organ systems. In the context of granulomatosis with polyangiitis, renal involvement such as glomerulonephritis (inflammation of the glomeruli (renal corpuscles)) or microaneurysms (bulges in the vascular wall of the capillaries) is present in about 80% of cases.
Sex ratio: males and females are equally affected.
Frequency peak: granulomatosis with polyangiitis can occur at any age.
The prevalence of granulomatosis with polyangiitis is approximately 5 cases per 100,000 persons.
The incidence (frequency of new cases) of granulomatosis with polyangiitis is approximately 0.9 cases per 100,000 people per year.
Course and prognosis: Due to the use of immunosuppressive therapy, the life expectancy of those affected has improved significantly in recent years.Recurrences are frequent, so patients should be monitored closely. Risk factors for recurrence include PR3-ANCA leading to a doubling of the recurrence rate, early cessation of glucocorticoid therapy, and lower total cyclophosphamide dose/duration of therapy. Patients with good renal function are also more at risk for recurrence. Infections can trigger relapse (recurrence of disease). For example, the bacterium Staphylococcus aureus increases the recurrence rate in ENT.
The 5-year survival rate for granulomatosis with polyangiitis without adequate therapy is a few months (< 6 months). With adequate therapy, it is > 85%. If organ damage (especially renal damage) occurs during the course of the disease, the prognosis worsens. The same is true for infections, which can develop more easily with immunosuppressive therapy.
