DIAGNOSIS AND MANAGEMENT OF PATIENTS WITH GRANULOMATOSIS WITH POLYANGIITIS

Granulomatosis with polyangiitis (GPA), previously known as Wegener’s granulomatosis, is a rare form of systemic vasculitis of unknown etiology, commonly associated with serum cANCA. Typical manifestations include necrotizing vasculitis and granulomatous inflammation which occur in the upper and lower respiratory tracts and kidneys, however the range of clinical manifestations of GPA can involve almost any organ. Epidemiological studies suggested that prevalence of GPA in Poland is 36/1,000,000 and is slightly lower than in other European countries. A statistically significant decrease in the GPA incidence was found within 2011–2015. Both sexes are affected equally, and a mean age at diagnosis is 40 yrs. Clinical manifestations are heterogeneous and the following presentation of the disease should be taken into consideration: cutaneous (leucocytoclastic vasculitis, purpura, digital infarcts and gangrene), oral (ulcers, granulomatous lesions, gingival hyperplasia with strawberry-like aspect), ocular (episcleritis, scleritis, conjunctivitis, keratitis, uveitis, retinal vasculitis or thrombosis, blindness, proptosis and orbital granulomatous masses), auricular (sensorineural hearing loss and conductive hearing loss), cardiovascular (small vessel vasculitis, occlusive vascular disease, pericarditis, cardiomyopathy, valvular heart disease, ischemic heart disease, heart failure), gastrointestinal (acute abdomen secondary to peritonitis or bowel ischemia), neurological (meningitis, seizures, cerebrovascular accidents, spinal cord lesions, cranial nerve palsies, sensory or motor peripheral neuropathy, mononeuritis multiplex, cerebral mass lesions) as well as musculoskeletal involvements. Therapy should be adjusted to individual patient. Various programs have been proposed for the induction of remission and its maintenance, and management of relapses. Glucocorticoids and cyclophosphamide are the most commonly used medication although rituximab is particularly useful in patients with refractory or relapsing disease, women of childbearing potential, and patients previously treated with cyclophosphamide as well as is more effective than cyclophosphamide for treating relapses. Azathioprine is also used for remission maintenance. Plasma exchange is indicated in patients with alveolar hemorrhage.