CASE REPORT: ANCA-ASSOCIATED VASCULITIS (WEGENER'S GRANULOMATOSIS) WITH MULTI-SYSTEM INVOLVEMENT

Abstract

Introduction. Granulomatosis with polyangiitis (GPA), formerly known as Wegener’s granulomatosis, is a rare disease of unknown etiology. It is characterized by necrotizing granulomatous inflammation and autoimmune-mediated vasculitis that primarily affects small- and medium-sized vessels, often with multiorgan involvement. The disease may present with diverse and nonspecific symptoms both at onset and during its full clinical manifestation, which complicates diagnosis, delays treatment, and impacts prognosis. The following clinical case illustrates the multisystem involvement of GPA, affecting the auditory, visual, respiratory, and urinary systems. Aim. To present a clinical case of GPA with multisystem involvement, highlighting the challenges of diagnosis and the effectiveness of treatment. Results. This article describes the case of a 35-year-old woman who presented with complaints of generalized body pain, lower back pain, myalgia, fatigue, hair loss, visual impairment, hearing loss, nasal crusting with bloody discharge, shortness of breath, exertional dyspnea, palpitations, unintentional weight loss of 5-6 kg, frequent urination, and severe headaches. After one year of outpatient observation, a diagnosis of ANCA-associated vasculitis was established based on clinical, biochemical, immunological, imaging, and histological findings. The diagnosis included upper respiratory tract involvement (sinusitis, otitis), ocular manifestations (uveitis), joint and skin involvement, positive anti-MPO titers, and renal pathology characterized by chronic nephritic syndrome and chronic kidney disease (CKD) stage G1A3 (eGFR 90 mL/min) with episodes of acute kidney injury during disease flares. This case highlights the diagnostic challenges of GPA due to its wide spectrum of clinical manifestations. Current treatment strategies are based on the latest European League Against Rheumatism (EULAR) and American College of Rheumatology (ACR) recommendations, in which rituximab (RTM) - based biologic therapy is the first-line treatment for severe GPA. Despite receiving treatment with glucocorticoids (GCs), the immunosuppressant mycophenolate mofetil (MMF), and RTM, the patient remains at high risk for relapse and requires long-term monitoring and therapeutic adjustment. Conclusions. Early diagnosis and timely initiation of immunosuppressive and biologic therapy are key factors in the successful management of GPA. This case underscores the importance of a multidisciplinary approach and continuous follow-up to prevent disease progression and complications in patients with GPA.