Granulomatosis with polyangiitis (Wegener's granulomatosis): An analysis of 59 patients

Abstract

Granulomatosis with polyangiitis (Wegener's granulomatosis) is a type of vasculitis which can be categorized as a subbranch of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides. This study examined the demographic, clinical, radiographic and laboratory characteristics of patients with granulomatosis with polyangiitis (GPA) in Imam Reza Hospital, Mahshad, Iran. The medical records of patients admitted to the Department and Clinic of Rheumatology who were diagnosed with granulomatosis with polyangiitis between January 1, 2006 and December 31, 2016 were retrospectively studied. Patients were classified according to the American College of Rheumatology (ACR) 1990 classification criteria for Wegener's granulomatosis. A total of 59 GPA-diagnosed patients (55.9% male, 44.1% female) were identified. The male-tofemale ratio of the patients was 1.32:1. The mean age was 35.58±15.09 years, with the youngest and oldest patients being 10 and 71 years old, respectively. The mean disease duration was 47.5±32.88 months. The most common involved organs were the upper respiratory tract in 77.9%, lower respiratory tract in 62.7%, kidney in 59.3%, joint/muscle in 49.15%, and eye in 28.8% of patients. Four (6.7%) patients died during the follow-up which had a mean of 3.9 years. In the studied region, GPA inflicts the younger population, and it is more common in males. Instances of upper respiratory tract, lower respiratory tract, and kidney involvement are less common in the present study than in most other studies. GPA might have less severe manifestations in the studied area than in other regions.

Submandibular salivary gland involvement in granulomatosis with polyangiitis

“…GPA commonly affects adult males [13], as in the present case. Approximately 90% of patients with active, generalized disease have positive C-ANCA [14].…”

“…The absence of renal affection does not preclude GPA. Renal involvement in GPA ranges from 85 to 59% in different studies [12,13]. Patients with limited disease can present without renal affection.…”

Granulomatosis Wegener dengan <i>Antineutrophil Cytoplasmic Antibodies</i> (ANCA) Negatif pada Laki-laki Usia 38 Tahun

“…Terapi granulomatosis Wegener terdiri atas 2 fase yaitu fase awal selama 3-6 bulan untuk mencapai remisi dan fase pemeliharaan selama 12-24 bulan untuk mencegah terjadinya kekambuhan. 7 Terapi standar baku emas untuk granulomatosis Wegener adalah kombinasi corticosteroid dan immunosuppressant, namun dapat juga diberikan salah satunya. 2 Pemberian cyclophosphamide dan corticosteroid merupakan terapi yang efektif untuk granulomatosis Wegener aktif.…”