Wegener's granulomatosis: a review of clinical features and an update in diagnosis and treatment

Abstract

Wegener's granulomatosis (WG) is an idiopathic, systemic inflammatory disease characterized by necrotizing granulomatous inflammation and pauci-immune small-vessel vasculitis of upper and lower respiratory tract and kidneys. The condition affects both genders equally, although some inconsistent gender differences have been observed. The aetiology of WG remains unknown although a number of exogenous factors have been suggested to be of aetiological relevance. Most clinical characteristics of this disease are non-specific, making clinical diagnosis challenging. Histopathological examination of lesional and peritoneal tissue is not pathognomonic, but is an essential investigation to confirm the presence of disease and exclude other disorders. At present, despite the increasingly wide range of potential therapies, cyclophosphamide plus corticosteroids remain the most recognized and effective means of inducing and sustaining remission of WG.

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

“…GPA is more likely to affect adults; a small number of young adults and children may be affected. The mean age of patients at diagnosis is between 40 and 55 years [16]. The mean age of patients in this study was lower in comparison with most other studies (35.58±15.09 years).…”

Orbital inflammation: Corticosteroids first

“…1,21,36 Hence, in the vast majority of cases, a better-defined diagnosis would not allow for a different or more targeted initial treatment. Furthermore, the cost of the more targeted therapeutic agents is thousands of times more than that of oral corticosteroid, and insurance providers will often not cover this cost unless corticosteroid therapy failure or contraindication can be proven.…”

“…36 Patients with granulomatosis with polyangiitis may have sinus, pulmonary, or renal symptoms. 1 Patients with histiocytosis may have asthma or yellow discoloration of the affected areas. 22 General physical examination may demonstrate diagnostically helpful findings, including enlarged lymph nodes, skin nodules, thyroid enlargement or tenderness, wheezing, heart murmurs, abdominal masses or enlargement of abdominal organs.…”

Wegener Granulomatosis: Case Report of Facial Prosthetic Rehabilitation

“…These sinus and ear changes are the most frequent. Strawberry gingivitis [1,6,9] in the oral cavity may arise as a rare sign, yet known as a pathognomonic sign of the disease. Gingival hyperplasia, described as erythematous gingivitis, presents widespread pain and easily bleeds when touched.…”

“…The most common signs and symptoms are fatigue, loss of appetite, weight loss, short breath, fever, bloody sputum through the upper airways, joint pain, sinusitis, skin nodules and ulcers, eyes and middle ear inflammation, as well as neuropathy [5]. The nonspecific manifestations may cause the diagnosis of this disease to be hard and particularly complex, thereby delaying the appropriate treatment determination [6]. As a consequence, sequel arises due to tissue degeneration of the affected area.…”