Clinic manifestations in granulomatosis with polyangiitis

Abstract

Granulomatosis with polyangiitis (GPA), formerly Wegener's granulomatosis (WG), is an uncommon immunologically mediated systemic small-vessel vasculitis that is pathologically characterised by an inflammatory reaction pattern (necrosis, granulomatous inflammation and vasculitis) that occurs in the upper and lower respiratory tracts and kidneys. Although the aetiology of GPA remains largely unknown, it is believed to be autoimmune in origin and triggered by environmental events on a background of genetic susceptibility.In Europe, the prevalence of GPA is five cases per 100,000 population, with greater incidence in Northern Europe. GPA can occur in all racial groups but predominantly affects Caucasians. Both sexes are affected equally. GPA affects a wide age range (age range, 8-99 years).Granulomatosis with polyangiitis is characterised by necrotising granulomatous lesions of the respiratory tract, vasculitis and glomerulonephritis. Classically, the acronym ELK is used to describe the clinical involvement of the ear, nose and throat (ENT); lungs; and kidneys. Because the upper respiratory tract is involved in 70-100% of cases of GPA, classic otorhinolaryngologic symptoms may be the first clinical manifestation of disease. The nasal cavity and the paranasal sinuses are the most common sites of involvement in the head and neck area (85-100%), whereas otological disease is found in approximately 35% (range, 19-61%) of cases.Diagnosis of GPA is achieved through clinical assessment, serological tests for anti-neutrophil cytoplasmic antibodies (ANCA) and histological analysis. The 10-year survival rate is estimated to be 40% when the kidneys are involved and 60-70% when there is no kidney involvement.The standard therapy for GPA is a combination of glucocorticoids and cyclophosphamide. In young patients, cyclophosphamide should be switched to azathioprine in the maintenance phase.A multidisciplinary approach, involving otorhinolaryngologists, oral and maxillofacial surgeons, oral physicians, rheumatologists, renal and respiratory physicians, and ophthalmologists, is necessary for the diagnosis and therapeutic treatment of GPA. ENT physicians have a determining role in recognising the early onset of the disease and starting an appropriate therapy.

Ear Nose and Throat (ENT) Manifestations in Granulomatosis with Polyangiitis in Patients from Saudi Arabia

“…Sininasal biopsies usually show nonspecific inflammation but could be the initial manifestation of GPA [16]. Para nasal sinus and nose biopsies are shown to be highly diagnostic of GPA [22,24]. In accordance with this sinonasal biopsies aided diagnosis in our GPA patients.…”

“…Overall, otologic manifestations are reported to constitute 36.4% of all cases while it was 13% in our GPA patients [21]. Otological manifestations namely otitis media and hearing loss can be the initial presentations of GPA [22][23][24]. It is reported that otitis media occurs in 25% of GPA patients and hearing loss in 6% while in our patients, otitis media was observed in 4.3% and hearing loss in 8.7% patients [22].…”

Difficulties in Recognizing Granulomatosis with Polyangiitis (GPA) in Elderly Patients Undergoing Diagnostic Thoracotomy Twice—A Report of Two Cases

“…It can occur at any age, although it is primarily a disease of older adults, with the peak age between 65 and 74 years old [2]. Because the upper respiratory tract is involved in 70-100% cases, classic otorhinolaryngologic symptoms -ear, nose and throat (ENT) manifestations -may be the first clinical presentation of the disease [3,4]. Some patients may progress to a more generalized form of disease, usually involving the lungs, skin and/or kidneys, with pauci-immune necrotizing crescentic glomerulonephritis [5].…”

Untypical Manifestations of Granulomatosis with Polyangiitis—A Review of the Literature

“…Granulomatosis with polyangiitis (GPA, previously known as Wegener's granulomatosis) affects small-sized to mediumsized vessels causing vasculitis [1,2]. Vessels of the upper or lower airways or whole respiratory tract and kidneys are involved with a prevalence of 90% [3] and 78% [4], respectively.…”

“…The disease affects all races. However, Caucasians are mostly affected [1]. An age-specific peak depends on a study.…”