Abstract
Background: Chronic and Atypical Sinusitis (AtS) is often the initial but frequently neglected presentation of Granulomatosis with Polyangiitis (GPA). These patients are primarily referred to otorhinolaryngology clinics and often receive prolonged antimicrobial medications before gradual development of other GPA symptoms. Objectives: This study was designed to find the number of patients with sinusitis fulfilling the ACR criteria for diagnosis of GPA (Wegener’s). Methods: This cross-sectional study recruited 23 patients with AtS and 32 patients with Typical Sinusitis (TyS). For every patient a through physical examination focusing primarily on the head and neck region, chest X-ray and sinus roentgenograms in Waters and Caldwell views, Complete Blood Count, ESR, BUN, Creatinine, CRP, c-ANCA, p-ANCA and Urinalysis were done. Histopathologic studies were performed on sinus biopsies in AtS patients. Results: Chronicity, recurrence and sinus masses are three major factors to make a sinusitis atypical. The most frequent complaint was purulent/bloody discharge from the nose found in 15 (65%) of patients with AtS. The most frequent signs and symptoms in TyS group were posterior nasal drainage and production of phlegm. Radiographic studies found 5 bony masses in the AtS group. One patient with AtS was found to have pansinusitis and a disappeared nasal septum. Chest radiographs of patients with TyS did not show any pathologic finding but in two patients with AtS ring shadows in the base of both lungs and calcified granulomas were detected respectively. Histopathologic study in two patients with AtS showed chronic inflammatory cells, granulomas and necrosis highly suggestive for GPA. In AtS group, ESR was elevated in three female and two male patients. c-ANCA was found to be very high in a male patient with elevated ESR, BUN, creatinine and CBC impairment. Urinalysis showed microhematuria in one patient and the same male patient mentioned above had RBC casts. No patient in TyS group met the ACR criteria of GPA but in the AtS group, three (13%) patients (two female and one male) were found to have GPA according to ACR criteria and yet another three (13%) were highly suggestive to have GPA but did not fulfill the ACR criteria. Conclusion: Early identification of this high number of patients with GPA, in a limited number of otorhinolaryngological patients confers the advantages of screening and early treatment. Rheumatologic consultation in the treatment of AtS patients is therefore inevitable.
Highlights
Wegener’s Granulomatosis (WG) was first described by a German medical student in 1931
Early identification of this high number of patients with Granulomatosis with Polyangiitis (GPA), in a limited number of otorhinolaryngological patients confers the advantages of screening and early treatment
ESR was elevated in three female and two male Atypical Sinusitis (AtS) patients
Summary
Wegener’s Granulomatosis (WG) was first described by a German medical student in 1931. Five years later Wegener described the same clinical presentation in three other patients [1]. The exact pathogenesis of WG is still unknown but it is an unusual form of vasculitis which almost always affects the sinopulmonary structures [2]. A triad of necrotizing granulomas of the upper and lower respiratory tract, glomerulitis and vasculitis constitute the systemic Wegener’s. Chronic and Atypical Sinusitis (AtS) is often the initial but frequently neglected presentation of Granulomatosis with Polyangiitis (GPA). These patients are primarily referred to otorhinolaryngology clinics and often receive prolonged antimicrobial medications before gradual development of other GPA symptoms
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