Granulomatosis with polyangiitis also known as wegener's Granulomatosis is a granulomatous disease which involves multiple organs as upper and lower respiratory tract, nasal, sinuses, kidneys, parotids are few of them. It is of two types -limited and systemic. Limited type is difficult to diagnose and commonly involves ENT organs as nose, ear and sinuses. ENT involvement as the presentation as the first symptom of the disease ranges from 63% and 72% of cases of patients. This review will thus focus on the sinonasal involvement in granulomatosis with polyangiitis.Hematologic abnormalities in GPA includes a leukocytosis, thrombocytosis (>400,000/mm3), and elevation of the erythrocyte sedimentation rate [17]. The plasma creatinine and urinalysis are normal in sinonasal GPA.Generally, in patients with sinonasal GPA involvement have normal ANCAs levels in the serum.
Imaging
Sinus computed tomographyMulti planar sinus CT scan is the preferred imaging modality for evaluating chronic rhinosinusitis and suspected rhino sinus involvement by WG [18,19]. Common findings on sinus CT are mucosal thickening in the nasal cavity and paranasal sinuses, bony destruction of the nasal cavity and paranasal sinuses, and bony thickening of the paranasal sinuses.
Nasal or sinus biopsyNasal and sinus biopsies are performed for evaluation and treatment of nasal obstruction due to soft tissue masses and chronic sinusitis [14,20]. Special stains and culture should also be sent for ruling out infections as fungal, mycobacterial. Histopathological infection should also be done.Patients with limited or sinonasal GPA, would not fulfill the American college of Rheumatology criteria 1990 classification criteria for GPA. So a high suspicion and regular follow up should be done to look for other organ involvement (Table 1)
TreatmentIn general, limited or sinonasal manifestations of patients with GPA usually respond well to immunosuppressive therapy (methotrexate or cyclophosphamide) along with tapering glucocorticoids. Maintenance therapy with methotrexate and azathioprine is useful. Frequent boluses of glucocorticoids to control reactivations can be used when required. Refractory disease may be treated with rituximab -anti CD20 monoclonal antibody [22].Surgical treatment does not change the course of the disease. It can ameliorate the consequences of tissue destruction in the head and neck region. Surgery should be done when the disease is under remission.
ConclusionPatients with GPA often present sinonasal manifestations. It is important to perform a systematic and ENT exploration of patients with suspected or confirmed diagnosis of GPA in order to contribute to an early diagnosis of these manifestations so as to prevent secondary complications which would worsen the quality of life of these patients.