Granulomatosis With Polyangiitis and Continuous Positive Airway Pressure—The Challenge of Interface Between Nose and Mask
The mortality of systemic vasculitis has changed in recent years after the appearance of new immune therapies. Because of increased survival, there are new concerns with these patients, such as quality of life and long-term complications of the disease and the treatment. Respiratory sleep disorders are a common cause of increased morbidity in this population, and the correct therapeutic approach leads to an improved quality and quantity of life. Patients with granulomatosis with polyangiitis (GPA) are especially complex. Craniofacial deformities can make the treatment of obstructive sleep apnea (OSA) more difficult with positive airway pressure devices. In the present study, we report a patient with comorbid diagnosis of GPA and OSA, emphasizing the challenge of interface between the patient and mask.
Granulomatosis with polyangiitis is a necrotizing granulomatous inflammation in the respiratory tract and glomerulonephritis. The mean survival time of untreated generalized GPA is 5 months because of renal or lung failure. After treatment, the 5-year survival rate ranges from 74% to 79%.1 However, the better outcome drives several complications after chronic inflammation. The nose and paranasal sinuses are involved in 80%. Clinical presentation can vary from mild obstruction to total nasal collapse. Nasal signs and symptoms include mucosal edema and consequent obstruction and epistaxis. Chronic complications such as nose deformity and septal perforation are prevalent.2
Rheumatology patients have increased risk of OSA due to aging, higher body mass index, upper neck circumference, and rheumatic disabilities.3 The overlap of OSA in rheumatic patients influences the severity of pain and fatigue, as well as increases the levels of inflammation. Continuous positive airway pressure (CPAP) is the best choice to treat patients with severe OSA. However, the treatment with CPAP in GPA patients can be a challenge because of anatomical abnormalities of the airway secondary to the disease. This study describes a case of GPA associated with OSA and the consequent difficulties of the treatment with CPAP.