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Granulomatosis With Polyangiitis and Continuous Positive Airway Pressure—The Challenge of Interface Between Nose and Mask To the Editor: T he mortality of systemic vasculitis has changed in recent years after the appear- ance 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 disor- ders are a common cause of increased mor- bidity 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 deformi- ties can make the treatment of obstructive sleep apnea (OSA) more difficult with pos- itive airway pressure devices. In the present study, we report a patient with comorbid di- agnosis 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 glomerulone- phritis. 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 inflam- mation. 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 rheu- matic 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. CASE REPORT A 39-year-old male patient had acute recur- rent rhinosinusitis for 4 years with conse- quent collapse of the nasal bridge. After 2 years, the patient developed several com- plications, such as blindness of the right eye, hearing loss in the right ear, abnormal eye movements, permanent hypoesthesia in his right side of the face, and pulmonary involvement with hemoptysis. The patient signed a consent form and authorized the use of photographic records. A biopsy performed during surgical treatment of chronic sinus disease confirmed GPA. Our patient had an important nasal deformity, as demonstrated in Figure 1. There was destruction of the medial wall of the maxillary sinus, anterior sphenoid sinus, anterior and posterior ethmoid cells, and papyraceous blades. The patient was initially immunosup- pressed with higher doses of corticosteroids, with occasional pulse therapy during clinical exacerbations. After introduction of metho- trexate and azathioprine, there was a gradual reduction of corticosteroid doses. After 12 months of immunosuppression, the pa- tient had a weight gain of 20 kg, and his wife described loud and continuous snoring with prolonged OSA episodes. Polysomnography has shown an apnea-hypopnea index of 16.6 events per hour, a moderate oxyhe- moglobin desaturation, and an important reduction of sleep efficiency. The second polysomnography with CPAP titration re- vealed resolution of respiratory events with 8-cm H 2 O pressure in use of oronasal mask (Simplus, Fisher and Paykel Healthcare Ltd, Auckland, New Zealand; size M) (Fig. 2). Continuous positive airway pressure treatment with 8 cm H 2 O and oronasal mask (Simplus size M) was carefully initi- ated because of fragile nasal walls and risk of barotrauma. After a month of therapy, the patient had no complaints about the use of the device and with a good improve- ment of the daytime sleepiness. Data of CPAP machine revealed a successful apnea- hypopnea control (0.8 event per hour), mean use of 5:28 hours per night, and a leak rate of 18 L/min. DISCUSSION Prevalence of nasal deformities with func- tional repercussion is frequent in patients with GPA. The continuous treatment of GPAwith corticosteroids increases the body mass index FIGURE 1. Nasal deformities caused by GPA. Color online-figure is available at http://www.jclinrheum.com. Letters to the Editor JCR: Journal of Clinical Rheumatology • Volume 24, Number 2, March 2018 102 www.jclinrheum.com © 2018 Wolters Kluwer Health, Inc. All rights reserved. Copyright © 2018 Wolters Kluwer Health, Inc. 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