Treatment issues in Granulomatosis with polyangiitis
- • Nasal irrigations to prevent sinus infections: regular use of saline rinses helps with symptoms of sinus congestion, nasal crusting, and may reduce the risk of infection. Some clinicians will recommend additives to the saline solution including:
- – 2% mupirocin (Bactroban) in 1 L of saline. This can be prepared by compounding pharmacies or patients can place a 0.5 to 1-inch strip of mupirocin into saline solution (the mupirocin does not dissolve easily, so it is recommended to mix it in warm saline and shake vigorously). Keep refrigerated after use. Irrigate sinuses with nasosinus lavage until clear return of fluid. Apply 2% mupirocin (Bactroban) ointment with Q-tip half way into nasal vestibule between irrigations to diminish the carriage of S. Aureus .
- – Baby shampoo (acts as a surfactant): a small amount (half tsp) can be added; loss of smell has been reported but is uncommon.
- – Steroids (budesonide 0.25 to 5-mg respule in rinse solution twice daily); risk of local tissue atrophy, hence should be directed by an ENT specialist.
- • Therapy to prevent osteoporosis should also be instituted according to guidelines.
- • Cardiovascular risk factors (high blood pressure, diabetes, and lipids) should be screened for and treated as the incidence of ischemic cardiovascular disease is two-fold higher in GPA.
- • Malignancy screening is recommended for patients who have received cyclophosphamide. Rates of malignancy (skin and bladder cancers and acute myeloid leukemia) are increased, particularly in patients receiving a total dose of >36 g.
- • Patients who receive multiple courses of rituximab may be at risk of hypogammaglobulinemia. Patients with IgG levels <300 mg/dL (or <500 mg/dL with infections) may be considered for replacement doses of IV IgG (0.4 g/kg monthly), as data in patients receiving rituximab for lymphoma suggest decreased risk of subsequent infections with this strategy.
- • Ovarian and sperm protection should be discussed if patient is to receive cyclophosphamide. The risk of ovarian failure is up to 50% in patients aged 20 to 30 years who receive a total dose of >20 g, among patients aged 30 to 40 years who receive a total dose of >10 g, and in those aged >40 years who receive a total dose of 5 g. Leuprolide (3.75 g monthly) has been used for ovarian protection. The risk of azoospermia occurs with a total dose of 6 to 10 g. Sperm banking is the only reliable method to assure future fertility. Some centers give testosterone 200 mg intramuscularly every 2 weeks while on cyclophosphamide therapy in an attempt to preserve testicular function.
