Drugs

What drugs have been reported to cause Eosinophilic Granulomatosis with Polyangiitis? The cysteinyl leukotriene type I receptor antagonists, zafirlukast (Accolate), montelukast (Singulair), and pranlukast, have been associated with EGPA. Whether there is a direct cause is controversial. Some clinicians believe that EGPA is unmasked when the patient uses these drugs and subsequently tapers their GCs. …

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Can Granulomatosis with polyangiitis patients who go into renal failure receive a kidney transplant? Yes. However, GPA should be under control and ideally the ANCA titer low or absent before transplant. The use of mycophenolate and cyclosporine post-transplant to prevent rejection should help prevent recurrence of GPA.

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 …

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What other immunosuppressive options are available in treatment resistant cases in Granulomatosis with polyangiitis? • Plasmapheresis or plasma exchange (PLEX) may be considered in life-threatening disease (rapidly progressive glomerulonephritis [RPGN] and DAH), although this is a controversial area. Data from the MEPEX trial has documented short-term improvement in the number of patients progressing to end-stage renal disease following PLEX, …

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Are prophylactic antibiotics appropriate during immunosuppressive treatment? Oral trimethoprim/sulfamethoxazole (one double-strength tablet thrice a week) provides prophylaxis against P. jiroveci in patients with vasculitis who are receiving high-dose (>15–20 mg/day prednisone) glucocorticoid (GC) therapy. This antibiotic therapy also limits recurrent sinus infections, which can cause exacerbations of GPA. Patients allergic to sulfa can receive dapsone, atovaquone, or …

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How does the therapy for HBV associated with PAN differ from that for PAN not associated with HBV? In countries with a comprehensive hepatitis B vaccination program, HBV-associated PAN now accounts for <10% cases of PAN. PAN usually occurs within 4 to 6 months of acquisition of hepatitis B. HBV-associated PAN has more orchitis, hypertension, …

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How is Polyarteritis nodosa treated? Decisions regarding the initial management of PAN without HBV infection depend on disease severity and organs involved (multiorgan versus single organ). For mild disease (constitutional symptoms, arthritis, anemia, skin lesions, normal glomerular filtration rate without cardiac, gastrointestinal, or neurologic involvement), initial treatment with prednisone 1 mg/kg per day for 4 …

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