Abstract

Objective: To characterize major infectious complications and analyze potential risk factors in Wegener’s granulomatosis (WG) patients.

Methods: Data from 113 WG patients (69 men) followed at least once between January 1984 and March 2006 in our internal medicine department were analyzed retrospectively.

Results: Thirty five patients (mean age at WG diagnosis: 50.2 (±13.05) years) developed 53 major infections. Infections were: bronchopneumonias (n = 19), herpes zoster recurrences (n = 9), cellulitis (n = 4), prostatitis (n = 4), spondylodiscitis and septic arthritis (n = 3), digestive tract infections (n = 2), Enterococcus faecalis or Staphylococcus aureus septicemia (n = 2), viral hepatitis B reactivations (n = 2), post-transfusion HIV infection with fatal cerebral toxoplasmosis, esophageal candidiasis, disseminated herpes simplex and cytomegalovirus infection, cytomegalovirus retinitis, herpetic keratitis, herpetic stomatitis, Serratia sp. node suppuration and fever resolving under broad spectrum antibiotics (n = 1 each). Half of the major infectious episodes occurred within the 3 years after WG diagnosis. Eight (7%) patients died, with two (2%) infection-related deaths. Patients diagnosed with WG before 1996 had a significantly higher rate of infections than those diagnosed later (48% vs. 24%, p = 0.02). Cyclophosphamide and corticosteroids were independently associated with significantly higher risk of major infection (p<0.05 and <0.001 respectively). All patients treated since 1993 received anti-pneumocystosis prophylaxis.

Conclusion: Cyclophosphamide and corticosteroids were associated with higher risk of infection. Despite systematic cotrimoxazole prophylaxis, major infections, mostly bronchopneumonias and herpes zoster recurrences, were still common in the course of WG.