Abstract | OBJECTIVES: METHODS: 159 eligible patients in the Wegener's Granulomatosis-Entretien (WEGENT) trial newly diagnosed with systemic or renal WG or MPA with ≥ 1 poor prognosis factors were included in this prospective study. Rescue treatment strategies and outcomes in patients with induction-refractory disease were analysed and patient characteristics at diagnosis were compared with those of induction-responders. RESULTS: Most patients (n=126, 79.2%) achieved remission; 1 stopped induction because of allergy and 32 were induction-refractory (24 WG and 8 MPA); 11 died rapidly within a median of 2.5 months, 6 of uncontrolled disease, 1 of an infectious complication and 4 of both. Treatment was discontinued in 1 patient with MPA with end-stage renal disease. Induction was switched to oral CYC in 20 patients, combined with infliximab in 1; 15 (75%) achieved remission or low disease activity state, 3 subsequently died of uncontrolled disease and 2 entered remission using several other agents including biological agents. Alveolar haemorrhage and a creatinine level >200 μmol/l were independently associated with induction-refractory disease. Among patients with induction-refractory disease, massive alveolar haemorrhage was associated with higher mortality. CONCLUSION: Switching to oral CYC can be an effective rescue treatment for patients with systemic forms of WG or MPA who fail to achieve remission with first-line CS and intravenous CYC. However, a more rapidly effective regimen remains to be identified for most severely affected patients whose outcomes can be rapidly fatal.
|
Authors | Raphaèle Seror, Christian Pagnoux, Marc Ruivard, Isabelle Landru, Denis Wahl, Sophie Rivière, Solveig Aussant, Alfred Mahr, Pascal Cohen, Luc Mouthon, Loïc Guillevin, French Vasculitis Study Group |
Journal | Annals of the rheumatic diseases
(Ann Rheum Dis)
Vol. 69
Issue 12
Pg. 2125-30
(Dec 2010)
ISSN: 1468-2060 [Electronic] England |
PMID | 20643762
(Publication Type: Journal Article, Multicenter Study, Randomized Controlled Trial)
|
Chemical References |
- Glucocorticoids
- Immunosuppressive Agents
- Cyclophosphamide
|
Topics |
- Administration, Oral
- Adult
- Aged
- Cyclophosphamide
(administration & dosage, therapeutic use)
- Drug Therapy, Combination
- Female
- Glucocorticoids
(therapeutic use)
- Granulomatosis with Polyangiitis
(drug therapy)
- Humans
- Immunosuppressive Agents
(administration & dosage, therapeutic use)
- Infusions, Intravenous
- Male
- Microscopic Polyangiitis
(drug therapy)
- Middle Aged
- Prognosis
- Prospective Studies
- Treatment Failure
- Treatment Outcome
|