Long-term follow-up of a randomized trial on 118 patients with polyarteritis nodosa or microscopic polyangiitis without poor-prognosis factors

Samson, M.; Puéchal, Xavier; Devilliers, H.; Ribi, Camillo; Cohen, Pascal; Bienvenu, Boris; Ruivard, M.; Terrier, Benjamin; Pagnoux, Christian; Mouthon, Luc; Guillevin, Loı̈c

doi:10.1016/j.autrev.2013.10.001

Cited by 76 publications

(41 citation statements)

References 28 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Indeed, 10-year relapse-free survival rates were only 26.3% and 33.5% in the AZA and MTX recipients, respectively. These low rates are consistent with those reported in other long-term studies in patients with GPA (24% at 5 years) (38) and patients with MPA (45.4% and 43.4% at 7 years) (7,8).…”

Section: Discussionsupporting

confidence: 81%

“…Among patients with granulomatosis with polyangiitis (Wegener's) (GPA), 74-80% now survive up to 5 years (2-4), compared to a 1-year mortality rate of ;70% in untreated patients (5). Similarly, in those with microscopic polyangiitis (MPA), the 5-year survival rate reaches 65-95%, depending on the prognostic factors considered (6)(7)(8). However, these advances have been achieved at the cost of treatment-related toxicity (9).…”

mentioning

confidence: 99%

See 1 more Smart Citation

Long‐Term Outcomes Among Participants in the WEGENT Trial of Remission‐Maintenance Therapy for Granulomatosis With Polyangiitis (Wegener's) or Microscopic Polyangiitis

Puéchal

Pagnoux

Perrodeau

et al. 2016

Arthritis & Rheumatology

Self Cite

111

View full text Add to dashboard Cite

Objective. Findings from the WEGENT trial and other short-term studies have suggested that azathioprine (AZA) or methotrexate (MTX) could effectively maintain remission of granulomatosis with polyangiitis (Wegener's) (GPA) or microscopic polyangiitis (MPA).This study was undertaken to examine whether differences in rates of relapse or adverse events would appear after discontinuation of these 2 maintenance regimens, when assessed over a longer followup period.Methods. Long-term outcomes in patients enrolled in the WEGENT trial were analyzed according to their

show abstract

Section: Discussionsupporting

confidence: 81%

mentioning

confidence: 99%

Long‐Term Outcomes Among Participants in the WEGENT Trial of Remission‐Maintenance Therapy for Granulomatosis With Polyangiitis (Wegener's) or Microscopic Polyangiitis

Puéchal

Pagnoux

Perrodeau

et al. 2016

Arthritis & Rheumatology

Self Cite

111

View full text Add to dashboard Cite

show abstract

“…Glucocorticoids can be used alone as firstline therapy to effectively induce remission in patients with non-severe MPA or non-severe EGPA (with a five-factor score of 0), on the basis of few cohort studies (74,75). However, more than half of these patients eventually require the addition of another immunosuppressant because of progressive, refractory, or relapsing disease.…”

Section: Treatmentmentioning

confidence: 99%

Updates in ANCA-associated vasculitis

2016

Self Cite

View full text Add to dashboard Cite

Antineutrophil cytoplasm antibody (ANCA)-associated vasculitides are small-vessel vasculitides that include granulomatosis with polyangiitis (formerly Wegener's granulomatosis), microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). Renal-limited ANCA-associated vasculitides can be considered the fourth entity. Despite their rarity and still unknown cause(s), research pertaining to ANCA-associated vasculitides has been very active over the past decades. The pathogenic role of antimyeloperoxidase ANCA (MPO-ANCA) has been supported using several animal models, but that of antiproteinase 3 ANCA (PR3-ANCA) has not been as strongly demonstrated. Moreover, some MPO-ANCA subsets, which are directed against a few specific MPO epitopes, have recently been found to be better associated with disease activity, but a different method than the one presently used in routine detection is required to detect them. B cells possibly play a major role in the pathogenesis because they produce ANCAs, as well as neutrophil abnormalities and imbalances in different T-cell subtypes [T helper (Th)1, Th2, Th17, regulatory cluster of differentiation (CD)4+ CD25+ forkhead box P3 (FoxP3)+ T cells] and/or cytokine-chemokine networks. The alternative complement pathway is also involved, and its blockade has been shown to prevent renal disease in an MPO-ANCA murine model. Other recent studies suggested strongest genetic associations by ANCA type rather than by clinical diagnosis. The induction treatment for severe granulomatosis with polyangiitis and microscopic polyangiitis is relatively well codified but does not (yet) really differ by precise diagnosis or ANCA type. It comprises glucocorticoids combined with another immunosuppressant, cyclophosphamide or rituximab. The choice between the two immunosuppressants must consider the comorbidities, past exposure to cyclophosphamide for relapsers, plans for pregnancy, and also the cost of rituximab. Once remission is achieved, maintenance strategy following cyclophosphamide-based induction relies on less toxic agents such as azathioprine or methotrexate. The optimal maintenance strategy following rituximab-based induction therapy remains to be determined. Preliminary results on rituximab for maintenance therapy appear promising. Efforts are still under way to determine the optimal duration of maintenance therapy, ideally tailored according to the characteristics of each patient and the previous treatment received.Keywords: Antineutrophil cytoplasmic antibody-associated vasculitis, granulomatosis with polyangiitis (Wegener's granulomatosis), microscopic polyangiitis, Churg-Strauss syndrome (eosinophilic granulomatosis with polyangiitis), cyclophosphamide, rituximab IntroductionAntineutrophil cytoplasm antibody (ANCA)-associated vasculitides are small-vessel vasculitides that include granulomatosis with polyangiitis (GPA; formerly Wegener's granulomatosis), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA; al...

show abstract

“…In a series of 26 patients, only 12% had CNS disease [73]. There are no studies investigating the efficacy of immunosuppressive agents in PAN-related CNS disease, however, because CNS disease increases the risk of both morbidity and mortality, an aggressive treatment with glucocorticoids and CYC is advisable [74].…”

Section: Polyarteritis Nodosamentioning

confidence: 98%