Cyclophosphamide Therapy of Severe Systemic Necrotizing Vasculitis

Fauci, Anthony S.; Katz, Paul; Haynes, Barton F.; Wolff, Sheldon M.

doi:10.1056/nejm197908023010503

Cited by 493 publications

(141 citation statements)

References 13 publications

Supporting

Mentioning

128

Contrasting

Unclassified

Order By: Relevance

“…The paradigm of AAV changed from a fatal diagnosis to a chronic disease after the introduction of immunosuppressive therapeutics [3]. The majority of patients, however, experience relapses during treatment or at remission.…”

Section: Introductionmentioning

confidence: 99%

Maintaining remission in patients with granulomatosis with polyangiitis or microscopic polyangiitis: the role of ANCA

Kemna¹,

Paassen²,

Damoiseaux³

et al. 2017

Expert Opinion on Orphan Drugs

View full text Add to dashboard Cite

Introduction: Granulomatosis with Polyangiitis (GPA; formerly Wegener's) and Microscopic Polyangiitis (MPA) are inflammatory disease entities affecting small to medium vessels. They are characterized by the presence of anti-neutrophil cytoplasmic antibodies (ANCA) and are frequently grouped together with Eosinophilic Granulomatosis with Polyangiitis (EGPA; formerly Churg Strauss Syndrome) under the term ANCA-associated Vasculitis (AAV). Due to effective immunosuppressive therapy that was introduced more than 45 years ago, AAV has become a chronic disease in which relapses occur frequently during maintenance treatment or later on after all immunosuppressive therapy is stopped. Since relapses are associated with morbidity and mortality, early detection and prevention of relapses is of great importance. Areas covered: This review focuses on the potential strategies to guide maintenance therapy in patients with GPA or MPA, with an emphasis on patient classifications and the role of serial ANCA measurements. Expert opinion: Risk factors for relapses, e.g., genetic background of the patient, have been studied during the last three decades. Only few of these risk factors directly influence the management of patients. One potentially important factor that may influence therapeutic decisions in AAV patients is serial measurement of ANCA. Recently, it became clear that serial ANCA measurement is an attractive approach in AAV patients with (a history of) capillaritis, e.g., renal involvement or alveolar haemorrhage, but not in patients with more limited disease. Whether ANCA rises can be used to guide therapy andhence-to prevent relapses has been a great debate during the last three decades. At present, we conclude that small studies do support such an approach but that these findings require further validation in larger randomized clinical trials. ARTICLE HISTORY

show abstract

Section: Introductionmentioning

confidence: 99%

Maintaining remission in patients with granulomatosis with polyangiitis or microscopic polyangiitis: the role of ANCA

Kemna¹,

Paassen²,

Damoiseaux³

et al. 2017

Expert Opinion on Orphan Drugs

View full text Add to dashboard Cite

show abstract

“…The introduction of cyclophosphamide (CYC) and glucocorticoids in the 1950s dramatically improved the prognoses in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs) (1). 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).…”

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

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

“…In humans, serologic abnormalities such as autoantibodies and immune complexes (14,15) as well as the presence of immunoglobulin, complement, and fibrinogen at the sites of tissue injury (16) suggest that systemic vasculitis is immunologically mediated. In hepatitis B-associated vasculitis, the antigen (HBsAg) has been demonstrated in the.…”

Section: Discussionmentioning

confidence: 99%

HLA antigen frequencies in systemic vasculitis: increase in HLA‐DR2 in wegener's granulomatosis

Elkon

Sutherland

Rees

et al. 1983

Arthritis & Rheumatism

View full text Add to dashboard Cite

Cyclophosphamide Therapy of Severe Systemic Necrotizing Vasculitis

Cited by 493 publications

References 13 publications

Maintaining remission in patients with granulomatosis with polyangiitis or microscopic polyangiitis: the role of ANCA

Maintaining remission in patients with granulomatosis with polyangiitis or microscopic polyangiitis: the role of ANCA

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

HLA antigen frequencies in systemic vasculitis: increase in HLA‐DR2 in wegener's granulomatosis

Contact Info

Product

Resources

About