Risks of treatments and long-term outcomes of systemic ANCA-associated vasculitis

Floßmann, Oliver

doi:10.1016/j.lpm.2015.02.019

Cited by 31 publications

(18 citation statements)

References 47 publications

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“…Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are necrotizing small vessel systemic diseases. Recent advances in the treatment of AAV have transformed these previously universally fatal diseases into chronic relapsing diseases, although substantial morbidity and mortality still exist related to vasculitis and treatment-associated side effects [ 1 - 2 ]. AAV therapy involves a two-stage approach consisting of remission induction followed by remission maintenance.…”

Section: Introductionmentioning

confidence: 99%

Long-term Clinical Course of Antineutrophil Cytoplasmic Antibody-associated Vasculitis Patients off Maintenance Therapy

Gapud¹,

Manno²,

Seo³

et al. 2018

Cureus

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ObjectivesThe optimal duration of maintenance immunosuppressive therapy in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is still controversial. The aim of our study is to describe the characteristics and outcomes of patients with AAV who were able to stop maintenance agents completely while remaining on daily prednisone (< 5 mg) for at least 36 months.Materials and methodsAAV patients treated at our center from 2000 to 2016 and who were not on maintenance agents while remaining on prednisone < 5 mg daily for at least 36 months were identified by the providers, and their records were retrospectively reviewed. Relapse was defined by the reinitiation of immunosuppressive therapy for biopsy-proven glomerulonephritis or any extra-renal organ involvement.ResultsOf the 18 patients who fulfilled the study inclusion criteria, 12 were male and 14 were Caucasian. The mean age at AAV diagnosis was 54 years. Seventeen patients had renal involvement and seven had lung involvement. Eleven patients received cyclophosphamide and eight patients received rituximab along with glucocorticoids for remission induction. Twelve patients were weaned completely off prednisone. The median duration of prednisone use was 20 months. Nine patients received maintenance therapy with azathioprine or mycophenolate mofetil. The median duration of maintenance therapy was 24 months. The mean follow-up time after stopping the maintenance agent was 64 months. During this period, three patients had disease relapse.ConclusionsStopping maintenance agents for > 36 months can be achieved in some patients with AAV. Prospective, randomized controlled trials are needed to confirm this finding.

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Section: Introductionmentioning

confidence: 99%

Long-term Clinical Course of Antineutrophil Cytoplasmic Antibody-associated Vasculitis Patients off Maintenance Therapy

Gapud¹,

Manno²,

Seo³

et al. 2018

Cureus

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“…Granulomatosis with polyangitis is a severe necrotizing vasculitis with high morbidity and mortality [18]. Disease-and treatment-related damages are common and relapse risk is as high as 50% [19]. Lack of markers that predict activity and relapses make management of GPA challenging and there is no clear consensus on the duration of maintenance treatment.…”

Section: Discussionmentioning

confidence: 99%

Serum ferritin as an activity marker for granulamotosis with polyangiitis

et al. 2017

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Background: Serum ferritin correlates well with the activities of systemic lupus erythematosus (SLE) and dermatomyositis, but it has not been previously studied in patients with vasculitis. Methods: Medical records of granulomatosis with polyangiitis (GPA, Wegener's granulomatosis) patients with at least six months of regular follow-up were evaluated. The activity of GPA was assessed with Birmingham Vasculitis Activity Score for Wegener's Granulomatosis (BVAS/WG). Serum ferritin and other acute phase markers were measured at initial presentation. Results: Serum ferritin levels were found to be the highest in GPA patients with alveolar hemorrhage, median (IQR) 1041 (1281) lg/L. Patients with renal disease also had high levels of ferritin and it was correlated with concurrent glomerular filtration rate (r ¼ À0.65, p < .001). Serum ferritin is also correlated well with the BVAS/WG scores (r ¼ 0.79, p < .001). Conclusions: Measurement of serum ferritin might help in assessing disease activity of GPA.

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“…Patients with antineutrophil cytoplasmic antibody (ANCA) associated vasculitis present a high risk of severe infections, sometimes fatal [8]. Prolonged corticosteroid treatment has been identified as one of the underlying factors [9].…”

Section: Immunosuppressive Treatments and Infectionsmentioning

confidence: 99%