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Rheumatology (Oxford, England) Aug 2011There are limited data on the long-term prognosis of microscopic polyangiitis (MPA). A systematic review was performed to estimate the survival, renal survival and... (Review)
Review
OBJECTIVE
There are limited data on the long-term prognosis of microscopic polyangiitis (MPA). A systematic review was performed to estimate the survival, renal survival and relapse rates in patients with MPA.
METHODS
Articles included in MEDLINE and EMBASE databases were reviewed. Randomized or non-randomized trials, cohort, case-control and cases-series studies of patients with MPA diagnosed according to Chapel Hill Consensus Conference definitions, a high rate of biopsy-confirmed diagnosis, follow-up >1 year and follow-up losses <10%. Two independent authors using a predefined questionnaire for evaluating the quality and risk of bias for each study extracted data.
RESULTS
Eighteen studies for MPA prognosis (n = 940) and six for MPA outcomes after transplantation (n = 65) were included. Survival rates were 77-100% at 1 year, 46-80% at 5 years and 60-80% at 10 years. Higher mortality density occurred within the first months after diagnosis. Vasculitis was the cause of death in 32-50% of patients. Relapses were detected in 19-39% of cases (median time to relapse 15-43 months). Renal graft survival was 85-94% at 1 year and 51-87% at 5 years. Age, renal involvement and immunosuppressive treatment were related to mortality. Lower relapse rate was achieved with 12 vs 6 CYC pulses.
CONCLUSION
Evidence regarding MPA prognosis is weak. MPA mortality is mainly concentrated in the first months after diagnosis. Fewer than 50% of deaths are related to MPA activity. MPA long-term prognosis is less severe, although relapses are frequent. End-stage renal failure is a frequent complication of MPA, and renal transplantation could be an effective therapy in these patients. Early diagnosis, early initiation of a tailored therapy according to risk factors and a longer follow-up of the patients are needed.
Topics: Cause of Death; Databases, Bibliographic; Humans; Kidney Transplantation; Microscopic Polyangiitis; Prognosis; Randomized Controlled Trials as Topic; Recurrence; Survival Rate; Time Factors
PubMed: 21406467
DOI: 10.1093/rheumatology/ker112 -
Expert Opinion on Pharmacotherapy Jun 2009The systemic vasculitides are characterized by inflammatory lesions in blood vessels. Therapeutic approaches should be based on the aetiology or pathophysiology of... (Review)
Review
The systemic vasculitides are characterized by inflammatory lesions in blood vessels. Therapeutic approaches should be based on the aetiology or pathophysiology of disease. Unfortunately, for many of these disorders neither is fully understood and empirical treatment based on clinical presentation and the pattern of organ involvement is used. This approach is effective in improving survival in the most serious forms. We undertook a systematic literature review to assess the evidence for using drug therapies in vasculitis. Glucocorticoids remain essential for many forms of vasculitis; indeed, in giant-cell arteritis, they may be the only therapy necessary. However, additional immunosuppressive agents are required for other forms of vasculitis: methotrexate in Takayasu's arteritis and non-renal small-vessel vasculitis and cyclophosphamide for classic Wegener's granulomatosis, microscopic polyangiitis, polyarteritis nodosa and Churg-Strauss syndrome with poor prognostic features. Subsequent disease control is with low-dose glucocorticoid and azathioprine or methotrexate. Biologic therapy is being used in resistant cases. Patients experience significant short- to medium-term toxicity, especially infection and steroid side effects. Late sequelae due to high cumulative doses of cyclophosphamide include infertility and malignancy. Such risks are being reduced due to more judicious use of short courses of cyclophosphamide followed by substitution by safer agents.
Topics: Humans; Vasculitis
PubMed: 19445559
DOI: 10.1517/14656560902946401 -
Clinical and Experimental Rheumatology 2008There has been a considerable improvement in the survival of patients with systemic vasculitis since the introduction of immunosuppressive therapy and improved... (Review)
Review
There has been a considerable improvement in the survival of patients with systemic vasculitis since the introduction of immunosuppressive therapy and improved diagnostic tools to allow earlier diagnosis. We review the published literature on current risk of mortality in patients with small vessel antineutrophil cytoplasm antibody- (ANCA) associated vasculitis including Wegener's granulomatosis (survival rate of approximately 75% at 5 years), microscopic polyangiitis (survival rate of 45% to 75% at 5 years), Churg-Strauss syndrome (survival rate of 68% to 100% at 5 years), and Henoch-Schönlein purpura (survival rate of 75% in adult-onset, greater in childhood onset); medium vessel vasculitis including polyarteritis nodosa (survival rate of 75% to 80% at 5 years), Kawasaki disease (survival rate of greater than 99% at 5 years); large vessel vasculitis including giant cell arteritis (survival rate equivalent to the age-matched population), and Takayasu arteritis (survival of 70% to 93% at 5 years). Mortality rates are falling as a result of more effective intervention but remain elevated substantially in severe disease. Early deaths are usually attributable to active vasculitis with multiorgan failure or infection, or both. The incidence of late deaths may be increased by long-term effects of therapy and development of comorbidities. These findings highlight the need to improve early diagnosis and initiation of targeted therapy, thereby reducing treatment-related toxicity and comorbidities.
Topics: Adult; Age Factors; Antibodies, Antineutrophil Cytoplasmic; Cause of Death; Child; Child, Preschool; Female; Humans; Male; Multiple Organ Failure; Risk; Severity of Illness Index; Sex Factors; Vasculitis
PubMed: 19026150
DOI: No ID Found -
Annals of the Rheumatic Diseases Jul 2008We undertook a systematic literature review as a background to the European League Against Rheumatism (EULAR) recommendations for conducting clinical trials in... (Review)
Review
Outcomes from studies of antineutrophil cytoplasm antibody associated vasculitis: a systematic review by the European League Against Rheumatism systemic vasculitis task force.
OBJECTIVES
We undertook a systematic literature review as a background to the European League Against Rheumatism (EULAR) recommendations for conducting clinical trials in anti-neutrophil cytoplasm antibody associated vasculitis (AAV), and to assess the quality of evidence for outcome measures in AAV.
METHODS
Using a systematic Medline search, we categorised the identified studies according to diagnoses. Factors affecting remission, relapse, renal function and overall survival were identified.
RESULTS
A total of 44 papers were reviewed from 502 identified by our search criteria. There was considerable inconsistency in definitions of end points. Remission rates varied from 30% to 93% in Wegener granulomatosis (WG), 75% to 89% in microscopic polyangiitis (MPA) and 81% to 91% in Churg-Strauss syndrome (CSS). The 5-year survival for WG, MPA and CSS was 74-91%, 45-76% and 60-97%. Relapse (variably defined) was common in the first 2 years but the frequency varied: 18% to 60% in WG, 8% in MPA, and 35% in CSS. The rate of renal survival in WG varied from 23% at 15 months to 23% at 120 months.
METHOD
used to assess morbidity varied between studies. Ignoring the variations in definitions of the stage of disease, factors influencing remission, relapse, renal and overall survival included immunosuppressive therapy used, type of organ involvement, presence of ANCA, older age and male gender.
CONCLUSIONS
Factors influencing remission, relapse, renal and overall survival include the type of immunosuppressive therapy used, pattern of organ involvement, presence of ANCA, older age and male gender. Methodological variations between studies highlight the need for a consensus on terminology and definitions for future conduct of clinical studies in AAV.
Topics: Antibodies, Antineutrophil Cytoplasmic; Churg-Strauss Syndrome; Cyclophosphamide; Evidence-Based Medicine; Glucocorticoids; Granulomatosis with Polyangiitis; Humans; Immunosuppressive Agents; Recurrence; Remission Induction; Risk Factors; Survival Analysis; Treatment Outcome; Vasculitis
PubMed: 17911225
DOI: 10.1136/ard.2007.071936