Background The systemic vasculitides continue to present the practising clinician with many challenges. The advent of effective immunosuppressive therapy (cyclophosphamide with high-dose corticosteroids) has markedly improved patients’ survival (78% at 5 years)1. New diagnostic tools have helped earlier diagnosis.2
Objectives Our aim was to study the patients under rheumatology care, with primary medium and small vessel vasculitis over the last 10 years and to analyse their presentations, diagnosis, treatment outcomes and to assess the impact of early remission induction therapy.
Methods Cases were retrieved using hospital records and a serology database using ANCA request. All new cases of GPA, EGPA, MPA, and PAN and other small to medium vasculitis between 2002 and 2012 were included, provided they met pre-defined criteria of Chapel Hill classification. Demographic profile, clinical features, laboratory data, treatment details, and clinical outcome of patients were analyzed retrospectively.
Results 52 patients were identified. Two patients were excluded due to lack of clarity of diagnosis. Mean age was 56.3 years, Female: Male= 29:21. Most frequent symptoms at diagnosis include arthralgia, vasculitic rash, cough, dyspnea and fever. Most had raised inflammatory markers (PV and CRP in 82%). 13 patients had elevated creatinine (112-187). ANCA was positive in 32/50.Diagnosis included the following: (50)
50% had IV glucocorticoids and cyclophosphamide (dose from 500mg-1gm as per weight) for induction therapy. We continued for 6 cycles and then switched to oral therapy. 6 patients had relapse, of which 5 had further cyclophosphamide and two achieved remission. 6/50 (one failed, another refused cyclo) patients needed Rituximab for adequate disease control. Other drugs used included Azathioprine (29) Mycophenolate (9) and Methotrexate (7)
The median time to achieve remission was 5 months since diagnosis. Median duration of follow up was 76.4 months (3-230). 30% of patients (15/50) were able to stop immunosuppression while maintaining remission.
Conclusions Cyclophosphamide and glucocorticoids have achieved high remission rates 80% (24/30), compared to Lane et al1 (78% at 5 years). Rituximab has achieved remission in refractory vasculitis. Aggressive early induction therapy and sustained remission have achieved a favourable impact on the long term outcome of our patient groups.
References S E Lane et al. Primary systemic vasculitis: Clinical features and mortality. QJM (2005) 98 (2): 97-111.
C Mukhtyar et al. Outcomes from studies of antineutrophil cytoplasm antibody associated vasculitis: a systematic review by the European League against Rheumatism systemic vasculitis task force. Ann Rheum Dis 2008;67:1004–1010
Disclosure of Interest None Declared
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