Vasculitis & polymyalgia rheumatica
ANCA-associated pulmonary-renal syndrome treated with cyclophosphamide, rituximab, repeated methyl-prednisolone pulses and a reduced oral glucocorticoid regime: an observational study
Authors' conclusion
Combination therapy with iv cyclophosphamide and rituximab, with repeated methyl-prednisolone pulses and a rapid prednisone taper, results in early disease control, with low mortality, chronic organ damage and infections.
Study context
- Population
- Patients diagnosed with pulmonary-renal syndrome due to ANCA-associated vasculitis (AAV), 2010 to 2020, Autoimmune Diseases Unit, Hospital Universitario Crucesmean age 62.71 years
- Participants
- 14 patients
- Outcome
- Mortality due to any cause within 24 months
- Follow-up
- 24 months
Methodological detailssingle-centre, routine clinical care data, All patients received repeated methyl-prednisolone pulses; 13 received iv cyclophosphamide 500 mg every two weeks; 12 received rituximab
- Single-centre
- Routine clinical care data
- All patients received repeated methyl-prednisolone pulses; 13 received iv cyclophosphamide 500 mg every two weeks; 12 received rituximab
Main finding
As reported in the abstract
All-cause mortality within 24 months
- No cases of death
Observational data: associations, not effects.
Secondary outcomes
End-stage kidney disease · 24 months
- No cases of end-stage kidney disease
Need for long-term oxygen therapy · 24 months
- No cases with need for long-term oxygen therapy
Relapse
- Patients with a relapse
- 3
Median serum creatinine · 24 months
- Median creatinine, mg/dl
- 1.30
- 2.46 mg/dl at diagnosis
Median Birmingham Vasculitis Activity Score (BVAS) · 24 months
- Median BVAS
- 0
- 24 at diagnosis
Safety
- Patients with major infections
- 5
- None of them opportunistic
Original publication
ANCA-associated pulmonary-renal syndrome treated with cyclophosphamide, rituximab, repeated methyl-prednisolone pulses and a reduced oral glucocorticoid regime: an observational study. Clin Exp Rheumatol. Published 26 December 2022. doi:10.
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