ANCA-associated pulmonary-renal syndrome treated with cyclophosphamide, rituximab, repeated methyl-prednisolone pulses and a reduced oral glucocorticoid regime: an observational study.

OBJECTIVES

To describe the clinical outcome of patients with pulmonary-renal syndrome (PRS) due to ANCA-associated vasculitis (AAV) from a single centre.

METHODS

Observational study of routine clinical care data of patients diagnosed with PRS due to AAV from 2010 to 2020 at the Autoimmune Diseases Unit, Hospital Universitario Cruces. Mortality due to any cause within 24 months was defined as the primary outcome. Secondary outcomes included end-stage kidney disease and the need for oxygen therapy at 24 months.

RESULTS

Fourteen patients were identified with a mean age at diagnosis of 62.71 years. At diagnosis, the median serum creatinine was 2.46 mg/dl and the median Birmingham Vasculitis Activity Score (BVAS) was 24. All patients were treated with repeated methyl-prednisolone pulses, 13 patients received iv cyclophosphamide 500 mg every two weeks and 12 patients received rituximab. The mean (SD) initial dose of oral prednisone was 25 (7) mg/d. A rapid tapering of oral prednisone was achieved in all patients as per protocol, with a mean (SD) dose of 10.6 (1.9) mg/d received within the first three months. No cases of death, end-stage kidney disease or with need for long-term oxygen therapy were seen. Three patients suffered a relapse and five patients had major infections, none of them opportunistic. The median creatinine and BVAS at 24 months were 1.30 mg/dl and 0, respectively.

CONCLUSIONS

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.