Abstract: PO1942
Clinical Features and Treatment Outcomes of Patients with Pauci-Immune Vasculitis with and Without a Medical History of Autoimmune Disease
Session Information
- Glomerular Diseases: Clinical, Outcomes, and Trials - 3
October 22, 2020 | Location: On-Demand
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1203 Glomerular Diseases: Clinical, Outcomes, and Trials
Authors
- Lionaki, Sophia, Nephrology, Laiko Hospital, Athens, Greece
- Marinaki, Smaragdi, Nephrology, Laiko Hospital, Athens, Greece
- Fragkioudaki, Sofia, Nephrology, Laiko Hospital, Athens, Greece
- Kalaitzakis, Emmanuel, Nephrology, Laiko Hospital, Athens, Greece
- Kalogeropoulos, Petros, Nephrology, Laiko Hospital, Athens, Greece
- Liapis, Georgios, Pathology, Laiko Hospital, Athens, Greece
- Tzioufas, Athanasios, Pathophysiology department, Laiko Hospital, Athens, Greece
- Boletis, Ioannis, Nephrology, Laiko Hospital, Athens, Greece
Background
A proportion of patients with pauci-immune vasculitis (PIV) report a past medical history (PMH) of other autoimmune disorder prior to the diagnosis of vasculitis. The purpose of this study was to identify the differences, if any, between patients with PIV with or without a PMH of other autoimmune disease prior to the onset of vasculitis.
Methods
Among 304 patients with biopsy proven PIV at any site, detailed information regarding their PMH was available in 235 patients (77.3%). Of these, 60 (25.5%) reported a PMH of other autoimmune disorder including Sjogren syndrome, Crohn’s disease, autoimmune thyroiditis, psoriasis, rheumatoid arthritis, and scleroderma.
Results
The clinical characteristics and outcomes of the two groups are displayed in [table 1].
Conclusion
Patients with a PMH of other autoimmune disorder prior to the diagnosis of PIV were predominantly P/MPO-ANCA positive, had less impaired kidney function at presentation and a lower probability of relapse after achieving remission, compared to patients without a PMH of autoimmunity.
| Characteristic Mean (sd) or N(%) | Patients with a PMH of autoimmune disease Ν=60 | Patients without a PMH of autoimmune disease Ν=175 | p-value |
| Age (years) | 53.9 (±16.03) | 51.2 (±15.08) | 0.88 |
| Gender (Male) | 26 (43.3) | 99 (56.5) | 0.67 |
| ANCA type | |||
| P/MPO-ANCA | 35 (58.6) | 73 (42) | 0.03 |
| C/PR3-ANCA | 23 (37.9) | 88 (55) | 0.61 |
| Negative | 2 (3.3) | 3 (1.8) | 0.47 |
| Clinical phenotype | |||
| Microscopic polyangiitis | 32 (53.4) | 71 (40.6) | 0.09 |
| Polyangiitis with Granulomatosis | 13 (22.4) | 81 (46.6) | 0.0012 |
| Renal limited disease | 14 (24.1) | 23 (13.1) | 0.057 |
| Organ involvement | |||
| Kidney | 42 (70) | 142 (80.9) | 0.8 |
| Pulmonary | (20) 33.3 | 78 (44.5) | 0.14 |
| Ear nose throat | 8 (13.3) | 55 (31.2) | 0.009 |
| Serum creatinine at biopsy (mg/dl) | 2.3 (±19.94) | 5.1 (±3.96) | <0.0001 |
| Outcome | |||
| Remission | 89.2 % | 85.5 % | 0.5 |
| Treatment resistance | 10.7 % | 15.2 % | 0.41 |
| Relapse | 16 % | 34.2 % | 0.017 |
| ESKD | 11.1 % | 13.4 % | 0.66 |
| Relapse by ANCA type | |||
| PR3-ANCA patients | 19 % | 47.7 % | 0.02 |
| MPO-ANCA patients | 10.7 % | 18.35 % | 0.37 |