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Kidney Week

Abstract: PO1942

Clinical Features and Treatment Outcomes of Patients with Pauci-Immune Vasculitis with and Without a Medical History of Autoimmune Disease

Session Information

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-ANCA35 (58.6)73 (42)0.03
C/PR3-ANCA23 (37.9)88 (55)0.61
Negative2 (3.3)3 (1.8)0.47
Clinical phenotype   
Microscopic polyangiitis32 (53.4)71 (40.6)0.09
Polyangiitis with Granulomatosis13 (22.4)81 (46.6)0.0012
Renal limited disease14 (24.1)23 (13.1)0.057
Organ involvement   
Kidney42 (70)142 (80.9)0.8
Pulmonary(20) 33.378 (44.5)0.14
Ear nose throat8 (13.3)55 (31.2)0.009
Serum creatinine at biopsy (mg/dl)2.3 (±19.94)5.1 (±3.96)<0.0001
Outcome   
Remission89.2 %85.5 %0.5
Treatment resistance10.7 %15.2 %0.41
Relapse16 %34.2 %0.017
ESKD11.1 %13.4 %0.66
Relapse by ANCA type   
PR3-ANCA patients19 %47.7 %0.02
MPO-ANCA patients10.7 %18.35 %0.37