Abstract: TH-PO181

ANCA Associated Vasculitis in Scleroderma: A Renal Perspective

Session Information

Category: Nephrology Education

  • 1302 Fellows and Residents Case Reports

Authors

  • Kant, Sam, University of Maryland, Baltimore, Maryland, United States
  • Geetha, Duvuru, John Hopkins Bayview Medical Center, Baltimore, Maryland, United States
Background

Overlap syndrome of ANCA associated vasculitis (AAV) and scleroderma (SS) is rare with conflicting data on renal outcomes. We describe the clinical characteristics and treatment outcome of ANCA glomerulonephriitis(GN) in SS patients followed at a single center

Methods

We conducted a retrospective study of 3840 patients in our SS database to identify SS patients who subsequently developed AAV with renal involvement. Patient demographics, serology, renal function with renal histology and treatment outcomes were assessed.

Results

Of the 3840 patients, we identified 5 patients who had ANCA GN. The median age at SS diagnosis was 52 years, all 5 patients were female and 4 had diffuse scleroderma. ANA was positive in all, with 3 of them having anti Scl 70 antibodies. Four patients had interstitial lung disease and gastrointestinal dysmotility as a part of the constellation of clinical characteristics of SS. Median time of onset of AAV from time of diagnosis of SS was 12 years and all of 5 patients were MPO positive. All patients had acute kidney injury, with biopsy proven crescentic glomerulonephritis and none requiring dialysis. One patient had sinus involvement while AAV was renal limited in the remaining 4 patients. Two patients were treated with cyclophosphamide(CYC) and steroids(GC) and three were treated with rituximab (RTX) and steroids. All patients achieved disease remission. The median follow up was 24 months, The mean GFR at diagnosis was 39 ml/min and at last follow up was 38 ml/min. Of the 5 patients, 2 did not receive maintenance immunosuppression and both experienced vasculitis relapse. None of the patients reached ESRD. Three patients died and of these 2 experienced relapse with fulminant alveolar hemorrhage

Conclusion

ANCA GN in SS is rare with disease manifestation and course similar to AAV. This case series demonstrates that disease remission can be achieved with standard induction therapy. However vasculitis relapse is common and associated with high mortality without remission maintenance therapy

Patient Characteristics
PatientInduction RxMaintenance RxVasculitis RelapseESRDDeath
1GC + RTXAZANoNoNo
2GC + CYCMMFNoNoYes
3GC + RTXNoneYes (GN + DAH)NoYes
4GC + CYCNoneYes (DAH)NoYes
5GC + RTXAZANoNoNo

AZA- Azathioprine; MMF- Mycophenolate mofetil; DAH- Diffuse alveolar hemorrhage