Abstract: TH-PO182

Renal Biopsy Teaching Case: A Patient with Scleroderma, Hypertension, AKI, and PR3 ANCA Positivity

Session Information

Category: Nephrology Education

  • 1302 Fellows and Residents Case Reports

Authors

  • Kant, Sam, University of Maryland Medical Center, Baltimore, Maryland, United States
  • Rosenberg, A Z, Johns Hopkins University Hospital, Baltimore, Maryland, United States
  • Geetha, Duvuru, Johns Hopkins University Hospital, Baltimore, Maryland, United States
Background

A 65 year old African-American male, with a history of limited scleroderma for 16 years complicated by severe gastrointestinal dysmotility and interstitial lung disease, presented to the clinic with elevated blood pressure of 190/100 mm Hg, on a background of previously well controlled blood pressure. He was found to have an acute kidney injury with a serum creatinine of 2.5 mg/dL, compared to his baseline of 1.3 mg/dL. Urine studies demonstrated microscopic hematuria, with 3.4 grams of proteinuria. His hemoglobin was 7.4 and he had no evidence of hemolysis and platelet count was normal. Serologies revealed a positive c-ANCA serology with PR3 positivity at 97.4 IU/mL with negative ANA, ds-DNA, Scl-70, anti-smith, anti-Ro, Anti-La, and RNP. A renal biopsy was performed which demonstrated arteriolar microangiopathy with fibrinoid necrosis and concentric lamellation with no evidence of ANCA GN (Fig1). He was treated with ACE inhibitor with improvement of his BP and improved serum creatinine of 1.7 mg/dl.

Conclusion

This case of late onset scleroderma renal crisis highlights that atypical presentations of scleroderma renal crisis can exist and ANCA positivity can be misleading in such situations. Therefore, clinical decisions for further management should be predicated on expedient renal biopsy.

A) Glomerulus (HE) and glomerular arteriole with entrapped RBCs (arrow) with vessel wall and surrounding interstitial edema and a consolidated ischemic glomerular appearance with potential entrapped RBCs (arrowhead).

B and C)

Glomerular arteriole (b, silver stain) shows lamination of the vessel wall and fibrinoid material entrapped within the vessel wall (Masson's trichrome).

D)

Small artery with subintimal mucoid change (HE)

E) Glomerulus (PAS) with synechial adhesion and associated glomerular solidification

F) Foci of interstitial inflammation including eosinophils (HE)

G) Ultrastructural evidence of microvascular injury including subendothelal widening with prominent cellular interposition.