Abstract: TH-PO186

Unusual Cause of Visceral Infarcts

Session Information

Category: Nephrology Education

  • 1302 Fellows and Residents Case Reports

Authors

  • Pandit, Amar, Westchester Medical Center, White Plains, New York, United States
  • Pawaskar, Aditya Sanjeev, Westchester Medical Center, White Plains, New York, United States
  • Goldberg, Randy A., New York Medical College , Valhalla, New York, United States
Background

Thrombosis is an increasingly recognized feature of ANCA vasculitis

Methods

An 18 year old lady with history of celiac disease presented with high grade fevers, 10 lb. weight loss, nausea and epigastric tenderness. Physical exam was negative for lymphadenopathy, murmur, adventitious lung sounds, hepatosplenomegaly, joint swelling/tenderness or rash. Labs revealed anemia, elevated ESR and CRP and normal white count, renal and liver function tests. Infectious vs. inflammatory processes were considered and she was started on antibiotics. CT abdomen revealed renal and splenic infarcts. Infective endocarditis was ruled out by negative blood cultures and a normal echocardiogram, following which antibiotics were stopped.
Urinalysis showed microscopic hematuria and 2+ proteinuria (Urine protein/creatinine ratio of 961), consistent with nephritic syndrome, likely due to collagen vascular disease vs. thrombotic disease. ANA and anti-PR3 were positive, and dsDNA, anti-Sm, anti-RNP, C3, C4, cryoglobulins, anti-GBM, anti-SS-A and anti-SS-B were negative. Hypercoagulable workup revealed decreased Protein S activity and negative anti-phospholipid antibodies. CT chest revealed a hemorrhagic infarct, but no nodules. Renal biopsy confirmed the presence of pauci-immune necrotizing crescentic glomerulonephritis, indicating GPA. She was started on IV methylprednisolone, rituximab and rivaroxaban with resolution of her symptoms.

Conclusion

Venous (DVT, PE) and arterial thrombosis are being increasingly recognized in GPA, although arterial involvement has predominantly been described in the coronaries. This predisposition to thrombosis is multifactorial: 1) Activation of neutrophils by antibodies and the interaction of activated neutrophils with the endothelium. There is also release of tissue factor which activates thrombosis. 2) Elevated thrombomodulin levels activate thrombosis and inhibition of conversion of plasminogen to plasmin inhibits fibrinolysis. There are no recommendations on use of anti-platelets or anti-coagulants at present.