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Abstract: FR-PO609

Old Friend in Camouflage: Polyarteritis Nodosa With Thrombotic Microangiopathy

Session Information

Category: Glomerular Diseases

  • 1302 Glomerular Diseases: Immunology and Inflammation

Authors

  • ElSheikhMohammed, Waleed A., Medical University of South Carolina, Charleston, South Carolina, United States
  • Pisoni, Roberto, Medical University of South Carolina, Charleston, South Carolina, United States
  • Campbell, Ruth C., Medical University of South Carolina, Charleston, South Carolina, United States
Introduction

PAN with TMA and AKI is an uncommon presentation rarely described in the literature. We report two cases below.

Case Description

1) 33 male with HTN presented to ER with abdominal pain, HTN urgency and severe anemia 6 g/dl. Abdominal CT revealed a liver hematoma requiring embolization. He represented in 2 weeks with HTN emergency, AKI and new thrombocytopenia 60 K/uL. U/A no RBC,UPCR 0.5 g/g. Schistocytes present on peripheral smear, LDH elevated and haptoglobin low. PLEX was urgently started with steroids. Shiga toxin, anti-factor H Ab, ADAMTS13, HBsAg and HCV Ab were normal/negative. ANA 1: 1280 + anti SSA but C3, C4, ANCA, cryoglobulins and anti-GBM all negative. Lymph node biopsy unremarkable. Kidney biopsy showed TMA with negative IF and no immune complex deposits. Colonoscopy showed multifocal areas of ischemia concerning for vasculitis. A diagnosis of PAN was made. Cyclophosphamide was added. Hematological markers improved but kidney function did not. He remained HD dependent. 2) 57 female presented with anasarca, Raynaud’s, HTN, anemia and AKI peak creatinine 2mg/dl with UPCR 0.3 g/g and albumin 3.7 g/L. Liver biopsy showed nodular regenerative hyperplasia. Viral hepatitides negative. Immunology + ANA 1:160, otherwise negative. Kidney biopsy revealed TMA. Patient was placed on ARB and underwent salpingoopherectomy for potential Meigs syndrome with no improvement in ascites, further complicated by spontaneous diverticular rupture. Conventional angiogram assessing for PAN was positive. Induction with steroids and cyclophosphamide followed with good clinical response. Creatinine at last follow up 1.2mg/dl.

Discussion

PAN first described in 1866 by Kussmaul and Maier has evolved from encompassing most vasculitides to a subset characterized by necrotizing inflammation of medium arteries. Its incidence and prevalence have declined due to improved hepatitis B prevention and vaccination. PAN may occur in the absence of hepatitis B. Kidneys are involved in 26-43% of cases and new or worsening HTN is a feature of renal involvement. Malignant HTN with TMA as presenting feature is reported only in a French case. Recognizing PAN as potential cause of TMA can be lifesaving as untreated, its 5-year survival is only 13%. CT angiography may be diagnostic but findings can be subtle. Renal failure requiring permanent dialysis is not common in PAN and impacts prognosis.