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Abstract: SA-PO666

A Rare Cause of Thrombotic Microangiopathy (TMA)

Session Information

Category: Glomerular Diseases

  • 1302 Glomerular Diseases: Immunology and Inflammation

Authors

  • Kethineni, Rama, University of Utah Health, Salt Lake City, Utah, United States
  • Gilligan, Sarah, University of Utah Health, Salt Lake City, Utah, United States
  • Abraham, Josephine, University of Utah Health, Salt Lake City, Utah, United States
Introduction

TMA can occur secondary to systemic autoimmune disorders such as SLE, Scleroderma, and APS. We present a case of TMA secondary to autoimmune myositis.

Case Description

A 48-year-old Hispanic female with a history of hypertension presented with headache, blurry vision, chest pain and dyspnea, with blood pressure of 230/140 mm hg. Her creatinine worsened from 0.8 mg/dl to 2.6 mg/dl with proteinuria of 1 g/g within a week despite improvement in blood pressure. Her labs were notable for microscopic hematuria and Platelets of 96k/ul and hemoglobin 10 g/dl. She had a troponin-I of 0.26 ng/ml, CK of 995 U/L and an ECHO with EF of 43%. Serologic work-up showed positive ANA of 1:1280 with speckled pattern, SSA-60 of 76 with negative SLE and Systemic sclerosis panel. Her hemolysis labs and SPEP were normal.

Her cardiac MRI showed findings of myocarditis. Renal biopsy showed TMA with scattered subendothelial immune complex deposits and acute tubular injury. Immunofluorescence showed show scant capillary and mesangial staining for predominantly IgM and C1q, with scant staining for other antibodies. Creatinine worsened to 11.5 mg/dl over the course of 5 weeks and dialysis was initiated.

Two months later she presented with fatigue, weakness, and diffuse muscle pain with CK of 4000 U/L and muscle biopsy showing necrotizing myopathy. She was started on high dose steroids and Cellcept with improvement of CK level. Her clinical course was complicated by bone marrow suppression, CMV viremia, and HLH with elevated ferritin of 25,527, SIL2r elevation of 19K with no response to Anakinra. She had worsening hemodynamic instability and ultimately passed away after her family opted to withdraw care.

Discussion

Distinguishing and treating of various causes of TMA can be very challenging especially when limited to the kidney. TMA frequently presents with hypertension, making it difficult to distinguish malignant hypertension from other causes. Literature on autoimmune myositis and TMA is rare. It can be very challenging to diagnose if TMA is caused early in the course of the disease as in our case.