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Kidney Week

Abstract: PO0296

Use of Eculizumab in Thrombotic Microangiopathy (TMA) Associated with PM/SCL100 and 75 and RP 155 Antibody-Positive Autoimmune Overlap Syndrome with Renal Crisis

Session Information

Category: Acute Kidney Injury

  • 102 AKI: Clinical, Outcomes, and Trials

Authors

  • Khan, Jahanzeb, Baptist Health Medical Center - North Little Rock, North Little Rock, Arkansas, United States
  • Mousa, Heba, Baptist Health Medical Center - North Little Rock, North Little Rock, Arkansas, United States
  • Sarwar, Mujtaba, Baptist Health Medical Center - North Little Rock, North Little Rock, Arkansas, United States
  • Bachu, Ramya, Baptist Health Medical Center - North Little Rock, North Little Rock, Arkansas, United States
  • Syed, Anum, Baptist Health Medical Center - North Little Rock, North Little Rock, Arkansas, United States
  • Bukhari, Sehrish W., Kidney Care Center, Little Rock, Arkansas, United States
  • Ijaz, Fakhar, Baptist Health Medical Center - North Little Rock, North Little Rock, Arkansas, United States
Introduction

TMA with associated PM/SCL100 & 75 and RP 155 antibody positive Autoimmune Overlap Syndrome with renal crisis has a very poor prognosis and limited therapeutic options. Use of Eculizumab has been reported in only a few cases in literature.

Case Description

A 44 years old female with hypertension and Raynaud's phenomenon presented with hypertensive emergency, recent sclerodactyly, hemolytic anemia, thrombocytopenia and renal failure. Routine serum markers including DsDNA Ab, complements, ANCA, SSA, SSB, Scl 70 Ab, Anti-centromere Ab, RNA polymerase 3 Ab, U-3 RNP Ab, lupus anticoagulant, beta 2 glycoprotein Ab and ADAMTS-13 were all negative. Pulse Steroids and plasmapheresis were initiated due to concern of aHUS. Renal biopsy showed onion skinning of arterioles with near complete occlusion of arterioles as shown in the figure. ACEI was added. Later she was found to have positive PM/SCL 100 & 75 and RP 155 antibodies. She was started on Eculizumab secondary to poor hematological response to the above measures. Our patient showed improvement of her thrombotic microangiopathy which helps to support the use of this drug in this rare disorder.

Discussion

Very limited theraputic options are available for cases of Autoimmune Overlap Syndrome. Our use of Eculizumab; an anti-CD5 monoclonal antibody, also supports the benefits of blocking the activation of the classical complement pathyway which may suggest the underline mechanism in this disease process.