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Kidney Week

Abstract: PO2205

Anti-MDA5 Dermatomyositis as a Paraneoplastic Syndrome of Myelodysplastic Syndrome After Kidney Transplant in Autosomal Dominant Polycystic Kidney Disease

Session Information

Category: Transplantation

  • 1902 Transplantation: Clinical

Authors

  • Martinez-Vazquez, Belen, Instituto Nacional de Cardiologia Ignacio Chavez, Mexico City, Ciudad de Mexico, Mexico
  • Gomez Johnson, Victor Hugo, Instituto Nacional de Cardiologia Ignacio Chavez, Mexico City, Ciudad de Mexico, Mexico
  • Rodríguez, Francisco, Instituto Nacional de Cardiologia Ignacio Chavez, Mexico City, Ciudad de Mexico, Mexico
Introduction

Malignancy after solid organ transplantation is increasing, there is 3 to 5-fold greater incidence than in general population. Major risk factors for non-cutaneous neoplasms are male sex, older age and Caucasians, there are no data on the prevalence of hematologic malignancies after kidney transplantation. We report a case of kidney allograft infarctions causing acute dysfunction secondary to myelodysplastic syndrome and amyopathic dermatomyositis as a paraneoplastic syndrome (PS).

Case Description

A 40-year-old man with chronic kidney disease (CKD) secondary to autosomal dominant polycystic kidney disease (ADPKD) was programmed for preemptive kidney transplant (KT); basiliximab induction and mycophenolate mofetil (1 year), tacrolimus and steroid was the maintenance therapy. Three years after KT he was admitted to the hospital with AKI (SCr 4.7mg/dL, baseline 1.7mg/dL), unexplained weight loss (17kg) and rash in face, hands and feet. Kidney biopsy showed cortical segmental infarction, focal hypoperfusion with negative C4d. MDA-5, Anti-Jo-1 and PL-7 were positive and Gottron papules were reported in skin biopsy, myopathy was excluded. PS was our conclusion, so PET-CT and BMA were done, with no metabolic activity and hypoplastic myelodysplastic syndrome with high risk for acute myeloid leukemia, respectively.

Discussion

Kidney infarctions were the etiology of AKI as a expression of a hypercoagulable state secondary to amyopathic dermatomyositis (paraneoplastic syndrome) presents and precedes hematologic malignancies in almost 50% cases, awarding poor prognosis at 1, 3 and 5 year with 96.9%, 78.1% and 51.4% overall survival, respectively. Dermatomyositis coexistence with kidney transplant has been described, nevertheless, ADPKD and dermatomyositis is anecdotal. In any case, the diagnosis of Anti-MDA5 dermatomyositis requires ruling out neoplasms.