ASN's Mission

ASN leads the fight to prevent, treat, and cure kidney diseases throughout the world by educating health professionals and scientists, advancing research and innovation, communicating new knowledge, and advocating for the highest quality care for patients.

learn more

Contact ASN

1401 H St, NW, Ste 900, Washington, DC 20005

email@asn-online.org

202-640-4660

The Latest on Twitter

Kidney Week

Abstract: TH-PO140

AKI Secondary to Thrombotic Microangiopathy (TMA) in a Myeloma Patient: Scleroderma Renal Crisis (SRC), A Paraneoplastic Phenomenon

Session Information

Category: Trainee Case Report

  • 103 AKI: Mechanisms

Authors

  • Visrodia, Parth, Albany Medical Center, Albany, New York, United States
  • Maheshwari, Ana, Albany Medical Center, Albany, New York, United States
  • Peredo, Ruben A., Albany Medical Center, Albany, New York, United States
  • Hongalgi, Krishnakumar D., Albany Medical Center, Albany, New York, United States
Introduction

AKI due to TMA in a myeloma patient is an uncommon presentation. Rheumatic paraneoplastic syndromes have atypical presentations and are typically a result of anti-tumor immune responses. These subsets of diseases occur simultaneously or in close temporal relation to the diagnosis of an underlying malignancy. Our case report describes scleroderma as a likely cause of AKI/TMA and a paraneoplastic manifestation of multiple myeloma.

Case Description

A 47-year-old man with no prior renal disease presented with peripheral neuropathy, hypertensive urgency, seizures, microangiopathic hemolytic anemia (MAHA), and acute renal failure. AKI work up revealed paraproteinemia and subsequent bone marrow biopsy confirmed multiple myeloma (MM). On examination, he was found to have findings suspicious for scleroderma including telangiectasia, limited cutaneous thickening, salt-and-pepper skin changes, and abnormal nailfold capillaroscopy. CT Abdomen revealed distal esophageal and rectal thickening and esophagram showed esophageal dysmotility. Worsening kidney function, new skin findings, and MAHA lead to a kidney biopsy, which demonstrated findings of severe thrombotic microangiopathy (TMA) with no evidence of cast nephropathy. Deep skin biopsy was compatible with papillary and mid dermal sclerosis. Although autoantibodies were negative, patient was diagnosed clinically with scleroderma with scleroderma renal crisis (SRC) which appeared to be a paraneoplastic presentation of MM. Patient was treated with captopril initially for SRC and subsequently Eculizumab. Interestingly, his skin findings have not progressed after treatment for MM but remains dialysis dependent.

Discussion

AKI due to TMA in a patient with MM and SRC is a diagnostic dilemma. Paraproteins are associated with mimics of rheumatic diseases; however, there are rare reports on the coexistence of MM and paraneoplastic scleroderma. TMA is known to be associated with both MM and SRC, and although the clinical presentation was suggestive of scleroderma, the negative serologies challenged the diagnosis. Further studies are required to determine whether MM can induce SRC and if chemotherapy can improve scleroderma outcomes in these patients.