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Kidney Week

Abstract: SA-PO671

Rapidly Progressive Kidney Failure in a Patient with Longstanding Juvenile Dermatomyositis

Session Information

  • Pediatric Nephrology - 2
    October 26, 2024 | Location: Exhibit Hall, Convention Center
    Abstract Time: 10:00 AM - 12:00 PM

Category: Pediatric Nephrology

  • 1900 Pediatric Nephrology

Authors

  • Rogal, Sarah, Children's National Hospital, Washington, District of Columbia, United States
  • Kwon, Donghyang, MedStar Georgetown University Hospital, Washington, District of Columbia, United States
  • Ahn, Sun-Young, Children's National Hospital, Washington, District of Columbia, United States
Introduction

Juvenile dermatomyositis (JDM) is an inflammatory myopathy characterized by proximal muscle weakness, heliotrope rash and Gottron papules. Collapsing focal segmental glomerulosclerosis (cFSGS) and glomerulocystic disease have not been previously reported with JDM.

Case Description

A 21 year-old male with JDM (diagnosed 9 years ago), osteoporosis, calcinosis, and restrictive lung disease presented with a 3-week history of left knee and foot pain concerning for cellulitis. He recently discontinued mycophenolic acid and tofacitinib for a low white blood cell count but continued hydroxychloroquine, prednisone, and monthly intravenous immunoglobulin. Initial labs were concerning for serum creatinine of 2.9 mg/dl, increased from baseline of 0.92 mg/dl, serum albumin 1.7 g/dl, and urine protein to creatinine ratio 4.8. An ultrasound showed increased renal echogenicity. C3 was 84(nl>90 mg/dl), C4 and creatinine kinase normal, ANA 1:80, while anti-dsDNA, HIV, hepatitis B and C were negative. His cellulitis was treated with IV clindamycin and cefepime. A kidney biopsy showed cFSGS, glomerulocystic changes, and tubules with microcystic dilatation(Fig 1). Widely scattered immune complex deposits were seen on electron microscopy and IV methylprednisolone was given for 3 days.However, his kidney function continued to worsen leading to the initiation of hemodialysis.

Discussion

Renal involvement in JDM is rare with acute kidney injury secondary to myoglobinuria and nephrotic syndrome reported. Collapsing FSGS has been reported in patients with parvovirus B19 and HIV, both of which were absent in our patient. It has also been observed in patients who received high doses of pamidronate; however, our patient’s cumulative dose(given for osteoporosis) was much lower than the reported cases(300 mg vs. 1170-3960 mg). This is the first report to our knowledge of cFSGS and glomerulocystic features in a patient with JDM. Further investigation is needed to elucidate whether there is a genetic or immunologic predisposition for patients with JDM to develop cFSGS and glomerulocysts.