Kidney Week

Abstract: FR-PO0444

Fulminant Multisystem Primary Sjögren Syndrome Presenting with Severe AKI, Extreme Rhabdomyolysis, and Aseptic Meningitis

Session Information

• AKI: Case Reports - TMA, Vasculitis, Immune-Mediated Injury, and Systemic Disease
  October 23, 2026 | Location: Exhibit Hall A, Convention Center
  Abstract Time: 10:00 AM - 12:00 PM

Category: Acute Kidney Injury

• 102 AKI: Clinical, Outcomes, and Trials

Authors

• Elrefy, Omar A., Jefferson Einstein Philadelphia Hospital, Philadelphia, Pennsylvania, United States

Omar A. Elrefy, MD

• Ahmed, Zahoor, Jefferson Einstein Philadelphia Hospital, Philadelphia, Pennsylvania, United States

Zahoor Ahmed, MD

• Anwar, Amna, Jefferson Einstein Philadelphia Hospital, Philadelphia, Pennsylvania, United States

Amna Anwar, MD

• Mubin, Fareeha, Jefferson Einstein Philadelphia Hospital, Philadelphia, Pennsylvania, United States

Fareeha Mubin, MD

• Saini, Manu Krishan Dev, Jefferson Einstein Philadelphia Hospital, Philadelphia, Pennsylvania, United States

Manu Krishan Dev Saini, MD

• Shin, Ji Young, Jefferson Einstein Philadelphia Hospital, Philadelphia, Pennsylvania, United States

Ji Young Shin, MD

• Chewaproug, Daranee, Jefferson Einstein Philadelphia Hospital, Philadelphia, Pennsylvania, United States

Daranee Chewaproug, DO, FASN

• Gupta, Saurabh, Jefferson Einstein Philadelphia Hospital, Philadelphia, Pennsylvania, United States

Saurabh Gupta, MD

Introduction

Sjögren syndrome is an autoimmune disease affecting exocrine glands with systemic involvement in 30–40% of cases. Renal disease occurs in 2.8–10% cases with tubulointerstitial nephritis accounting for most cases, leading to chronic kidney disease, and less commonly glomerular disease associated with cryoglobulinemia.
Neurological involvement, which can precede sicca symptoms, is reported in up to 70% of cases with non-focal or focal central nervous system deficits.
Myositis is rare at 1.28–3%, and extreme CPK elevation without necrotizing autoimmune myopathy is exceptionally uncommon. We present a very rare case of a patient who presented with severe acute kidney injury, rhabdomyolysis, and aseptic meningitis, and was found to have primary Sjogren syndrome.

Case Description

An 18-year-old woman with no prior autoimmune disease presented with altered mental status, aseptic meningitis, and severe rhabdomyolysis with profound muscle weakness. She developed acute kidney injury with a peak creatinine of 3.5 mg/dL and CPK of 250,633 IU/L, progressing to anuric renal failure. Workup revealed positive ANA (1:160), anti–SSA/SSB, and anti–smooth muscle antibodies, with otherwise negative infectious, immune, and myositis panels. Kidney biopsy showed interstitial nephritis with edema and superimposed acute tubular necrosis, while muscle biopsy was inconclusive without necrosis. She required continuous venovenous hemodialysis transitioned to intermittent hemodialysis and was started on an oral prednisone 60 mg. She achieved full renal recovery and marked improvement in muscle injury, allowing dialysis discontinuation within two weeks.

Discussion

This case highlights an exceptionally rare multisystem presentation of primary Sjögren syndrome with aseptic meningitis, severe rhabdomyolysis, and biopsy-proven tubulointerstitial nephritis with ATN.
Sjögren syndrome without sicca symptoms is typically seen in young patients with strong serologic positivity.
Despite the initial concern for rhabdomyolysis-induced ATN, kidney biopsy revealed interstitial nephritis with superimposed ATN, guiding corticosteroid therapy and resulting in rapid renal recovery, highlighting its importance in this clinical context.
Myoglobin is poorly cleared by iHD; CVVHD with a high-cutoff dialyzer is most effective, while high-flux modalities with ~24-hour filter exchange are reasonable alternatives.