Abstract: PUB257
Double Trouble or Better Together? Double Antibody-Positive Pulmonary-Renal Syndrome
Session Information
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics
Authors
- Eris, Tansu, Icahn School of Medicine at Mount Sinai, New York, New York, United States
- Liranzo Tejera, Niurka, Icahn School of Medicine at Mount Sinai, New York, New York, United States
- Sedlacek, Martin, Icahn School of Medicine at Mount Sinai, New York, New York, United States
Introduction
Pulmonary-renal syndrome (PRS) is characterized by DAH and RPGN most commonly due to ANCA-associated vasculitis (AAV) and anti-GBM disease. In double-positive PRS, it is more likely that patients have treatable disease than with anti-GBM disease alone.
Case Description
86-year-old woman with MGUS presented from PCP’s office after routine labs revealed severe kidney injury (Cr 0.77 to 8.6 mg/dL over two months). Patient reported dark-colored urine for weeks. She was euvolemic with clear lung sounds bilaterally. UA revealed sheets of RBCs, and a muddy brown cast. Renal ultrasound showed normal-sized kidneys, and increased echogenicity. Anti-GBM antibody was >8 U/mL and anti-MPO antibody was 4.9 U/mL (Table 2). Chest CT was consistent with DAH (Figure 1). DAH was confirmed with bronchoscopy. Kidney biopsy revealed crescentic glomerulonephritis, fibrinoid necrosis, RBC casts, prominent linear capillary loop IgG reactivity. Treatment is urgently initiated (Figure 2). At six-month follow-up, creatinine improved to 2.09 mg/dL.
Discussion
In double positive RRS, it is more likely that patients still have treatable disease for the same creatinine elevation on presentation and significant recovery is possible with prompt diagnosis and treatment.