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Abstract: FR-PO672

Hydrocarbons to Pulmonary Renal Syndrome: A Risky Road

Session Information

Category: Glomerular Diseases

  • 1303 Glomerular Diseases: Clinical‚ Outcomes‚ and Trials

Authors

  • Morales-Alvarez, Martha Catalina, Beth Israel Deaconess Medical Center, Boston, Massachusetts, United States
  • Eley, Samuel Theodore, Harvard Medical School, Boston, Massachusetts, United States
  • Stillman, Isaac Ely, Beth Israel Deaconess Medical Center, Boston, Massachusetts, United States
  • Chopra, Bhavna, Beth Israel Deaconess Medical Center, Boston, Massachusetts, United States
Introduction

Anti-GBM syndrome is rare small vessel vasculitis with an incidence of <2 cases/million population and a bimodal age distribution in the third and sixth decades of life. Environmental factors such as URIs, smoking, and inhalation of hydrocarbons in genetically susceptible individuals are associated with increased anti-GBM antibodies production. We present a rare case of a young patient with Toluene exposure manifesting anti-GBM syndrome in a background of PLA2R membranous nephropathy (MN).

Case Description

A 23-year-old man with new onset HTN and headaches presented to the ED with hemoptysis for 2 days. He was hemodynamically stable with normal oxygen saturation. Initial laboratory results revealed a Hb of 5.7 g/dL and SCr of 19.7 mg/dL. CXR revealed bilateral diffuse infiltrates and urine sediment showed several dysmorphic RBCs. Total urine Pr/Cr ratio was 6.2 g/g. Due to concern for pulmonary-renal syndrome with diffuse alveolar hemorrhage and hypoxic respiratory failure he was initiated on pulse dose steroids, plasmapheresis (total x5), and hemodialysis within 1 day of admission. Serologies returned positive only for anti-GBM antibodies. A kidney biopsy showed active crescentic GN with minimal IFTA and granular staining of PLA2R in the capillary loops by IF (Fig 1). Oral cyclophosphamide was initiated on day 5. He reported repeated high exposure to toluene since childhood while repairing bikes. During a 3-mo follow up, he was still HD dependent.

Discussion

This case exhibits a unique presentation of anti-GBM syndrome following repeated hydrocarbon exposure in a setting of underlying MN. Inhaled toxins promote recurrent localized inflammation and increased capillary permeability unmasking hidden epitopes and allowing antibody formation, potentially manifesting as anti-GBM syndrome. Early diagnosis and appropriate use of immunosuppressive and extracorporeal therapies is necessary to prevent morbidity and to improve survival of this rare condition.