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Kidney Week

Abstract: FR-PO0757

Pulmonary-Renal Syndrome from Double-Seropositive Vasculitis with Anti-Myeloperoxidase (MPO) and Anti-GBM Antibodies: A Case Report from a Hospital in Guadalajara, Mexico

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics

Authors

  • Bejarano Flores, Andrea Carolina, Instituto Mexicano del Seguro Social, Guadalajara, Jalisco, Mexico
  • Aguilar, Brenda, Instituto Mexicano del Seguro Social, Guadalajara, Jalisco, Mexico
Introduction

Pulmonary renal syndrome is a life-threatening condition which includes the combination of diffuse alveolar haemorrhage and rapidly progressive glomerulonephritis. ANCA positive vasculitis and anti-GBM disease are the most common etiologies. The coexistence of these two conditions occurs in 30% of patients with PRS, however, report cases in Mexico are scarce

Case Description

We present a 65 year-old male, resident of Gualadajara, Mexico, with a 5-year history of systemic hypertension who attended the emergency room with abdominal pain, macroscopic hematuria and nonproductive cough for the last 3 weeks. He denied fever, dermic lesions, dyspnea or edema. Laboratory test results were as follows: hemoglobin, 8.6gr/dL; serum creatinine, 15.1mg/dL (previously of 0.83mg/dL in 2023); blood urea nitrogen, 149mg/dL; and serum potassium, 6.2mmol/L. Urinalysis revealed proteinuria (2+), hematuria, dysmorphic red blood cells and leucocytes in the urinary sediment. Heymodialysis was initiated shortly after hospitalization due to hyperkalemia and metabolic acidosis. During the hospitalization the patient presented bloody sputum, which increased in the following 48 hours. We requested a thoracic CT Scan that suggested diffuse alveolar haemorrhage. ANCA anti-MPO became positive, so we decided to perform a kidney biopsy and initiated empiric immunosuppressive management with intravenous pulses of methylprednisolone and cyclophosphamide. However, the poor clinical outcome and respiratory conditions restricted the procedure. 24 hours later, we obtained the result of anti-GMB antibodies, which were positive. The patient was transferred to the intensive care unit for receiving therapeutic plasma exchange on two occasions. Nonetheless, he progressed negatively with massive pulmonary hemorrhage, requiring advanced airway management. 72 hours later, he presented a cardiac arrest, without any return of spontaneous circulation despite advanced cardiopulmonary resuscitation maneuvers

Discussion

Dual positivity for anti-GBM and ANCA antibodies demonstrates the critical nature of the disease. The specific autoimmune phenotype combines features of both entities and requires an aggressive and individualized approach. Although kidney biopsy and serology confirms the diagnosis, treatment should not be delayed when pulmonary and renal involvement are present

Acknowledgment

Chávez-Durán, Alejandro; Parra-Michel, Renato; Reséndez-González, Rosa María