Luis R. Álvarez-Velazquez, Servicio de Nefrologia, Hospital General de Zona No 83, Morelia, Michoacán, Mexico
Luis R. Álvarez-Bribiesca, Servicio Urgencias, Cínica Instituto de Seguridad y Servicios Sociales de los Trabajadores del Estado (ISSSTE) La Piedad. Michoacán, Mexico
Primary biliary cholangitis (PBC) is a chronic autoimmune cholestatic liver disease in which extrahepatic renal involvement is uncommon. We describe a woman with non–anion-gap metabolic acidosis and inappropriately alkaline urine. Urinary indices supported a diagnosis of distal renal tubular acidosis (dRTA) in the context of PBC, and a structured evaluation excluded other causes of distal acidification defects. Management combined alkali therapy with electrolyte monitoring and disease-directed treatment. This case highlights when to suspect dRTA in cholestatic autoimmunity, offers a pragmatic diagnostic approach, and summarizes key therapeutic principles. Greater awareness of PBC-associated dRTA may facilitate earlier recognition, multidisciplinary care, and individualized follow-up.
Keywords: Primary biliary cholangitis. Distal renal tubular acidosis. Non�??anion-gap metabolic acidosis. Hypokalaemia. Autoimmunity.