Primary biliary cirrhosis with refractory hypokalemia

Abstract

Rationale:Renal tubular acidosis (RTA) represents a class of metabolic disorders characterized by metabolic acidosis with a normal plasma anion gap. As a rare complication of primary biliary cirrhosis (PBC), RTA is easily overlooked, likely leading to misdiagnosis.Patient concerns:A 32-year-old woman who had been diagnosed with PBC at our hospital was found to have hypokalemia due to repeated fatigue for 2 years, and the etiology was unknown.Diagnoses:Due to the laboratory test results, radiographic findings, and pathologic results, she was diagnosed with PBC associated with RTA.Interventions:She was then treated with ursodeoxycholic acid, potassium citrate, and calcium supplements together with activated vitamin D.Outcomes:Thus far, the patient showed a good response to ursodeoxycholic acid, and the clinical symptoms and liver function were significantly improved.Lessons:Physicians that encounter refractory hypokalemia in a patient with PBC should be aware of the presence of RTA. The early diagnosis and treatment of such patients are of paramount importance to alleviate clinical symptoms and delay disease progression.

Distal Renal Tubular Acidosis in Patients with Autoimmune Diseases—An Update on Pathogenesis, Clinical Presentation and Therapeutic Strategies

“…Aiming for a better understanding of the disease, we performed a search of the PubMed database using MeSH descriptors (Acidosis, Renal Tubular; Lupus Erythematosus, Systemic; Sjogren’s Syndrome, Systemic Vasculitis, Rheumatoid Vasculitis, Arthritis, Rheumatoid, Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis, Churg-Strauss Syndrome, IgA Vasculitis, Spondylitis, Ankylosing, Cryoglobulins, Hepatitis, Autoimmune, Liver Cirrhosis, Biliary), and we identified 37 individual case reports published since December 2016 with the association of distal renal tubular acidosis and the following autoimmune disorders: SLE, Sjögren’s syndrome, autoimmune hepatitis, primary biliary cirrhosis and rheumatoid arthritis (see Table 1 ) [ 6 , 10 , 11 , 13 , 15 , 16 , 17 , 18 , 20 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 ]. We also identified a case of hereditary autoimmune polyendocrine syndrome (DRTA, hypoparathyroidism, Addison disease), published in a series of cases, which was not included in our analysis, due to the lack of individual data availability [ 39 ].…”

“…In only one case out of these 33 reports, cyclophosphamide was used, with eGFR improvement and potassium value amelioration [ 33 ]. Immunosuppressive therapy was not used in those three cases of autoimmune hepatitis, primary biliary cirrhosis and rheumatoid arthritis included in our analysis [ 36 , 37 ]. A meta-analysis published in 2021 which included patients with renal tubular acidosis and liver pathology (autoimmune hepatitis and alcoholic hepatitis) showed that corticosteroids improved short term mortality in this group, but it is not mentioned any effect on the evolution of DRTA [ 55 ].…”