Patients with hepatitis C and inherited bleeding disorders frequently developed end-stage liver disease, with advanced age, HIV coinfection and alcohol abuse significantly increasing their risk in a recent study.
Researchers retrospectively analyzed data from 863 patients with HCV and inherited bleeding disorders during a median follow-up of 31.4 years from HCV infection. Seventy-six percent of the cohort (94% men) had hemophilia A, and 25% of patients were coinfected with HIV.
Spontaneous HCV clearance (positive anti-HCV without HCV RNA on at least two assessments without antiviral therapy) occurred in 19% of patients. Eighty-one percent developed chronic HCV, and of those, 13% later developed end-stage liver disease (ESLD). Three percent developed hepatocellular carcinoma, 41% of which occurred during the final 6 years of analysis.
Antiviral therapy was administered in 361 cases; 53% of patients achieved sustained virologic response. At completion of follow-up, 487 patients survived, and 49% did not receive optimal conventional antiviral therapy. Death was due to liver-related causes in 28% of cases, with liver-related mortality occurring more commonly in untreated patients (28%) and those treated unsuccessfully (44%) than patients with successful therapy (0%) or spontaneous clearance (4%).
Factors associated with ESLD included age at infection (HR=1.09; 95% CI, 1.07-1.11 per year increase), HIV coinfection (HR=10.85; 95% CI, 6.06-19.44), history of alcohol abuse (HR=4.34; 95% CI, 2.33-8.06) and successful antiviral therapy (HR=0.14; 95% CI, 0.03-0.58).
“The increasing number of patients diagnosed with ESLD and the large beneficial effect of successful antiviral treatment on reducing ESLD risk underline the importance of careful follow-up of the extent of liver damage and timely consideration of antiviral treatment in HCV-infected patients with inherited bleeding disorders,” the researchers wrote. “In many patients who previously declined treatment or who underwent unsuccessful treatment in the past, treatment options could be reconsidered in light of the availability of new treatment regimens with increasing success rates.”
Disclosure: The researchers report no relevant financial disclosures.