INTRODUCTION: Sarcoidosis is a non-caseating, granulomatous disease of incompletely understood aetiology that can affect nearly all organs including the liver. Hepatic involvement is thought to occur in 50-90% of patients but may remain undiagnosed in many cases. Evidence-based guidelines for the treatment of sarcoidosis of the liver are lacking. Patients usually receive no treatment or are treated pragmatically with corticosteroids. However, treatment with systemic corticosteroids has had mixed results. The use of ursodeoxycholic acid (UDCA) in the treatment of sarcoidosis-associated cholestasis has been reported by several groups, and is empirically prescribed to sarcoidosis patients with hepatic involvement. METHODS: The effect of UDCA on symptoms and serum liver tests was investigated in a retrospective cohort study in which hepatic sarcoidosis patients had received either no treatment, prednisolone treatment or UDCA treatment. For all patients, laboratory results on ASAT, ALAT, AP and GGT were collected. Patients described the severity of their symptoms before and after treatment on a numerical scale. RESULTS: A total of 17 patients participated in the study. Serum liver tests in the group treated with UDCA had improved as compared with the other groups. Also, symptomatic improvement of pruritus and fatigue was reported in the group treated with UDCA. CONCLUSION: This retrospective cohort study supports the empirical first-line use of UDCA in the treatment of sarcoidosis of the liver, especially in symptomatic patients. Prospective randomised trials are needed to adequately support this concept.
INTRODUCTION:Sarcoidosis is a non-caseating, granulomatous disease of incompletely understood aetiology that can affect nearly all organs including the liver. Hepatic involvement is thought to occur in 50-90% of patients but may remain undiagnosed in many cases. Evidence-based guidelines for the treatment of sarcoidosis of the liver are lacking. Patients usually receive no treatment or are treated pragmatically with corticosteroids. However, treatment with systemic corticosteroids has had mixed results. The use of ursodeoxycholic acid (UDCA) in the treatment of sarcoidosis-associated cholestasis has been reported by several groups, and is empirically prescribed to sarcoidosispatients with hepatic involvement. METHODS: The effect of UDCA on symptoms and serum liver tests was investigated in a retrospective cohort study in which hepatic sarcoidosispatients had received either no treatment, prednisolone treatment or UDCA treatment. For all patients, laboratory results on ASAT, ALAT, AP and GGT were collected. Patients described the severity of their symptoms before and after treatment on a numerical scale. RESULTS: A total of 17 patients participated in the study. Serum liver tests in the group treated with UDCA had improved as compared with the other groups. Also, symptomatic improvement of pruritus and fatigue was reported in the group treated with UDCA. CONCLUSION: This retrospective cohort study supports the empirical first-line use of UDCA in the treatment of sarcoidosis of the liver, especially in symptomatic patients. Prospective randomised trials are needed to adequately support this concept.
Authors: Elliott D Crouser; Lisa A Maier; Kevin C Wilson; Catherine A Bonham; Adam S Morgenthau; Karen C Patterson; Eric Abston; Richard C Bernstein; Ron Blankstein; Edward S Chen; Daniel A Culver; Wonder Drake; Marjolein Drent; Alicia K Gerke; Michael Ghobrial; Praveen Govender; Nabeel Hamzeh; W Ennis James; Marc A Judson; Liz Kellermeyer; Shandra Knight; Laura L Koth; Venerino Poletti; Subha V Raman; Melissa H Tukey; Gloria E Westney; Robert P Baughman Journal: Am J Respir Crit Care Med Date: 2020-04-15 Impact factor: 21.405