Abstract
We report the case of a 48-year-old male with a history of pulmonary and ocular sarcoidosis. Non-caseating granulomas, identified histologically, are the most characteristic manifestation of sarcoidosis. Hepatic sarcoidosis is difficult to diagnose using radiological imaging. In the patient reported in this study, ultrasound and contrast-enhanced computed tomography scans identified multiple intra-abdominal lymphadenopathies, with evidence of liver and splenic infiltrations. The first liver biopsy revealed non-caseating granulomatous hepatitis consistent with hepatic sarcoidosis. The patient was treated with ursodeoxycholic acid (UDCA), but his laboratory parameters did not improve. Prednisone was initiated at a dose of 30 mg daily and slowly tapered. At a dose of 12.5 mg daily, marked improvements in the fibrotic and sarcoid-like lesions were noted at the second biopsy. A third biopsy was performed, with the patient on a prednisone taper of 5 mg/day showed mild fibrous expansion in the portal tracts and mild parenchymal necro-inflammatory lesions. However, overall, fibrosis marker levels remained stable over the course of treatment. A fourth biopsy was performed after a 5-year course of 5 mg/day prednisone. This revealed minimal lobular inflammation without fibrosis. Thus, treatment of this patient with corticosteroids and UDCA resulted in marked improvements in his biochemical and histological parameters.
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References
Hattori T, Konno S, Shijubo N, et al. Nationwide survey on the organ-specific prevalence and its interaction with sarcoidosis in Japan. Sci Rep. 2018;8:9440.
Deutsch-Link S, Fortuna D, Weinberg EM. A comprehensive review of hepatic sarcoid. Semin Liver Dis. 2018;38:284–97.
Morimoto T, Azuma A, Abe S, et al. Epidemiology of sarcoidosis in Japan. Eur Respir J. 2008;31:372–9.
Karagiannidis A, Karavalaki M, Koulaouzidis A. Hepatic sarcoidosis. Ann Hepatol. 2006;5:251–6.
Studdy P, Bird R, James DG. Serum angiotensin-converting enzyme (SACE) in sarcoidosis and other granulomatous disorders. Lancet. 1978;2:1331–4.
Cremers J, Drent M, Driessen A, et al. Liver-test abnormalities in sarcoidosis. Eur J Gastroenterol Hepatol. 2012;24:17–24.
Ayyala US, Padilla ML. Diagnosis and treatment of hepatic sarcoidosis. Curr Treat Opt Gastroenterol. 2006;9:475–83.
Baughman RP, Valeyre D, Korsten P, et al. ERS clinical practice guidelines on treatment of sarcoidosis. Eur Respir J. 2021;58.
Putra J, Toor A, Suriawinata AA. The utility of repeat liver biopsy in autoimmune hepatitis: a series of 20 consecutive cases. Pathology. 2016;48:449–53.
Patel K, Sebastiani G. Limitations of non-invasive tests for assessment of liver fibrosis. JHEP Rep. 2020;2: 100067.
Atzori SM, Pasha Y, Maurice JB, et al. Prospective evaluation of liver shearwave elastography measurements with 3 different technologies and same day liver biopsy in patients with chronic liver disease. Dig Liver Dis. 2023.
Graf C, Arncken J, Lange CM, et al. Hepatic sarcoidosis: clinical characteristics and outcome. JHEP Rep. 2021;3: 100360.
Ennaifer R, Ayadi S, Romdhane H, et al. Hepatic sarcoidosis: a case series. Pan Afr Med J. 2016;24:209.
Yoshiji H, Kitagawa K, Noguchi R, et al. A histologically proven case of progressive liver sarcoidosis with variceal rupture. World J Hepatol. 2011;3:271–4.
Bhargava P, Vaidya S, Kolokythas O, et al. Pictorial review. Hepatic vascular shunts: embryology and imaging appearances. Br J Radiol. 2011;84:1142–52.
Iwakiri Y. Pathophysiology of portal hypertension. Clin Liver Dis. 2014;18:281–91.
Moller DR. Treatment of sarcoidosis—from a basic science point of view. J Intern Med. 2003;253:31–40.
Czaja AJ, Carpenter HA. Decreased fibrosis during corticosteroid therapy of autoimmune hepatitis. J Hepatol. 2004;40:646–52.
Imoto K, Kohjima M, Hioki T, et al. Clinical Features of liver injury induced by immune checkpoint inhibitors in Japanese patients. Can J Gastroenterol Hepatol. 2019;2019:6391712.
Tadros M, Forouhar F, Wu GY. Hepatic sarcoidosis. J Clin Transl Hepatol. 2013;1:87–93.
Vannozzi G, Tozzi A, Chibbaro G, et al. Hepatic and mesenteric sarcoidosis without thoracic involvement: a case of severe noncirrhotic portal hypertension and successful pregnancy. Eur J Gastroenterol Hepatol. 2008;20:1032–5.
Cengiz C, Rodriguez-Davalos M, deBoccardo G, et al. Recurrent hepatic sarcoidosis post-liver transplantation manifesting with severe hypercalcemia: a case report and review of the literature. Liver Transpl. 2005;11:1611–4.
Bakker GJ, Haan YC, Maillette de Buy Wenniger LJ, et al. Sarcoidosis of the liver: to treat or not to treat? Neth J Med. 2012;70:349–56.
Amber KT, Bloom R, Mrowietz U, et al. TNF-alpha: a treatment target or cause of sarcoidosis? J Eur Acad Dermatol Venereol. 2015;29:2104–11.
Keely SJ, Steer CJ, Lajczak-McGinley NK. Ursodeoxycholic acid: a promising therapeutic target for inflammatory bowel diseases? Am J Physiol Gastrointest Liver Physiol. 2019;317:G872–81.
Ko WK, Lee SH, Kim SJ, et al. Anti-inflammatory effects of ursodeoxycholic acid by lipopolysaccharide-stimulated inflammatory responses in RAW 264.7 macrophages. Plos One. 2017;12:e0180673.
Angulo P. Use of ursodeoxycholic acid in patients with liver disease. Curr Gastroenterol Rep. 2002;4:37–44.
Ungprasert P, Crowson CS, Simonetto DA, et al. Clinical characteristics and outcome of hepatic sarcoidosis: a population-based study 1976–2013. Am J Gastroenterol. 2017;112:1556–63.
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The authors would like to thank the Department of Gastroenterology and Hepatology and the Department of Pathology at Nara Medical University. The authors would also like to thank Enago for the English language editing.
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12328_2023_1918_MOESM1_ESM.pptx
Fig. S1 (A) Chronological course of contrast-enhanced abdominal computed tomography of a 48-year-old Japanese man with hepatic sarcoidosis. The liver was grossly enlarged with numerous low-contrast nodules in the left and right lobes. The spleen was markedly enlarged and multiple hypointense nodules were apparent after contrast injection. (B) Chronological course of computed tomography of the lung of a 48-year-old Japanese man with hepatic sarcoidosis (PPTX 4198 KB)
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Kikuchi, M., Koizumi, A., Namisaki, T. et al. Improvement of liver histology in hepatic sarcoidosis due to treatment with corticosteroids and ursodeoxycholic acid: a case report. Clin J Gastroenterol 17, 327–333 (2024). https://doi.org/10.1007/s12328-023-01918-3
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DOI: https://doi.org/10.1007/s12328-023-01918-3