Improvement of liver involvement in familial Mediterranean fever after introduction of canakinumab: a case report Liver involvement in familial Mediterranean fever
Main Article Content
Keywords
Familial Mediterranean fever, autoinflammation, periodic fever, steatosis, hepatitis, colchicine, interleukin-1, innovative biotechnologies, anakinra, canakinumab, personalized medicine
Abstract
A 44-year-old Jewish woman with familial Mediterranean fever (FMF) developed non-alcoholic steato-hepatitis during colchicine treatment (2,5 mg per day), confirmed by both elastographic study and liver biopsy. A combined therapy with the interleukin-1 (IL-1) blocking agent canakinumab (150 mg every 4 weeks) and colchicine (at a reduced dose of 1.5 mg per day) was started. Three months later transaminases became normal, and six months later there was a marked improvement of liver fibrosis on the elastographic study. Hepatic involvement in FMF occurs ranging from nonspecific increase in liver enzymes to cryptogenic cirrhosis. Liver is mostly involved in patients bearing the homozygous M694V MEFV mutation, as in our case. IL-1 blockade has the power to halt or mitigate liver involvement in FMF patients, though further experience is required to assess its therapeutic potential in the most severe patients with hepatic disease who are partially responsive to long-term colchicine.
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References
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31) Wree A, Eguchi A, McGeough MD, et al. NLRP3 inflammasome activation results in hepatocyte pyroptosis, liver inflammation and fibrosis in mice. Hepatology 2014; 59: 898-910. doi: 10.1002/hep.26592 PMid: 23813842
32) Rimar D, Rosner I, Rozenbaum M, Zuckerman E. Familial Mediterranean fever: an association with non-alcoholic fatty liver disease. Clin Rheumatol 2011; 30: 987-91. doi: 10.1007/s10067-011-1718-1 PMid: 21360101
33) Sarkis C, Caglar E, Ugurlu S, et al. Nonalcoholic fatty liver disease and familial Mediterranean fever: are they related? Srp Arh Celok Lek 2012; 140: 589-94. doi: 10.2298/sarh1210589s PMid: 23289274
34) Lee SS, Park SH. Radiologic evaluation of nonalcoholic fatty liver disease. World J Gastroenterol 2014; 20: 7392-402. doi: 10.3748/wjg.v20.i23.7392 PMid: 24966609
35) Tweezer-Zaks N, Doron-Libner A, Weiss P, et al. Familial Mediterranean fever and cryptogenic cirrhosis. Medicine (Baltimore) 2007; 86: 355-62. doi: 10.1097/MD.0b013e31815be056 PMid: 18004180
36) La Regina M, Ben-Chetrit E, Gasparyan AY, et al. Current trends in colchicine treatment in familial Mediterranean fever. Clin Exp Rheumatol 2013; 31(3 Suppl 77): 41-6. PMid: 24064013
37) Leung YY, Yao Hui LL, Kraus VB. Colchicine-update on mechanisms of action and therapeutic uses. Semin Arthritis Rheum 2015; 45: 341-50. doi: 10.1016/j.semarthrit.2015.06.013 PMid: 26228647
38) Stack J, Ryan J, McCarthy G. Colchicine: new insights to an old drug. Am J Ther 2015; 22(5): e151-e157. doi: 10.1097/01.mjt.0000433937.07244.e1 PMid: 24100258
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40) Terkeltaub RA, Furst DE, Digiacinto JL, et al. Novel evidence-based colchicine dose-reduction algorithm to predict and prevent colchicine toxicity in the presence of cytochrome P450 3A4/P-glycoprotein inhibitors. Arthritis Rheum. 2011; 63: 2226-2237. doi: 10.1002/art.30389 PMid: 21480191
41) Rigante D, Emmi G, Fastiggi M, et al. Macrophage activation syndrome in the course of monogenic autoinflammatory disorders. Clin Rheumatol 2015; 34: 1333-9. doi: 10.1007/s10067-015-2923-0 PMid: 25846831
42) Stabile A, Bertoni B, Ansuini V, et al. The clinical spectrum and treatment options of macrophage activation syndrome in the pediatric age. Eur Rev Med Pharmacol Sci 2006; 10: 53-9. PMid: 16705949
43) Cantarini L, Iacoponi F, Lucherini OM, et al. Validation of a diagnostic score for the diagnosis of autoinflammatory diseases in adults. Int J Immunopathol Pharmacol 2011; 24: 695-702. doi: 10.1177/039463201102400315 PMid: 21978701
44) Ter Haar NM, Annink KV, Al-Mayouf SM, et al. Development of the autoinflammatory disease damage index (ADDI). Ann Rheum Dis 2017; 76: 821-30. doi: 10.1136/annrheumdis-2016-210092 PMid: 27811147
45) Ter Haar NM, van Delft ALJ, Annink KV, et al. In silico validation of the Autoinflammatory Disease Damage Index. Ann Rheum Dis 2018; 77: 1599-605. doi: 10.1136/annrheumdis-2018-213725 PMid: 30077992
46) Pras E, Livneh A, Balow JE Jr, et al. Clinical differences between North African and Iraqi Jews with familial Mediterranean fever. Am J Med Genet 1998; 75(2): 216–219. doi: 10.1002/(sici)1096-8628(19980113)75:2<216::aid-ajmg20>3.0.co;2-r PMid: 9450890
47) Vitale A, Rigante D, Lucherini OM, et al. Biological treatments: new weapons in the management of monogenic autoinflammatory disorders. Mediators Inflamm 2013; 2013: 939847. doi: 10.1155/2013/939847 PMid: 23970817
48) Vitale A, Insalaco A, Sfriso P, et al. A snapshot on the on-label and off-label use of the interleukin-1 inhibitors in Italy among rheumatologists and pediatric rheumatologists: a nationwide multi-center retrospective observational study. Front Pharmacol 2016; 7: 380. doi: 10.3389/fphar.2016.00380 PMid: 27822185
49) Cantarini L, Lucherini OM, Frediani B, et al. Bridging the gap between the clinician and the patient with cryopyrin-associated periodic syndromes. Int J Immunopathol Pharmacol 2011; 24: 827-36. doi: 10.1177/039463201102400402 PMid: 22230390
50) Fleischmann RM, Tesser J, Schiff MH, et al. Safety of extended treatment with anakinra in patients with rheumatoid arthritis. Ann Rheum Dis 2006; 65: 1006-12. doi: 10.1136/ard.2005.048371 PMid: 16396977
51) van der Hilst JC, Moutschen M, Messiaen PE, et al. Efficacy of anti-IL-1 treatment in familial Mediterranean fever: a systematic review of the literature. Biologics 2016; 10: 75-80. doi: 10.2147/BTT.S102954 PMid: 27110096
2) Livneh A, Langevitz P. Diagnostic and treatment concerns in familial Mediterranean fever. Best Pract Res Clin Rheumatol 2000;14:477-98. doi: 10.1053/berh.2000.0089 PMid: 10985982
3) Sohar E, Gafni J, Pras M, et al. Familial Mediterranean fever. A survey of 470 cases and review of the literature. Am J Med 1967;43:227-53. PMid: 534064
4) Tunca M, Akar S, Onen F, et al. Familial Mediterranean fever (FMF) in Turkey: results of a nationwide multicenter study. Medicine 2005;84:1-11. doi: 10.1097/01.md.0000152370.84628.0c PMid: 15643295
5) Rigante D. A systematic approach to autoinflammatory syndromes: a spelling booklet for the beginner. Expert Rev Clin Immunol 2017;13:571-97. doi: 10.1080/1744666X.2017.1280396 PMid: 28064547
6) Rigante D. The broad-ranging panorama of systemic autoinflammatory disorders with specific focus on acute painful symptoms and hematologic manifestations in children. Mediterr J Hematol Infect Dis 2018;10:e2018067. doi: 10.4084/MJHID.2018.067 PMid: 30416699
7) Manna R, Rigante D. Familial Mediterranean fever: assessing the overall clinical impact and formulating treatment plans. Mediterr J Hematol Infect Dis 2019;11:e2019027. doi: 10.4084/MJHID.2019.027 PMid: 31205631
8) Kees S, Langevitz P, Zemer D, et al. Attacks of pericarditis as a manifestation of familial Mediterranean fever. Int J Med 1997;90:643-7. doi: 10.1093/qjmed/90.10.643 PMid: 9415347
9) Rigante D, Cantarini L, Imazio M, et al. Autoinflammatory diseases and cardiovascular manifestations. Ann Med 2011;43:341-6. doi: 10.3109/07853890.2010.547212 PMid: 21284530
10) Rigante D, Frediani B, Galeazzi M, et al. From the Mediterranean to the sea of Japan: the transcontinental odyssey of autoinflammatory diseases. Biomed Res Int 2013;2013:485103. doi: 10.1155/2013/485103 PMid: 23971037
11) Kasifoglu T, Bilge SY, Sari I, et al. Amyloidosis and its related factors in Turkish patients with familial Mediterranean fever: a multi-centre study. Rheumatology 2014; 53: 741-5. doi: 10.1093/rheumatology/ket400 PMid: 24369413
12) Ben-Chetrit E, Yazici H. The liver in familial Mediterranean fever: is it involved? Clin Exp Rheumatol 2017; 35 Suppl 108: 108-12. PMid: 28598780
13) Fraisse T, Savey L, Hentgen V, et al. Non-amyloid liver involvement in familial Mediterranean fever: a systematic literature review. Liver Int 2020 Mar 20 - Epub ahead of print - doi: 10.1111/liv.14445 PMid: 32196885
14) Goldfinger SE. Colchicine for familial Mediterranean fever. N Engl J Med 1972; 287: 1302. doi: 10.1056/NEJM197212212872514 PMid: 4636899
15) Rigante D, La Torraca I, Avallone L, et al. The pharmacological basis of treatment with colchicine in children with familial Mediterranean fever. Eur Rev Med Pharmacol Sci 2006; 10: 173-8. PMid: 16910346
16) Dinarello CA, Wolfe SM, Goldfinger SE, et al. Colchicine therapy for familial Mediterranean fever: a double-blind trial. N Engl J Med 1974; 291: 934-7. doi: 10.1056/NEJM197410312911804 PMid: 4606353
17) Ozen S, Demirkaya E, Erer B, et al. EULAR recommendations for the management of familial Mediterranean fever. Ann Rheum Dis 2016; 75: 644-51. doi: 10.1136/annrheumdis-2015-208690 PMid: 26802180
18) Ozen S, Koné-Paut I, Gu?l A. Colchicine resistance and intolerance in familial Mediterranean fever: definition, causes, and alternative treatments. Semin Arthritis Rheum 2017; 47: 115-20. doi: 10.1016/j.semarthrit.2017.03.006 PMid: 28413100
19) Laskari K, Boura P, Dalekos GN, et al. Long-term beneficial effect of canakinumab in colchicine-resistant familial Mediterranean fever. J Rheumatol 2017; 44: 102-9. doi: 10.3899/jrheum.160518 PMid: 28042127
20) Ben-Zvi I, Kukuy O, Giat E, et al. Anakinra for colchicine-resistant familial Mediterranean fever: a randomized double-blind placebo-controlled trial. Arthritis Rheumatol 2017; 69: 854-62. doi: 10.1002/art.39995 PMid: 27860460
21) De Benedetti F, Gattorno M, Anton J, et al. Canakinumab for the treatment of autoinflammatory recurrent fever syndromes N Engl J Med 2018; 378: 1908-19. doi: 10.1056/NEJMoa1706314 PMid: 29768139
22) Kacar M, Savic S, van der Hilst JCH. The efficacy, safety and tolerability of canakinumab in the treatment of familial Mediterranean fever: a systematic review of the literature. J Inflamm Res 2020; 13: 141?9. doi: 10.2147/JIR.S206204 PMid: 32210604
23) Ben-Chetrit E, Touitou I. Familial Mediterranean fever in the world. Arthritis Rheum 2009; 61: 1447-53. doi: 10.1002/art.24458 PMid: 19790133
24) Rigante D. New mosaic tiles in childhood hereditary autoinflammatory disorders. Immunol Lett 2018; 193: 67-76. doi: 10.1016/j.imlet.2017.11.013 PMid: 29198619
25) Rigante D. A developing portrait of hereditary periodic fevers in childhood. Expert Opin Orphan Drugs 2018; 6: 47-55. doi: 10.1080/21678707.2018.1406797
26) Lidar M, Doron A, Barzilai A, et al. Erysipelas-like erythema as the presenting feature of familial Mediterranean fever. J Eur Acad Dermatol Venereol 2013; 27: 912-5. doi: 10.1111/j.1468-3083.2011.04442.x PMid: 22243424
27) van der Hilst JC, Simon A, Drenth JPH. Hereditary periodic fever and reactive amyloidosis. Clin Exp Med 2005; 5: 87-98. doi: 10.1007/s10238-005-0071-6 PMid: 16284730
28) Ebert EC, Nagar M. Gastrointestinal manifestations of amyloidosis. Am J Gastroenterol 2008; 103: 776-87. doi: 10.1111/j.1572-0241.2007.01669.x PMid: 18076735
29) Tilg H, Wilmer A, Vogel W, et al. Serum levels of cytokines in chronic liver diseases. Gastroenterology 1992; 103: 264-74. doi: 10.1016/0016-5085(92)91122-k PMid: 1612333
30) Ludwiczek O, Vannier E, Moschen A, et al. Impaired counter-regulation of interleukin-1 by the soluble IL-1 receptor type II in patients with chronic liver disease. Scand J Gastroenterol 2008; 43: 1360-5. doi: 10.1080/00365520802179925 PMid: 18609176
31) Wree A, Eguchi A, McGeough MD, et al. NLRP3 inflammasome activation results in hepatocyte pyroptosis, liver inflammation and fibrosis in mice. Hepatology 2014; 59: 898-910. doi: 10.1002/hep.26592 PMid: 23813842
32) Rimar D, Rosner I, Rozenbaum M, Zuckerman E. Familial Mediterranean fever: an association with non-alcoholic fatty liver disease. Clin Rheumatol 2011; 30: 987-91. doi: 10.1007/s10067-011-1718-1 PMid: 21360101
33) Sarkis C, Caglar E, Ugurlu S, et al. Nonalcoholic fatty liver disease and familial Mediterranean fever: are they related? Srp Arh Celok Lek 2012; 140: 589-94. doi: 10.2298/sarh1210589s PMid: 23289274
34) Lee SS, Park SH. Radiologic evaluation of nonalcoholic fatty liver disease. World J Gastroenterol 2014; 20: 7392-402. doi: 10.3748/wjg.v20.i23.7392 PMid: 24966609
35) Tweezer-Zaks N, Doron-Libner A, Weiss P, et al. Familial Mediterranean fever and cryptogenic cirrhosis. Medicine (Baltimore) 2007; 86: 355-62. doi: 10.1097/MD.0b013e31815be056 PMid: 18004180
36) La Regina M, Ben-Chetrit E, Gasparyan AY, et al. Current trends in colchicine treatment in familial Mediterranean fever. Clin Exp Rheumatol 2013; 31(3 Suppl 77): 41-6. PMid: 24064013
37) Leung YY, Yao Hui LL, Kraus VB. Colchicine-update on mechanisms of action and therapeutic uses. Semin Arthritis Rheum 2015; 45: 341-50. doi: 10.1016/j.semarthrit.2015.06.013 PMid: 26228647
38) Stack J, Ryan J, McCarthy G. Colchicine: new insights to an old drug. Am J Ther 2015; 22(5): e151-e157. doi: 10.1097/01.mjt.0000433937.07244.e1 PMid: 24100258
39) Abbott CE, Xu R, Sigal SH. Colchicine-induced hepatotoxicity. ACG Case Rep J 2017; 4: e120. doi: 10.14309/crj.2017.120 PMid: 29201931
40) Terkeltaub RA, Furst DE, Digiacinto JL, et al. Novel evidence-based colchicine dose-reduction algorithm to predict and prevent colchicine toxicity in the presence of cytochrome P450 3A4/P-glycoprotein inhibitors. Arthritis Rheum. 2011; 63: 2226-2237. doi: 10.1002/art.30389 PMid: 21480191
41) Rigante D, Emmi G, Fastiggi M, et al. Macrophage activation syndrome in the course of monogenic autoinflammatory disorders. Clin Rheumatol 2015; 34: 1333-9. doi: 10.1007/s10067-015-2923-0 PMid: 25846831
42) Stabile A, Bertoni B, Ansuini V, et al. The clinical spectrum and treatment options of macrophage activation syndrome in the pediatric age. Eur Rev Med Pharmacol Sci 2006; 10: 53-9. PMid: 16705949
43) Cantarini L, Iacoponi F, Lucherini OM, et al. Validation of a diagnostic score for the diagnosis of autoinflammatory diseases in adults. Int J Immunopathol Pharmacol 2011; 24: 695-702. doi: 10.1177/039463201102400315 PMid: 21978701
44) Ter Haar NM, Annink KV, Al-Mayouf SM, et al. Development of the autoinflammatory disease damage index (ADDI). Ann Rheum Dis 2017; 76: 821-30. doi: 10.1136/annrheumdis-2016-210092 PMid: 27811147
45) Ter Haar NM, van Delft ALJ, Annink KV, et al. In silico validation of the Autoinflammatory Disease Damage Index. Ann Rheum Dis 2018; 77: 1599-605. doi: 10.1136/annrheumdis-2018-213725 PMid: 30077992
46) Pras E, Livneh A, Balow JE Jr, et al. Clinical differences between North African and Iraqi Jews with familial Mediterranean fever. Am J Med Genet 1998; 75(2): 216–219. doi: 10.1002/(sici)1096-8628(19980113)75:2<216::aid-ajmg20>3.0.co;2-r PMid: 9450890
47) Vitale A, Rigante D, Lucherini OM, et al. Biological treatments: new weapons in the management of monogenic autoinflammatory disorders. Mediators Inflamm 2013; 2013: 939847. doi: 10.1155/2013/939847 PMid: 23970817
48) Vitale A, Insalaco A, Sfriso P, et al. A snapshot on the on-label and off-label use of the interleukin-1 inhibitors in Italy among rheumatologists and pediatric rheumatologists: a nationwide multi-center retrospective observational study. Front Pharmacol 2016; 7: 380. doi: 10.3389/fphar.2016.00380 PMid: 27822185
49) Cantarini L, Lucherini OM, Frediani B, et al. Bridging the gap between the clinician and the patient with cryopyrin-associated periodic syndromes. Int J Immunopathol Pharmacol 2011; 24: 827-36. doi: 10.1177/039463201102400402 PMid: 22230390
50) Fleischmann RM, Tesser J, Schiff MH, et al. Safety of extended treatment with anakinra in patients with rheumatoid arthritis. Ann Rheum Dis 2006; 65: 1006-12. doi: 10.1136/ard.2005.048371 PMid: 16396977
51) van der Hilst JC, Moutschen M, Messiaen PE, et al. Efficacy of anti-IL-1 treatment in familial Mediterranean fever: a systematic review of the literature. Biologics 2016; 10: 75-80. doi: 10.2147/BTT.S102954 PMid: 27110096