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Investigation of Hepatic and Splenic Shear-wave Elastography Findings in Patients with Familial Mediterranean Fever

Yıl 2025, Cilt: 9 Sayı: 1, 37 - 43, 21.04.2025
https://doi.org/10.46332/aemj.1455102

Öz

Purpose: Familial Mediterranean Fever (FMF) is an autoinflammatory multisystemic disorder that may impact internal organs. This study aims to evaluate the impact of FMF on the liver and spleen utilizing shear wave elastography (SWE).

Materials and Methods: Our study included patients diagnosed with FMF according to the Tel-Hashomer criteria, aged ≥18 years, who did not have AA amyloidosis or any conditions affecting liver or spleen tissue. Electronic medical records were examined retrospectively. A radiologist with a minimum of five years of expertise in shear wave elastography conducted the procedure. Shear wave elastography results were compared between patients with FMF and healthy controls.

Results: We examined fifty-one cases of FMF (25 females, 26 males) with a mean age of 32.3 years, alongside 14 healthy persons (4 females, 10 males) with a mean age of 36.9 years. The predominant symptom was fever, observed in 98% of cases, whereas 64.7% had the M694V gene mutation. No significant difference was seen between the mean SWE values of FMF cases and the control group. A positive connection between hepatic stiffness and age was identified (r=0.319, p=0.023). The age at disease onset exhibited a correlation with hepatic stiffness and velocity (r=0.474, p=0.001; r=0.386, p=0.007, respectively).

Conclusion: A consensus on non-invasive methods to follow up patients with FMF is lacking. Further studies are necessary for the quantitative and routine application of SWE measurements in solid organs affected by inflammatory disorders, including FMF.

Kaynakça

  • 1. Sohar E, Gafni J, Pras M, Heller H. Familial Mediterranean fever: A survey of 470 cases and review of the literature. Am J Med. 1967;43(2):227-253.
  • 2. Ben-Chetrit E, Levy M. Familial Mediterranean Fever. Lancet. 1998;351(9103):659-664.
  • 3. Onen F, Sumer H, Turkay S, Akyurek O, Tunca M, Ozdogan H. Increased frequency of familial Mediterranean fever in Central Anatolia, Turkey. Clin Exp Rheumatol. 2004;22(34):31-33.
  • 4. Ancient missense mutations in a new member of the RoRet gene family are likely to cause familial Mediterranean fever. The International FMF Consortium. Cell. 1997;90(4):797-807.
  • 5. Touitou I, Picot MC, Domingo C, et al. The MICA region determines the first modifier locus in familial Mediterranean fever. Arthritis Rheum. 2001;44(1): 163-169.
  • 6. Cazeneuve C, Ajrapetyan H, Papin S, et al. Identification of MEFV-independent modifying genetic factors for familial Mediterranean fever. Am J Hum Genet. 2000;67(5):1136-1143.
  • 7. Ozen S, Aktay N, Lainka E, Duzova A, Bakkaloglu A, Kallinich T. The significance of paired MEFV mutations in individuals without symptoms of familial Mediterranean fever. Ann Rheum Dis. 2009;68(2): 246-248.
  • 8. Touitou I, Sarkisian T, Medlej-Hashim M, et al. Country as the primary risk factor for renal amyloidosis in familial Mediterranean fever. Arthritis Rheum. 2007;56(5):1706-1712.
  • 9. Delplanque M, Benech N, Rolhion N, et al. Gut microbiota alterations are associated with phenotype and genotype in familial Mediterranean fever. Rheumatology (Oxford). 2024:63(4):1039-1048.
  • 10. Ozdogan H, Ugurlu S, Hatemi G. Colchicine compliance and amyloidosis. Pediatr Rheumatol Online J. 2013;11:15.
  • 11. Ozen S, Demirkaya E, Erer B, et al. EULAR recommendations for the management of familial Mediterranean fever. Ann Rheum Dis. 2016;75(4):644-651.
  • 12. Ben-Zvi I, Livneh A. Chronic inflammation in FMF: markers, risk factors, outcomes and therapy. Nat Rev Rheumatol. 2011;7(2):105-112.
  • 13. Ahbap E, Kara E, Sahutoglu T, et al. Outcome of 121 patients with renal amyloid a amyloidosis. J Res Med Sci. 2014;19(7):644-649.
  • 14. Balci B, Tinaztepe K, Yilmaz E, et al. MEFV gene mutations in familial Mediterranean fever phenotype II patients with renal amyloidosis in childhood: a retrospective clinicopathological and molecular study. Nephrol Dial Transplant. 2002;17(11):1921-1923.
  • 15. Ophir J, Garra B, Kallel F, et al. Elastographic imaging. Ultrasound Med Biol. 2000;26(1):23-29.
  • 16. Bercoff J, Tanter M, Fink M. Supersonic shear imaging: a new technique for soft tissue elasticity mapping. IEEE Trans Ultrason Ferroelectr Freq Control. 2004;51(4):396-409.
  • 17. Taljanovic MS, Gimber LH, Becker GW, et al. Shear-Wave Elastography: Basic Physics and Musculoskeletal Applications. Radiographics. 2017;37(3): 855-870.
  • 18. Nakao T, Ushigome H, Nakamura T, et al. Transplant Proc. Evaluation of renal allograft fibrosis by transient elastography (Fibro Scan). Transplant. Proc. 2015;47 (3):640-643.
  • 19. Zeng J, Zheng J, Jin JY, et al. Shear wave elastography for liver fibrosis in chronic hepatitis B: adapting the cut-offs to alanine aminotransferase levels improves accuracy. Eur Radiol. 2019;29(2):857-865.
  • 20. Leong SS, Wong JHD, Md Shah MN, et al. Shear wave elastography accurately detects chronic changes in renal histopathology. Nephrology (Carlton). 2021;26(1):38-45.
  • 21. Horvat U, Kozinc Ž. The Use of Shear-Wave Ultrasound Elastography in the Diagnosis and Monitoring of Musculoskeletal Injuries. Crit Rev Biomed Eng. 2024;52(2):15-26.
  • 22. Livneh A, Langevitz P, Zemer D, et al. Criteria for the Diagnosis of Familial Mediterranean Fever. Arthritis Rheum. 1997;40(10):1879-1885.
  • 23. Kasifoglu T, Bilge SY, Sari I, et al. Amyloidosis and its related factors in Turkish patients with familial Mediterranean fever: a multicentre study. Rheumatology (Oxford). 2014;53(4):741-745.
  • 24. Bayramoglu Z, Akyol Sari ZN, Koker O, Adaletli I, Eker Omeroglu R. Shear wave elastography evaluation of liver, pancreas, spleen and kidneys in patients with familial mediterranean fever and amyloidosis. Br J Radiol. 2021;94(1128):20210237.
  • 25. Akti S, Albayrak E. Evaluation of Liver and Spleen With Shear Wave Elastography in Adult Patients With Familial Mediterranean Fever. Ultrasound Q. 2023;39(3):165-170.
  • 26. Kayalı A, Öztürk Keleş F, Seyfettin A, Dirican E, Çelik MM. An evaluation with shear wave elastography of kidney elasticity in patients with familial Mediterranean fever. J Clin Ultrasound. 2023;51(1):177-183.
  • 27. Ozmen Z, Kasap T, Aktas F, Ozmen ZC. Shear wave elastography evaluation of kidneys in children with familial mediterranean fever. Niger J Clin Pract. 2023;26(7):957-962.
  • 28. Urfali M, Yilmaz G, Özkul B, Urfali FE. Indirect evaluation of amyloid deposition by ultrasonography and its relationship with MEFV gene mutation in FMF patients. Clin Ultrasound. 2023;51(4):715-722.
  • 29. Akpolat T, Özkaya O, Özen S. Homozygous M694V as a risk factor for amyloidosis in Turkish FMF patients. Gene. 2012;492(1):285-289.
  • 30. Bayramoglu Z, Koker O, Kandemirli SG, Adaletli I, Eker Omeroglu R. Shear wave elastography of salivary and thyroid glands in pediatric patients with FMF and amyloidosis. J Ist Faculty Med. 2020;83(2): 92-99.
  • 31. Turkish FMF Study Group. Familial Mediterranean fever (FMF) in Turkey: results of a nationwide multicenter study. Medicine (Baltimore). 2005;84(1):1-11.

Ailevi Akdeniz Ateşi Hastalarında Karaciğer ve Dalak Shearwave Elastografi Bulgularının İncelenmesi

Yıl 2025, Cilt: 9 Sayı: 1, 37 - 43, 21.04.2025
https://doi.org/10.46332/aemj.1455102

Öz

Amaç: Ailevi Akdeniz Ateşi (AAA), iç organları etkileyebilen otoinflamatuar multisistemik bir hastalıktır. Bu çalışma, AAA'nın karaciğer ve dalak üzerindeki etkisini shear wave elastografisi (SWE) kullanarak değerlendirmeyi amaçlamaktadır.

Araçlar ve Yöntem: Çalışmamıza Tel-Hashomer kriterlerine göre AAA tanısı alan, 18 yaş ve üzeri, amiloidoz gelişmemiş, karaciğer ya da dalak dokusunu etkileyen herhangi bir durumu olmayan hastalar dahil edilmiştir. Elektronik tıbbi kayıtlar retrospektif olarak incelenmiştir. Shear wave elastografi incelemesi en az beş yıllık SWE deneyimi olan bir radyolog tarafından yapıldı. Shear wave elastografi bulguları AAA olguları ve sağlıklı grup arasında karşılaştırıldı.

Bulgular: Yaş ortalaması 32.3 yıl olan 51 AAA olgusu (25 kadın, 26 erkek) ile yaş ortalaması 36.9 yıl olan 14 sağlıklı birey (4 kadın, 10 erkek) incelendi. En sık görülen semptom ateş olup vakaların %98'inde görülürken, %64.7'sinde M694V gen mutasyonu vardı. Hasta ve kontrol grubunun ortalama SWE değerleri arasında anlamlı bir fark bulunmadı. Karaciğer sertliği ve yaş arasında hafif pozitif bir korelasyon olduğunu saptadık (r=0.319, p=0.023). Hastalık başlangıç yaşı, hepatik sertlik (r=0.474, p=0.001) ve hepatik velosite (r=0.386, p=0.007) ile koreleydi.

Sonuç: Ailevi Akdeniz Ateşi hastalarını takip etmek için non-invaziv yöntemler konusunda bir fikir birliği bulunmamaktadır. AAA’de dahil olmak üzere enflamatuar hastalıklardan etkilenen solid organlarda SWE ölçümlerinin kantitatif ve rutin olarak uygulanması için daha fazla çalışma yapılması gerekmektedir.

Kaynakça

  • 1. Sohar E, Gafni J, Pras M, Heller H. Familial Mediterranean fever: A survey of 470 cases and review of the literature. Am J Med. 1967;43(2):227-253.
  • 2. Ben-Chetrit E, Levy M. Familial Mediterranean Fever. Lancet. 1998;351(9103):659-664.
  • 3. Onen F, Sumer H, Turkay S, Akyurek O, Tunca M, Ozdogan H. Increased frequency of familial Mediterranean fever in Central Anatolia, Turkey. Clin Exp Rheumatol. 2004;22(34):31-33.
  • 4. Ancient missense mutations in a new member of the RoRet gene family are likely to cause familial Mediterranean fever. The International FMF Consortium. Cell. 1997;90(4):797-807.
  • 5. Touitou I, Picot MC, Domingo C, et al. The MICA region determines the first modifier locus in familial Mediterranean fever. Arthritis Rheum. 2001;44(1): 163-169.
  • 6. Cazeneuve C, Ajrapetyan H, Papin S, et al. Identification of MEFV-independent modifying genetic factors for familial Mediterranean fever. Am J Hum Genet. 2000;67(5):1136-1143.
  • 7. Ozen S, Aktay N, Lainka E, Duzova A, Bakkaloglu A, Kallinich T. The significance of paired MEFV mutations in individuals without symptoms of familial Mediterranean fever. Ann Rheum Dis. 2009;68(2): 246-248.
  • 8. Touitou I, Sarkisian T, Medlej-Hashim M, et al. Country as the primary risk factor for renal amyloidosis in familial Mediterranean fever. Arthritis Rheum. 2007;56(5):1706-1712.
  • 9. Delplanque M, Benech N, Rolhion N, et al. Gut microbiota alterations are associated with phenotype and genotype in familial Mediterranean fever. Rheumatology (Oxford). 2024:63(4):1039-1048.
  • 10. Ozdogan H, Ugurlu S, Hatemi G. Colchicine compliance and amyloidosis. Pediatr Rheumatol Online J. 2013;11:15.
  • 11. Ozen S, Demirkaya E, Erer B, et al. EULAR recommendations for the management of familial Mediterranean fever. Ann Rheum Dis. 2016;75(4):644-651.
  • 12. Ben-Zvi I, Livneh A. Chronic inflammation in FMF: markers, risk factors, outcomes and therapy. Nat Rev Rheumatol. 2011;7(2):105-112.
  • 13. Ahbap E, Kara E, Sahutoglu T, et al. Outcome of 121 patients with renal amyloid a amyloidosis. J Res Med Sci. 2014;19(7):644-649.
  • 14. Balci B, Tinaztepe K, Yilmaz E, et al. MEFV gene mutations in familial Mediterranean fever phenotype II patients with renal amyloidosis in childhood: a retrospective clinicopathological and molecular study. Nephrol Dial Transplant. 2002;17(11):1921-1923.
  • 15. Ophir J, Garra B, Kallel F, et al. Elastographic imaging. Ultrasound Med Biol. 2000;26(1):23-29.
  • 16. Bercoff J, Tanter M, Fink M. Supersonic shear imaging: a new technique for soft tissue elasticity mapping. IEEE Trans Ultrason Ferroelectr Freq Control. 2004;51(4):396-409.
  • 17. Taljanovic MS, Gimber LH, Becker GW, et al. Shear-Wave Elastography: Basic Physics and Musculoskeletal Applications. Radiographics. 2017;37(3): 855-870.
  • 18. Nakao T, Ushigome H, Nakamura T, et al. Transplant Proc. Evaluation of renal allograft fibrosis by transient elastography (Fibro Scan). Transplant. Proc. 2015;47 (3):640-643.
  • 19. Zeng J, Zheng J, Jin JY, et al. Shear wave elastography for liver fibrosis in chronic hepatitis B: adapting the cut-offs to alanine aminotransferase levels improves accuracy. Eur Radiol. 2019;29(2):857-865.
  • 20. Leong SS, Wong JHD, Md Shah MN, et al. Shear wave elastography accurately detects chronic changes in renal histopathology. Nephrology (Carlton). 2021;26(1):38-45.
  • 21. Horvat U, Kozinc Ž. The Use of Shear-Wave Ultrasound Elastography in the Diagnosis and Monitoring of Musculoskeletal Injuries. Crit Rev Biomed Eng. 2024;52(2):15-26.
  • 22. Livneh A, Langevitz P, Zemer D, et al. Criteria for the Diagnosis of Familial Mediterranean Fever. Arthritis Rheum. 1997;40(10):1879-1885.
  • 23. Kasifoglu T, Bilge SY, Sari I, et al. Amyloidosis and its related factors in Turkish patients with familial Mediterranean fever: a multicentre study. Rheumatology (Oxford). 2014;53(4):741-745.
  • 24. Bayramoglu Z, Akyol Sari ZN, Koker O, Adaletli I, Eker Omeroglu R. Shear wave elastography evaluation of liver, pancreas, spleen and kidneys in patients with familial mediterranean fever and amyloidosis. Br J Radiol. 2021;94(1128):20210237.
  • 25. Akti S, Albayrak E. Evaluation of Liver and Spleen With Shear Wave Elastography in Adult Patients With Familial Mediterranean Fever. Ultrasound Q. 2023;39(3):165-170.
  • 26. Kayalı A, Öztürk Keleş F, Seyfettin A, Dirican E, Çelik MM. An evaluation with shear wave elastography of kidney elasticity in patients with familial Mediterranean fever. J Clin Ultrasound. 2023;51(1):177-183.
  • 27. Ozmen Z, Kasap T, Aktas F, Ozmen ZC. Shear wave elastography evaluation of kidneys in children with familial mediterranean fever. Niger J Clin Pract. 2023;26(7):957-962.
  • 28. Urfali M, Yilmaz G, Özkul B, Urfali FE. Indirect evaluation of amyloid deposition by ultrasonography and its relationship with MEFV gene mutation in FMF patients. Clin Ultrasound. 2023;51(4):715-722.
  • 29. Akpolat T, Özkaya O, Özen S. Homozygous M694V as a risk factor for amyloidosis in Turkish FMF patients. Gene. 2012;492(1):285-289.
  • 30. Bayramoglu Z, Koker O, Kandemirli SG, Adaletli I, Eker Omeroglu R. Shear wave elastography of salivary and thyroid glands in pediatric patients with FMF and amyloidosis. J Ist Faculty Med. 2020;83(2): 92-99.
  • 31. Turkish FMF Study Group. Familial Mediterranean fever (FMF) in Turkey: results of a nationwide multicenter study. Medicine (Baltimore). 2005;84(1):1-11.
Toplam 31 adet kaynakça vardır.

Ayrıntılar

Birincil Dil İngilizce
Konular Romatoloji ve Artrit
Bölüm Bilimsel Araştırma Makaleleri
Yazarlar

Kadir Çabalak 0009-0001-5393-7492

Muhammet Murat Çelik 0000-0002-4874-1241

Alperen Kayalı 0000-0002-9862-8925

Mete Pekdiker 0000-0003-3089-1564

Erken Görünüm Tarihi 16 Nisan 2025
Yayımlanma Tarihi 21 Nisan 2025
Gönderilme Tarihi 19 Mart 2024
Kabul Tarihi 10 Kasım 2024
Yayımlandığı Sayı Yıl 2025 Cilt: 9 Sayı: 1

Kaynak Göster

APA Çabalak, K., Çelik, M. M., Kayalı, A., Pekdiker, M. (2025). Investigation of Hepatic and Splenic Shear-wave Elastography Findings in Patients with Familial Mediterranean Fever. Ahi Evran Medical Journal, 9(1), 37-43. https://doi.org/10.46332/aemj.1455102
AMA Çabalak K, Çelik MM, Kayalı A, Pekdiker M. Investigation of Hepatic and Splenic Shear-wave Elastography Findings in Patients with Familial Mediterranean Fever. Ahi Evran Med J. Nisan 2025;9(1):37-43. doi:10.46332/aemj.1455102
Chicago Çabalak, Kadir, Muhammet Murat Çelik, Alperen Kayalı, ve Mete Pekdiker. “Investigation of Hepatic and Splenic Shear-Wave Elastography Findings in Patients With Familial Mediterranean Fever”. Ahi Evran Medical Journal 9, sy. 1 (Nisan 2025): 37-43. https://doi.org/10.46332/aemj.1455102.
EndNote Çabalak K, Çelik MM, Kayalı A, Pekdiker M (01 Nisan 2025) Investigation of Hepatic and Splenic Shear-wave Elastography Findings in Patients with Familial Mediterranean Fever. Ahi Evran Medical Journal 9 1 37–43.
IEEE K. Çabalak, M. M. Çelik, A. Kayalı, ve M. Pekdiker, “Investigation of Hepatic and Splenic Shear-wave Elastography Findings in Patients with Familial Mediterranean Fever”, Ahi Evran Med J, c. 9, sy. 1, ss. 37–43, 2025, doi: 10.46332/aemj.1455102.
ISNAD Çabalak, Kadir vd. “Investigation of Hepatic and Splenic Shear-Wave Elastography Findings in Patients With Familial Mediterranean Fever”. Ahi Evran Medical Journal 9/1 (Nisan 2025), 37-43. https://doi.org/10.46332/aemj.1455102.
JAMA Çabalak K, Çelik MM, Kayalı A, Pekdiker M. Investigation of Hepatic and Splenic Shear-wave Elastography Findings in Patients with Familial Mediterranean Fever. Ahi Evran Med J. 2025;9:37–43.
MLA Çabalak, Kadir vd. “Investigation of Hepatic and Splenic Shear-Wave Elastography Findings in Patients With Familial Mediterranean Fever”. Ahi Evran Medical Journal, c. 9, sy. 1, 2025, ss. 37-43, doi:10.46332/aemj.1455102.
Vancouver Çabalak K, Çelik MM, Kayalı A, Pekdiker M. Investigation of Hepatic and Splenic Shear-wave Elastography Findings in Patients with Familial Mediterranean Fever. Ahi Evran Med J. 2025;9(1):37-43.

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