Bektaş Murat, Çavuş Bilger, Ağargün Besim Fazıl, Şenkal İbrahim Volkan, Koca Nevzat, İnce Burak, Sarı Selma, Dirim Ahmet Burak, Mastanzade Metban Güzel, Dağcı Gizem, Özer Pelin Karaca, Aydoğan Mehmet, Büyük Melek, Yalçınkaya Yasemin, Artım-Esen Bahar, İnanç Murat, Güllüoğlu Mine, Yazıcı Halil, Beşışık Sevgi Kalayoğlu, Beşışık Selman Fatih, Gül Ahmet
Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University, Turgut Özal Millet Cd, 34093, Istanbul, Fatih, Turkey.
Division of Rheumatology, Department of Internal Medicine, Istanbul Aydin University, Istanbul, Turkey.
Rheumatol Int. 2025 Jul 1;45(7):162. doi: 10.1007/s00296-025-05906-3.
To evaluate the utility of elasticity imaging techniques, such as transient elastography (FibroScan), in assessing the liver and transplanted kidney in patients with AA amyloidosis (AA-A). This study was conducted in patients with AA-A, while patients with immunoglobulin light chain amyloidosis (AL-A), Familial Mediterranean Fever (FMF) without amyloidosis, and healthy controls (HC) were included as comparison groups for liver stiffness (LS) measurements. Additionally, kidney stiffness (KS) was measured in renal transplant recipients (RTRs) with transplants due to AA-A or other causes of chronic renal failure. LS evaluations were performed in 65 patients with AA-A, 14 with AL-A, 20 with FMF, and 27 with HC. LS (kPa) was significantly higher [median (IQR)] in patients with AA-A [6.4 (5.4)] and AL-A [9.8 (11)] compared to HC [4.7 (1.7)] (p < 0.001). However, the difference between AL-A and AA-A was not statistically significant. LS values were comparable in FMF-AA patients with [6.8 (6.6)] and without [5.7 (3.6)] liver involvement, and FMF patients without amyloidosis [7.15 (4.6)]. The median KS values were comparable in 19 AA-A and 16 disease controls. However, KS values were significantly higher in patients with recurrent amyloidosis in the transplanted kidney [29.3 (18.9)] compared to those without recurrence [10.9 (7.7)] (p = 0.003). In our study, increased liver stiffness measurements were observed in patients with AA-A, AL-A, and FMF compared to HC. Transient elastography using FibroScan appears to be a promising non-invasive tool for assessing liver involvement in AA-A and may aid in detecting recurrence of amyloidosis in transplanted kidneys. Further studies are needed to validate the utility of FibroScan in evaluating the involvement of the liver and kidneys in patients with AA-A.
评估弹性成像技术,如瞬时弹性成像(FibroScan),在评估AA淀粉样变性(AA-A)患者的肝脏和移植肾方面的效用。本研究针对AA-A患者开展,同时纳入免疫球蛋白轻链淀粉样变性(AL-A)患者、无淀粉样变性的家族性地中海热(FMF)患者以及健康对照(HC)作为肝脏硬度(LS)测量的比较组。此外,对因AA-A或其他慢性肾衰竭病因接受移植的肾移植受者(RTR)测量肾脏硬度(KS)。对65例AA-A患者、14例AL-A患者、20例FMF患者和27例HC进行了LS评估。与HC组[4.7(1.7)]相比,AA-A组[6.4(5.4)]和AL-A组[9.8(11)]的LS(kPa)显著更高(中位数[四分位间距])(p < 0.001)。然而,AL-A组和AA-A组之间的差异无统计学意义。有肝脏受累的FMF-AA患者[6.8(6.6)]和无肝脏受累的患者[5.7(3.6)]以及无淀粉样变性的FMF患者[7.15(4.6)]的LS值具有可比性。19例AA-A患者和16例疾病对照的KS中位数具有可比性。然而,移植肾发生复发性淀粉样变性的患者的KS值[29.3(18.9)]显著高于无复发患者[10.9(7.7)](p = 0.003)。在我们的研究中,与HC相比,AA-A、AL-A和FMF患者的肝脏硬度测量值升高。使用FibroScan的瞬时弹性成像似乎是评估AA-A患者肝脏受累情况的一种有前景的非侵入性工具,并且可能有助于检测移植肾淀粉样变性的复发。需要进一步研究来验证FibroScan在评估AA-A患者肝脏和肾脏受累情况方面的效用。