Objectives: Proteinuria, amyloidosis, and kidney failure are the main long-term renal complications of familial Mediterranean fever (FMF). This study assesses their risk factors, independent of ethnicity or residence. Materials and methods: Patients’ data were drawn from the International AIDA Network registry for monogenic autoinflammatory diseases. Results: A total of 598 FMF patients were enrolled, with 80 having proteinuria, 61 amyloidosis, and 25 kidney failure. At multivariate regression analysis, proteinuria was associated with out-of-flares thrombocytosis (OR: 4.78, 95%CI 1.54-14.8, p=0.007), increased out-of-flares erythrocyte sedimentation rate (ESR) (OR: 2.7, 95%CI 1.3-5.6, p=0.008), homozygous M694V mutation (OR: 2.27, 95%CI 1.1-4.66, p=0.025), and heterozygous M694V mutation (OR: 0.29, 95%CI 0.09-0.86, p=0.026); amyloidosis was associated with the disease duration (OR: 1.034, 95% CI1.004-1.065, p=0.027), during-flares anemia (OR: 2.9, 95%CI 1.18-7.19, p=0.021), out-of-flares leukocytosis (OR: 7.47, 95%CI 1.6-34.7, p=0.01), increased out-of-flares ESR (OR: 3.6, 95%CI 1.48-8.81, p=0.005), and heterozygous M694V mutation (OR: 13 0.18, 95%CI 0.035-0.9, p=0.04); kidney failure was associated with the age at diagnosis (1.04, 1.0003-1.19, p=0.048), the disease duration in years (OR: 1.07, 95%CI 1.02-1.12, p=0.005), attack frequency per year (OR: 15 1.04, 95%CI 1.007-1.076, p=0.019), anemia out-of-flares (OR: 4.7, 95% CI: 1.004-22.1, p=0.049), and out of-flares leukocytosis (OR: 25.8, 95%CI 2.75-242, p=0.004). The intraclass correlation coefficient related to ethnicity and country of residence was 6.7% and 6.8% for amyloidosis, respectively, and 0% for proteinuria and kidney failure. Conclusions: FMF patients with older age at diagnosis, longer disease duration, anemia, leukocytosis, thrombocytosis, elevated ESR, and homozygous M694V mutation are at higher risk of kidney complications.
Vitale, A., Caggiano, V., Sbalchiero, J., Tufan, A., Batu, E., Ragab, G., Portincasa, P., Conti, G., Aragona, E., Sota, J., Gavioli, F., Gaggiano, C., De Paulis, A., Şahin, A., Maggio, M., Rigante, D., Olivieri, A., Yildirim, D., Kucuk, H., Kardas, R., Vasi, I., Ozen, S., Bilginer, Y., Sener, S., Emreol, H., Mahmoud, A., Ghanema, M., Maher, A., Saad, M., Jaber, N., Khalil, M., Di Ciaula, A., De Palma, L., Cuzzola, R., Affronti, A., Gambino, F., Della Casa, F., Mormile, I., Babayiğit, A., Çelik, N., Gicchino, M., Jahnz-Rozyk, K., La Torre, F., Insalaco, A., Recke, A., Del Giudice, E., Barone, P., Cattalini, M., Brucato, A., Opris-Belinski, D., Sfikakis, P., Li Gobbi, F., Emmi, G., Giardini, H., Hernández-Rodríguez, J., Parronchi, P., Ruscitti, P., Costi, S., Tharwat, S., Karamanakos, A., Sfriso, P., Conforti, A., Verrecchia, E., Lopalco, G., Ogunjimi, B., Wiesik-Szewczyk, E., Balistreri, A., Fabiani, C., Frediani, B., Cantarini, L., Predictors of proteinuria, amyloidosis and kidney failure in familial Mediterranean fever: data from the International AIDA Network Registry., <<RHEUMATOLOGY>>, 2025; 2025 (64(8)): 4776-4787. [doi:10.1093/rheumatology/keaf181] [https://hdl.handle.net/10807/320021]
Predictors of proteinuria, amyloidosis and kidney failure in familial Mediterranean fever: data from the International AIDA Network Registry.
Rigante, Donato;Verrecchia, Elena;
2025
Abstract
Objectives: Proteinuria, amyloidosis, and kidney failure are the main long-term renal complications of familial Mediterranean fever (FMF). This study assesses their risk factors, independent of ethnicity or residence. Materials and methods: Patients’ data were drawn from the International AIDA Network registry for monogenic autoinflammatory diseases. Results: A total of 598 FMF patients were enrolled, with 80 having proteinuria, 61 amyloidosis, and 25 kidney failure. At multivariate regression analysis, proteinuria was associated with out-of-flares thrombocytosis (OR: 4.78, 95%CI 1.54-14.8, p=0.007), increased out-of-flares erythrocyte sedimentation rate (ESR) (OR: 2.7, 95%CI 1.3-5.6, p=0.008), homozygous M694V mutation (OR: 2.27, 95%CI 1.1-4.66, p=0.025), and heterozygous M694V mutation (OR: 0.29, 95%CI 0.09-0.86, p=0.026); amyloidosis was associated with the disease duration (OR: 1.034, 95% CI1.004-1.065, p=0.027), during-flares anemia (OR: 2.9, 95%CI 1.18-7.19, p=0.021), out-of-flares leukocytosis (OR: 7.47, 95%CI 1.6-34.7, p=0.01), increased out-of-flares ESR (OR: 3.6, 95%CI 1.48-8.81, p=0.005), and heterozygous M694V mutation (OR: 13 0.18, 95%CI 0.035-0.9, p=0.04); kidney failure was associated with the age at diagnosis (1.04, 1.0003-1.19, p=0.048), the disease duration in years (OR: 1.07, 95%CI 1.02-1.12, p=0.005), attack frequency per year (OR: 15 1.04, 95%CI 1.007-1.076, p=0.019), anemia out-of-flares (OR: 4.7, 95% CI: 1.004-22.1, p=0.049), and out of-flares leukocytosis (OR: 25.8, 95%CI 2.75-242, p=0.004). The intraclass correlation coefficient related to ethnicity and country of residence was 6.7% and 6.8% for amyloidosis, respectively, and 0% for proteinuria and kidney failure. Conclusions: FMF patients with older age at diagnosis, longer disease duration, anemia, leukocytosis, thrombocytosis, elevated ESR, and homozygous M694V mutation are at higher risk of kidney complications.
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