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Background The spectrum of Interferon-beta-associated nephropathy (IFN-β) remains poorly described and the potential features of this uncommon association remain to be determined. Methods In this study, we retrospectively analyzed the clinical, laboratory, histological and therapeutic data of patients with biopsy-proven renal disease in a context of IFN-β treatment administered since at least 6 months. Results Eighteen patients (13 women, median age 48 years) with biopsy-proven renal disease occurring during IFN-β therapy were included. The median exposure to IFN-β (14 patients were treated by IFN-β1a and four patients by IFN-β1b) was 67 months (ranged from 23 to 165 months). The clinical presentation consists in hypertension (HT) (83%), malignant HT (44%), proteinuria >1g/g (94%), reduced renal function (78%), biological hallmark suggesting thrombotic microangiopathy (TMA) (61%), edematous syndrome (17%), or nephritic syndrome (11%). The pathological findings included typical features of isolated TMAs in 11 cases, isolated focal segmental glomerulosclerosis (FSGS) lesions in two cases, and five cases with concomitant TMA and FSGS lesions. An exploration of the alternative complement pathway performed in 10 cases (63%) did not identify mutations in genes that regulate complement system. The statistical analysis highlighted that the occurrence of IFN-β-associated TMA was significantly associated with Rebif®, with a weekly dose >50 µg and with multiple weekly injections. In all cases IFN-β therapy was consequently discontinued. Patients with TMA lesions received other therapies included corticosteroids (44%), eculizumab (13%) or plasmatic exchanges (25%). At the end of a 36-month median follow-up, persistent hypertension and persistent proteinuria were observed in 61% and 22% of patients. Estimated glomerular filtration rate <60 ml/min/1.73m2 was present in 61% of patients. Conclusion IFN-β-associated nephropathy must be sought in the case of hypertension and/or proteinuria onset during treatment. When TMA and/or FSGS are observed on renal biopsy, early discontinuation of IFN-β is essential.
Background The spectrum of Interferon-beta-associated nephropathy (IFN-β) remains poorly described and the potential features of this uncommon association remain to be determined. Methods In this study, we retrospectively analyzed the clinical, laboratory, histological and therapeutic data of patients with biopsy-proven renal disease in a context of IFN-β treatment administered since at least 6 months. Results Eighteen patients (13 women, median age 48 years) with biopsy-proven renal disease occurring during IFN-β therapy were included. The median exposure to IFN-β (14 patients were treated by IFN-β1a and four patients by IFN-β1b) was 67 months (ranged from 23 to 165 months). The clinical presentation consists in hypertension (HT) (83%), malignant HT (44%), proteinuria >1g/g (94%), reduced renal function (78%), biological hallmark suggesting thrombotic microangiopathy (TMA) (61%), edematous syndrome (17%), or nephritic syndrome (11%). The pathological findings included typical features of isolated TMAs in 11 cases, isolated focal segmental glomerulosclerosis (FSGS) lesions in two cases, and five cases with concomitant TMA and FSGS lesions. An exploration of the alternative complement pathway performed in 10 cases (63%) did not identify mutations in genes that regulate complement system. The statistical analysis highlighted that the occurrence of IFN-β-associated TMA was significantly associated with Rebif®, with a weekly dose >50 µg and with multiple weekly injections. In all cases IFN-β therapy was consequently discontinued. Patients with TMA lesions received other therapies included corticosteroids (44%), eculizumab (13%) or plasmatic exchanges (25%). At the end of a 36-month median follow-up, persistent hypertension and persistent proteinuria were observed in 61% and 22% of patients. Estimated glomerular filtration rate <60 ml/min/1.73m2 was present in 61% of patients. Conclusion IFN-β-associated nephropathy must be sought in the case of hypertension and/or proteinuria onset during treatment. When TMA and/or FSGS are observed on renal biopsy, early discontinuation of IFN-β is essential.
Interferon (IFN) is an effective therapy for multiple disorders. An infrequently reported side effect is thrombotic microangiopathy (TMA): thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). We published the first comprehensive review analyzing this association with the following observations: (1) there was a higher incidence of IFN-induced TMA in myeloproliferative disorders (chronic myelogenous leukemia (CML)) than that in nonmalignant disorders (multiple sclerosis (MS), chronic hepatitis C virus infection (HCV)); (2) mean age at diagnosis was 47 years; (3) there was rare association with hairy cell leukemia (HCL), Sezary syndrome (one case each) and no cases reported for polycythemia vera (PV); (4) sex distribution was balanced (exception of higher prevalence in females for MS); (5) TMA was insidious in onset with long incubation periods (average treatment duration 40.4 months); (6) comparative analysis of mean time (months) to onset of TMA ensuing cumulative IFN exposure was: MS 68.6 vs. CML 35.5 vs. HCV 30.4; (7) confirmed TTP (low ADAMTS 13 levels) was associated with the presence of an inhibitor; (8) outcome analysis revealed complete remission in 27 (40%), persistent chronic kidney disease in 28 (42%) and fatality in 12 patients (18%); (9) corticosteroids, plasma exchange (PEX) and rituximab are effective therapies.
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