Universite Libre de Bruxelles, Belgium
Posters & Accepted Abstracts: J Nephrol Ther
Introduction: Tubulointerstitial Nephritis (TIN) is a manifestation of IgG4-related diseases, which are characterized by infiltration of target organs by IgG4+ plasma cells and severe fibrosis. Cortico-sensitivity is one of the diagnostic criteria, but the treatment of steroid resistant and dependent forms is not well defined. Case Report: We present a case of a 47-years-old patient with IgG4-related NTI followed for 72 months. He complained of fatigue and recurrent postprandial abdominal pain. With the exception of elevated levels of Gamma-Glutamyl Transferase (GGT), transaminases and IgG4, kidney function remained normal (serum creatinine ├ó┬?┬Ą 0.9 mg/dL). After 2 cures of methyl prednisolone (2010-11) azathioprine was associated in 2012. Due to the corticodependence and persistence of bilateral focal renal lesions detected by diffusion-weighted magnetic resonance imaging (DW-MRI), Rituximab (RTX) was given (2 ├?┬? 376 mg/m├?┬▓/15 days) in 2013. Before the first injection, Positron Emission Tomography (PET) showed metabolic hyperactivity corresponding to axillary and abdominal aorta lymph nodes but not in the kidney. After 4 months of RTX, the patient became asymptomatic. All biological alterations disappeared. PET showed a decrease in metabolic activity at extrarenal lesions described above. A dramatic regression of bilateral renal lesions was noted by DW-MRI: the apparent diffusion coefficient had almost doubled (0.776 vs. 1.111x10-3 mm├?┬▓/sec) and the volume of renal lesions was reduced by 50%, which was never observed under other treatments. Results: Our observations demonstrate: (1) the clinical, biological and radiological efficacy of rituximab in a steroid-dependent form of IgG4- related TIN and (2) the interest of DW-MRI as a non-nephrotoxic radiological and PET complementary approach not only in monitoring the effectiveness of immunosuppression but also in the early detection of renal involvement during IgG4 related disease.
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