A Challenging Presentation of Familial Mediterranean Fever- Associated Glomerulonephritis: A Case Report
Author(s): Bassil Leghrouz, Osama J. Makhamreh, Alhareth M. Amro*, Suad Abughazala, Afnan W. M. Jobran, Fawzy M Abunejma
Familial Mediterranean Fever (FMF) is a hereditary autoinflammatory disorder characterized by recurrent episodes of fever and abdominal pain. We report a case of a 13-year-old boy who developed kidney manifestations with a history of FMF from two years of age. The patient was diagnosed with FMF, but he did not comply with colchicine therapy. He presented with acute glomerulonephritis with gross hematuria, hypertension, nephrotic-range proteinuria, and impaired kidney function. Previous evaluations at several hospitals resulted in varying diagnoses because FMF-associated glomerulonephritis (GN) is rare. He was initially diagnosed with post-infectious glomerulonephritis and lupus nephritis, but there was no improvement despite various management strategies. After a comprehensive review, the patient was referred to our center and was diagnosed with FMF-associated membranoproliferative glomerulonephritis (MPGN). However, we could not exclude concurrent FMF-associated amyloidosis because of the absence of an Electron microscopy study of the kidney biopsy. Treatment with pulse Methylprednisolone, Colchicine, Enalapril, and interleukin-1 receptor antagonist (Anakinra) resulted in the resolution of proteinuria and hematuria and normalization of the kidney function. This case highlights the difficulties in diagnosis, timing of early intervention, and comprehensive treatment, including unconventional modalities in FMF-associated glomerular diseases. Further research is needed to optimize the management of FMF-associated kidney complications in pediatric patients.