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Spectrum of renal involvement in familial Mediterranean fever
Abstract:
Kidney biopsies were performed on fifteen patients with a long-standing history of familial Mediterranean fever (FMF) and evidence of renal involvement. On light microscopy, seven patients were found to have amyloidosis, six mesangial proliferative glomerulonephritis (MsPGN) and two patients rapid progressive glomerulonephritis (RPGN). Immunofluorescent studies of the six biopsies with MsPGN were positive for mesangial IgA deposits (IgA nephropathy) in three patients and IgM mesangial deposits in three (IgM nephropathy). We conclude that in patients with FMF and renal involvement, non-amyloid renal lesions (IgA nephropathy, IgM nephropathy and RPGN) should be considered in the differential diagnosis in addition to amyloidosis.
Insights
Familial Mediterranean Fever (FMF) patients with kidney issues may have non-amyloid conditions like IgA nephropathy or IgM nephropathy, not just amyloidosis. Early diagnosis of these varied kidney diseases is crucial for FMF patients.
Area of Science:
- Nephrology
- Rheumatology
- Genetics
Background:
- Familial Mediterranean Fever (FMF) is a genetic autoinflammatory disorder.
- Renal involvement is a significant complication in long-standing FMF.
- Amyloidosis is a known renal manifestation of FMF.
Observation:
- Kidney biopsies were analyzed from fifteen FMF patients with renal involvement.
- Light microscopy revealed amyloidosis in seven, mesangial proliferative glomerulonephritis (MsPGN) in six, and rapid progressive glomerulonephritis (RPGN) in two patients.
- Immunofluorescence identified IgA nephropathy in three and IgM nephropathy in three of the MsPGN cases.
Findings:
- Non-amyloid renal lesions are present in FMF patients.
- IgA nephropathy, IgM nephropathy, and RPGN are identified as differential diagnoses.
- These findings expand the spectrum of renal pathology in FMF.
Implications:
- Clinicians should consider a broader range of kidney diseases beyond amyloidosis in FMF patients.
- Timely diagnosis of non-amyloid renal lesions can guide appropriate treatment strategies.
- Further research into the pathogenesis of these varied renal manifestations in FMF is warranted.