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Amyloidosis and Glomerular Diseases in Familial Mediterranean Fever

Rossella Siligato1, Guido Gembillo1,2, Vincenzo Calabrese1

  • 1Unit of Nephrology and Dialysis, Department of Clinical and Experimental Medicine, University of Messina, 98125 Messina, Italy.

Medicina (Kaunas, Lithuania)
|October 23, 2021
PubMed

Insights

Familial Mediterranean fever (FMF) can lead to kidney damage through amyloidosis. Early detection with markers like NGAL and new therapies like IL-1 inhibitors offer hope for better patient outcomes.

Area of Science:

  • Genetics
  • Immunology
  • Nephrology

Background:

  • Familial Mediterranean fever (FMF) is an autosomal recessive autoinflammatory disorder causing periodic fever and serositis.
  • A severe complication is secondary amyloidosis (AA amyloidosis) due to amyloid A deposition, leading to nephrotic syndrome and end-stage kidney disease in up to 8.6% of patients.
  • Other kidney diseases, including IgA vasculitis and polyarteritis nodosa, can also affect FMF patients.

Purpose of the Study:

  • To highlight the importance of differential diagnosis for nephrotic syndrome in FMF patients.
  • To discuss the role of emerging biomarkers like Neutrophil Gelatinase-Associated Lipocalin (NGAL) in predicting renal outcomes.
  • To explore the pathogenic role of Serum Amyloid A protein (SAA) and novel therapeutic strategies for FMF and AA amyloidosis.

Main Methods:

  • Review of current literature on FMF, AA amyloidosis, and nephrotic syndrome.
  • Analysis of the diagnostic and prognostic significance of microalbuminuria and NGAL.
  • Evaluation of the role of SAA in inflammation and disease progression.
  • Assessment of novel therapeutic agents including IL-1 and IL-6 inhibitors.

Main Results:

  • Microalbuminuria is an early sign of kidney damage in FMF.
  • NGAL shows potential as a marker to improve renal outcomes.
  • SAA is a reliable indicator of inflammation and treatment adherence, and may predict AA amyloidosis course.
  • Monoclonal antibodies targeting IL-1 and IL-6 offer new treatment avenues for FMF and AA amyloidosis.

Conclusions:

  • Accurate diagnosis is crucial for managing nephrotic syndrome in FMF.
  • Biomarkers like NGAL and SAA are vital for monitoring kidney health and disease activity.
  • Targeted therapies, including IL-1 and IL-6 inhibitors, represent a significant advancement in FMF and AA amyloidosis management.
Keywords:
AA amyloidosisFamilial Mediterranean feverNGALanakinracanakinumabglomerulonephritisnephrotic syndromeserum amyloid Atocilizumabvasculitis

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