Amyloidosis and Glomerular Diseases in Familial Mediterranean Fever
Abstract
:1. Introduction
- Recurrent fever attacks with serositis;
- AA amyloidosis, without other predisposing diseases;
- Symptoms responsive to colchicine;
- Recurrent fever episodes;
- The presence of erysipelas-like erythema;
- Family history of FMF;
- (1)
- Type 1, with short episodes of inflammation and serositis;
- (2)
- Type 2, characterized by the onset of secondary amyloidosis without any other overt systemic symptoms of FMF;
- (3)
- Type 3 noted as “silent homozygous or compound heterozygote state” without relevant symptoms.
2. A Focus on Secondary Amyloidosis
- hereditary forms, due to genetic variants of amyloid precursors such as transthyretin (TTR), fibrinogen Aα, ApoAI, ApoAII, ApoAIV, ApoCII, ApoCIII, lysozyme, gelsolin, β2-microglobulin (β2M) and cystatin;
- primary forms due to acquired plasma cells disorders with over-expression of monoclonal immunoglobulin (Ig) light chains (AL), rarely of Ig heavy chains (AH) or both (AHL);
- secondary amyloidosis (AA) due to chronic inflammatory processes which enhance the synthesis of acute-phase reactants such as SAA or CRP production and progressive deposition;
- senile amyloidosis, due to misfolding wild-type TTR (ATTRwt);
- Aβ2M wild-type, especially in patients requiring renal replacement therapy (RRT) with hemodialysis.
3. The Role of SAA in FMF and AA Amyloidosis Follow-Up
4. Glomerular Diseases and FMF
5. The Nephrologist’s Point of View
6. Future Perspectives: Biomarkers and New Therapeutic Strategies
7. Conclusions
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Conflicts of Interest
References
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Siligato, R.; Gembillo, G.; Calabrese, V.; Conti, G.; Santoro, D. Amyloidosis and Glomerular Diseases in Familial Mediterranean Fever. Medicina 2021, 57, 1049. https://doi.org/10.3390/medicina57101049
Siligato R, Gembillo G, Calabrese V, Conti G, Santoro D. Amyloidosis and Glomerular Diseases in Familial Mediterranean Fever. Medicina. 2021; 57(10):1049. https://doi.org/10.3390/medicina57101049
Chicago/Turabian StyleSiligato, Rossella, Guido Gembillo, Vincenzo Calabrese, Giovanni Conti, and Domenico Santoro. 2021. "Amyloidosis and Glomerular Diseases in Familial Mediterranean Fever" Medicina 57, no. 10: 1049. https://doi.org/10.3390/medicina57101049