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Urinary glycosaminoglycans in the course of familial Mediterranean fever
Esra Baskin1, Umit Saatçi, Gönenç Ciliv
1Baskent University Hospital, Department of Paediatric Nephrology, 6. Cadde, 72/3 Bahcelievler, 06490, Ankara, Turkey. esrabaskin@hotmail.com
Unlabelled:
Familial Mediterranean fever (FMF) is characterised by recurrent fever and serositis. The most important complication of the disease is amyloidosis. Cheap and non-invasive methods would be important in predicting or establishing the early diagnosis of amyloidosis. For this purpose, we studied the role of urinary glycosaminoglycans (GAG). The study group included 123 FMF patients without an attack and 11 patients with FMF associated amyloidosis. Ten healthy children and ten patients with primary nephrotic syndrome served as controls. In patients with amyloidosis, urinary GAG were lower than in patients with FMF, patients with nephrotic syndrome and controls (median and range: 8.54 mg hexuronic acid/g creatinine (1.87-25.5), 5.8 (1.7-17.26), 23.12 (8.74-28.06) and 19.25 (14.2-26.9) respectively, P<0.01). There was a significant negative correlation between the duration of the disease and urinary GAG ( r= -043, P=0.002). In 49 FMF patients with a low GAG, urinary GAG increased significantly after an increase in the colchicine dose (median and range: 6.64 mg hexuronic acid/g creatinine (1.77-19.39) and 9.45 mg hexuronic acid/g creatinine (2.36-28.9), P<0.01).
Conclusion:
These results suggest that urinary glycosaminoglycan levels may be a predictor of amyloidosis in patients with familial Mediterranean fever. We also suggest that effective colchicine doses may be monitored by following urinary glycosaminoglycan excretion.
Insights
Urinary glycosaminoglycans (GAG) may predict amyloidosis in Familial Mediterranean Fever (FMF) patients. Monitoring urinary GAG can also help optimize colchicine dosage for FMF treatment.
Area of Science:
- Rheumatology
- Nephrology
- Biochemistry
Background:
- Familial Mediterranean Fever (FMF) is an autoinflammatory disease characterized by recurrent fever and serositis.
- Amyloidosis is a serious complication of FMF, leading to organ damage.
- Early detection and prediction of amyloidosis are crucial for effective management.
Purpose of the Study:
- To investigate the role of urinary glycosaminoglycans (GAG) as potential biomarkers for predicting amyloidosis in FMF patients.
- To explore the correlation between urinary GAG levels and disease duration in FMF.
- To assess the impact of colchicine dosage adjustments on urinary GAG excretion.
Main Methods:
- A study involving 123 FMF patients without active attacks and 11 FMF patients with amyloidosis.
- Inclusion of healthy children and patients with primary nephrotic syndrome as control groups.
- Measurement of urinary GAG levels and correlation analysis with disease duration and colchicine treatment.
Main Results:
- Urinary GAG levels were significantly lower in FMF patients with amyloidosis compared to FMF patients without amyloidosis, nephrotic syndrome patients, and healthy controls.
- A significant negative correlation was observed between the duration of FMF and urinary GAG levels.
- In FMF patients with initially low urinary GAG, levels increased significantly after an increase in colchicine dose.
Conclusions:
- Urinary GAG levels show potential as predictive biomarkers for amyloidosis in Familial Mediterranean Fever.
- Urinary GAG excretion can serve as an indicator for monitoring the effectiveness of colchicine therapy in FMF.
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