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Purpose of this study is to determine whether keeping SAA on normal or near normal level will delay progression of renal failure in patients with amyloidosis secondary to FMF.
FMF is an inherited inflammatory disorder typically presented in most causes as recurrent episodes of fever and serositis. Phenotype II, another kind of this disorder, has atypical courses, when the inflammation proceeds without any clinical sign.
Each FMF attack is accompanied by sharp elevation of inflammatory markers in the serum, and serum amyloid A (SAA) one of them. The level of these inflammatory markers returns to normal with termination of the attack. The SAA is the main component of amyloids fibrils and constantly high level of SAA after the attack (as occurs in undiagnosed or undertreated disease) is the significant risk factor responsible for development of amyloidosis. On the other hand, in patients with phenotype II the amyloidosis occurs despite absolute absence of the attacks.
The kidney is one of the first organ suffers from amyloid deposits. The spectrum of kidney damage spread wildly from mild proteinuria to obvious nephrotic syndrome with disturbance in renal function and progression to end stage renal failure.
It is well known that deterioration of renal disease in AA amyloidosis links to level of SAA in serum. The permanently high SAA level is a major factor responsible to progression of renal disease. Occasionally, however, decline in the renal function occurred despite normal or near normal levels of SAA. Renal impairment in these cases may be explained by mechanisms existing in other kidney diseases when uncontrolled proteinuria aggravates renal dysfunction. The purpose of the study is to find whether a cohort of patients followed in our clinic and receiving colchicine for FMF- amyloidosis according to the SAA levels, monitored periodically, have better prognosis than an historical cohort receiving colchicine according to the attack status
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| SAA monitored group | FMF-Amyloidosis patients receiving colchicine with a purpose to normalize SAA levels | ||
| Historical control group | FMF-Amyloidosis patients receiving colchicine at a dose determined to stop FMF attacks. obtained from the Fibrillex study |
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Inclusion Criteria:
Exclusion Criteria:
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20 FMF patients with AA amyloidosis demonstrated by positive biopsy of any target organs
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| Name | Affiliation | Role |
|---|---|---|
| Avi Livneh, MD | Sheba Medical Center | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Sheba Medical Center | Tel Litwinsky | 52621 | Israel |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 17554117 | Background | Lachmann HJ, Goodman HJ, Gilbertson JA, Gallimore JR, Sabin CA, Gillmore JD, Hawkins PN. Natural history and outcome in systemic AA amyloidosis. N Engl J Med. 2007 Jun 7;356(23):2361-71. doi: 10.1056/NEJMoa070265. | |
| 11454373 | Background | Gillmore JD, Lovat LB, Persey MR, Pepys MB, Hawkins PN. Amyloid load and clinical outcome in AA amyloidosis in relation to circulating concentration of serum amyloid A protein. Lancet. 2001 Jul 7;358(9275):24-9. doi: 10.1016/S0140-6736(00)05252-1. |
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| ID | Term |
|---|---|
| D010505 | Familial Mediterranean Fever |
| C000718787 | AA amyloidosis |
| D051437 | Renal Insufficiency |
| D009404 | Nephrotic Syndrome |
| ID | Term |
|---|---|
| D056660 | Hereditary Autoinflammatory Diseases |
| D030342 | Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D007674 | Kidney Diseases |
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| 17103173 | Background | Yalcinkaya F, Cakar N, Acar B, Tutar E, Guriz H, Elhan AH, Ozturk S, Kansu A, Ince E, Atalay S, Girgin N, Dogru U, Aysev D, Ekim M. The value of the levels of acute phase reactants for the prediction of familial Mediterranean fever associated amyloidosis: a case control study. Rheumatol Int. 2007 Apr;27(6):517-22. doi: 10.1007/s00296-006-0265-6. Epub 2006 Nov 14. |
| D014570 | Urologic Diseases |
| D052776 | Female Urogenital Diseases |
| D005261 | Female Urogenital Diseases and Pregnancy Complications |
| D000091642 | Urogenital Diseases |
| D052801 | Male Urogenital Diseases |
| D009401 | Nephrosis |