Related Experiment Videos
[Familial Mediterranean fever with amyloidosis. Recent pathogenetic and therapeutic aspects]
Abstract:
Familial Mediterranean fever was diagnosed in a 34-year-old Turkish patient with severe nephrotic oedema. Immunohistochemical classification of a biopsy specimen showed amyloidosis of the AA-type. There was a definite increase of serum amyloid-A-protein (SAA). The typical recurrent fever, attacks of abdominal pain with symptoms of subileus and joint swelling could be treated successfully with colchicine, the oedema with diuretics. The progression of renal failure and proteinuria as indicator of the degree of amyloid-induced renal damage remained unaffected by this treatment. With dimethyl-sulfoxide (DMSO) a marked improvement in renal function and a lowering of the SAA level could be achieved. Thus this treatment inhibits the progression of amyloidosis of the AA-type in Mediterranean fever and may be considered for other forms of AA-type amyloidoses. It is possible that the lowering of the SAA-serum concentration and the improvement of renal function is due to an antiphlogistic effect of DMSO, the mechanism of action of which is so far unknown.
Insights
Familial Mediterranean Fever (FMF) patients with AA-type amyloidosis experienced severe nephrotic edema. Dimethyl sulfoxide (DMSO) treatment improved renal function and lowered serum amyloid-A protein (SAA) levels.
Area of Science:
- Nephrology
- Rheumatology
- Internal Medicine
Background:
- Familial Mediterranean Fever (FMF) is a genetic autoinflammatory disorder.
- AA-type amyloidosis, characterized by serum amyloid-A protein (SAA) deposition, can be a severe complication of FMF.
- Nephrotic edema indicates significant renal involvement in AA-type amyloidosis.
Observation:
- A 34-year-old Turkish patient with FMF presented with severe nephrotic edema and biopsy-confirmed AA-type amyloidosis.
- Standard treatments (colchicine, diuretics) managed FMF symptoms and edema but did not halt renal failure progression.
- Elevated serum amyloid-A protein (SAA) levels were noted in the patient.
Findings:
- Dimethyl sulfoxide (DMSO) treatment led to marked improvement in renal function.
- DMSO therapy significantly reduced serum amyloid-A protein (SAA) levels.
- The progression of renal failure and proteinuria remained unaffected by colchicine and diuretics.
Implications:
- DMSO may inhibit the progression of AA-type amyloidosis in FMF.
- DMSO's antiphlogistic properties could be responsible for its therapeutic effects.
- This treatment warrants consideration for other forms of AA-type amyloidosis.