Familial Mediterranean fever

Plasma cell disorders Plasma cell disorders Inflammation-associated, familial Mediterranean fever Familial amyloidotic neuropathy, systemic senile amyloidosis Dialysis-associated amyloidosis Familial amyloidotic neuropathy, aortic amyloidosis Familial systemic amyloidosis Familial systemic amyloidosis Familial systemic amyloidosis Familial cerebral hemorrhage with amyloidosis Sporadic and familial Alzheimer s disease, familial cerebral hemorrhage with amyloidosis Spongiform encephalopathies C-cell thyroid tumors Insulinoma, type II diabetes Atrial amyloidosis Prolactinomas pituitary amyloidosis Iatrogenic amyloidosis Corneal amyloidosis ... 

A geographic area (for example, familial Mediterranean fever, which occurs mainly in populations bordering the Mediterranean Sea)... 

Colchicine [Antigout Agent/Colchicum Alkaloid] Uses Acute gouty arthritis prevention of recurrences familial Mediterranean fever primary biliary cirrhosis Action -1- Migration of leukocytes X leukocyte lactic acid production Dose Initial 0.6—1.2 mg PO, then 0.6 mg ql-2h until rehef or GI SE develop (max 8 mg/d) do not r eat for 3 d /U 1-3 mg, then 0.5 mg q6h until rehef (max 4 mg/d) do not rqjeat for 7 d Prophylaxis PO 0.6 mg/d or 3-4 d/wk ... 

Familial Mediterranean fever. A patient with familial Mediterranean fever was presented with chronic relapsing pain and... 

CS447 Holdcroft, A., M. Smith, A. Jacklin, et al. Pain relief with oral cannab-inoids in familial Mediterranean fever. Anaesthesia 1997 52(5) 483-486. 

Unlabeled Uses To reduce frequency of recurrence of familial Mediterranean fever treatment of acute calcium pyrophosphate deposition, amyloidosis, biliary cirrhosis, recurrent pericarditis, sarcoid arthritis... 

Transient diabetes and hyperlipidemia have been reported. Metabolic acidosis is probably a consequence of heavy, cholera-like diarrhea. Progressive reduction of libido was attributed to colchicine in patients with familial Mediterranean fever (312). 

Peters RS, Lehman TJ, Schwabe AD. Colchicine use for familial Mediterranean fever. Observations associated with long-term treatment. West J Med 1983 138(l) 43-6. 

Daily administration of colchicine is useful for the prevention of attacks of familial Mediterranean fever (familial paroxysmal polyserositis) and for prevention and treatment of amyloidosis in such patients. Colchicine appears to benefit patients with primary biliary cirrhosis in terms of improvement of liver function tests and perhaps of survival. Colchicine also has been employed to treat a variety of skin disorders, including psoriasis and Behcet s syndrome. 

Colchicine is another possible candidate for the treatment of Alzheimer s disease. This drug effectively treats familial Mediterranean fever, a condition in which recurrent inflammation and renal amyloidosis occur. Although the amyloid constituents in familial Mediterranean fever and Alzheimer s disease differ, both illnesses involve chronic inflammation, elevated acute-phase proteins, and abnormal processing of a precursor protein leading to deposition of insoluble amyloid fragments. These similarities suggest the potential therapeutic efficacy of colchicine for patients with Alzheimer s disease. 

Therapeutically effective doses of colchicine in gout are 2-3 mg per patient on day 1 with smaller doses later, and 0.5-1 mg/day for 1-3 years given orally in familial Mediterranean fever (131). Side effects such as ileal dysfunction, decreased vitamin Bi2 absorption, and an increased absorption of steroids occur after intake of 7-10 mg/day, which if continued for 4-5 days result in dehydration and renal shutdown (96). The three congeners of allocolchicine, jerusalemine, salimine, and suhailamine, which all had (—)-rotations were isolated from Colchicum decaisnei Boiss. (141). 

The alkaloid colchicine (FI) isolated from the medicinal plant Colchicum autumnale L. (Liliaceae family) still is used to treat gout and familial Mediterranean fever. FI and thiocolchicine (F2) (SCH3 rather than OCH3 at C-10), which is more stable and more potent but slightly more toxic, are mitotic inhibitors that inhibit polymerization of tubulin (69). Although they show antileukemic activity, they are too toxic to use as anticancer agents, which prompts the synthesis of new, less toxic analogs. 

This is an alkaloid derived from the autumn crocus (Colchicum). Colchicine rapidly relieves the pain and inflammation of an acute attack of gout. Such swift relief is considered to confirm the diagnosis because non-gouty arthritis is unaffected, though failure does not prove the patient is free of gout. It is most effective if given within 24 h of onset and is useful in patients in whom NSAIDs are contraindicated. It is also used in recurrent hereditary polyserositis (Familial Mediterranean Fever) when it may prevent attacks and the development of amyloid. The t) is 1 h. 

AA amyloid appears in a generalized form and is mainly stored perireticularly in the kidney, spleen and liver. This kind of amyloidosis occurs as (1.) a congenital form in cases of familial Mediterranean fever, (2.) an idiopathic (= primary) form without any associated basic disease, and (i.) a reactive (= secondary) form in chronic inflammations or tumours (e.g. Hodgkin s disease) as well as in drug abuse and AIDS. 

There is controversy about the long-term toxicity of colchicine. In familial Mediterranean fever, low dosages of colchicine (1-2 mg/day) for 15-18 years have been well tolerated, even by young patients (SEDA-16,114). 

Life-threatening hyperkalemia secondary to the use of standard doses of co-trimoxazole has been reported in two renal transplant recipients who developed end-stage renal disease secondary to familial Mediterranean fever, who may be at increased risk of hyperkalemia because of concurrent renal insufficiency, concomitant use of... 

An increase in the frequency of attacks of familial Mediterranean fever has been reported after the start of systemic therapy with isotretinoin for nodulocystic acne (108). 

Colchicine is used in the treatment of acute gouty arthritis. Unlabeled uses include treatment of familial Mediterranean fever, neoplasms of the skin, and cirrhosis of the liver. It is available as a pesticide for moles and gofers. 

The glycosaminoglycans present in abnormally high amounts in amyloid extracts of amyloid-laden organs from cases of rheumatoid arthritis, familial Mediterranean fever, Hodgkin s disease, and tuberculosis are largely dermatan sulfate and heparan sulfate (P7). The glycosaminoglycans are increased in the livers of cases of Gm,-gangliosidosis type I (C2), and keratan sulfate is predominantly in excess. This occurrence... 

Colchicine is marketed in tablets used for treatment of gout and familial Mediterranean fever and is found in certain plants autumn crocus or meadow saffron (Colchicum autumnale) and glory lily Gloriosa superba). A colchicine overdose is ex-... 

IV. Diagnosis. A syndrome beginning with severe gastroenteritis, leucocytosis, shock, rhabdomyolysis, and acute renal failure and, several days later, leukopenia and thrombocytopenia should suggest colchicine poisoning. A history of gout or familial Mediterranean fever in the patient or a family member is also suggestive. 

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