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Amyloidosis in Familial Mediterranean Fever
Amyloidosis and Familial Mediterranean Fever Syndrome (heller)

Presentation

Renal involvement is the main clinical complication and it mostly presents with nephrotic syndrome and chronic renal failure. [ncbi.nlm.nih.gov]

Entire Body System

  • Fever

    METHODS: Ninety-four patients with familial Mediterranean fever and 60 healthy volunteers were included in the study. Of the patients, 12 had familial Mediterranean fever related amyloidosis. [ncbi.nlm.nih.gov]

    (FMF) is an autosomal recessive disease characterised by recurrent attacks of fever and serositis. [ard.bmj.com]

Treatment

Treatment of the underlying disease decreases the frequency of this complication however if it develops there is no established treatment of AA amyloidosis. [acrabstracts.org]

Although a number of agents have been considered, there is no established treatment of AA amyloidosis. [ojrd.biomedcentral.com]

Treatment The treatment for FMF is a drug called colchicine. Colchicine was originally derived from a flower called the autumn crocus or meadow saffron. This flower has been known to possess medicinal powers since the time of ancient Greece. [amyloidosis.org.uk]

Anti-interleukin-1 drugs are the most important agents of biological treatments. In this review, colchicine resistance and treatment options will be evaluated. [ingentaconnect.com]

In this report, we have described a renal transplant recipient with delayed gastric emptying secondary to amyloidosis due to familial Mediterranean fever, which improved with erithromycin treatment. [ncbi.nlm.nih.gov]

Prognosis

The key take-home message is that, since the introduction of colchicine, the prognosis for patients with FMF is excellent. [dermatologyadvisor.com]

NAKD is common in FMF and, compared to amyloidosis, it is featured with milder course and better prognosis. Contrary to common practice, it is highly recommended to obtain a kidney biopsy from patients with FMF and proteinuria more than 0.5 g/24 h. [jrheum.org]

Prognosis Currently, except for those who get amyloidosis and chronic arthritis, people with FMF have a good quality of life between episodes and an excellent prognosis. [the-medical-dictionary.com]

Prognosis For those individuals who are diagnosed early enough and take colchicine consistently, the prognosis is excellent. [medical-dictionary.thefreedictionary.com]

Etiology

We believe that periodontitis may be an additive factor to traditional etiological factors for systemic AA amyloidosis as well as being a primary etiological factor. [hypothesisjournal.com]

The etiology of colchicine resistance (CR) remains largely unknown. Studies have shown that low vitamin D levels are more prevalent in patients with FMF compared to healthy controls. [vitamindcouncil.org]

(Etiology) Defective mutation on a particular gene called the MEFV gene, is the principal cause of Familial Mediterranean Fever The symptoms of FMF are due to a defective protein that causes inflammation of the joints and other body regions. [dovemed.com]

Etiology FMF is an autosomal recessive disorder, resulting from mutations in the MEFV gene. FMF carrier frequencies are extremely high in the eastern Mediterranean. Pathophysiology MEFV encodes a protein called pyrin. [dermatologyadvisor.com]

J Rheumatol. vol. 38. 2011. pp. 516 Ongoing controversies regarding etiology, diagnosis, treatment Long term risks/benefits of treatment with biologics, given uncertain future risks, particularly for malignancies, opportunistic infections, autoimmune [clinicaladvisor.com]

Epidemiology

In Armenian persons (based on epidemiology among Armenian populations in Lebanon and southern California), the estimated prevalence of FMF is 1 case per 500 population, with a gene frequency of 1 per 7. [emedicine.medscape.com]

Epidemiology FMF is common in people from eastern Mediterranean countries but is not restricted to these ethnic groups. [ 5 ] Male-to-female ratio is 1.5-2:1. Presentation [ 6, 7, 8 ] The majority present in the first decade of life. [patient.info]

[…] with colchicine-resistant FMF and in two additional autoinflammatory recurrent fever syndromes: mevolonate kinase deficiency (hyper- immunoglobulin D syndrome, or HIDS ) and tumor necrosis factor receptor-associated periodic syndrome ( TRAPS ). [19] Epidemiology [en.wikipedia.org]

American Journal of Epidemiology 175 (10): 1054–1061. Yilmaz E, Ozen S, Balci B, et al. (2001) Mutation frequency of familial Mediterranean fever and evidence for a high carrier rate in the Turkish population. [els.net]

Pathophysiology

Pathophysiology Associated with mutations in the MEFV (Mediterranean fever) gene located on chromosome 16. [ 2 ] Although FMF was originally defined as an autosomal recessive disorder, approximately 10-20% of FMF patients do not carry any FMF gene (MEFV [patient.info]

Moreover, as the pathophysiology of this disorder becomes less of a hypothesis and more of an understood entity, it is likely that treatment options will broaden beyond the use of daily prophylactic colchicine. [thedoctorsdoctor.com]

Pathophysiology MEFV encodes a protein called pyrin. Wild-type pyrin appears to have an important role in the regulation of the pro-inflammatory pathways of the innate immune system. [dermatologyadvisor.com]

Whether this is due to modifier genes or environmental factors remains to be established. [12] Pathophysiology [ edit ] Virtually all cases are due to a mutation in the Mediterranean Fever (MEFV) gene on the sixteenth chromosome, which codes for a protein [en.wikipedia.org]

Masters SL, Simon A, Aksentijevich I, et al. (2009) Horror autoinflammaticus: the molecular pathophysiology of autoinflammatory disease. Annual Review of Immunology 27: 621–628. [els.net]

Prevention

As well as preventing FMF attacks, regular colchicine treatment is effective in preventing development of AA amyloidosis in most patients with FMF. [amyloidosis.org.uk]

Therefore, it may prevent the deposition of serum amyloid A fibers. [link.springer.com]

Colchicine prevents attacks and renal amyloidosis.5% to 10% of the patients with FMF are resistant or intolerant to colchicine. Colchicine resistant patients may receive biological therapies. [ingentaconnect.com]

May be sufficient to prevent attacks, but may or may not prevent renal amyloidosis. [clinicaladvisor.com]

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