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

• 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 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]

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]

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]

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 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]

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]