Cyclosporine and Familial Mediterranean Fever Amyloidosis

Siegal, B; Zemer, Debora; Pras, Mordechai

doi:10.1097/00007890-198606000-00027

Cited by 10 publications

(1 citation statement)

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“…However, the fact that several other categories of chronic renal disease, apart from amyloidosis, are associated with RHP should be borne in mind. Use of the immunosuppressive agent cyclosporin, in patients with RHP complicated by amyloidosis, who are receiving colchicine [101], is limited by poor tolerance, including gastrointestinal side-effects and muscle weakness [102].…”

Section: Managementmentioning

confidence: 99%

Recurrent Hereditary Polyserositis or Familial Mediterranean Fever: An Overview

Cook

1991

Ann Saudi Med

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The etiology and pathogenesis of recurrent hereditary polyserositis (RHP) remain undetermined. There is good evidence for a genetic basis (transmission being by a recessive Mendelian mode of inheritance), but not all cases have a family history and an infective (or other environmental) component has not been excluded. The marked variation in presentation and complications (in particular the prevalence of amyloid deposition) in different series suggests that this might not be a homogeneous entity. Vascular involvement is a basic prerequisite for the periodic attacks, and evidence for transient immunological abnormalities is incontrovertible. There are no reliable diagnostic techniques. Colchicine is of value in treatment, but its mode of action is unclear. More macro-and microepidemiological studies are required and will be of paramount importance. Clinical observations and molecular genetic studies should address the possibility that two or more immunological or metabolic defects might be interwoven; why for example do only some affected groups develop AA amyloidosis? Simple, non-invasive diagnostic tests are required for the uncomplicated disease and also for the presence of amyloid deposition. Preventive strategies might eventually involve genetic engineering techniques, but in the immediate future, education of doctors and other health care workers-to raise the "index of awareness" of RHP, in order that colchicine treatment can be commenced early in the disease-forms an important strategy. Colchicine therapy is not without complications and an alternative chemotherapeutic agent should be sought. GC Cook, Recurrent Hereditary Polyserositis or Familial Mediterranean Fever: An Overview. 1991; 11(5): 576-584 Many aspects of recurrent hereditary polyserositis (RHP) have recently been reviewed [1][2][3][4]. The first description of a familial condition comprising "recurring attacks of a peculiar nature" was made by Janeway and Mosenthal in 1908. In that report, a 16-year-old Jewish schoolgirl "without special neurotic inheritance" had suffered febrile bouts associated with abdominal pain, which consistedof prodromal, crescendo, and recovery phases, since the age of 2 weeks [5]. In view of the dramatic nature of many attacks, it is perhaps surprising that no records of the disease have been discovered in early medical (and also lay) literature from the Mediterranean and Middle East, including the work of Hippocrates, the Bible, and the Quran. Subsequent accounts of RHP followed

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Section: Managementmentioning

confidence: 99%

Recurrent Hereditary Polyserositis or Familial Mediterranean Fever: An Overview

Cook

1991

Ann Saudi Med

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Familial Mediterranean fever in the colchicine era: The fate of one family

et al. 1993

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In order to demonstrate the effect of prophylactic colchicine treatment on the natural history of familial Mediterranean fever (FMF), a family is presented with 6 out of 9 siblings affected by FMF. Each patient represents a different stage of the amyloidotic kidney disease of FMF and the effect of continuous colchicine treatment on its course. Considered together, the members of this family present an almost complete clinical, genetic, and behavioral picture of the disease.

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Renal Transplantation in Systemic Inherited and Metabolic Disease

Lederer¹,

Suki²

1991

Therapy of Renal Diseases and Related Disorders

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Cyclosporine and Familial Mediterranean Fever Amyloidosis

Cited by 10 publications

References 0 publications

Recurrent Hereditary Polyserositis or Familial Mediterranean Fever: An Overview

Recurrent Hereditary Polyserositis or Familial Mediterranean Fever: An Overview

Familial Mediterranean fever in the colchicine era: The fate of one family

Renal Transplantation in Systemic Inherited and Metabolic Disease

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