Familial Mediterranean Fever and Cryptogenic Cirrhosis

Tweezer-Zaks, Nurit; Doron-Libner, Anat; Weiss, Perez; Ben‐Horin, Shomron; Barshack, Iris; Lidar, Merav; Livneh, Avi

doi:10.1097/md.0b013e31815be056

Cited by 27 publications

(23 citation statements)

References 39 publications

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“…In a minority of cases, discontinuation of colchicine will lead to normalisation of the enzyme values, but in most there proves to be no clear association with the drug. Tweezer-Zaks et al , reported that cryptogenic cirrhosis is more common among patients with FMF compared with its prevalence in the general population 56. Rimar et al 57 reported that non-alcoholic cirrhosis is more common in FMF, perhaps as a result of uncontrolled inflammation.…”

Section: Resultsmentioning

confidence: 99%

EULAR recommendations for the management of familial Mediterranean fever

et al. 2016

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Familial Mediterranean fever (FMF) is the most common monogenic autoinflammatory disease, but many rheumatologists are not well acquainted with its management. The objective of this report is to produce evidence-based recommendations to guide rheumatologists and other health professionals in the treatment and follow-up of patients with FMF. A multidisciplinary panel, including rheumatologists, internists, paediatricians, a nurse, a methodologist and a patient representative, was assembled. Panellists came from the Eastern Mediterranean area, Europe and North America. A preliminary systematic literature search on the pharmacological treatment of FMF was performed following which the expert group convened to define aims, scope and users of the guidelines and established the need for additional reviews on controversial topics. In a second meeting, recommendations were discussed and refined in light of available evidence. Finally, agreement with the recommendations was obtained from a larger group of experts through a Delphi survey. The level of evidence (LoE) and grade of recommendation (GR) were then incorporated. The final document comprises 18 recommendations, each presented with its degree of agreement (0-10), LoE, GR and rationale. The degree of agreement was greater than 7/10 in all instances. The more controversial statements were those related to follow-up and dose change, for which supporting evidence is limited. A set of widely accepted recommendations for the treatment and monitoring of FMF is presented, supported by the best available evidence and expert opinion. It is believed that these recommendations will be useful in guiding physicians in the care of patients with FMF.

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Section: Resultsmentioning

confidence: 99%

EULAR recommendations for the management of familial Mediterranean fever

et al. 2016

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“…In this setting, mitochondrial heteroplasmy (variable distribution of weak mitochondria) may play a role in variable organ susceptibility to common oxidative stress [69]. Familial Mediterranean fever has also been reported to be associated with cryptogenic cirrhosis [70], and cryptogenic cirrhosis may also be seen in systemic lupus erythematosus, presumably related to an autoimmune process [71]. We have observed cryptogenic cirrhosis in a 27-year-old male with moderate obesity, type 2 diabetes, late-onset loss of sight and hearing, and chronic renal failure who was subsequently found to suffer Alstrom syndrome (a form of celiopathy) [72,73].…”

Section: What Are We Missing?mentioning

confidence: 98%

Cryptogenic Cirrhosis: What Are We Missing?

Caldwell

2010

Curr Gastroenterol Rep

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Cryptogenic cirrhosis remains a common clinical condition although recent advances have allowed for a better understanding of underlying conditions and associations. The evolving terminology applied to this condition has resulted in some confusion and persistent variation among pathologists and clinicians. Typical patients are middle aged with only minor liver enzyme abnormalities. Presentations range from incidentally discovered cirrhosis to complications of advanced portal hypertension and hepatocellular cancer. Clinicopathologic analysis of these patients indicates that the leading causes include previously unrecognized nonalcoholic steatohepatitis, silent autoimmune hepatitis, non-B, non-C viral hepatitis, and occult past ethanol exposure. In this article, we review these associations as well as a proposed classification system for cryptogenic cirrhosis and other lesser known genetic and syndromic associations that warrant consideration when evaluating these individuals.

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“…All patients showed clinical and laboratory improvement upon initiation of colchicine therapy. In addition, nine cases of cryptogenic cirrhosis, cirrhosis without an identifi able cause, were reported in FMF patients by Tweezer-Zaks et al further supporting an association between liver disease and FMF [ 154 ]. Taken together, these fi ndings suggest that FMF should be considered in the differential diagnosis of cryptogenic chronic hepatitis/cirrhosis, especially in regions where this infl ammatory disease is prevalent [ 153 ].…”

Section: Chronic Liver Diseasementioning

confidence: 71%

Clinical Picture in Adulthood and Unusual and Peculiar Clinical Features of FMF

Ben-Zvi

Lidar

Giat

et al. 2015

Rare Diseases of the Immune System

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4 IntroductionFamilial Mediterranean fever (FMF) is classically manifested with painful, irregularly recurrent, short-lasting bouts of serosal infl ammation (mainly peritonitis, synovitis or pleuritis), accompanied by fever, and resolving spontaneously [ 1 ]. A rise then fall of acute-phase reactants is typical, but some patients might suffer from continuous infl ammation and its associated manifestations [ 2 ]. The disease onset is usually in childhood or adolescence, but, in some patients, the initial symptoms develop later in life, during the ages of 20-40 years and sometimes even later [ 3 ]. In general, late-onset FMF has a mild phenotype and its genotype lacks the homozygous M694V genotype [ 3 ]. The clinical manifestations may vary between patients, including members of the same family and even between identical twins 48 [ 4 , 5 ]. Clinical diversity may also be seen in the same individual, with different site of attacks in different episodes. Approximately 50 % of FMF patients experience a prodrome over the day preceding the attack, usually manifested as discomfort at the impending attack site and various constitutional, emotional, and physical manifestations [ 6 ]. Many factors have been described as triggers of the FMF attack, including stressful experiences [ 7 ], exposure to cold weather, surgical operations, drugs, certain food items, and menstrual cycles [ 8 , 9 ]. The typical length of the attacks is 24-72 h. The most frequent clinical manifestations of FMF are peritonitis (in ~95 % of FMF patients), fever (>90 %), acute arthritis or arthralgia (~50 %), myalgia (~40 %), pleuritis (~30 %), and erysipelas-like erythema (ELE) (~5 %) [ 10 , 11 ]. Other clinical features are less frequent, including pericarditis, protracted febrile myalgia, protracted arthritis, and tunica vaginalitis (resembling orchitis).In this chapter, we describe the classic manifestations of adult FMF, as well as other manifestations, usually not in the mainstream of the FMF routine daily doings, involving the neurologic, skin, cardiovascular (CV), renal, hepatobiliary, and pulmonary systems. Classic Manifestations of FMF The Peritoneal AttackThe most common site of acute FMF attack is the abdomen, and the majority of abdominal attacks are accompanied by fever. The abdominal attack is caused by infl ammation of the peritoneum, and therefore, the classical attack in its most severe presentation simulates acute abdomen of a perforated viscera. This fact is important when contemplating a diagnosis of FMF. The presence of peritoneal irritation turns recurrent episodes of abdominal pain from a highly nonspecifi c manifestation into a diagnostic clue. In fact, many FMF patients have a history of appendectomy, with an erroneous removal of benign appendix (white appendix). During the abdominal attack, physical examination will elicit peritoneal signs (rebound, rigidity), distention, and decreased peristalsis. A plain abdominal fi lm may show air-fl uid levels in the intestine. Usually constipation characterizes the abdominal attack, but i...

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Familial Mediterranean Fever and Cryptogenic Cirrhosis

Cited by 27 publications

References 39 publications

EULAR recommendations for the management of familial Mediterranean fever

EULAR recommendations for the management of familial Mediterranean fever

Cryptogenic Cirrhosis: What Are We Missing?

Clinical Picture in Adulthood and Unusual and Peculiar Clinical Features of FMF

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