The grandfather’s fever

Ricci, Paola; Stella, Alessandro; Settimo, Enrica; Passerini, Francesca; Minerva, Francesco; Belfiore, Anna; Palmieri, Vincenzo; Pugliese, Stefania; Scaccianoce, Giuseppe; Portincasa, Piero

doi:10.1007/s10067-019-04741-9

Cited by 7 publications

(4 citation statements)

References 39 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Furthermore, colchicine may be useful in treating fibrosing disorders such as pulmonary fibrosis, scleroderma, retroperitoneal fibrosis and even cirrhosis 73 . Similarly, in the case reports and series previously described, initiation of colchicine led to normalization of liver biochemical tests, suggesting its beneficial effect on the liver 11,38–40,43 …”

Section: Discussionsupporting

confidence: 59%

See 1 more Smart Citation

Non‐amyloid liver involvement in familial Mediterranean fever: A systematic literature review

Fraisse

Savey

Hentgen

et al. 2020

Liver International

View full text Add to dashboard Cite

Introduction: Familial Mediterranean fever (FMF), the most frequent autoinflammatory disease, is caused by mutations in the MEFV gene. It is characterized by recurrent febrile attacks of polyserositis. Liver abnormalities may develop during its course, but they remain poorly defined.Objective: To describe liver involvement in FMF patients. Methods:A systematic search was conducted through PubMed/Medline and Embase from 1946 to January 2020. All articles describing children and adults with FMF and liver involvement were included. Patients with amyloidosis were excluded. The selected full-text articles were independently reviewed by three investigators.Results: Forty-three articles were identified, of which 20 articles with a total of 99 patients were included: 74 adults, 23 children and two patients of unknown age. Ten patients had cryptogenic cirrhosis, 48 had nonalcoholic fatty liver disease (NAFLD), four had Budd-Chiari syndrome (BCS), 12 had isolated hyperbilirubinaemia and 25 had elevated liver enzymes. Conclusion:Despite a low prevalence of metabolic risk factors, FMF may be associated with NAFLD and cryptogenic cirrhosis as a consequence of chronic or recurrent inflammation. FMF patients should be regularly screened for liver injury. The latter may be prevented and treated by daily colchicine intake. The evidence was insufficient to establish an association with BCS, hyperbilirubinaemia or autoimmune hepatitis. K E Y W O R D Scolchicine, cytolysis, familial Mediterranean fever, inflammation, nonalcoholic fatty liver disease, nonalcoholic steatohepatitis

show abstract

Section: Discussionsupporting

confidence: 59%

“…Extensive investigations revealed no alternative cause. Ricci et al 43 described a patient with late‐onset FMF and elevation of cholestatic markers during inflammatory episodes. As in the previous cases, colchicine led to significant improvement of cholestasis, such that further investigations were not pursued.…”

Section: Resultsmentioning

confidence: 99%

Non‐amyloid liver involvement in familial Mediterranean fever: A systematic literature review

Fraisse

Savey

Hentgen

et al. 2020

Liver International

View full text Add to dashboard Cite

show abstract

“…Lebensjahr. Meist sind dies Patient*innen mit heterozygoten Mutationsformen, mildem Krankheitsverlauf und häufig nur inkompletter Symptommanifestation [15].…”

Section: Besonderheiten Im Erwachsenenalterunclassified

Autoinflammation – Unterschiede bei Kindern und Erwachsenen

Krusche

Kallinich

2021

Z Rheumatol

View full text Add to dashboard Cite

Autoinflammation-differences between children and adultsAutoinflammatory diseases present as multisystemic inflammation and often manifest in early childhood. In contrast, in a few diseases, e.g., the recently described VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome, the first symptoms occur exclusively in adulthood. This article describes how the phenotypic expression and severity of individual autoinflammatory diseases differ depending on age. Furthermore, differences in the development of organ damage in children and adults are pointed out. In addition to the hereditary periodic fever syndromes, the clinical picture of deficiency of adenosine deaminase 2, the interferonopathies, periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome as well as VEXAS and Schnitzler syndromes are highlighted.

show abstract

“…Many patients with FMF describe their first attack in early childhood, i.e., before the ages of 10 (65%) and 20 years (90%). Depending on genetic penetrance and phenotypic characteristics, however, the initial attack can occur in subjects aged older than 50 years [ 23 ] with extremely delayed onset represented by one case diagnosed at the age of 86 [ 25 ].…”

Section: Familial Mediterranean Fevermentioning

confidence: 99%

Gut Microbiota between Environment and Genetic Background in Familial Mediterranean Fever (FMF)

Ciaula

Stella

Bonfrate

et al. 2020

Genes

Self Cite

View full text Add to dashboard Cite

The gastrointestinal tract hosts the natural reservoir of microbiota since birth. The microbiota includes various bacteria that establish a progressively mutual relationship with the host. Of note, the composition of gut microbiota is rather individual-specific and, normally, depends on both the host genotype and environmental factors. The study of the bacterial profile in the gut demonstrates that dominant and minor phyla are present in the gastrointestinal tract with bacterial density gradually increasing in oro-aboral direction. The cross-talk between bacteria and host within the gut strongly contributes to the host metabolism, to structural and protective functions. Dysbiosis can develop following aging, diseases, inflammatory status, and antibiotic therapy. Growing evidences show a possible link between the microbiota and Familial Mediterranean Fever (FMF), through a shift of the relative abundance in microbial species. To which extent such perturbations of the microbiota are relevant in driving the phenotypic manifestations of FMF with respect to genetic background, remains to be further investigated.

show abstract

The grandfather’s fever

Cited by 7 publications

References 39 publications

Non‐amyloid liver involvement in familial Mediterranean fever: A systematic literature review

Non‐amyloid liver involvement in familial Mediterranean fever: A systematic literature review

Autoinflammation – Unterschiede bei Kindern und Erwachsenen

Gut Microbiota between Environment and Genetic Background in Familial Mediterranean Fever (FMF)

Contact Info

Product

Resources

About