scholarly journals In familial Mediterranean fever, soluble TREM-1 plasma level is higher in case of amyloidosis

2019 ◽  
Vol 25 (8) ◽  
pp. 487-490 ◽  
Author(s):  
Clémence Gorlier ◽  
Jérémie Sellam ◽  
Ludivine Laurans ◽  
Tabassome Simon ◽  
Irina Giurgea ◽  
...  
Keyword(s):  
Serum Level ◽  
Familial Mediterranean Fever ◽  
Protein Level ◽  
Demographic Data ◽  
Normal Serum ◽  
Soluble Form ◽  
Blood Levels ◽  
Aa Amyloidosis ◽  
Amyloid A ◽  
Mediterranean Fever

We aimed to explore triggering receptor expressed on myeloid cells-1 (TREM-1) activation in familial Mediterranean fever (FMF), the most frequent monogenic auto-inflammatory disease, through the measurement of its serum soluble form, named sTREM-1. Blood samples from patients with FMF according to Livneh criteria followed in the French FMF national center and carrying two pathogenic MEFV mutations were collected. Serum level of sTREM-1 was assessed using ELISA. Demographic data, presence of FMF attack, association with histologically proven AA amyloidosis, and blood levels of C-reactive protein (CRP), serum amyloid A (SAA) protein, and creatinine were collected. TREM-1 was available in 56 patients (33.9% male, mean age 43 yr); AA amyloidosis was associated in six patients (19.6% in FMF). Mean sTREM-1 level did not differ significantly between patients having an attack or not and there was also no significant correlation between the level of sTREM-1 and CRP and SAA protein. However, the mean rate of sTREM-1 was significantly higher among FMF patients with AA amyloidosis versus without, though the concomitant SAA protein level was normal. Serum level of sTREM-1 was higher in patients with amyloidosis even though the concomitant SAA protein level was normal. sTREM-1 plasma levels could be an accurate tool to specifically identify FMF patients with amyloidosis.

scholarly journals AB0055 SOLUBLE TREM-1 LEVELS IN FAMILIAL MEDITERRANEAN FEVER RELATED AA-AMYLOIDOSIS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1329.2-1330
Author(s):  
S. Ugurlu ◽  
B. H. Egeli ◽  
I. M. Bolayirli ◽  
H. Ozdogan
Keyword(s):  
Familial Mediterranean Fever ◽  
Demographic Data ◽  
Enzyme Linked Immunosorbent Assay ◽  
Aa Amyloidosis ◽  
Control Groups ◽  
Tertiary Center ◽  
Healthy Control ◽  
Mediterranean Fever ◽  
The Mean

Background:Triggering Receptor Expressed on Myeloid cells-1 (TREM-1) is a monocyte and neutrophil receptor functioning in innate immunity. TREM-1 produces proinflammatory cytokines and serves for neutrophil degranulation. TREM-1 activity is well known in the pathogenesis of sepsis; hence it can be also present in autoinflammatory diseases such as the most common monogenic one, Familial Mediterranean Fever (FMF).Objectives:The objective of this study is to measure soluble TREM-1 (sTREM-1) activity in severe FMF cases complicated with systemic AA-Amyloidosis.Methods:The cohort of the study includes regularly followed FMF related AA-Amyloidosis patients in a tertiary center outpatient rheumatology clinic. Soluble TREM-1 levels were measured using enzyme-linked immunosorbent assay (ELISA). In addition, demographic data, renal function tests, acute phase reactants, and medical prescription history was also noted and analyzed. None of the FMF diagnosed patients had an attack during the collection of the blood samples.Results:The patients were categorized into 4 groups: FMF related AA-Amyloidosis patients (A(+) FMF(+)), FMF unrelated AA-Amyloidosis (FMF(-) A(+)), FMF patients without Amyloidosis diagnosis (FMF(+) A (-)), and healthy controls (HC). The mean ages, TREM-1, C - reactive protein (CRP), and Creatinine levels of each group are shown in Table 1. TREM-1 levels were found to be significantly higher in A(+) FMF(+) group than FMF(+) A (-), and healthy control groups (p= 0.001 and 0.002). Nevertheless, this difference was not found in between A(+) FMF(+) and FMF(-) A(+) (p= 0.447). In addition, the TREM-1 levels of FMF(+) A (-), and healthy control groups were not different (0.532). In A(+) FMF(+) group, 36 patients used colchicine with the mean dose of 1.9±0.8 mg/day, 14 patients used anakinra, and 9 patients used canakinumab. In FMF(+) A (-) group all 20 patients used colchicine with the mean dose of 2.8±0.9 mg/day, 1 patient used anakinra, and 2 patients used canakinumab.Table 1.Clinical Features of Patients and TREM-1 levelsA(+) FMF(+)(n= 42)FMF(-) A(+)(n=5)FMF(+) A(-)(n=20)HC(n=20)Age43.9±12.954.8±1935.3±9.6435.4±6.57TREM-1735.3±566.51247.1±1349.2414.3±142.3439.2±104.6CRP11.1±14.251.3±98.325.8±541.8±1.7Creatinine1.6±1.83.28±4.170.7±0.150.7±0.15Conclusion:In conclusion, TREM-1 is a proinflammatory marker found significantly high in AA-amyloidosis patients regardless of their FMF diagnosis. TREM-1 may be useful in AA-amyloidosis follow-up and early diagnosis since currently there is a deficit of an early diagnostic marker of amyloidosis. This study is a cross-sectional one so it is hard to reach a conclusion on the effectiveness of TREM-1 during regular FMF follow-up for the secondary prevention of amyloidosis. However, the sensitivity of TREM-1 as a marker cannot be denied in amyloidosis.Disclosure of Interests:None declared

scholarly journals Amyloidosis and Glomerular Diseases in Familial Mediterranean Fever

Medicina ◽  
2021 ◽  
Vol 57 (10) ◽  
pp. 1049
Author(s):  
Rossella Siligato ◽  
Guido Gembillo ◽  
Vincenzo Calabrese ◽  
Giovanni Conti ◽  
Domenico Santoro
Keyword(s):  
Nephrotic Syndrome ◽  
Familial Mediterranean Fever ◽  
Periodic Fever ◽  
Rapid Progression ◽  
Secondary Amyloidosis ◽  
Aa Amyloidosis ◽  
Glomerular Diseases ◽  
Amyloid A ◽  
Serum Amyloid A Protein ◽  
Mediterranean Fever

Familial Mediterranean fever (FMF) is a genetic autoinflammatory disease with autosomal recessive transmission, characterized by periodic fever attacks with self-limited serositis. Secondary amyloidosis due to amyloid A renal deposition represents the most fearsome complication in up to 8.6% of patients. Amyloidosis A typically reveals a nephrotic syndrome with a rapid progression to end-stage kidney disease still. It may also involve the cardiovascular system, the gastrointestinal tract and the central nervous system. Other glomerulonephritis may equally affect FMF patients, including vasculitis such as IgA vasculitis and polyarteritis nodosa. A differential diagnosis among different primary and secondary causes of nephrotic syndrome is mandatory to determine the right therapeutic choice for the patients. Early detection of microalbuminuria is the first signal of kidney impairment in FMF, but new markers such as Neutrophil Gelatinase-Associated Lipocalin (NGAL) may radically change renal outcomes. Serum amyloid A protein (SAA) is currently considered a reliable indicator of subclinical inflammation and compliance to therapy. According to new evidence, SAA may also have an active pathogenic role in the regulation of NALP3 inflammasome activity as well as being a predictor of the clinical course of AA amyloidosis. Beyond colchicine, new monoclonal antibodies such as IL-1 inhibitors anakinra and canakinumab, and anti-IL-6 tocilizumab may represent a key in optimizing FMF treatment and prevention or control of AA amyloidosis.

Long term administration of tocilizumab improves renal amyloid A (AA) amyloidosis deposition in Familial Mediterranean fever

2020 ◽  
Vol 4 (2) ◽  
pp. 310-311
Author(s):  
Keita Inui ◽  
Naoki Sawa ◽  
Tatsuya Suwabe ◽  
Hiroki Mizuno ◽  
Masayuki Yamanouchi ◽  
...  
Keyword(s):  
Familial Mediterranean Fever ◽  
Aa Amyloidosis ◽  
Amyloid A ◽  
Term Administration ◽  
Mediterranean Fever

Thyroid disorders in familial Mediterranean fever: think about AA amyloidosis!

2021 ◽  
Author(s):  
Hélène Vergneault ◽  
Rim Bourguiba ◽  
Samuel Ardois ◽  
Anael Dumont ◽  
Léa Savey ◽  
...  
Keyword(s):  
Familial Mediterranean Fever ◽  
Thyroid Disorders ◽  
Aa Amyloidosis ◽  
Mediterranean Fever

scholarly journals Familial Mediterranean Fever: an unusual cause of liver disease

2019 ◽  
Vol 45 (1) ◽  
Author(s):  
Maria Cristina Maggio ◽  
Maria Castiglia ◽  
Giovanni Corsello
Keyword(s):  
Abdominal Pain ◽  
Liver Disease ◽  
Familial Mediterranean Fever ◽  
Serum Amyloid A ◽  
Mefv Gene ◽  
Serum Amyloid ◽  
Aphthous Stomatitis ◽  
Homozygous Mutation ◽  
Amyloid A ◽  
Mediterranean Fever

Abstract Background Familial Mediterranean Fever is an autoinflammatory disease typically expressed with recurrent attacks of fever, serositis, aphthous stomatitis, rash. Only a few reports describe the association with hepatic involvement. Case presentation We describe the clinical case of a child affected, since the age of 1 year, by recurrent fever, aphthous stomatitis, rash, arthralgia, associated with abdominal pain, vomiting, lymphadenopathy. The diagnosis of Familial Mediterranean Fever was confirmed by the genetic study of MEFV gene; the homozygous mutation M694 V in exon was documented. A partial control of attacks was obtained with colchicine. The child continued to manifest only recurrent episodes of abdominal pain without fever, however serum amyloid A persisted high, in association with enhanced levels of CRP, AST and ALT (1.5 x n.v.). The dosage of colchicine was increased step by step and the patient achieved a better control of symptoms and biochemical parameters. However, the patient frequently needed an increase in the dose of colchicine, suggesting the possible usefulness of anti-interleukin-1 beta treatment. Conclusions The unusual presentation of Familial Mediterranean Fever with liver disease suggests the role of inflammasome in hepatic inflammation. Colchicine controls systemic inflammation in most of the patients; however, subclinical inflammation can persist in some of them and can manifest with increased levels of CRP, ESR, serum amyloid A also in attack-free intervals.

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