Complete remission of nephrotic syndrome in secondary amyloidosis of familial Mediterranean fever following colchicine treatment Ailevi Akdeniz ateşi ve sekonder amiloidozise bağlı nefrotik sendromun

Objective Recurrent attacks of peritonitis of Familial Mediterranean fever (FMF), may lead to peritoneal adhesions and fallopian tube obstruction. Colchicine - the treatment of choice for FMF - may disturb cell division. Secondary amyloidosis - a complication of untreated FMF - may involve the testes and ovaries. Thus, FMF and colchicine may potentially affect fertility and pregnancy in FMF patients. The aims of the study are to evaluate the causes of infertility and pregnancy outcome in FMF patients and to compare them with two groups: non-FMF patients with peritoneal female genital tuberculosis (FGTB) and normal healthy control. Methods This is a retrospective study in which FMF patients with reproductive disorders were recruited from the National Center of Medical Genetics and Primary Health Care in Yerevan, Armenia. The FGTB patients and the normal control patients with reproductive problems were recruited successively from a large gynecology clinic in Yerevan. Genetic analyses for FMF were performed using ViennaLab Diagnostics GmbH Strip Assay. Results The FMF group (211 patients) resembles the FGTB group (127 patients) regarding etiologies of infertility. However, in vitro fertilization (IVF) success rate and pregnancy outcome were comparable between the FMF patients and the control group (167patients). Infertility in FMF patients was clearly associated with a more severe disease and a lack of adequate colchicine treatment. Conclusion Colchicine medication and controlled FMF disease do not adversely affect the reproductive system and pregnancy outcome. However, a lack of an appropriate colchicine treatment may cause infertility and poor pregnancy outcome.

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Amyloidosis and Glomerular Diseases in Familial Mediterranean Fever

Medicina ◽

10.3390/medicina57101049 ◽

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.

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Complete remission of nephrotic syndrome in a woman with renal amyloidosis due to Familial Mediterranean Fever

Nefrología (English Edition) ◽

10.1016/j.nefroe.2016.06.009 ◽

2016 ◽

Vol 36 (3) ◽

pp. 331-332

Author(s):

Manuel Heras ◽

Ana Saiz ◽

María José Fernández-Reyes ◽

Álvaro Molina ◽

María Astrid Rodríguez ◽

...

Keyword(s):

Nephrotic Syndrome ◽

Complete Remission ◽

Familial Mediterranean Fever ◽

Renal Amyloidosis ◽

Mediterranean Fever

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Lack of Correlation between Amyloid a Protein Levels and Clinical Parameters in Aspirated Fat Tissue of Familial Mediterranean Fever Patients with Secondary Amyloidosis

XIth International Symposium on Amyloidosis ◽

10.1201/9781420043358.ch24 ◽

2007 ◽

pp. 75-77

Author(s):

A Livneh ◽

B Hazenberg ◽

J Bijzet ◽

R Kedem ◽

M Lidar ◽

...

Keyword(s):

Familial Mediterranean Fever ◽

Secondary Amyloidosis ◽

Clinical Parameters ◽

Fat Tissue ◽

Protein Levels ◽

Amyloid A ◽

Mediterranean Fever

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Ultrasonographic evaluation of femoral cartilage thickness in patients with familial Mediterranean fever

Turkish Journal of Physical Medicine and Rehabilitation ◽

10.5606/tftrd.2021.6094 ◽

2021 ◽

Vol 67 (4) ◽

pp. 416-420

Author(s):

Ayşe Aydemir Ekim ◽

Fulya Bakılan ◽

Emel Gönüllü ◽

Hatice Hamarat

Keyword(s):

Familial Mediterranean Fever ◽

Colchicine Treatment ◽

Left Knee ◽

Chronic Arthritis ◽

Cartilage Thickness ◽

Femoral Cartilage ◽

Mediterranean Fever ◽

Femoral Cartilage Thickness ◽

The Right ◽

Femoral Condyles

Objectives: This study aims to determine femoral cartilage thickness using ultrasonography in familial Mediterranean fever (FMF). Patients and methods: A total of 45 patients (16 males, 29 females; mean age: 38.5±9.1 years; range, 24 to 49 years) with the diagnosis of FMF and 31 healthy individuals (6 males, 25 females; mean age: 37.0±8.7 years; range, 25 to 47 years) between January 2016 and July 2016 were included in this study. Clinical data and demographic characteristics of the patients were recorded. All FMF cases in the study were in remission with colchicine treatment. The thickness of femoral cartilage in both knees were evaluated using ultrasonography. Three measurements (mid-point) were taken from both knees (at the medial/lateral femoral condyles and intercondylar area). Results: Ultrasonographical measurements revealed that cartilage measurements of FMF patients were significantly thinner at both the medial/lateral femoral condyles and intercondylar area on the right knee and at the medial/lateral femoral condyles on the left knee (p<0.001). The cartilage measurements in FMF patients were significantly thinner at the intercondylar area on left knee, compared to those in controls (p=0.023). Conclusion: Our study showed decreased femoral cartilage thickness in FMF patients. These findings indicate that even if these patients do not have an attack, they may have subacute and chronic arthritis in their joints, and their femoral cartilage thickness can be affected.

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