Familial Mediterranean fever and membranous glomerulonephritis: report of a case

Çeri, Mevlüt; Unverdi, Selman; Altay, Mustafa; Ünverdǐ, Hatǐce; Ensarı, Arzu; Duranay, Murat

doi:10.3109/08860221003640090

Cited by 5 publications

(3 citation statements)

References 11 publications

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“…Proteinuria of another case improved with cyclophosphamide. Ceri et al reported that a patient who was diagnosed with FMF and MGN initially presented with 2.5 gr/day proteinuria and with colchicine treatment proteinuria decreased 50% after six months and the patient was completely cured after 12 months [22] . Immunologic mechanisms have an important role on FMF pathogenesis.…”

Section: Discussionmentioning

confidence: 99%

Biopsy Proven Non-Amyloid Glomerular Diseases in Patients With Familial Mediterranean Fever

Hüzmelí

Koçkara

Candan

et al. 2015

Journal of Nephrology Research

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treatment, patients with more aggressive kidney involvement may require immunosuppressive therapy. Further studies are needed for revealing the relationship between FMF gene mutation and nonamyloid glomerular diseases.© 2015 ACT. All rights reserved. . Mutations of the MEFV gene, on the short arm of chromosome 16, codes for pyrin or marenostrin proteins is responsible for the disease. The MEFV mutation is found in less than 70% of patients diagnosed with FMF. Four mutations have been defined in 85% of patients from the mentioned 4 ethnic groups (M694V, M680I, M694I V726A) [3][4][5] . AA type amyloidosis is the result of serum amyloid A protein deposition in tissues. It is the best understood and the most serious reason for kidney failure in patients with FMF. In a report from Turkey, amyloidosis rates among FMF patients were found to be 7% [6] . There is no publication proving an increase of glomerulonephritis among FMF patients. ABSTRACT AIM: Kidney involvement is the most serious organ involvement of Familial Mediterranean Fever (FMF), while the most common cause is AA type amyloidosis with serum amyloid AA deposition. However, vasculitis and other types of glomerulonephritis (GN) also have been reported. With this study we evaluated biopsy proven non-amyloid glomerular diseases in patients with FMF. METHODS: At our Nephrology clinic, total 950 patients followed by diagnosis of FMF have been evaluated. Nine patients who had positive proteinuria ( >500 mg/day) but had negative amyloidosis in the tissue biopsy were made renal biopsy. RESULTS: Nine patients underwent a renal biopsy, two patients were found to have IgA nephropathy (IGAN), three mesangioproliferative glomerulonephritis (MsPGN), three membranous glomerulonephritis (MGN) and one had immune complex glomerulonephritis. Two patients with IGAN and one patient with MGN with non-nephrotic proteinuria exhibited significant improvement with colchicine and angiotensin receptor blocker (ARB) therapy. Other patients were treated with colchicine, an ARB and immunosuppressive drugs. Upon evaluation for the FMF gene mutation, different mutations have been found for the same glomerulonephritis type. CONCLUSIONS: Other glomerular causes also must be investigated alongside amyloidosis in the case of kidney involvement in FMF patients. While patients with IGAN and other glomerulonephrities with non-nephrotic proteinuria can respond well to colchicine

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Section: Discussionmentioning

confidence: 99%

Biopsy Proven Non-Amyloid Glomerular Diseases in Patients With Familial Mediterranean Fever

Hüzmelí

Koçkara

Candan

et al. 2015

Journal of Nephrology Research

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“…Since 1989, cases of other glomerulopathies than amyloidosis have been reported in FMF patients. Several associations between FMF regard IgA nephropathy (IgAN) [ 34 , 35 , 36 , 37 ], membranous nephropathy (MN) [ 36 , 38 ], membranoproliferative glomerulonephritis (MPGN) [ 36 , 39 , 40 ], mesangioproliferative glomerulonephritis (MsPGN) [ 36 , 41 , 42 , 43 ], focal and segmental glomerulosclerosis (FSGS) [ 36 , 37 ], vasculitis [ 44 , 45 , 46 , 47 ]. Rarer cases are represented by and rapid progressive glomerulonephritis (RPGN) [ 48 ], IgM nephropathy [ 49 , 50 ] and fibrillary glomerulopathy [ 51 ].…”

Section: Glomerular Diseases and Fmfmentioning

confidence: 99%

Amyloidosis and Glomerular Diseases in Familial Mediterranean Fever

et al. 2021

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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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“…Familial Mediterranean Fever (FMF) is an autoinflammatory disease characterized by transient febrile episodes accompanied by abdominal and chest pain, arthralgia, myalgia, arthritis, and skin rash symptoms (1). Mediterranean fever gene (MEFV) is localized on the short arm of chromosome 16 and encodes a 781-amino-acid protein known as pyrin (2). FMF is also known as an autosomal recessive condition prevalent in Armenian, Turkish, and Arab populations.…”

Section: Introductionmentioning

confidence: 99%

Mediterranean fever gene mutations in patients with idiopathic mesangial proliferative glomerulonephritis

Etemadi¹,

Majidi²,

Motavalli³

et al. 2017

J Nephropathol

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Familial Mediterranean fever and membranous glomerulonephritis: report of a case

Cited by 5 publications

References 11 publications

Biopsy Proven Non-Amyloid Glomerular Diseases in Patients With Familial Mediterranean Fever

Biopsy Proven Non-Amyloid Glomerular Diseases in Patients With Familial Mediterranean Fever

Amyloidosis and Glomerular Diseases in Familial Mediterranean Fever

Mediterranean fever gene mutations in patients with idiopathic mesangial proliferative glomerulonephritis

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