Familial Mediterranean fever and glomerulonephritis and review of the literature

Tekin, Mustafa; Akpolat, İlkser; Karagöz, Filiz; Yılmaz, Engin; Kandemır, Bedrı; Özen, Seza

doi:10.1007/s00296-003-0329-9

Cited by 35 publications

(31 citation statements)

References 14 publications

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“…Four patients using regular colchicine, two were found to have MsPGN and two were found to have MGN. These findings are inconsistent with other publications that suggest a protective role of regular colchicine for GN [25] . Homozygosity for the M694V mutation is a major risk factor for renal amyloidosis in FMF patients [14] .…”

Section: Discussioncontrasting

confidence: 57%

“…With colchicine and angiotensin converting enzyme inhibitors, a marked improvement was seen in patients with amyloidosis, but no improvement was seen in patients with FSGS [24] . Akpolat T et al have suggested that colchicine prevents glomerular disease, and that the irregular use of this drug triggers GN attacks [25] . In our study, four of nine patients were diagnosed as FMF and were receiving regular colchicine before biopsy.…”

Section: Discussionmentioning

confidence: 99%

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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: Discussioncontrasting

confidence: 57%

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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“…9 Akpolat et al described a 25-year-old patient, whose proteinuria remitted partially with prednisolone and azathioprine, who had membranoproliferative glomerulonephritis with FMF. 12 Immune complexes are exhibited in 50% of FMF patients. 6 IgM, IgA, and C3 deposits found in the mesangium in previously reported cases 3,6,11 are supportive of immunologic mechanism.…”

Section: Discussionmentioning

confidence: 99%

“…Prednisolone, cyclophosphamide, and azathioprine have been rarely used in cases with FMF-related glomerulonephritis, 3,12 but colchicine is the most common drug used to treat FMF-associated glomerulonephritis. 12 Said et al documented important improvement in three patients with IgM nephropathy and FMF who took regular treatment with colchicine.…”

Section: Discussionmentioning

confidence: 99%

Familial Mediterranean fever and membranous glomerulonephritis: report of a case

Çeri¹,

Unverdi²,

Altay³

et al. 2010

Renal Failure

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Familial Mediterranean fever (FMF) is an autosomal recessive genetic disease characterized by recurrent attacks of fever and painful episodes of sterile polyserositis. Kidney involvement may occur as a result of secondary amyloidosis during the course of FMF. Previously, different types of glomerulopathies such as IgM and IgA nephropathy, crescentic glomerulonephritis, diffuse proliferative glomerulonephritis, minimal change disease, and membranoproliferative glomerulonephritis were rarely reported. We herein represent a first case of membranous glomerulonephritis who had complete remission with colchicine treatment in the course of familial Mediterranean fever.

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“…Several case reports showed that FMF patients with non-amyloid renal involvement -including IgG and IgM mesangial proliferative glomerulonephritis and IgA nephropathy -also responded to colchicine therapy alone 13,14 . This observation suggests that colchicine may suppress the inflammatory process affecting the kidneys.…”

Section: Rheumatologymentioning

confidence: 99%

The Kidney in Familial Mediterranean Fever

Peleg

Ben-Chétrit

2013

J Rheumatol

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Familial Mediterranean fever and glomerulonephritis and review of the literature

Cited by 35 publications

References 14 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

Familial Mediterranean fever and membranous glomerulonephritis: report of a case

The Kidney in Familial Mediterranean Fever

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