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.jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117 Medicine+(Baltimore)
2015 ; 94
(30
): e1243
Nephropedia Template TP
gab.com Text
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English Wikipedia
Membranous Nephropathy Associated With Immunological Disorder-Related Liver
Disease: A Retrospective Study of 10 Cases
#MMPMID26222864
Dauvergne M
; Moktefi A
; Rabant M
; Vigneau C
; Kofman T
; Burtey S
; Corpechot C
; Stehlé T
; Desvaux D
; Rioux-Leclercq N
; Rouvier P
; Knebelmann B
; Boffa JJ
; Frouget T
; Daugas E
; Jablonski M
; Dahan K
; Brocheriou I
; Remy P
; Grimbert P
; Lang P
; Chazouilleres O
; Sahali D
; Audard V
Medicine (Baltimore)
2015[Jul]; 94
(30
): e1243
PMID26222864
show ga
The association between membranous nephropathy (MN) and immunological
disorder-related liver disease has not been extensively investigated, and the
specific features of this uncommon association, if any, remain to be
determined.We retrospectively identified 10 patients with this association. We
aimed to describe the clinical, biological, and pathological characteristics of
these patients and their therapeutic management. The possible involvement of the
phospholipase A2 receptor (PLA2R) in these apparent secondary forms of MN was
assessed by immunohistochemistry with renal and liver biopsy specimens.The mean
delay between MN and liver disease diagnoses was 3.9 years and the interval
between the diagnosis of the glomerular and liver diseases was <1.5 years in 5
patients. MN was associated with a broad spectrum of liver diseases including
primary biliary cirrhosis (PBC), autoimmune hepatitis (AIH), and primary
sclerosing cholangitis (PSC). AIH whether isolated (n = 3) or associated with PBC
(n = 2) or PSC (n = 2) was the most frequent autoimmune liver disease.
Circulating PLA2R antibodies were detected in 4 out of 9 patients but the test
was performed under specific immunosuppressive treatment in 3 out of 9 patients.
Seven of the 9 patients with available renal tissue specimens displayed enhanced
expression of PLA2R in glomeruli whereas PLA2R was not expressed in liver
parenchyma from these patients or in normal liver tissue. The study of
immunoglobulin (Ig) subclasses of deposits in glomeruli revealed that the most
frequent pattern was the coexistence of IgG1 and IgG4 immune deposits with IgG4
predominating.Detection of PLA2R antibodies in glomeruli but not in liver
parenchyma is a common finding in patients with MN associated with autoimmune
liver disease, suggesting that these autoantibodies are not exclusively detected
in idiopathic MN.