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Statements

Subject Item
n2:RIV%2F00216208%3A11150%2F13%3A10173407%21RIV14-MSM-11150___
rdf:type
n9:Vysledek skos:Concept
rdfs:seeAlso
http://download.springer.com/static/pdf/855/art%253A10.1007%252Fs00428-013-1480-7.pdf?auth66=1392201175_27eff28118401c974372fc078e55ee3d&ext=.pdf
dcterms:description
Retroperitoneal fibrosis (RF) is a rare disease characterized by inflammation and fibrosis of retroperitoneal soft tissues. It is classified into 2 types: idiopathic (iRF), and secondary (sRF). The aim of the study was to investigate the relationship between iRF and IgG4-related disease (IgG4-RD) and to eventually extend the clinicopathological features of this condition by analysis of the sample comprising 6 iRF- and 6 sRF-patients. The iRF-patients included 4 males and 2 females, aged 12-62 years (median 55 years). Two lesions were periaortic, 1 was periureteral and 3 cases showed both periaortic and periureteral localization. Two patients had increased serum levels of IgG4. None of the patients developed any manifestation of IgG4-RD during the follow-up period ranging for 15 - 133 months (median 43 months). Microscopically, in 2 iRF-cases fibrosis was highly cellular encircling vessels, nerves and paraganglia. Phlebitis was found in all cases being obliterative in 4. Lymphocytic inflammation with formation of follicles and plasma cell infiltration were scored as severe in 5 iRF-cases. The numbers of IgG-positive plasma cells ranged 0-373 per 1 HPF (median 132) and of IgG4-positive plasma cells 0-238 per 1 HPF (median 91). The IgG4/IgG ratio values ranged 0.38-0.74 (median 0.68). Two of the iRF-cases were diagnosed as definite and 3 cases as probable IgG4-RD. To the contrary, none of the sRF-cases met the diagnostic criteria for either definite, probable or possible IgG4-related disease. Our results indicate that a substantial portion of iRF-cases, including some of very rare pediatric cases, is a manifestation of IgG4-RD Retroperitoneal fibrosis (RF) is a rare disease characterized by inflammation and fibrosis of retroperitoneal soft tissues. It is classified into 2 types: idiopathic (iRF), and secondary (sRF). The aim of the study was to investigate the relationship between iRF and IgG4-related disease (IgG4-RD) and to eventually extend the clinicopathological features of this condition by analysis of the sample comprising 6 iRF- and 6 sRF-patients. The iRF-patients included 4 males and 2 females, aged 12-62 years (median 55 years). Two lesions were periaortic, 1 was periureteral and 3 cases showed both periaortic and periureteral localization. Two patients had increased serum levels of IgG4. None of the patients developed any manifestation of IgG4-RD during the follow-up period ranging for 15 - 133 months (median 43 months). Microscopically, in 2 iRF-cases fibrosis was highly cellular encircling vessels, nerves and paraganglia. Phlebitis was found in all cases being obliterative in 4. Lymphocytic inflammation with formation of follicles and plasma cell infiltration were scored as severe in 5 iRF-cases. The numbers of IgG-positive plasma cells ranged 0-373 per 1 HPF (median 132) and of IgG4-positive plasma cells 0-238 per 1 HPF (median 91). The IgG4/IgG ratio values ranged 0.38-0.74 (median 0.68). Two of the iRF-cases were diagnosed as definite and 3 cases as probable IgG4-RD. To the contrary, none of the sRF-cases met the diagnostic criteria for either definite, probable or possible IgG4-related disease. Our results indicate that a substantial portion of iRF-cases, including some of very rare pediatric cases, is a manifestation of IgG4-RD
dcterms:title
Idiopathic vs. secondary retroperitoneal fibrosis: a clinicopathological study of 12 cases, with emphasis to possible relationship to IgG4-related disease Idiopathic vs. secondary retroperitoneal fibrosis: a clinicopathological study of 12 cases, with emphasis to possible relationship to IgG4-related disease
skos:prefLabel
Idiopathic vs. secondary retroperitoneal fibrosis: a clinicopathological study of 12 cases, with emphasis to possible relationship to IgG4-related disease Idiopathic vs. secondary retroperitoneal fibrosis: a clinicopathological study of 12 cases, with emphasis to possible relationship to IgG4-related disease
skos:notation
RIV/00216208:11150/13:10173407!RIV14-MSM-11150___
n9:predkladatel
n17:orjk%3A11150
n3:aktivita
n8:I n8:P
n3:aktivity
I, P(LM2010004)
n3:cisloPeriodika
5
n3:dodaniDat
n10:2014
n3:domaciTvurceVysledku
n4:1166603 n4:1141147 n4:5436478 n4:1239570 n4:3136418 n4:8678022 n4:6953891
n3:druhVysledku
n13:J
n3:duvernostUdaju
n21:S
n3:entitaPredkladatele
n20:predkladatel
n3:idSjednocenehoVysledku
78793
n3:idVysledku
RIV/00216208:11150/13:10173407
n3:jazykVysledku
n16:eng
n3:klicovaSlova
Autoimmune disease; IgG4-related disease; Fibrosis; Retroperitoneum
n3:klicoveSlovo
n12:IgG4-related%20disease n12:Retroperitoneum n12:Fibrosis n12:Autoimmune%20disease
n3:kodStatuVydavatele
DE - Spolková republika Německo
n3:kontrolniKodProRIV
[E8E597CCF47F]
n3:nazevZdroje
Virchows Archiv : an international journal of pathology
n3:obor
n19:FP
n3:pocetDomacichTvurcuVysledku
7
n3:pocetTvurcuVysledku
8
n3:projekt
n5:LM2010004
n3:rokUplatneniVysledku
n10:2013
n3:svazekPeriodika
463
n3:tvurceVysledku
Novák, Ivo Hácová, Mária Dobeš, Daniel Broďák, Miloš Ryška, Aleš Kamarádová, Kateřina Laco, Jan Podhola, Miroslav
n3:wos
000326929400013
s:issn
0945-6317
s:numberOfPages
10
n14:doi
10.1007/s00428-013-1480-7
n15:organizacniJednotka
11150