Warning: file_get_contents(https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&id=23793110
&cmd=llinks): Failed to open stream: HTTP request failed! HTTP/1.1 429 Too Many Requests
in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 215
Deprecated: Implicit conversion from float 231.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Warning: imagejpeg(C:\Inetpub\vhosts\kidney.de\httpdocs\phplern\23793110
.jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117 Medicine+(Baltimore)
2013 ; 92
(4
): 206-216
Nephropedia Template TP
gab.com Text
Twit Text FOAVip
Twit Text #
English Wikipedia
IgG4-related disease and hypertrophic pachymeningitis
#MMPMID23793110
Wallace ZS
; Carruthers MN
; Khosroshahi A
; Carruthers R
; Shinagare S
; Stemmer-Rachamimov A
; Deshpande V
; Stone JH
Medicine (Baltimore)
2013[Jul]; 92
(4
): 206-216
PMID23793110
show ga
Hypertrophic pachymeningitis (HP) is an inflammatory condition in which the dura
mater of the cranium or spine becomes thickened, leading to symptoms that result
from mass effect, nerve compression, or vascular compromise. The differential
diagnosis of HP includes immune-mediated conditions such as rheumatoid arthritis
and vasculitis, malignancies, and infections. Many times, no diagnosis is
reached; in such cases, the disease has been described as idiopathic HP.
IgG4-related disease (IgG4-RD) is a recently described inflammatory condition
known to cause tumefactive lesions at myriad anatomical locations. Both IgG4-RD
and idiopathic HP share similar demographics, histopathology, and natural
history. We hypothesized that IgG4-RD is a common cause of idiopathic HP.To
investigate this hypothesis, we identified all pathology specimens diagnosed as
noninfectious HP during 25 years at our institution. Fourteen cases had stained
slides and paraffin blocks to permit review of the original hematoxylin and eosin
stained slides as well as immunostaining of cell blocks. Recently published
consensus guidelines describing characteristic histopathology and the necessary
quantity of IgG4+ plasma cell infiltrate were used to diagnose IgG4-RD.Four cases
(66.6%) that had been regarded previously as representing idiopathic HP were
diagnosed as IgG4-RD; of all the reviewed cases, IgG4-RD represented 29% of
cases. Of the remaining cases, 3 cases were associated with granulomatosis with
polyangiitis (GPA), 2 with lymphoma, and 1 each with rheumatoid arthritis, giant
cell arteritis, and sarcoidosis. Two of the cases could not be diagnosed more
precisely and were classified as undifferentiated HP. Clinical history, serologic
tests, cerebrospinal fluid studies, and radiology alone could not identify the
cause of HP. Rather, biopsy with histopathology and immunostaining was necessary
to reach an accurate diagnosis. Significant IgG4+ plasma cell infiltrates were
observed in rheumatoid arthritis, granulomatosis with polyangiitis, and lymphoma,
underscoring the importance of histopathology in making the diagnosis of
IgG4-RD.This case series demonstrates that IgG4-RD may be the most common
etiology of noninfectious HP and highlights the necessity of biopsy for accurate
diagnosis.
free
free
free
