Warning: imagejpeg(C:\Inetpub\vhosts\kidney.de\httpdocs\phplern\25906201
.jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117 Am+J+Respir+Crit+Care+Med
2015 ; 192
(1
): 17-29
Nephropedia Template TP
gab.com Text
Twit Text FOAVip
Twit Text #
English Wikipedia
Diffuse Cystic Lung Disease Part II
#MMPMID25906201
Gupta N
; Vassallo R
; Wikenheiser-Brokamp KA
; McCormack FX
Am J Respir Crit Care Med
2015[Jul]; 192
(1
): 17-29
PMID25906201
show ga
The diffuse cystic lung diseases have a broad differential diagnosis. A wide
variety of pathophysiological processes spanning the spectrum from airway
obstruction to lung remodeling can lead to multifocal cyst development in the
lung. Although lymphangioleiomyomatosis and pulmonary Langerhans cell
histiocytosis are perhaps more frequently seen in the clinic, disorders such as
Birt-Hogg-Dubé syndrome, lymphocytic interstitial pneumonia, follicular
bronchiolitis, and light-chain deposition disease are increasingly being
recognized. Obtaining an accurate diagnosis can be challenging, and management
approaches are highly disease dependent. Unique imaging features, genetic tests,
serum studies, and clinical features provide invaluable clues that help
clinicians distinguish among the various etiologies, but biopsy is often required
for definitive diagnosis. In part II of this review, we present an overview of
the diffuse cystic lung diseases caused by lymphoproliferative disorders, genetic
mutations, or aberrant lung development and provide an approach to aid in their
diagnosis and management.
free
free
free
