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2016 ; 17
(ä): 30-3
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The inter-connection between fibrosis and microvascular remodeling in idiopathic
pulmonary fibrosis: Reality or just a phenomenon
#MMPMID27222781
Mlika M
; Bacha S
; Braham E
; El Mezni F
Respir Med Case Rep
2016[]; 17
(ä): 30-3
PMID27222781
show ga
BACKGROUND: Idiopathic pulmonary fibrosis is the most frequent interstitial
disease with the worst prognosis. It is characterized by an uncontrolled fibrosis
which is difficult to manage. The pathogenesis of this disease remains unclear
with many theories resulting in multiple target therapies. The relation between
fibrosis and vascular remodeling has been debated in the literature with
different results that may seem contradictory. AIM: We target to evaluate the
connection between fibrosis and vascular remodeling in usual interstitial
pneumonia. MATERIAL AND METHODS: 26 cases of idiopathic pulmonary fibrosis were
reviewed by 2 pathologists and the diagnosis of UIP was retained according to the
American Thoracic Society's criteria. Fibrotic changes and vascular remodeling
were evaluated blindly. The fibrotic changes were classified as severe,
intermediate and mild. Vascular occlusion was graded in 4 grades extending from
medial hypertrophy (grade 1) to plexiform lesions of the vascular wall (grade 4).
RESULTS: We noticed that severe degrees of fibrosis were correlated with severe
grades of vascular obstruction. In fact, our 26 cases were classified as severe
fibrosis in 11 cases with grade IV vascular lesions in 6 cases, intermediate
fibrosis in 12 cases with grade II vascular lesions in 8 cases and mild fibrosis
in 3 cases with grade I vascular lesions in all cases. CONCLUSION: Many theories
have been reported concerning the UIP's pathogenesis. Recently, many authors
reported that the primum movens of these lesions was an epithelial/endothelial
injury which induces uncontrolled fibrosis and microvascular remodeling using
different pathways. This puts emphasis on the necessity of multi-target therapies
in order to improve the management of this fatal disease.