Open AccessCapillary Proliferation in Systemic‐Sclerosis‐Related Pulmonary Fibrosis: Association with Pulmonary Hypertension
Author(s) -
Seki Atsuko,
Anklesaria Zafia,
Saggar Rajeev,
Dodson Mark W.,
Schwab Kristin,
Liu MingChang,
Charan Ashana Deepshikha,
Miller William D.,
Vangala Sitaram,
DerHovanessian Ariss,
Channick Richard,
Shaikh Faisal,
Belperio John A.,
Weigt Stephen S.,
Lynch Joseph P.,
Ross David J.,
Sullivan Lauren,
Khanna Dinesh,
Shapiro Shelley S.,
Sager Jeffrey,
Gargani Luna,
Stanziola Anna,
Bossone Eduardo,
Schraufnagel Dean E.,
Fishbein Gregory,
Xu Haodong,
Fishbein Michael C.,
Wallace William D.,
Saggar Rajan
Publication year - 2019
Publication title -
acr open rheumatology
Language(s) - English
Resource type - Journals
ISSN - 2578-5745
DOI - 10.1002/acr2.1003
Subject(s) - medicine , microcirculation , pulmonary hypertension , lung , pathology , pulmonary fibrosis , fibrosis , cardiology
Objective We sought to determine if any histopathologic component of the pulmonary microcirculation can distinguish systemic sclerosis ( SS c)‐related pulmonary fibrosis ( PF ) with and without pulmonary hypertension ( PH ). Methods Two pulmonary pathologists blindly evaluated 360 histologic slides from lungs of 31 SS c‐ PF explants or autopsies with (n = 22) and without (n = 9) PH . The presence of abnormal small arteries, veins, and capillaries (pulmonary microcirculation) was semiquantitatively assessed in areas of preserved lung architecture. Capillary proliferation ( CP ) within the alveolar walls was measured by its distribution, extent ( CP % involvement), and maximum number of layers (maximum CP ). These measures were then evaluated to determine the strength of their association with right heart catheterization–proven PH . Results Using consensus measures, all measures of CP were significantly associated with PH . Maximum CP had the strongest association with PH ( P = 0.013; C statistic 0.869). Maximum CP 2 or more layers and CP % involvement 10% or greater were the optimal thresholds that predicted PH , both with a sensitivity of 56% and specificity of 91%. The CP was typically multifocal rather than focal or diffuse and was associated with a background pattern of usual interstitial pneumonia. There was a significant but weaker relationship between the presence of abnormal small arteries and veins and PH . Conclusion In the setting of advanced SS c‐ PF , the histopathologic feature of the pulmonary microcirculation best associated with PH was capillary proliferation in architecturally preserved lung areas.