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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
04:44

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

Published on: June 16, 2020

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Expanded acceptance of acute exacerbation of nonspecific interstitial pneumonia, including 7 additional cases with

Atsushi Miyamoto1, Amita Sharma2, Michiya Nishino1

  • 1Department of Pathology, Massachusetts General Hospital, Boston, MA, USA.

Pathology International
|May 5, 2018
PubMed

Insights

Acute exacerbation in nonspecific interstitial pneumonia (NSIP) is linked to specific lung biopsy findings. Identifying organizing pneumonia, alveolar hemorrhage, fibroblastic foci, and hyaline membranes can help predict exacerbations.

Area of Science:

  • Pulmonary Medicine
  • Pathology
  • Respiratory Diseases

Background:

  • Acute exacerbation is infrequently diagnosed in patients with nonspecific interstitial pneumonia (NSIP).
  • Pathologic features of acute exacerbation in NSIP are less understood compared to idiopathic pulmonary fibrosis.
  • Surgical lung biopsy is a key diagnostic tool for interstitial lung diseases.

Purpose of the Study:

  • To investigate the clinicopathologic features of acute exacerbation in patients with histopathologically proven NSIP.
  • To determine if specific pathological findings in lung biopsies are associated with acute exacerbation of NSIP.
  • To assess the utility of identifying organizing pneumonia, alveolar hemorrhage, fibroblastic foci, and hyaline membranes in predicting acute exacerbation.

Main Methods:

  • Retrospective analysis of 14 consecutive NSIP cases diagnosed via surgical lung biopsy.
  • Clinical confirmation of acute exacerbation diagnosis.
  • Evaluation of lung biopsy slides for the presence of organizing pneumonia lesions, alveolar hemorrhage, numerous fibroblastic foci, and focal hyaline membranes.

Main Results:

  • Acute exacerbation was clinically diagnosed in 8 out of 14 NSIP cases.
  • Pathologic features observed in acute exacerbation cases included organizing pneumonia (7/8), alveolar hemorrhage (7/8), many fibroblastic foci (6/8), and focal hyaline membranes (3/8).
  • The presence of more than two of these pathologic components was significantly associated with acute exacerbation (p<0.05).

Conclusions:

  • Specific pathologic features in lung biopsies, including organizing pneumonia, alveolar hemorrhage, numerous fibroblastic foci, and focal hyaline membranes, are more prevalent in acute exacerbations of NSIP.
  • Identifying a combination of these features (more than two components) in NSIP lung biopsies may aid in predicting the likelihood of acute exacerbation.
  • Further research into these clinicopathologic correlations can improve the management of NSIP patients prone to exacerbations.
Keywords:
acute exacerbationfibroblastic fociidiopathic interstitial pneumonianonspecific interstitial pneumoniapathologysurgical lung biopsy

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