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Cicatricial organising pneumonia mimicking a fibrosing interstitial pneumonia
Author(s) -
Churg Andrew,
Wright Joanne L,
Bilawich AnaMaria
Publication year - 2018
Publication title -
histopathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.626
H-Index - 124
eISSN - 1365-2559
pISSN - 0309-0167
DOI - 10.1111/his.13443
Subject(s) - granulation tissue , usual interstitial pneumonia , medicine , idiopathic interstitial pneumonia , lung biopsy , pathology , pneumonia , calcification , lung , biopsy , interstitial pneumonia , interstitial lung disease , soft tissue , pathological , radiology , surgery , wound healing
Aims Organising pneumonia (OP) is composed of loose granulation tissue plugs in distal airspaces; these disappear with steroid treatment. Recently a variant labelled ‘cicatricial’ OP has been described in which the granulation tissue organised to much denser fibrous tissue but still retained the usual pattern of OP. Here we report 10 patients thought to have an interstitial lung disease, and who on biopsy had a variant of cicatricial OP characterised by linear bands or small nodular masses of dense fibrous tissue that does not resemble ordinary OP. Methods and results The bands/nodules were usually distributed randomly but occasionally resembled fibrotic non‐specific interstitial pneumonia in local areas. Small foci of loose granulation tissue at the edge of the fibrotic bands sometimes mimicked fibroblast foci. Recognisable conventional OP was always present, but often in very small amounts. Four cases, including one patient with Ehlers–Danlos syndrome, showed formation of bone in the fibrotic bands and nodules. On computerised tomography (CT) scan of the chest some cases looked like typical OP, but some demonstrated only irregularly distributed linear opacities, sometimes with associated calcification. Follow‐up imaging on six cases showed that the process either markedly improved or remained stable over time; no case had progressive disease. Conclusions Cicatricial OP with this pathological pattern represents an uncommon form of OP that appears to be a generally benign process which may have persisting linear opacities on CT scan but that does not progress; however, it can be confused on biopsy and CT with a fibrosing interstitial pneumonia.