An Autopsy Case of Sarcoidosis Associated with Refractory Pulmonary Fibrosis

Sugino, Keishi; Isobe, Kazutoshi; Iwata, Motohide; Ito, Takafumi; Wada, Tomohiro; Kaburaki, Kyohei; Gocho, Kyoko; Ishida, Fumiaki; Yamakoshi, Shiho; Sato, Daisuke; Sakaguchi, Shinji; Sato, Keita; Sano, Go; Sakamoto, Susumu; Takai, Yujiro; Akasaka, Yoshikiyo; Shibuya, Kazutoshi; Uekusa, Toshimasa; Takemura, Tamiko; Eishi, Yoshinobu; Honma, Sakae

doi:10.7878/jjsogd.30.33

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“…Concerning the second observation, the honeycomb lung-like structures seemed to be the result of clustering of traction bronchiectasis and small cysts at the distal side ( Figure 2). To our knowledge, this finding has not been reported to date, partly because the number of reports that investigated such patients are limited [8][9][10][11][12][13][14]. However, considering the relatively low incidence of honeycomb lung-like structures (30%) in this study, there remains a possibility that this architecture does not play a crucial role in disease prognosis.…”

Section: Discussionmentioning

confidence: 68%

Computed Tomography Images of Fibrotic Pulmonary Sarcoidosis Leading to Chronic Respiratory Failure

Sawahata

Johkoh

Kawanobe

et al. 2020

JCM

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Background: There is currently no consensus on the morphology of severe fibrotic pulmonary sarcoidosis, and we examined computed tomography (CT) findings and progression. Methods: We analyzed findings in 10 consecutive patients (three men, seven women) with pulmonary sarcoidosis requiring oxygen therapy for chronic respiratory failure, who were extracted from >2500 sarcoidosis patients (three hospitals, 2000–2018). Patients with comorbidities causing chronic respiratory failure were excluded. Results: Predominant findings were consolidations along the bronchovascular bundles comprising ‘central-peripheral band’, traction bronchiectasis, peripheral cysts/bullae, and upper lobe shrinkage. Traction bronchiectasis arose from opacities comprising ‘central-peripheral band’. Clustering of traction bronchiectasis at the distal side formed honeycomb lung-like structures in three patients. Upper lobe shrinkage progressed in seven patients together with progression of consolidations, ‘central-peripheral band’, traction bronchiectasis clusters, and cysts, while patients without shrinkage included two patients with severe multiple cysts without traction bronchiectasis. Restrictive ventilatory impairment developed in most patients. Pulmonary hypertension (PH) was detected radiologically in five patients, and chronic progressive pulmonary aspergillosis (CPPA) in four patients. Conclusions: During progression, consolidations comprising ‘central-peripheral band’ progressed together with traction bronchiectasis clusters and peripheral cysts, resulting in upper lobe shrinkage. This may lead to respiratory failure with possible complications such as PH and CPPA.

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