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

Abstract

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.

Progression of Central-peripheral Band and Traction Bronchiectasis Clusters Leading to Chronic Respiratory Failure in a Patient with Fibrotic Pulmonary Sarcoidosis

“…Regarding the second observation, the mechanisms underlying how peripheral cysts form after the disappearance of granular/nodular opacities and consolidations around the bronchovascular bundles may include stenosis of the bronchi with granulomatous involvement and peribronchial fibrosis, and a further check-valve mechanism of bronchiolar involvement of granulomata and fibrosis (7). This notion is supported by the fact that a decrease in the size of the enlarged cysts over time is sometimes observed in fibrotic pulmonary sarcoidosis (5), as was seen in this case. Furthermore, pulmonary sarcoidosis cases have been detected with multiple severe cysts occupying more than half of the total lung volume (5), suggesting the possibility of other factors.…”

“…Indeed, a pathological study of 66 autopsy cases (7) showed that bronchovascular bundle fibrosis was frequently observed (58%) and was accompanied by peribronchial atelectasis. In the abovementioned study (5), traction bronchiectasis arose from granular/nodular opacities (8). Until recently, obstructive ventilatory impairment rather than restrictive ventilatory impairment had been of interest in pulmonary sarcoidosis; however, the present case, as well as other cases included in the abovementioned study (5), showed progression of restrictive ventilatory impairment as fibrosis progressed.…”

“…In the abovementioned study (5), traction bronchiectasis arose from granular/nodular opacities (8). Until recently, obstructive ventilatory impairment rather than restrictive ventilatory impairment had been of interest in pulmonary sarcoidosis; however, the present case, as well as other cases included in the abovementioned study (5), showed progression of restrictive ventilatory impairment as fibrosis progressed. Neither pulmonary hypertension nor chronic progressive pulmonary aspergillosis, which are frequently observed in fibrotic pulmonary sarcoidosis, were detected in this case.…”

Radiographic and Histopathologic Features in Sarcoidosis: A Pictorial Display

“…Central airway obstruction may reflect bronchial distortion or extrinsic bronchial compression from enlarged lymph nodes. 49,55,61 The distribution of fibrosis, bullae, and cystic lesions preferentially involve the upper lobes and perihilar regions 50,[62][63][64] (►Figs. 26 and 27) in a distinct lymphangitic pattern 65 (►Fig.…”

Management of Sarcoidosis, a Selection of Topical Items Updating

“…Advanced pulmonary sarcoidosis is the main cause of dangerous sarcoidosis. In their paper, Sawahata et al had the original idea of comparing the thorax CT in patients with supplemental oxygen need with a previous one done several years before for a better understanding of the radiological findings’ significance [ 41 ]. They proved that in many cases, what resembled honeycombing was actually bronchiectasis.…”