Central Bronchial Deformity in Pulmonary Sarcoidosis: A Finding Suggestive of an Upper Lobe Fibrotic Phenotype on Chest Images.

RATIONALE AND OBJECTIVES: Bronchial and lung parenchymal structural remodeling may occur due to disease progression in patients with pulmonary sarcoidosis; however, its mechanisms remain unclear. Central bronchial deformity (CBD) associated with shrinkage in the upper lobe (SUL) is often observed in such patients. This study aimed to examine the association between CBD and structural remodeling to identify features indicating disease severity on chest images. MATERIALS AND METHODS: This retrospective cohort study included 72 patients with pulmonary sarcoidosis, excluding patients with only bilateral hilar lymphadenopathy. The participants were divided into with and without CBD groups to examine the association between CBD and other structural remodeling, including SUL, cyst and/or low attenuation area-like emphysema (Cyst/LAA), pleural/sub-pleural thickening (PT), and traction bronchiectasis (TrBE), in the upper lobe on chest images. The association of CBD phenotype with respiratory dysfunction was also examined. RESULTS: CBD was highly associated with SUL (81.4% vs. 8.9%), Cyst/LAA (44.4% vs. 6.7%), and PT (59.2% vs. 3.7%). The respective odds ratios in the univariable and multivariable analyses were as follows: SUL, 45.1 and 39.9; Cyst/LAA, 11.2 and 14.2; and PT, 64.0 and 68.7. TrBE was frequently associated with CBD (22.25% vs. 4.4%); the odds ratio was 6.14 in the univariable analysis. Furthermore, participants with CBD exhibited lower %FVC and %DLCO. CONCLUSION: CBD is significantly associated with lung remodeling (SUL, Cyst/LAA, TrBE, and PT) and respiratory dysfunction. CBD may be a crucial clinical phenotype to identify upper lobe fibrotic changes.

A 33-Year-Old Man With Hemoptysis and Renal Dysfunction.

CASE PRESENTATION: A 33-year-old man presented with a 10-day history of fever, dry cough, and dyspnea. He reported small amounts of frank hemoptysis that occurred several times a day for the past 3 days and a reduction in urine volume. There was no joint pain, skin rash, muscle weakness, or bleeding symptoms, except for the hemoptysis. He had a medical history of childhood asthma and untreated hypertension for the past 2 years. He had no history of smoking, recent travel, medication use, or occupational inhalation.

Paradoxical Formation of a Pleuroparenchymal Fibroelastosis-like Lesion in the Chronic Course of Pulmonary Sarcoidosis.

We herein report the long-term changes in chest computed tomography (CT) findings from early sarcoidosis lesions to pleuroparenchymal fibroelastosis (PPFE)-like lesions in a 30-year-old man with granulomas on a transbronchial lung biopsy. Multiple bilateral micronodular and nodular opacities around the bronchovascular bundle in the upper lobes detected by chest CT in 2004 disappeared, but paradoxically, peripheral consolidations continued to grow at the periphery of the original lesions. Chest CT in 2017 confirmed the progression of bilateral shrinkage of the upper lobe, spread of peripheral consolidations and wedge-shaped opacities below the first rib, and bronchiectatic air bronchograms, confirming PPFE-like lesions.

Honeycomb-like structures in sarcoidosis pathologically showing granulomas in walls of clustered bronchioles.

In the clinical setting, it is often difficult to judge whether honeycomb-like structures represent progression of fibrosis in pulmonary sarcoidosis or a complication by interstitial pneumonitis. This report described a valuable case in which pathology of video-assisted thoracoscopic surgery specimens collected from the lungs with honeycomb-like structures that were continuous with the dilated bronchioles on chest computed tomography (CT) showed granulomas in the membranous bronchiole walls, thereby demonstrating that the honeycomb-like structures were lung lesions of sarcoidosis. Pathological features of these structures on chest CT included cystic changes attributable to incorporation of peripheral alveoli into dilated bronchioles in lobules: these findings in lung sarcoidosis were different from those corresponding to honeycomb lung in idiopathic pulmonary fibrosis/usual interstitial pneumonia. Radiological and pathological findings showed the possibility that progressive clustering of dilated bronchi and bronchioles causes cystic changes, resulting in the formation of honeycomb-like structures as fibrosis progresses in sarcoidosis with lung involvement.

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

We herein report a rare case of pulmonary sarcoidosis leading to chronic respiratory failure with restrictive ventilatory impairment during a 53-year-long observation period. Nine years after the histological diagnosis of stage I sarcoidosis on chest X-ray in a woman in her 20s, she developed bilateral reticular and granular opacities on chest computed tomography and was started on prednisone for 18 years. Seven years after prednisone withdrawal, these persisting opacities around the bronchovascular bundle, including a central-peripheral band, had progressed, forming traction bronchiectasis clusters and peripheral cysts, some of which developed continuously at the distal side of these clusters, with eventual upper lobe shrinkage.

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

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.

"Possible primary antiphospholipid syndrome" with concurrent diffuse alveolar hemorrhaging and Libman-Sacks endocarditis mimicking catastrophic antiphospholipid syndrome.

Although antiphospholipid antibody syndrome (APS) is an autoimmune condition that is primarily characterized by arterial or venous thrombosis or pregnancy morbidity and the presence of antiphospholipid antibodies (aPL), recent reviews have introduced non-thromboembolic manifestations. We describe the case of a 58-year-old woman with vegetation on the aortic valve, whose initial presentation of APS abruptly developed into diffuse pulmonary hemorrhage. Despite consecutive plasma exchange procedures and the administration of corticosteroids and high-dose intravenous immunoglobulin, multiple brain infarctions developed, and the patient died of pneumonia. Although anecdotal, this case might serve as a useful example of the non-standard complications of fulminant APS.