Metabolic and storage lung diseases: spectrum of imaging appearances

Renapurkar, Rahul D.; Kanne, Jeffrey P.

doi:10.1007/s13244-013-0289-x

Cited by 17 publications

(34 citation statements)

References 73 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…In addition, parenchymal abnormalities such as bronchiectasis and lobar collapse may be evident especially in distal disease 15. HRCT helps in defining the extent and degree of luminal narrowing and planning therapy 14. The HRCT in our patient also showed circumferential thickening of the airway wall, endoluminal irregularities and an MLS.…”

Section: Discussionmentioning

confidence: 53%

Middle lobe syndrome: an intriguing presentation of tracheobronchial amyloidosis

et al. 2017

View full text Add to dashboard Cite

Pulmonary involvement in amyloidosis is a distinct rarity. This clinical entity usually presents as tracheobronchial amyloidosis (TBA). A 32-year-old, never-smoker man presented with episodic dyspnoea and wheezing along with cough and mucoid sputum. The chest radiograph was suggestive of a middle lobe syndrome (MLS). High-resolution CT (HRCT) of the chest confirmed the presence of MLS. In addition, HRCT showed circumferential thickening of the trachea and the main bronchi, with thickening of the posterior membranous wall of trachea. Fibrebronchoscopy, done to evaluate MLS, visualised multiple small polypoidal lesions in the lower part of trachea and carina. Endobronchial biopsies showed homogeneous, acellular amorphous deposit in the subepithelial region, which was congophilic in nature. A diagnosis of TBA presenting as MLS was made. To the best of our knowledge, this is the first detailed report of MLS as a presentation of TBA in the English literature.

show abstract

Section: Discussionmentioning

confidence: 53%

Middle lobe syndrome: an intriguing presentation of tracheobronchial amyloidosis

et al. 2017

View full text Add to dashboard Cite

show abstract

“…110,114,112 Chest x-ray and CT scan usually reveal reticolonodular markings easily misdiagnosed as other interstitial lung disease/fibrosis or as traction bronchiectasis. 42,116,117 When linear or micronodular calcific opacities are evidenced, 58,60,116 they are indistinguishable from those visible in other types of diffuse parenchymal calcifications. 118,119 Diffuse parenchymal calcification with possible accompanying ossification mainly occurs in diseases with abnormal calcium/phosphorus metabolism, such as hyperparathyroidism (both primary and secondary, especially in patients with chronic renal failure on periodic hemodyalisis), hypervitaminosis D and milk-alkali syndrome, infectious granulomatous disorders, including fungal and parasitic diseases, pneumoconiosis, alveolar proteinosis, and pulmonary alveolar microlithiasis.…”

Section: Authors' Commentmentioning

confidence: 99%

“…118,119 Diffuse parenchymal calcification with possible accompanying ossification mainly occurs in diseases with abnormal calcium/phosphorus metabolism, such as hyperparathyroidism (both primary and secondary, especially in patients with chronic renal failure on periodic hemodyalisis), hypervitaminosis D and milk-alkali syndrome, infectious granulomatous disorders, including fungal and parasitic diseases, pneumoconiosis, alveolar proteinosis, and pulmonary alveolar microlithiasis. 1,89,116,117,[120][121][122][123][124][125] Intraoperatively or in video-assisted thoracoscopy, the irregular lung surface because of the DPO has sometimes been rightly analogized to a coral-reef appearance 64 or to a balloon bound with wire nettings. 71 The histologic diagnosis of diffuse pulmonary ossification is straightforward, and the differential diagnosis between the 2 main forms (dendriform vs. nodular) is based either on the branching appearance of spicules, the presence of fatty or hematopoietic marrow within the intertrabecular spaces, or the presence of fibrous tissue surrounding bone in the interstitium, which are typically seen in DPO and usually absent in the nodular form.…”

Section: Authors' Commentmentioning

confidence: 99%

Selected Case From the Arkadi M. Rywlin International Pathology Slide Series

Bisceglia¹,

Chiaramonte

Panniello

et al. 2015

Advances in Anatomic Pathology

View full text Add to dashboard Cite

Two cases of diffuse dendriform pulmonary ossification (DPO) are presented, one of the secondary type and the other of the idiopathic type. Case 1 was an adult female patient who underwent thoracic surgery to remove a posterior mediastinal bronchogenic cyst, which was discovered on a computed tomography scan performed after an episode of pneumonia when traction bronchiectasis with interstitial lung disease/fibrosis was also suspected in the lungs. Histologic examination performed on the resected lung tissue revealed numerous scattered small osseous spicules on a background of intense interstitial inflammation and fibrosis, leading to further clinical and laboratory investigations and the final diagnosis of DPO secondary to lung involvement by scleroderma. Case 2 was an adult male patient who underwent thoracoscopic exploration after a computed tomography scan, which revealed traction bronchiectasis with linear thickening of the interstitial lung tissue. Histologic examination of the lung tissue specimen revealed numerous osseous spicules in the absence of interstitial septal inflammation. Noteworthy in this case were also some nodules of collagenized tissue similar to those seen in the lungs of patients affected by Ehlers-Danlos syndrome. The absence of any clinical sign or symptoms related to Ehlers-Danlos syndrome attest to the nonspecificity of these pulmonary fibrous nodules. No case of DPO secondary to scleroderma has been reported in the literature so far, although around half of the patients with scleroderma manifest pulmonary diseases. Idiopathic DPO is even rarer, usually discovered postmortem, with only 20 cases diagnosed in life with lung biopsies taken by open surgery or through a thoracoscopic approach. DPO is often misdiagnosed radiologically as bronchiectasis and/or interstitial lung disease/fibrosis.

show abstract

“…Three main forms and CT patterns of pulmonary amyloidosis have been described: tracheobronchial amyloidosis, diffuse interstitial amyloidosis and nodular pulmonary amyloidosis [1]. Tracheobronchial amyloidosis is characterized by mural nodules and calcification of the thickened tracheobronchial wall, localized or multifocal.…”

Section: Commentmentioning

confidence: 99%