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Interstitial lung disease (ILD), or diffuse parenchymal lung disease (DPLD), [3] is a group of respiratory diseases affecting the interstitium (the tissue) and space around the alveoli (air sacs) of the lungs. [4] It concerns alveolar epithelium, pulmonary capillary endothelium, basement membrane, and perivascular and perilymphatic tissues. It ...
These multifocal lesions are observed in tuberous sclerosis, [6] [7] and can be associated with lymphangioleiomyomatosis [8] and perivascular epithelioid cell tumour (PEComa or clear cell "sugar tumor")). [9] It can be diagnosed through lung biopsy using thoracoscopy. [10]
Alveolar capillary dysplasia (ACD) is a rare, congenital diffuse lung disease characterized by abnormal blood vessels in the lungs that cause highly elevated pulmonary blood pressure and an inability to effectively oxygenate and remove carbon dioxide from the blood.
Restrictive lung diseases are a category of extrapulmonary, pleural, or parenchymal respiratory diseases that restrict lung expansion, [2] resulting in a decreased lung volume, an increased work of breathing, and inadequate ventilation and/or oxygenation. Pulmonary function test demonstrates a decrease in the forced vital capacity.
Because the hyperplasia of PNE cells can be seen as a reaction to chronic lung disease, surrounding solitary bronchial carcinoids and adenocarcinoma of the lung, these causes must be excluded prior to a DIPENCH diagnosis. [1] Obstructive bronchiolitis has been reported as a characteristic histopathologic finding in patients with DIPNECH. [8]
It occurs most often in infants and young children [1] but also has been reported in adults. [2] In a retrospective review of 204 children with lung tumors, pleuropulmonary blastoma and carcinoid tumor were the most common primary tumors (83% of the 204 children had secondary tumors spread from cancers elsewhere in the body). [ 1 ]
There is no cause for most cases of neuroendocrine hyperplasia. However, a known cause is a rapid increase of pulmonary endocrine cells in the lungs in children under the age of 2 years. An increase in pulmonary endocrine cells is usually seen in adults with a history of smoking, COPD, or cystic fibrosis.
LAM cells appear to arise from an extrapulmonary source and migrate to the lung. [21] Increased LAM cell migration and invasiveness is rescued by TSC2 re-expression. [49] The cellular and molecular mechanisms of neoplastic transformation and lung parenchymal destruction by LAM cells remain unknown.