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Sarcoidosis (/ ˌ s ɑːr k ɔɪ ˈ d oʊ s ɪ s /; also known as Besnier–Boeck–Schaumann disease) is a disease involving abnormal collections of inflammatory cells that form lumps known as granulomata. [2]
The differential diagnosis includes other types of lung disease that cause similar symptoms and show similar abnormalities on chest radiographs. Some of these diseases cause fibrosis, scarring or honeycomb change. The most common considerations include: chronic hypersensitivity pneumonitis; non-specific interstitial pneumonia; sarcoidosis
Idiopathic interstitial pneumonia (IIP), or noninfectious pneumonia [1] are a class of diffuse lung diseases. These diseases typically affect the pulmonary interstitium, although some also have a component affecting the airways (for instance, cryptogenic organizing pneumonitis). There are seven recognized distinct subtypes of IIP. [2]
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 ...
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.
Acute interstitial pneumonitis (also known as acute interstitial pneumonia) is a rare, severe lung disease that usually affects otherwise healthy individuals. There is no known cause or cure. Acute interstitial pneumonitis is often categorized as both an interstitial lung disease and a form of acute respiratory distress syndrome (ARDS).
In a cohort of 59 CVID patients with granulomatous disease, 30 (51%) of whom had lung involvement, complete remission of disease was obtained in 5 of 25 attempts using corticosteroids (three patients), methotrexate (1 patient) and cyclophosphamide (1 patient). [2] Partial responses were also seen with rituximab and hydroxychloroquine.
The yearly incidence of AE-IPF is between 10 and 15% of all patients. The prognosis of AE-IPF is poor, with mortality ranging from 78% to 96%. [63] Other causes of AE-IPF such as pulmonary embolism, congestive heart failure, pneumothorax, or infection need to be excluded. Pulmonary infection have to be ruled out by endotracheal aspirate or BAL.