See also lysyl oxidase-type enzymes in the “enzyme” section
Classification:
Pulmonary fibrotic disorders are classified into the following groups, in order of their relative occurrence: idiopathic pulmonary fibrosis (IPF), nonspecific interstitial pneumonia (NSIP), respiratory bronchiolitis-associated interstitial lung disease, desquamative interstitial pneumonia, cryptogenic organizing pneumonia, acute interstitial pneumonia, and lymphocytic interstitial pneumonia (LIP) as well as acute respiratory disress syndrome (ARDS), scleroderm-associated lung fibrosis and fibrotic damage as a sequelae of sarcoidosis. (US application No: 12/860834).
Etiology and symptoms:
Pulmonary fibrotic disorders are characterized by inflammation and fibrosis of the lung parenchyma. Fibrosis is the abnormal accumulation of fibrous tissue that can occur as a part of the wound healing process in damaged tissue. Such tissue damage may result from physical injury, inflammation, infection, exposure to toxins, and other causes. The etiology of these diseases has not been established, and prognosis is generally poor. Symptoms include decreased body weight, increased lung weight, presence of activated fibroblasts or fibrocytes, presence of fibrocyte precursor cells (e.g., cells that express both CD45 and collagen), abnormal lung architecture (including alveolar thickening, proliferation and expansion of pneumocytes, and honeycomb lung. Molecular symptoms include increase in one or more of the following proteins: LOXL2 (see US2009/0053224, US2009/0104201), alpha-smooth muscle actin, transforming growth factor beta-1, stromal dervied factor-1beta, stromal derirved factor-1, endothelin-1 and phosphorylated SMAD2.
Pulmonary fibrosis is a common disorder thought to be due to the destructive effects of products released from leukocytes. Pulmonayr fibrosis may depend upon the recruitment and activation of lymphotyces (Huang (US 2007/0148173).
Treatment of Pulmonary fibrotic disorders
Spangler (US12/860834) discloses inhibitors of the lysyl oxidase related protein-2 (LOXL2) such as anti-LOXL2 antibodies for the treatment of pulmonary fibrotic disorders such as IPF, interstitial pneumonia and acute respiratory distress syndrom (ARDS).
Specific Diseases
Pulmonary fibrotic disorders are classified in the following groups, arranged in order of their frequency of occurrence: (1) IPF, nonspecific interstitial pneumonia (NSIP), respiratory bronchiolitis-associated interstitial lung disease, desquamative interstitial pneumonia, cryptogenic organizing pneumonia, acute interstitial pneumonia, and lympocytic interstitial pneumonia (LIP). ARDS has also been identified as a pulmonary fibrotic disorder.
Acute Respiratory Distress Syndrome (ARDS):
Acute interstitial pneumonia:
Cryptogenic organizing pneumonia:
Idiopathic pulmonary fibrosis (IPF): see outline
Lymphocytic interstitial pneumonia (LIP):
Nonspecific interstitial pneumonia (NSIP):