Restrictive Lung Diseases
DiseaseMechanism
PneumoconiosesInhaled inorganic dusts trigger an interstitial inflammatory reaction with fibrosis
Silicosis
Inhaled silica dust produces a fibrogenic response in a nodular pattern ("silicotic nodules")
Asbestosis
Inhaled asbestos fibers become encrusted with calcium and iron ("ferruginous bodies"). Pleural plaques (often diaphgragmatic) and interstitial fibrosis occur. The risk of bronchogenic carcinoma is increased, particularly in smokers. Mesotheliomas can occur but are rare
Coal Worker's Pneumoconiosis
The amount of carbonaeous dust inhaled to produce CWP is far in excess of the simple anthracosis that nearly everyone has. "Coal macules" induce fibrosis
Berylliosis
In this rare condition the inhaled dusts contain beryllium that produces a granulomatous response with "sarcoid-like" granulomas
SarcoidosisThis idiopathic condition is characterized by granuloma formation. The granulomas are typically non-caseating. Hilar lymph nodes are nearly always involved and can become quite large
Idiopathic Pulmonary FibrosisIPF includes a variety of entities, of which usual interstitial pneumonitis (UIP) is most common, all characterized by progressive fibrosis
Autoimmune ConditionsThe damage can occur with autoantibodies that produce inflammation involving mesothelial surfaces and vascular basement membranes
Scleroderma
Diffuse scleroderma can lead to interstitial fibrosis with minimal inflammation
Rheumatoid Arthritis
Focal necrotizing granulomatous inflammation ("rheumatoid nodules") can form on pleural surfaces and in the parenchyma
Wegener's Granulomatosis
There is vasculitis involving pulmonary arteries
Goodpasture SyndromeThere is antiglomerular basement membrane antibody (that also is directed at pulmonary capillary basement membranes) that causes a form of type II hypersensitivity reaction which damages alveolar capillary basement membranes with subsequent hemorrhage into alveoli
Therapeutic ComplicationsThe lungs can be damaged by radiation and pharmacologic agents used to treat various conditions
Radiation
External beam radiation therapy, particularly mantle radiation for Hodgkin disease, can result in progressive fibrosis of interstitium and pleura
Drugs
The chemotherapeutic agents bleomycin and busulfan in particular induce pulmonary fibrosis
Hypersensitivity PneumonitisInhaled allergens produce localized antigen-antibody complexes that lead to an acute extrinsic allergic alveolitis, a form of type III hypersensitivity. If chronic, there can be a type IV hypersensitivity response
Diffuse Alveolar DamageDAD is an acute process that can result from any type of severe lung injury. Clinically, it is known as adult respiratory distress syndrome (ARDS)
Phase 1
In the first few days following an acute lung injury, there is exudation with hyaline membrane formation in alveoli
Phase 2
Toward the end of the first week following acute lung injury, pulmonary macrophages proliferate and interstitial edema with some mixed inflammatory infiltrates occurs
Phase 3
Over weeks to months following acute lung injury, there is increasing interstitial fibrosis leading to "honeycomb" change