Merck Manual

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Causes of Interstitial Lung Diseases

Causes of Interstitial Lung Diseases

Type

Examples

Autoimmune disorders

Ankylosing spondylitis Ankylosing Spondylitis Ankylosing spondylitis is a spondyloarthritis characterized by inflammation of the spine (spondylitis), large joints, and fingers and toes, resulting in stiffness and pain. Prolonged joint pain... read more Ankylosing Spondylitis (rare), autoimmune myositis Autoimmune Myositis Autoimmune myositis causes inflammation and weakness in the muscles (polymyositis) or in the skin and muscles (dermatomyositis). Muscle damage may cause muscle pain and muscle weakness may cause... read more Autoimmune Myositis , Behçet disease Behçet Disease Behçet disease is chronic blood vessel inflammation (vasculitis) that can cause painful mouth and genital sores, skin lesions, and eye problems. The joints, nervous system, and digestive tract... read more Behçet Disease (very rare), Goodpasture syndrome Goodpasture Syndrome Goodpasture syndrome is an uncommon autoimmune disorder in which bleeding into the lungs and progressive kidney failure occur. People usually have difficulty breathing and may cough up blood... read more , mixed connective tissue disease Mixed Connective Tissue Disease (MCTD) Mixed connective tissue disease is a term used by some doctors to describe a disorder characterized by features of systemic lupus erythematosus, systemic sclerosis, and polymyositis. Raynaud... read more Mixed Connective Tissue Disease (MCTD) , relapsing polychondritis Relapsing Polychondritis Relapsing polychondritis is a rare autoimmune rheumatic disorder characterized by episodes of painful, destructive inflammation of the cartilage and other connective tissues in many organs.... read more Relapsing Polychondritis , rheumatoid arthritis Rheumatoid Arthritis (RA) Rheumatoid arthritis is an inflammatory arthritis in which joints, usually including those of the hands and feet, are inflamed, resulting in swelling, pain, and often destruction of joints.... read more Rheumatoid Arthritis (RA) , systemic sclerosis Systemic Sclerosis Systemic sclerosis is a rare, chronic autoimmune rheumatic disorder characterized by degenerative changes and scarring in the skin, joints, and internal organs and by blood vessel abnormalities... read more Systemic Sclerosis , Sjögren syndrome Sjögren Syndrome Sjögren syndrome is a common autoimmune rheumatic disorder and is characterized by excessive dryness of the eyes, mouth, and other mucous membranes. White blood cells can infiltrate and damage... read more Sjögren Syndrome , and systemic lupus erythematosus Systemic Lupus Erythematosus (SLE) Systemic lupus erythematosus is a chronic autoimmune inflammatory connective tissue disorder that can involve joints, kidneys, skin, mucous membranes, and blood vessel walls. Problems in the... read more Systemic Lupus Erythematosus (SLE) (lupus)

Organic dust

Bird droppings and molds

Drug-related

Amiodarone, bleomycin, busulfan, carbamazepine, checkpoint inhibitors, chlorambucil, cocaine, cyclophosphamide, gold, methotrexate, nitrofurantoin, sulfasalazine, and sulfonamides

Chemical-related

Aluminum powder, asbestos, beryllium, metals, sulfur dioxide, talc

Genetic disorders

Fabry disease Fabry Disease Fabry disease is a type of lysosomal storage disorder called a sphingolipidosis. It is caused by a buildup of glycolipid in tissues. This disease causes skin growths, pain in the extremities... read more Fabry Disease , familial pulmonary fibrosis, Gaucher disease Gaucher Disease Gaucher disease is a type of lysosomal storage disorder called a sphingolipidosis. It is caused by a buildup of glucocerebrosides in tissues. Children who have the infantile form usually die... read more , Hermansky-Pudlak syndrome, neurofibromatosis Neurofibromatosis Neurofibromatosis is a group of genetic disorders in which many soft, fleshy growths of nerve tissue (neurofibromas) form under the skin and in other parts of the body, and flat spots that are... read more Neurofibromatosis , Niemann-Pick disease Niemann-Pick Disease Niemann-Pick disease is type of lysosomal storage disorder. Types A and B are sphingolipidoses and are caused by a buildup of sphingomyelin in the tissues. Type C is a lipidosis that is caused... read more (rare), pulmonary alveolar microlithiasis, pulmonary alveolar proteinosis Pulmonary Alveolar Proteinosis Pulmonary alveolar proteinosis is a rare disorder in which the air sacs of the lungs (alveoli) become plugged with a protein- and fat-rich fluid. Pulmonary alveolar proteinosis typically affects... read more , and tuberous sclerosis Tuberous Sclerosis Complex Tuberous sclerosis complex is a hereditary disorder that causes abnormal growths in the brain, changes in the skin, and sometimes tumors in vital organs, such as the heart, kidneys, and lungs... read more Tuberous Sclerosis Complex

Idiopathic* interstitial pneumonias

Acute interstitial pneumonia Acute Interstitial Pneumonia Acute interstitial pneumonia is an idiopathic interstitial pneumonia that develops suddenly and is severe. (See also Overview of Idiopathic Interstitial Pneumonias.) Acute interstitial pneumonia... read more , cryptogenic organizing pneumonia Cryptogenic Organizing Pneumonia Cryptogenic organizing pneumonia is a rapidly developing idiopathic interstitial pneumonia characterized by lung inflammation and scarring that obstructs the small airways (bronchioles) and... read more , desquamative interstitial pneumonia Respiratory Bronchiolitis–Associated Interstitial Lung Disease and Desquamative Interstitial Pneumonia Respiratory bronchiolitis-associated interstitial lung disease and desquamative interstitial pneumonia are rare conditions that cause chronic lung inflammation and occur mostly in current or... read more , idiopathic pleuroparenchymal fibroelastosis Idiopathic Pleuroparenchymal Fibroelastosis Idiopathic pleuroparenchymal fibroelastosis is a rare idiopathic interstitial pneumonia that is slowly progressive. People often have recurrent infections, shortness of breath, and a dry cough... read more , idiopathic pulmonary fibrosis Idiopathic Pulmonary Fibrosis Idiopathic pulmonary fibrosis is the most common form of idiopathic interstitial pneumonia. Idiopathic pulmonary fibrosis affects mostly people over the age of 50, usually former smokers. People... read more , lymphoid interstitial pneumonia Lymphoid Interstitial Pneumonia Lymphoid interstitial pneumonia is an uncommon lung disease in which mature lymphocytes (a type of white blood cell) accumulate in the air sacs of the lungs (alveoli). People usually cough and... read more , nonspecific interstitial pneumonia Nonspecific Interstitial Pneumonia Nonspecific interstitial pneumonia is an idiopathic interstitial pneumonia that occurs mainly in women, people who do not smoke, and people younger than 50 years. (See also Overview of Idiopathic... read more , and respiratory bronchiolitis-associated interstitial lung disease Respiratory Bronchiolitis–Associated Interstitial Lung Disease and Desquamative Interstitial Pneumonia Respiratory bronchiolitis-associated interstitial lung disease and desquamative interstitial pneumonia are rare conditions that cause chronic lung inflammation and occur mostly in current or... read more

Therapeutic or industrial radiation-related

Radiation therapy for cancer

Other disorders

Amyloidosis Amyloidosis Amyloidosis is a rare disease in which abnormally folded proteins form amyloid fibrils that accumulate in various tissues and organs, sometimes leading to organ dysfunction, organ failure, and... read more Amyloidosis , chronic aspiration Aspiration Pneumonia and Chemical Pneumonitis Aspiration pneumonia is lung infection caused by inhaling mouth secretions, stomach contents, or both. Chemical pneumonitis is lung irritation caused by inhalation of substances irritating or... read more , eosinophilic pneumonia Eosinophilic Pneumonia Eosinophilic pneumonia comprises a group of lung diseases in which eosinophils (a type of white blood cell) appear in increased numbers in the lungs and usually in the bloodstream. Certain disorders... read more , lymphangioleiomyomatosis Lymphangioleiomyomatosis Lymphangioleiomyomatosis (LAM) is a rare, slowly progressive growth of smooth muscle cells throughout the lungs. (See also Overview of Interstitial Lung Diseases.) Lymphangioleiomyomatosis ... read more , pulmonary Langerhans cell histiocytosis Pulmonary Langerhans Cell Histiocytosis Pulmonary Langerhans cell histiocytosis is a disorder in which cells called histiocytes and eosinophils (types of white blood cells) proliferate in the lungs, often causing scarring. People... read more , sarcoidosis Sarcoidosis Sarcoidosis is a disease in which abnormal collections of inflammatory cells (granulomas) form in many organs of the body. Sarcoidosis usually develops in people aged 20 to 40, most often people... read more Sarcoidosis , and vasculitic disorders (which cause inflammation of blood vessels) such as eosinophilic granulomatosis with polyangiitis Eosinophilic Granulomatosis with Polyangiitis Eosinophilic granulomatosis with polyangiitis is inflammation of small- and medium-sized blood vessels that damages organs and that usually occurs in adults with a history of asthma, nasal allergies... read more , Takayasu arteritis Takayasu Arteritis Takayasu arteritis causes chronic blood vessel inflammation, mainly of the aorta (the artery that connects directly with the heart), the arteries that branch off from it, and the pulmonary arteries... read more , and granulomatosis with polyangiitis Granulomatosis with Polyangiitis Granulomatosis with polyangiitis often begins with inflammation of small- and medium-sized blood vessels and tissues in the nose, sinuses, throat, lungs, or kidneys. The cause is unknown. The... read more Granulomatosis with Polyangiitis

* Idiopathic means with no known cause.