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The morbidity associated with DIPNECH is due to the associated obstructive lung disease. The lung disease tends to be slowly progressive, but given enough time can lead to significant disability and require supplemental oxygen therapy. There have been reports of lung transplantation in the setting of end-stage DIPNECH.
According to a recent study, the main risk factors for RA-ILD are advancing age, male sex, greater RA disease activity, rheumatoid factor (RF) positivity, and elevated titers of anticitrullinated protein antibodies such as anticyclic citrullinated peptide. Cigarette smoking also appears to increase risk of RA-ILD, especially in patients with human leukocyte antigen DRB1.
A recently published retrospective study by a team from Beijing Chao-Yang Hospital in Beijing, China, supported three of the risk factors listed for RA-ILD and identified an additional risk factor. In that study of 550 RA patients, logistic regression analysis of data collected on the 237 (43%) with ILD revealed that age, smoking, RF positivity, and elevated lactate dehydrogenase closely correlated with ILD.
Recent studies have identified risk factors for disease progression and mortality. A retrospective study of 167 patients with RA-ILD determined that the usual interstitial pneumonia (UIP) pattern on high-resolution computed tomography (HRCT) was a risk factor for progression, as were severe disease upon diagnosis and rate of change in pulmonary function test results in the first 6 months after diagnosis.
A study of 59 RA-ILD patients found no median survival difference between those with the UIP pattern and those without it. But the UIP group had more deaths, hospital admissions, need for supplemental oxygen, and decline in lung function.
PAM is one of the rare lung diseases currently being studied by the Rare Lung Diseases Consortium (RLDC). Pulmonary Alveolar Microlithiasis patients, families, and caregivers are encouraged to join the NIH Rare Lung Diseases Consortium Contact Registry. This is a privacy protected site that provides up-to-date information for individuals interested in the latest scientific news, trials, and treatments related to rare lung diseases.
Asbestos can cause lung cancer that is identical to lung cancer from other causes. Exposure to asbestos is associated with all major histological types of lung carcinoma (adenocarcinoma, squamous cell carcinoma, large-cell carcinoma and small-cell carcinoma). The latency period between exposure and development of lung cancer is 20 to 30 years. It is estimated that 3%-8% of all lung cancers are related to asbestos. The risk of developing lung cancer depends on the level, duration, and frequency of asbestos exposure (cumulative exposure). Smoking and individual susceptibility are other contributing factors towards lung cancer. Smokers who have been exposed to asbestos are at far greater risk of lung cancer. Smoking and asbestos exposure have a multiplicative (synergistic) effect on the risk of lung cancer. Symptoms include chronic cough, chest pain, breathlessness, haemoptysis (coughing up blood), wheezing or hoarseness of the voice, weight loss and fatigue. Treatment involves surgical removal of the cancer, chemotherapy, radiotherapy, or a combination of these (multimodality treatment). Prognosis is generally poor unless the cancer is detected in its early stages. Out of all patients diagnosed with lung cancer, only 15% survive for five years after diagnosis.
Pulmonary fibrosis may be a secondary effect of other diseases. Most of these are classified as interstitial lung diseases. Examples include autoimmune disorders, viral infections and bacterial infection like tuberculosis which may cause fibrotic changes in both lungs upper or lower lobes and other microscopic injuries to the lung. However, pulmonary fibrosis can also appear without any known cause. In this case, it is termed "idiopathic". Most idiopathic cases are diagnosed as "idiopathic pulmonary fibrosis". This is a diagnosis of exclusion of a characteristic set of histologic/pathologic features known as usual interstitial pneumonia (UIP). In either case, there is a growing body of evidence which points to a genetic predisposition in a subset of patients. For example, a mutation in surfactant protein C (SP-C) has been found to exist in some families with a history of pulmonary fibrosis.
Diseases and conditions that may cause pulmonary fibrosis as a secondary effect include:
- Inhalation of environmental and occupational pollutants, such as metals in asbestosis, silicosis and exposure to certain gases. Coal miners, ship workers and sand blasters among others are at higher risk.
- Hypersensitivity pneumonitis, most often resulting from inhaling dust contaminated with bacterial, fungal, or animal products.
- Cigarette smoking can increase the risk or make the illness worse.
- Some typical connective tissue diseases such as rheumatoid arthritis, SLE and scleroderma
- Other diseases that involve connective tissue, such as sarcoidosis and granulomatosis with polyangiitis.
- Infections
- Certain medications, e.g. amiodarone, bleomycin (pingyangmycin), busulfan, methotrexate, apomorphine, and nitrofurantoin
- Radiation therapy to the chest
Five million people worldwide are affected by pulmonary fibrosis. A wide range of incidence and prevalence rates have been reported for pulmonary fibrosis. The rates below are per 100,000 persons, and the ranges reflect narrow and broad inclusion criteria, respectively.
Based on these rates, pulmonary fibrosis prevalence in the United States could range from more than 29,000 to almost 132,000, based on the population in 2000 that was 18 years or older. The actual numbers may be significantly higher due to misdiagnosis. Typically, patients are in their forties and fifties when diagnosed while the incidence of idiopathic pulmonary fibrosis increases dramatically after the age of fifty. However, loss of pulmonary function is commonly ascribed to old age, heart disease or to more common lung diseases.
To date there have been no clinical trials to determine effective treatment for this disease. Some patients have been treated with somatostatin analogs. Although the cough associated with DIPNECH tends to diminish on this treatment, improvement in pulmonary function has not been clearly demonstrated. There are also reports of symptomatic treatment with long- and short-acting beta agonists. Although steroids, both oral and inhaled, have been used in the setting of DIPNECH, there is no clear improvement with this treatment.
It is not uncommon for typical carcinoids to arise within DIPNECH. Due to presence of these tumors, DIPNECH is classified as a pre-malignant condition. Although there have been reports of atypical carcinoids with local lymph node involvement, there are no reports of more aggressive neuroendocrine tumors, such as large cell neuroendocrine or small cell lung cancer, associated with DIPNECH. When isolated bronchial carcinoids are diagnosed, oncology guidelines recommend surgical resection with lymph node sampling. However, as multiple carcinoids may develop in the setting of DIPNECH, a more conservative approach is often considered to preserve lung function.
Since the disease was first described in 1918, over 500 case reports have appeared in the literature. PAM is associated with consanguinity. The incidence is higher in Turkey, Japan, India and Italy. The mean age at diagnosis is 35 years based on the cases reported in the literature.
ILD may be classified according to the cause. One method of classification is as follows:
1. Inhaled substances
- Inorganic
- Silicosis
- Asbestosis
- Berylliosis
- printing workers (eg. carbon bblack, ink mist)
- Organic
- Hypersensitivity pneumonitis
2. Drug-induced
- Antibiotics
- Chemotherapeutic drugs
- Antiarrhythmic agents
3. Connective tissue and Autoimmune diseases
- Rheumatoid arthritis
- Systemic lupus erythematosus
- Systemic sclerosis
- Polymyositis
- Dermatomyositis
4. Infection
- Atypical pneumonia
- Pneumocystis pneumonia (PCP)
- Tuberculosis
- "Chlamydia" trachomatis
- Respiratory Syncytial Virus
5. Idiopathic
- Sarcoidosis
- Idiopathic pulmonary fibrosis
- Hamman-Rich syndrome
- Antisynthetase syndrome
6. Malignancy
- Lymphangitic carcinomatosis
7. Predominantly in children
- Diffuse developmental disorders
- Growth abnormalities deficient alveolarisation
- Infant conditions of undefined cause
- ILD related to alveolar surfactant region
Interstitial lung disease (ILD), or diffuse parenchymal lung disease (DPLD), is a group of lung diseases affecting the interstitium (the tissue and space around the air sacs of the lungs). It concerns alveolar epithelium, pulmonary capillary endothelium, basement membrane, perivascular and perilymphatic tissues. It may occur when an injury to the lungs triggers an abnormal healing response. Ordinarily, the body generates just the right amount of tissue to repair damage. But in interstitial lung disease, the repair process goes awry and the tissue around the air sacs (alveoli) becomes scarred and thickened. This makes it more difficult for oxygen to pass into the bloodstream. The term ILD is used to distinguish these diseases from obstructive airways diseases.
In children, several unique forms of ILD exist which are specific for the young age groups. The acronym chILD is used for this group of diseases and is derived from the English name, Children’s Interstitial Lung Diseases – chILD.
Prolonged ILD may result in pulmonary fibrosis, but this is not always the case. Idiopathic pulmonary fibrosis is interstitial lung disease for which no obvious cause can be identified (idiopathic), and is associated with typical findings both radiographic (basal and pleural based fibrosis with honeycombing) and pathologic (temporally and spatially heterogeneous fibrosis, histopathologic honeycombing and fibroblastic foci).
In 2013 interstitial lung disease affected 595,000 people globally. This resulted in 471,000 deaths.
Many cases of restrictive lung disease are idiopathic (have no known cause). Still, there is generally pulmonary fibrosis. Examples are:
- Idiopathic pulmonary fibrosis
- Idiopathic interstitial pneumonia, of which there are several types
- Sarcoidosis
- Eosinophilic pneumonia
- Lymphangioleiomyomatosis
- Pulmonary Langerhans' cell histiocytosis
- Pulmonary alveolar proteinosis
Conditions specifically affecting the interstitium are called interstitial lung diseases.
The exact cause of rheumatoid lung disease is unknown. However, associated factors could be due largely to smoking. Sometimes, the medicines used to treat rheumatoid arthritis, especially methotrexate, may result in lung disease.
Prevention's:
- Stop smoking: Chemicals found in cigarettes can irritate already delicate lung tissue, leading to further complications.
- Having regular checkups: The doctor could listen to lungs and monitor breathing, because lung problems that are detected early can be easier to treat.
Tobacco smoking is by far the main contributor to lung cancer. Cigarette smoke contains at least 73 known carcinogens, including benzo["a"]pyrene, NNK, 1,3-butadiene and a radioactive isotope of polonium, polonium-210. Across the developed world, 90% of lung cancer deaths in men during the year 2000 were attributed to smoking (70% for women). Smoking accounts for about 85% of lung cancer cases.
Passive smoking—the inhalation of smoke from another's smoking—is a cause of lung cancer in nonsmokers. A passive smoker can be defined as someone living or working with a smoker. Studies from the US, Europe and the UK have consistently shown a significantly increased risk among those exposed to passive smoke. Those who live with someone who smokes have a 20–30% increase in risk while those who work in an environment with secondhand smoke have a 16–19% increase in risk. Investigations of sidestream smoke suggest it is more dangerous than direct smoke. Passive smoking causes about 3,400 deaths from lung cancer each year in the USA.
Marijuana smoke contains many of the same carcinogens as those in tobacco smoke. However, the effect of smoking cannabis on lung cancer risk is not clear. A 2013 review did not find an increased risk from light to moderate use. A 2014 review found that smoking cannabis doubled the risk of lung cancer.
In most patients, the number and size of cherry angiomas increases with advancing age. They are harmless, having no relation to cancer at all.
Pulmonary diseases may also impact newborns, such as pulmonary hyperplasia, pulmonary interstitial emphysema (usually preterm births), and infant respiratory distress syndrome,
Restrictive lung diseases may be due to specific causes which can be intrinsic to the parenchyma of the lung, or extrinsic to it.
Typical pulmonary carcinoid tumour is a subtype of pulmonary carcinoid tumour. It is an uncommon low-grade malignant lung mass that is most often in the central airways of the lung.
It is also known as typical lung carcinoid tumour, lung carcinoid, and typical lung carcinoid.
Respiratory disease is a common and significant cause of illness and death around the world. In the US, approximately 1 billion "common colds" occur each year. A study found that in 2010, there were approximately 6.8 million emergency department visits for respiratory disorders in the U.S. for patients under the age of 18. In 2012, respiratory conditions were the most frequent reasons for hospital stays among children.
In the UK, approximately 1 in 7 individuals are affected by some form of chronic lung disease, most commonly chronic obstructive pulmonary disease, which includes asthma, chronic bronchitis and emphysema.
Respiratory diseases (including lung cancer) are responsible for over 10% of hospitalizations and over 16% of deaths in Canada.
In 2011, respiratory disease with ventilator support accounted for 93.3% of ICU utilization in the United States.
Microvenular hemangioma (also known as "Microcapillary hemangioma") is an acquired benign vascular neoplasm that presents as an asymptomatic, slowly growing, 0.5- to 2.0 cm reddish lesion on the forearms or other sites of young to middle-aged adults.
Conditions which commonly involve hemoptysis include bronchitis and pneumonia, lung cancers and tuberculosis. Other possible underlying causes include aspergilloma, bronchiectasis, coccidioidomycosis, pulmonary embolism, pneumonic plague, and cystic fibrosis. Rarer causes include hereditary hemorrhagic telangiectasia (HHT or Rendu-Osler-Weber syndrome), Goodpasture's syndrome, and granulomatosis with polyangiitis. In children, hemoptysis is commonly caused by the presence of a foreign body in the airway. The condition can also result from over-anticoagulation from treatment by drugs such as warfarin.
Blood-laced mucus from the sinus or nose area can sometimes be misidentified as symptomatic of hemoptysis (such secretions can be a sign of nasal or sinus cancer, but also a sinus infection). Extensive non-respiratory injury can also cause one to cough up blood. Cardiac causes like congestive heart failure and mitral stenosis should be ruled out.
The origin of blood can be identified by observing its color. Bright-red, foamy blood comes from the respiratory tract, whereas dark-red, coffee-colored blood comes from the gastrointestinal tract. Sometimes hemoptysis may be rust-colored.
The most common cause of minor hemoptysis is bronchitis.
- Lung cancer, including both non-small cell lung carcinoma and small cell lung carcinoma.
- Sarcoidosis
- Aspergilloma
- Tuberculosis
- Histoplasmosis
- Pneumonia
- Pulmonary edema
- Pulmonary embolism
- Foreign body aspiration and aspiration pneumonia
- Goodpasture's syndrome
- Granulomatosis with polyangiitis
- Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)
- Bronchitis
- Bronchiectasis
- Pulmonary embolism
- Anticoagulant use
- Trauma
- Lung abscess
- Mitral stenosis
- Tropical eosinophilia
- Bleeding disorders
- Hughes-Stovin Syndrome and other variants of Behçet's disease
- Squamous Cell Carcinoma Of Esophagus
Asbestosis is a chronic lung disease caused by scarring of lung tissue, which results from prolonged exposure to asbestos. It is defined as diffuse interstitial pulmonary fibrosis secondary to asbestos exposure. It initially affects the lung bases and usually manifests after 15 or more years from initial exposure. It occurs after high intensity and/or long-term exposure to asbestos. Asbestos-related fibrosis is progressive because it continues to progress in the lung even if no further asbestos is inhaled. The scar tissue causes the alveolar walls to thicken, reducing the lung capacity which leads to the patient experiencing shortness of breath (dyspnea). Sufferers are at an increased risk for heart failure and certain malignancies.
The most common cause is post-surgical atelectasis, characterized by splinting, i.e. restricted breathing after abdominal surgery.
Another common cause is pulmonary tuberculosis. Smokers and the elderly are also at an increased risk. Outside of this context, atelectasis implies some blockage of a bronchiole or bronchus, which can be within the airway (foreign body, mucus plug), from the wall (tumor, usually squamous cell carcinoma) or compressing from the outside (tumor, lymph node, tubercle). Another cause is poor surfactant spreading during inspiration, causing the surface tension to be at its highest which tends to collapse smaller alveoli. Atelectasis may also occur during suction, as along with sputum, air is withdrawn from the lungs. There are several types of atelectasis according to their underlying mechanisms or the distribution of alveolar collapse; resorption, compression, microatelectasis and contraction atelectasis.
Pulmonary alveolar proteinosis (PAP) is a rare lung disease in which an abnormal accumulation of pulmonary surfactant occurs within the alveoli (microscopic air sacs in the lung), interfering with the lungs' ability to exchange oxygen from the air, and carbon dioxide from the blood. PAP can occur in a primary form or secondarily in the settings of certain cancers (such as myeloid leukemia), lung infections, or environmental exposure to dusts or chemicals. Rare familial forms have also been recognized, suggesting a genetic component in those cases.
The disease is more common in males and in tobacco smokers.
In a recent epidemiologic study from Japan, Autoimmune PAP has an incidence and prevalence higher than previously reported and is not strongly linked to smoking, occupational exposure, or other illnesses.
Endogenous lipoid pneumonia and non-specific interstitial pneumonitis has been seen prior to the development of PAP in a child.
A tufted angioma (also known as an "Acquired tufted angioma," "Angioblastoma," "Angioblastoma of Nakagawa," "Hypertrophic hemangioma," "Progressive capillary hemangioma," and "Tufted hemangioma") usually develops in infancy or early childhood on the neck and upper trunk, and is an ill-defined, dull red macule with a mottled appearance, varying from 2 to 5 cm in diameter.