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Non-specific Interstitial Pneumonia
Non-specific interstitial pneumonia (NSIP) is a form of idiopathic interstitial pneumonia. Symptoms Symptoms include cough, difficulty breathing, and fatigue. Causes It has been suggested that idiopathic nonspecific interstitial pneumonia has an autoimmune mechanism, and is a possible complication of undifferentiated connective tissue disease; however, not enough research has been done at this time to find a cause. Patients with NSIP will often have other unrelated lung diseases like COPD or emphysema, along with other auto-immune disorders. Diagnosis A full clinical diagnosis can only be made from a lung biopsy of the tissue, fully best performed by a VATS, done by a cardio-thoracic surgeon. Some pulmonologists may first attempt a bronchoscopy; however, this frequently fails to give a full or correct diagnosis. Lung biopsies performed on patients with NSIP reveal two different disease patterns – cellular and fibrosing – which are associated with different prognoses. The ce ...
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Idiopathic Interstitial Pneumonia
Idiopathic interstitial pneumonia (IIP), or noninfectious pneumonia 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. Diagnosis Classification can be complex, and the combined efforts of clinicians, radiologists, and pathologists can help in the generation of a more specific diagnosis. Idiopathic interstitial pneumonia can be subclassified based on histologic appearance into the following patterns:Leslie KO, Wick MR. Practical Pulmonary Pathology: A Diagnostic Approach. Elsevier Inc. 2005. . Usual interstitial pneumonia is the most common type. Development Table 1: Development of the (histologic) idiopathic interstitial pneumonia classification UIP=usual interstitial pneumonia; DAD=diffuse alveolar damage; NSIP=non-specific interstitial pneumonia; D ...
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Cough
A cough is a sudden expulsion of air through the large breathing passages that can help clear them of fluids, irritants, foreign particles and microbes. As a protective reflex, coughing can be repetitive with the cough reflex following three phases: an inhalation, a forced exhalation against a closed glottis, and a violent release of air from the lungs following opening of the glottis, usually accompanied by a distinctive sound. Frequent coughing usually indicates the presence of a disease. Many viruses and bacteria benefit, from an evolutionary perspective, by causing the host to cough, which helps to spread the disease to new hosts. Most of the time, irregular coughing is caused by a respiratory tract infection but can also be triggered by choking, smoking, air pollution, asthma, gastroesophageal reflux disease, post-nasal drip, chronic bronchitis, lung tumors, heart failure and medications such as angiotensin-converting-enzyme inhibitors (ACE inhibitors). Treatment sho ...
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Dyspnea
Shortness of breath (SOB), also medically known as dyspnea (in AmE) or dyspnoea (in BrE), is an uncomfortable feeling of not being able to breathe well enough. The American Thoracic Society defines it as "a subjective experience of breathing discomfort that consists of qualitatively distinct sensations that vary in intensity", and recommends evaluating dyspnea by assessing the intensity of its distinct sensations, the degree of distress and discomfort involved, and its burden or impact on the patient's activities of daily living. Distinct sensations include effort/work to breathe, chest tightness or pain, and "air hunger" (the feeling of not enough oxygen). The tripod position is often assumed to be a sign. Dyspnea is a normal symptom of heavy physical exertion but becomes pathological if it occurs in unexpected situations, when resting or during light exertion. In 85% of cases it is due to asthma, pneumonia, cardiac ischemia, interstitial lung disease, congestive heart fai ...
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Fatigue (medical)
Fatigue describes a state of tiredness that does not resolve with rest or sleep. In general usage, fatigue is synonymous with extreme tiredness or exhaustion that normally follows prolonged physical or mental activity. When it does not resolve after rest or sleep, or occurs independently of physical or mental exertion, it may be a symptom of a medical condition that may become severe or progressive. Fatigue can be a feature of a mental disorder such as depression; may be associated with conditions of chronic pain such as fibromyalgia; it may also feature in conditions of chronic low-level inflammation, and be a disease-related symptom in many other conditions. Fatigue often has no known cause, and is recognised as being very complex in nature. Fatigability describes a susceptibility to fatigue. Physical fatigue results from muscle fatigue brought about by intense physical activity. Mental fatigue results from prolonged periods of cognitive activity which impairs cognitive ab ...
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Undifferentiated Connective Tissue Disease
Undifferentiated connective tissue disease (UCTD) is a disease in which the body mistakenly attacks its own tissues. It is diagnosed when there is evidence of an existing autoimmune condition which does not meet the criteria for any specific autoimmune disease, such as systemic lupus erythematosus or scleroderma. Latent lupus and incomplete lupus are alternative terms that have been used to describe this condition. The term is sometimes used interchangeably with mixed connective tissue disease, an overlap syndrome. However, MCTD is thought by some researchers to be a clinically distinct entity and is strongly associated with the presence of high titers of ribonucleoprotein (RNP) antibodies. It is estimated that up to 25 percent of people with systemic autoimmune disease could be considered to have UCTD. Signs and symptoms Disease presentation varies widely from patient to patient, as UCTD is by definition nonspecific. Symptoms typically include constitutional complaints that a ...
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COPD
Chronic obstructive pulmonary disease (COPD) is a type of progressive lung disease characterized by long-term respiratory symptoms and airflow limitation. The main symptoms include shortness of breath and a cough, which may or may not produce mucus. COPD progressively worsens, with everyday activities such as walking or dressing becoming difficult. While COPD is incurable, it is preventable and treatable. The two most common conditions of COPD are emphysema and chronic bronchitis and they have been the two classic COPD phenotypes. Emphysema is defined as enlarged airspaces (alveoli) whose walls have broken down resulting in permanent damage to the lung tissue. Chronic bronchitis is defined as a productive cough that is present for at least three months each year for two years. Both of these conditions can exist without airflow limitation when they are not classed as COPD. Emphysema is just one of the structural abnormalities that can limit airflow and can exist without air ...
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Emphysema
Emphysema, or pulmonary emphysema, is a lower respiratory tract disease, characterised by air-filled spaces ( pneumatoses) in the lungs, that can vary in size and may be very large. The spaces are caused by the breakdown of the walls of the alveoli and they replace the spongy lung parenchyma. This reduces the total alveolar surface available for gas exchange leading to a reduction in oxygen supply for the blood. Emphysema usually affects the middle aged or older population because it takes time to develop with the effects of tobacco smoking, and other risk factors. Alpha-1 antitrypsin deficiency is a genetic risk factor that may lead to the condition presenting earlier. When associated with significant airflow limitation, emphysema is a major subtype of chronic obstructive pulmonary disease ( COPD), a progressive lung disease characterized by long-term breathing problems and poor airflow. Without COPD, the finding of emphysema on a CT lung scan still confers a higher mortal ...
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Auto-immune Disorder
An autoimmune disease is a condition arising from an abnormal immune response to a functioning body part. At least 80 types of autoimmune diseases have been identified, with some evidence suggesting that there may be more than 100 types. Nearly any body part can be involved. Common symptoms can be diverse and transient, ranging from mild to severe, and generally include low grade fever and feeling tired. The cause is unknown. Some autoimmune diseases such as lupus run in families, and certain cases may be triggered by infections or other environmental factors. Some common diseases that are generally considered autoimmune include celiac disease, diabetes mellitus type 1, graves' disease, inflammatory bowel disease, multiple sclerosis, alopecia areata, addison’s disease, pernicious anemia, psoriasis, rheumatoid arthritis, and systemic lupus erythematosus. The diagnosis can be difficult to determine. Treatment depends on the type and severity of the condition. Nonsteroida ...
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Lung Biopsy
A lung biopsy is an interventional procedure performed to diagnose lung pathology by obtaining a small piece of lung which is examined under a microscope. Beyond microscopic examination for cellular morphology and architecture, special stains and cultures can be performed on the tissue obtained. Types A lung biopsy can be performed percutaneously (through the skin, typically guided by a CT Scan), via bronchoscopy with ultrasound guidance, or by surgery, either open or by video-assisted thoracoscopic surgery (VATS). Reasons to perform A lung biopsy is performed when a lung lesion is suspicious for lung cancer, or when cancer cannot be distinguished from another disease, such as aspergillosis. Lung biopsy also plays a role in the diagnosis of interstitial lung disease. Risks Any approach to lung biopsy risks causing a pneumothorax. Careful technique can limit this risk, which ranges from less than 1% to about 10%. The precise risk of pneumothorax depends on technique and on ...
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Bronchoscopy
Bronchoscopy is an endoscopic technique of visualizing the inside of the airways for diagnostic and therapeutic purposes. An instrument (bronchoscope) is inserted into the airways, usually through the nose or mouth, or occasionally through a tracheostomy. This allows the practitioner to examine the patient's airways for abnormalities such as foreign bodies, bleeding, tumors, or inflammation. Specimens may be taken from inside the lungs. The construction of bronchoscopes ranges from rigid metal tubes with attached lighting devices to flexible optical fiber instruments with realtime video equipment. History The German laryngologist Gustav Killian is attributed with performing the first bronchoscopy in 1897. Killian used a rigid bronchoscope to remove a pork bone. The procedure was done in an awake patient using topical cocaine as a local anesthetic. From this time until the 1970s, rigid bronchoscopes were used exclusively. Chevalier Jackson, refined the rigid bronchoscope in the ...
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Prednisone
Prednisone is a glucocorticoid medication mostly used to suppress the immune system and decrease inflammation in conditions such as asthma, COPD, and rheumatologic diseases. It is also used to treat high blood calcium due to cancer and adrenal insufficiency along with other steroids. It is taken by mouth. Common side effects with long-term use include cataracts, bone loss, easy bruising, muscle weakness, and thrush. Other side effects include weight gain, swelling, high blood sugar, increased risk of infection, and psychosis. It is generally considered safe in pregnancy and low doses appear to be safe when breastfeeding. After prolonged use, prednisone needs to be stopped gradually. Prednisone is a prodrug and must be converted to prednisolone by the liver before it becomes active. Prednisolone then binds to glucocorticoid receptors, activating them and triggering changes in gene expression. Prednisone was patented in 1954 and approved for medical use in the Unite ...
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Imuran
Azathioprine (AZA), sold under the brand name Imuran, among others, is an immunosuppressive medication. It is used in rheumatoid arthritis, granulomatosis with polyangiitis, Crohn's disease, ulcerative colitis, and systemic lupus erythematosus, and in kidney transplants to prevent rejection. It is listed by the International Agency for Research on Cancer as a group 1 carcinogen (carcinogenic to humans). It is taken by mouth or injected into a vein. Common side effects include bone-marrow suppression and vomiting. Bone-marrow suppression is especially common in people with a genetic deficiency of the enzyme thiopurine S-methyltransferase. Other serious risk factors include an increased risk of certain cancers. Use during pregnancy may result in harm to the baby. Azathioprine is in the purine analogue and antimetabolite family of medications. It works via 6-thioguanine to disrupt the making of RNA and DNA by cells. Azathioprine was first made in 1957. It is on the World ...
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