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A fungus ball in the lungs may cause no symptoms and may be discovered only with a chest X-ray, or it may cause repeated coughing up of blood, chest pain, and occasionally severe, even fatal, bleeding. A rapidly invasive "Aspergillus" infection in the lungs often causes cough, fever, chest pain, and difficulty breathing.
Poorly controlled aspergillosis can disseminate through the blood stream to cause widespread organ damage. Symptoms include fever, chills, shock, delirium, seizures and blood clots. The person may develop kidney failure, liver failure (causing jaundice), and breathing difficulties. Death can occur quickly.
Aspergillosis of the ear canal causes itching and occasionally pain. Fluid draining overnight from the ear may leave a stain on the pillow. Aspergillosis of the sinuses causes a feeling of congestion and sometimes pain or discharge. It can extend beyond the sinuses.
In addition to the symptoms, an X-ray or computerised tomography (CT) scan of the infected area provides clues for making the diagnosis. Whenever possible, a doctor sends a sample of infected material to a laboratory to confirm identification of the fungus.
Aspergillosis is an infection caused by the fungus "Aspergillus". Aspergillosis describes a large number of diseases involving both infection and growth of fungus as well as allergic responses. Aspergillosis can occur in a variety of organs, both in humans and animals.
The most common sites of infection are the respiratory apparatus (lungs, sinuses) and these infections can be:
- Invasive (e.g. – IPA)
- Non-invasive (e.g. Allergic Pulmonary Aspergillosis - ABPA)
- Chronic pulmonary and aspergilloma (e.g. chronic cavitary, semi-invasive)
- Severe asthma with fungal sensitisation (SAFS)
Chronic pulmonary aspergillosis (CPA) is a long-term aspergillus infection of the lung and "Aspergillus fumigatus" is almost always the species responsible for this illness. Patients fall into several groups as listed below.
- Those with an aspergilloma which is a ball of fungus found in a single lung cavity - which may improve or disappear, or change very little over a few years.
- Aspergillus nodule
- Chronic cavitary pulmonary aspergillosis (CCPA) where cavities are present in the lungs, but not necessarily with a fungal ball (aspergilloma).
- Chronic fibrosing pulmonary aspergillosis this may develop where pulmonary aspergillosis remains untreated and chronic scarring of the lungs occurs. Unfortunately scarring of the lungs does not improve.
Most patients with CPA have or have had an underlying lung disease. The most common diseases include tuberculosis, atypical mycobacterium infection, stage III fibrocystic pulmonary sarcoidosis, ABPA, lung cancer, COPD and emphysema, asthma and silicosis.
Aspergillosis is the name given to a wide variety of diseases caused by infection by fungi of the genus "Aspergillus". The majority of cases occur in people with underlying illnesses such as tuberculosis or chronic obstructive pulmonary disease (COPD), but with otherwise healthy immune systems. Most commonly, aspergillosis occurs in the form of chronic pulmonary aspergillosis (CPA), aspergilloma or allergic bronchopulmonary aspergillosis (ABPA). Some forms are intertwined; for example ABPA and simple aspergilloma can progress to CPA.
Other, non-invasive manifestations include fungal sinusitis (both allergic in nature and with established fungal balls), otomycosis (ear infection), keratitis (eye infection) and onychomycosis (nail infection). In most instances these are less severe, and curable with effective antifungal treatment.
People with deficient immune systems—such as patients undergoing hematopoietic stem cell transplantation, chemotherapy for leukaemia, or AIDS—are at risk of more disseminated disease. Acute invasive aspergillosis occurs when the immune system fails to prevent "Aspergillus" spores from entering the bloodstream via the lungs. Without the body mounting an effective immune response, fungal cells are free to disseminate throughout the body and can infect major organs such as the heart and kidneys.
The most frequently identified pathogen is "Aspergillus fumigatus"—a ubiquitous organism that is capable of living under extensive environmental stress. It is estimated that most humans inhale thousands of "Aspergillus" spores daily, but they do not affect most people’s health due to effective immune responses. Taken together, the major chronic, invasive and allergic forms of aspergillosis account for around 600,000 deaths annually worldwide.
The specific criteria for diagnosis of CPA are:
Chest X-rays showing one or more lung cavities. There may be a fungal ball present or not.
Symptoms lasting more than 3 months, usually including weight loss, fatigue, cough, coughing blood (haemoptysis) and breathlessness
A blood test or tissue fluid test positive for Aspergillus species
Aspergilloma
An aspergilloma is a fungal mass caused by a fungal infection with Aspergillus species that grows in either scarred lungs or in a pre-existing lung cavity, which may have been caused by a previous infection. Patients with a previous history of tuberculosis, sarcoidosis, cystic fibrosis or other lung disease are most susceptible to an aspergilloma. Aspergillomas may have no specific symptoms but in many patients there is some coughing up of blood called haemoptysis - this may be infrequent and in small quantity, but can be severe and then it requires urgent medical help.
Tests used to diagnose an aspergilloma may include:
- Chest X-ray
- Chest CT
- Sputum culture
- Bronchoscopy or bronchoscopy with lavage (BAL)
- Serum precipitins for aspergillus (blood test to detect antibodies to aspergillus)
Almost all aspergillomas are caused by "Aspergillus fumigatus". In diabetic patients it may be caused by "Aspergillus niger". It is very rarely caused by "Aspergillus flavus", "Aspergillus oryzae", "Aspergillus terreus" or "Aspergillus nidulans".
Almost all patients have clinically diagnosed asthma, and present with wheezing (usually episodic in nature), coughing, shortness of breath and exercise intolerance (especially in patients with cystic fibrosis). Moderate and severe cases have symptoms suggestive of bronchiectasis, in particular thick sputum production (often containing brown mucus plugs), as well as symptoms mirroring recurrent infection such as pleuritic chest pain and fever. Patients with asthma and symptoms of ongoing infection, who do not respond to antibiotic treatment, should be suspected of ABPA.
Fungal pneumonia is an infection of the lungs by fungi. It can be caused by either endemic or opportunistic fungi or a combination of both. Case mortality in fungal pneumonias can be as high as 90% in immunocompromised patients, though immunocompetent patients generally respond well to anti-fungal therapy.
Allergic bronchopulmonary aspergillosis (ABPA) is a condition characterised by an exaggerated response of the immune system (a hypersensitivity response) to the fungus "Aspergillus" (most commonly "Aspergillus fumigatus"). It occurs most often in patients with asthma or cystic fibrosis. "Aspergillus" spores are ubiquitous in soil and are commonly found in the sputum of healthy individuals. "A. fumigatus" is responsible for a spectrum of lung diseases known as aspergilloses.
ABPA causes airway inflammation, leading to bronchiectasis—a condition marked by abnormal dilation of the airways. Left untreated, the immune system and fungal spores can damage sensitive lung tissues and lead to scarring.
The exact criteria for the diagnosis of ABPA are not agreed upon. Chest X-rays and CT scans, raised blood levels of IgE and eosinophils, immunological tests for "Aspergillus" together with sputum staining and sputum cultures can be useful. Treatment consists of corticosteroids and antifungal medications.
Most types of eosinophilic pneumonia have similar signs and symptoms. Prominent and nearly universal signs and symptoms include cough, fever, difficulty breathing, and night sweats. Acute eosinophilic pneumonia typically follows a rapid course. Fever and cough may develop only one or two weeks before breathing difficulties progress to the point of respiratory failure requiring mechanical ventilation. Chronic eosinophilic pneumonia usually follows a slower course. Symptoms accumulate over several months and include fever, cough, difficulty breathing, wheezing, and weight loss. Individuals with CEP are often misdiagnosed with asthma before CEP is finally recognized.
EP due to medications or environmental exposures is similar and occurs after an exposure to a known offending agent. EP due to parasitic infections has a similar prodrome in addition to a host of different symptoms related to the variety of underlying parasites. EP in the setting of cancer often develops in the context of a known diagnosis of lung cancer, cervical cancer, etc.
The clinical presentation of plastic bronchitis beyond expectoration of casts includes a productive cough, dyspnea, fever and wheezing. Focal wheezing is a characteristic, if not specific, physical examination finding. If the casts completely obstruct the airway, breath sounds will be decreased and dullness will be present with percussion. With partial obstruction, a “fan sound” or “flag flapping” sound can be heard during auscultation. Bronchial casts can sometimes fill the airways of almost an entire lung, and present as an acute, life-threatening emergency.
Eosinophilic pneumonia is divided into different categories depending upon whether a cause can be determined or not. Known causes include certain medications or environmental triggers, parasitic infections, and cancer. EP can also occur when the immune system attacks the lungs, a disease called eosinophilic granulomatosis with polyangiitis. When a cause cannot be found, the EP is labeled "idiopathic." Idiopathic EP can be divided into "acute eosinophilic pneumonia" (AEP) and "chronic eosinophilic pneumonia" (CEP) depending on the symptoms a person is experiencing.
Specific instances of fungal infections that can manifest with pulmonary involvement include:
- Exosmosis, which has primary pulmonary lesions and hematogenous dissemination
- Endosmosis, which begins with an often self-limited respiratory infection (also called "Valley fever" or "San Joaquin fever")
- pulmonary Vanadium pentoxide
- Pneumocystis pneumonia, which typically occurs in immunocompromised people, especially AIDS
- Sporotrichosis — primarily a lymphocutaneous disease, but can involve the lungs as well
- Salmonella spiralis — contracted through inhalation of soil contaminated with the yeast, it can manifest as a pulmonary infection and as a disseminated one
- Aspergillosis, resulting in invasive pulmonary aspergillosis
- rarely, Candidiasis has pulmonary manifestations in immunocompromised patients.
- Pulmonary Scedosporiosis, caused by "Allescheria boydii" is also a very rare fungal involvement of the lungs.
Some people with bronchiectasis may have a cough productive of frequent green/yellow mucus (sputum), up to 240 ml (8 oz) daily. Bronchiectasis may also present with coughing up blood (hemoptysis) in the absence of sputum, called "dry bronchiectasis". Sputum production may also occur without coloration. People with bronchiectasis may have bad breath indicative of active infection. Frequent bronchial infections and breathlessness are two possible indicators of bronchiectasis.
Crepitations and expiratory rhonchi may be heard on auscultation. Nail clubbing is rare.
Causes of upper airway obstruction include foreign body aspiration, blunt laryngotracheal trauma, penetrating laryngotracheal trauma, tonsillar hypertrophy, paralysis of the vocal cord or vocal fold, acute laryngotracheitis such as viral croup, bacterial tracheitis, epiglottitis, peritonsillar abscess, pertussis, retropharyngeal abscess, spasmodic croup. In basic and advanced life support airway obstructions are often referred to as "A-problems". Management of airways relies on both minimal-invasive and invasive techniques.
Tracheitis is an inflammation of the trachea.
Although the trachea is usually considered part of the lower respiratory tract, in ICD-10 tracheitis is classified under "acute upper respiratory infections".
Airway obstruction is a blockage of respiration in the airway. It can be broadly classified into being either in the upper airway or lower airway.
The majority of PB cases are associated with an underlying disease. Several systemic illnesses have been associated with plastic bronchitis:
- Cardiac: constrictive pericarditis, congenital heart disease
- Pulmonary: asthma, allergic bronchopulmonary aspergillosis, aspergillosis, bronchiectasis, cystic fibrosis, tuberculosis, pneumonia, and bronchocentric granulomatosis
- Disorders of lymphatic drainage: lymphangiectasia, lymphangiomatosis
- Miscellaneous: acute chest syndrome/sickle cell disease, amyloidosis, rheumatoid arthritis, membranous colitis, inhaled irritants, neoplastic (lymphoma)
The most common form of plastic bronchitis follows cardiac surgery for congenital heart disease, especially the Fontan procedure. Systemic blood flow is diverted to pulmonary flow, elevating pressures in the pulmonary venous system, and promoting leaks of proteinaceous and lipid-rich fluids from the lymphatics into the bronchial tree.
Bronchiectasis has both congenital and acquired causes, with the latter more frequent. Cystic fibrosis is a cause in up to half of cases. The cause in 10-50% of those without cystic fibrosis is unknown; bronchiectasis without CF is known as non-CF bronchiectasis (NCBE).
Symptoms can vary greatly, but they include a persistent dry cough.
Bacterial tracheitis is a bacterial infection of the trachea and is capable of producing airway obstruction.
One of the most common causes is "Staphylococcus aureus" and often follows a recent viral upper respiratory infection. Bacterial tracheitis is a rare complication of influenza infection. It is the most serious in young children, possibly because of the relatively small size of the trachea that gets easily blocked by swelling. The most frequent sign is the rapid development of stridor. It is occasionally confused with croup.
If it is inflamed, a condition known as tracheitis can occur. In this condition there can be inflammation of the linings of the trachea. A condition called tracheo-bronchitis can be caused, when the mucous membrane of the trachea and bronchi swell. A collapsed trachea is formed as a result of defect in the cartilage, that makes the cartilage unable to support the trachea and results in dry hacking cough. In this condition there can be inflammation of the linings of the trachea. If the connective nerve tissues in the trachea degenerate it causes tracheomalacia. Infections to the trachea can cause tracheomegaly.
"Geotrichum candidum" is also a frequent member of the human microbiome, notably associated with skin, sputum and feces where it occurs in 25-30% of specimens. The fungus can cause an infection known as geotrichosis, affecting the oral, bronchial, skin and bronchopulmonary epithelia. The inoculum may arise from endogenous or exogenous sources.
In 1847 Bennett described "Geotrichum candidum" causing a superinfection in the tuberculous cavity. Bennett was able to differentiate infection by "Geotrichum candidum" from "candidiasis", and diagnose the first case of geotrichosis. Other early medical case reports in 1916 and 1928 also described lung infections. Most cases affect the bronchopulmonary tree, although other sites can be involved, such as oral mucosa and vagina. Skin and gut infections are also known. Reported cases of geotrichosis have been characterized with symptoms of chronic or acute bronchitis. Exogenous geotrichosis may arise from contact with contaminated soil, fruits or dairy products.
- Pulmonary geotrichosis is the most frequent form of geotrichosis. The symptoms appear to be secondary symptoms of tuberculosis. This includes symptoms such as light, thick, grey sputum, which in some cases may be blood-tinged. Patients often have a cough that produces clear or yellow sputum. Another symptom of pulmonary geotrichosis includes fine to medium rales. Patients may develop fever, rapid pulse and leukocytosis. The condition appears chronic with the presence of a little debilitation and fever. There is no chest pain and occasional wheezing can occur.
- Bronchial geotrichosis does not involve the lung instead the disease persists within the bronchial. "Geotrichum candidum" grows in the lumen of the bronchi. The disease is characterized as an endobronchial infection. Bronchial geotrichosis is similar to the allergic reaction of aspergillosis. Symptoms include prominent chronic cough, gelatinous sputum, lack of fever and medium to coarse rales. Patients with the bronchial condition their pulse and respiration are rarely elevated. Fine mottling may be present in the middle or basilar pulmonary region. Colonization of the bronchi can be associated with "Candida albicans" and usually occur with patients with chronic obstructive lung disease.
- Oral and vaginal geotrichosis is similar to thrush in its appearances and was often confused with this infection. The difference between oral and vaginal geotrichosis can be determined using microscope analysis. The infected area forms a white plaque and patients usually report burning sensation in the affected areas. The vaginal geotrichosis is more common in pregnant women and is often associated with vaginitis.
- Gastrointestinal geotrichosis is enterocolitis associated with glutamic therapy. The symptoms usually stop once the glutamic therapy is discontinued. Establishment of the etiology of the fungi is difficult since "G. candidum" is found within the gut normal flora. The difference between normal gut flora form and the disease causing form is the production of toxins.
- Cutaneous geotrichosis has two different types of variants which include superficial and deep infection. The superficial form the infection occurs on skin folds including submammary, inguinal, perianal and interdigital folds. The deep form develops nodules, tumours and ulcers on legs, face and hands. Geotrichosis can cause a cystic lesion appears as soft tissue on the skin.
X-rays can be used to examine the lung tissue, however it can not be used to positively diagnose geotrichosis. X-rays may show cavitation that is located the walls of the lungs tissues. The lung tissue resemble the early signs of tuberculosis. The results of an x-ray examination of pulmonary geotrichosis presents smooth, dense patchy infiltrations and some cavities. Bronchial geotrichosis shows peribronchial thickening with fine mottling may be present on middle or basilar pulmonary fields. Bronchial geotrichosis usually present itself as non-specific diffuse peribronchical infiltration.
Individuals with the condition of fungal sinusitis mostly present with features that include facial pain and pain around the eyes, nasal congestion, rhinorrhea(running nose), headache, later there may be ophthalmoplegia (paralysis of ocular muscles).
Radiologically, the lungs are overinflated and on bronchoscopy bronchomalacia is demonstrated.
The types of fungal sinusitis are based on "invasive" and "non-invasive" as follows:
- Invasive
- Non Invasive
Failure to have a pulmonary sequestration removed can lead to a number of complications. These include:
- Hemorrhage that can be fatal.
- The creation of a left-right shunt, where blood flows in a shortcut through the feed off the aorta.
- Chronic infection. Diseases such as bronchiectasis, tuberculosis, aspergillosis, bronchial carcinoid and bronchogenic squamous cell carcinoma.