Treatment of Ilds and Assessment

Interstitial lung disease is a blanket term for a big group of disorders categorized by progressive scarring of both the lung tissue supporting and between the air sacs. This tissue is called the interstitium. The interstitium consists of the region between the alveolar space and the capillaries. The scarring causes inflammation and damage in the lung tissue followed by lung stiffness, meaning the air sacs cannot expand as much as before. Lung stiffness makes it harder to breathe. People affected by the condition are not able to get enough oxygen from the lungs into their bloodstream.

Although some potential causes have been researched, there may be no underlying cause for development of interstitial lung disease. If there is no known cause, it is called idiopathic interstitial lung disease. IPF or idiopathic pulmonary fibrosis remains the most common type of this illness. Of the causes most widely recognized, cigarette smoking is said to potentially make the disease more severe and the progression and onset more rapid. Although there are treatments, once lung scarring begins there is no means of reversing the damage.

Symptoms vary, but generally include a dry cough as well as shortness of breath either from after exertion or at rest. Other symptoms are often restricted to the respiratory tract. The condition is long-term lasting years or for some, can be lifelong. People most affected by the disorder is anyone over the age of 19 with many experiencing it after the age of 60. While it is a rare disorder, there are 200,000 cases reported annually in the United States. When people with a disease that falls within this category undergo physical examination, they will typically have bilateral inspiratory fine crackles. These are seen clearly seen at the lung bases. What should not be expected when examining is expiratory wheezing as it is relatively uncommon. If any skin rashes or joint deformities are present, this may signal underlying connective tissue disease.

Causes

There are various causes for interstitial lung disease. Some people develop it just from injuring the lungs and healing abnormally. Other triggers include autoimmune diseases, certain medications, exposure to inorganic and organic agents that may happen at home or in the workplace, and some kinds of radiation. When it comes to environmental and occupational factors, certain inorganic and organic materials may cause appearance of interstitial lung disease after long-term exposure.

Such materials are:

1. Asbestos fibers

2. Coal dust

3. Grain dust

4. Silica dust

5. Mold from shower, previous water damage, and hot tubs

6. Bird protein

The list of medications and radiation that could damage the lungs are:

1. Heart medications (Inderal, Innopran, Inderide, Cordarone, Nexterone, Pacerone)

2. Some antibiotics (sulfasalazine and nitrofurantoin)

3. Chemotherapy/immunomodulating drugs (cyclophosphamide and methotrexate)

4. Radiation for breast or lung cancer can lead to damage in the lungs months, even years after the first treatment.

Cigarette smoke can cause lung damage and as previously stated, may accelerate the progression of the lung damage. All these potential causes may not be what causes interstitial lung disease in every patient. Some do not have any known cause.

Some risk factors that may attribute to interstitial lung disease include family history due to some forms of it being heritable, age, degree of exposure to environmental and occupational toxins, smoking, and radiation. Although children and infants are not likely to develop interstitial lung disease, it can happen, but it is very rare. The main cause seems to be exposure to toxins and many cases from the 200,000 diagnosed each year point to these irritants/toxins as being the cause for disease development (Fischer & du Bois, 2012).

Assessment

Identifying ILDs in patients can be difficult. Early detection is frequently missed and diagnosis comes after serious progression of the disease. This is due to the symptoms being ascribed to more typical diseases like COPD while in the primary care setting. Furthermore, a pulmonologist would need to see the patient to detect whether the symptoms match interstitial lung disease.

Initial assessment begins with a structure history as well as physical examination. The history should include information on where the person worked, and what kind of exposure the person had to any toxins. Most often, a surgical lung biopsy may be performed to confirm diagnosis.

However, HRCT or high resolution computer tomography chest scans are in use and act as an essential in diagnosis of ILDs. Recent times has brought about the use of new technology such as transthoracic ultrasound. "The usefulness of transthoracic ultrasound in the evaluation of lung diseases has been highlighted in the past decades. Transthoracic ultrasound of the lung might be a sensitive non-invasive tool to observe early stage interstitial lung disease in rheumatic diseases" (Moazedi-Fuerst et al., 2015, p. S87). This tool is not only sensitive, but also non-invasive and can observe early stages of interstitial lung disease within patients exhibiting rheumatic diseases. This is a clear step in the right direction towards assessing ILDs early on without the need for a surgical biopsy.

In terms of getting the details of a differential diagnosis, acute signs are initial hypersensitivity reactions, eosinophilic pneumonia, infection, and cryptogenic organizing pneumonia. Chronic signs are IPF, Silica/asbestos related, and chronic HP. Those with advanced versions of the disease may show signs of heart failure. These are just some signs that doctors (pulmonologists) look for to determine if a patient has interstitial lung disease.

Treatment

A clear sign of interstitial lung disease is inflammation. This and/or fibrosis of the aforementioned interstitial space increases the likelihood of gas exchange abnormalities that result in dyspnoea. In the past, doctors have used immunosuppression as the main form of treatment. However, recent innovation brought on by the latest results from clinical studies and research suggest combined use of azathioprine and prednisolone is harmful for patients suffering from idiopathic pulmonary fibrosis. Therefore, some licensing has been implemented for a new anti-fibrotic drug named pirfenidone. This drug is what some consider an evolution in treatment and may help those suffering from ILDs.