Dr. Kaner began his presentation by speaking about the challenges physicians face when diagnosing pulmonary issues in people with lupus. There are a large number of different disease processes associated with lupus, which can also cause pulmonary issues, as well as impact treatment. For these reasons, patients with lupus can be among the most complex patients that pulmonologists see.
In his presentation, Dr. Kaner emphasized the importance of partnering with your rheumatologist and pulmonologist to arrive at an accurate diagnosis.
When people present with a re-occurring cough, only 50% of the time does it have to do with your lungs. Dr. Kaner spoke with the group about how the lung itself, is not often a cause of specific symptoms, like coughing. The cough, he conveyed, is usually due to the upper nasal track, which can cause a cough as well as other symptoms.
In order to discuss the types of lung problems that someone with SLE may experience, a description of the lung is necessary. The trachea, or windpipe, sits in the back of the throat, and divides into the lungs. The trachea is the beginning of a branching airway formation, Dr. Kaner explained, that then continue to branch out within each lung, growing smaller and smaller until they end. At the end, there is an air-exchanging cavity made up of many alveoli, where blood vessels meet with capillaries. On the surface of the alveoli is a thin membrane which allows the exchange of oxygen with blood vessels outside of the lung. This is how oxygen gets into your blood.
The most common disease symptoms related to the lung that SLE patients notice, Dr. Kaner explained, include:
Different approaches are administered by physicians to evaluate each symptom thoroughly. As a lupus patient, one should communicate with your rheumatologist or pulmonologist if you have any of these symptoms.
Dr. Kaner spoke with the group about the common forms of lung involvement that people with lupus may encounter. These categories include:
Pulmonologists approach chest pain by asking questions about where the pain is, how long the pain has been happening, what type of pain it is, and if it stays in one place. Pleuritic chest pain, or pain when you take a breath, is very common in people with SLE. This commonly happens with pain in the chest wall for SLE patients and always in the same location, which can be very easily diagnosed by doctors. This can be due to costochondritis, inflammation of the cartilage that connects the rib to the sternum, and happens more commonly in people who have lupus than the overall population. This, Dr. Kaner stated, can be “exquisitely painful,” but can usually be treated with moist heat and anti-inflammatory drugs.
Patients with SLE are more likely than most to experience upper respiratory tract infections, due to their immune system being suppressed (including from the use of Steroids or other immune-suppressing medications). These infections, however, can usually be quickly diagnosed with currently available technology. In most cases, Dr. Kaner explained, there is only a need for a nasal swab, which allows the microbiology lab to identify the cause. Due to the different causes, treatment for upper respiratory tract infections depends on what virus or bacteria is causing the infection.
Acute pneumonitis, known as inflammation and/or infection in the lung tissue, can be caused in two different ways.
1) Acute pneumonitis usually caused by a bacterial infection. This can be problematic for people with lupus because the medications that cause immune suppression, such as those used to treat lupus, increase the risk for bacterial infections. This is especially true for the bacteria that don’t generally make people sick but which can cause serious infection problems for those with lupus.
2) Patients with SLE can develop inflammation in their lung that is associated with their lupus. This should be treated with increased suppression of the immune system. Dr. Kaner stressed that, “in order to optimize your treatment with lupus, you really need an accurate diagnosis”. You must work with your doctor towards the correct diagnosis, as the treatments for each cause are entirely different.
Interstitial lung disease, which is chronic inflammation and scarring of the lung tissue, is generally not curable but it is treatable. This disease occurs when an infection or another substance stays in the alveoli, preventing the exchange of oxygen to the blood. If untreated and worsened, this can lead to a condition called pulmonary fibrosis, scarring of the lung tissue. When a person undergoes a chest CT scan, scarring can be visible within the lung tissue. As this scarring progresses, it can cause structurally misshapen parts, which can lead to additional problems. If the disease has advanced, cysts can form, which can block the view in x-rays making a diagnosis difficult.
Pulmonary Hypertension can be considered both a cardiovascular and pulmonary problem, because it involves both heart and lung systems. Much like acute pneumonitis, there are two ways in which pulmonary hypertension can develop with someone who has lupus.
1) Pulmonary hypertension involves an increased pressure in the blood vessels inside the lung. When blood goes into the lungs, Dr. Kaner explained, it first goes into the right ventricle, flowing into the pulmonary artery then into the smaller branches that reach the surface of the alveoli. Once the blood has been oxygenated, it then flows back into the left atrium, then to the left ventricle and then delivered to the rest of the body. When there is scarring in the lung, more pressure can be needed for the heart to push the blood from the right ventricle into the lung. This increased pressure is called pulmonary hypertension, related to disease of the lung tissue.
2) Another way for someone to develop pulmonary hypertension with SLE is when the disease directly affects the capillaries in the lung, causing them to no longer allow for maximum blood flow. This is called pulmonary artery hypertension. Dr. Kaner spent a great deal of time discussing this as he said “It is potentially fatal, but incredibly treatable.” Again, this reflects why it is important to have a correct diagnosis as it will determine the mode of treatment for someone with lupus and pulmonary hypertension.
Shrinking lung syndrome is completely unique to SLE, said Dr. Kaner. Shrinking lung syndrome is described as lung volume decreasing over time, resulting in smaller lungs. This reduction in size can then cause shortness of breath. Dr. Kaner emphasized that although this syndrome is very uncommon, but important to diagnose.
Pulmonary hemorrhage, or bleeding in the lung, is a very serious condition that can be life threatening. When there is bleeding in the lung, it eventually blocks the airways, causing one to try to cough it out. This is problematic because if there is too much bleeding and the patient is not able to cough it out fast enough, then the patient can lose the ability to get enough oxygen. Due to this risk, pulmonary hemorrhage is taken very seriously by pulmonologists. For some, as Dr. Kaner explained, the hemorrhage can be very deceptive, resulting in little coughing of blood or no coughing at all.
In pulmonary medicine, as Dr. Kaner discussed, the pulmonologist’s history and exam still accounts for 90% of the diagnosis.
To aid in diagnosis, there are often several diagnostic tests that may be recommended by the pulmonologist for someone with lupus. These tests are discussed below:
Pulmonary evaluation nearly always begins with a chest x-ray. For problems that are more subtle, if the doctor is not able to see what is causing the problem, a CT scan may be ordered. CT scans are done in cross sections of the body so the pulmonologist can view more specific parts of the lung more clearly.
Chest CT’s with intravenous contrast are often utilized in patients with SLE if they become suddenly short of breath and have chest pain, which could imply a pulmonary embolism. The Chest CT can allow for the doctor to quickly assess if there are any blood clots that have traveled to the lung. Additionally, for interstitial lung disease, a high-resolution chest CT can be performed for further evaluation and diagnosis. This allows for the pulmonologist to see the structure of the lung more clearly.
The pulmonary function test is completely non-invasive and can help your doctor follow your lung disease over time, which is very beneficial for people with SLE and lung issues. The most basic test, called Spirometry, measures how much the patient is able to breathe in and out and how fast they are able to do this. The patient exhales into a mouth piece connected to a machine. This can help to initially diagnose and later to monitor the progress of one’s asthma, Chronic Obstructive Pulmonary Disease (COPD), and pulmonary fibrosis. For diagnosis, the pulmonologist finds it useful to graph the flow and volume of the person’s breath so they can see if there is airflow obstruction, as can be seen with COPD with asthma.
The Lung Diffusion Capacity test is similar to Spirometry in that the patient exhales into a mouthpiece. The difference for the Lung Diffusion Capacity is that certain gases, such as a low concentration of carbon monoxide, are used to trace the amount of oxygen that is passed into the blood from the lungs. Through the readings of the carbon monoxide after it has been inhaled and exhaled, the pulmonologist can determine what area for oxygen exchange is available within the lung. This can help detect, diagnose, and measure how much surface area of the lungs are damaged, such as with emphysema or pulmonary fibrosis.
The 6-minute walk test is an evaluation for people who experience shortness of breath. This test involves having the patient walk down a hallway for 6 minutes with an oximeter on. This allows the pulmonologist to see how well the lung saturates the blood with oxygen. This, Dr. Kaner stated, is a very reliable way to look at one’s functional ability.
If the pulmonologist thinks infection may be causing your lung problem, as is a risk in lupus, a bronchoscopy may be advised. This test requires local anesthesia, and is helpful for the diagnosis in people with lupus especially in view of uncommon infections that may occur. The bronchoscopy allows pulmonologists to see into the airways of the lung, and to collect samples for culture and other tests.
The Right Heart Catheterization test is an invasive procedure that a pulmonologist may recommend to aid in the diagnosis of pulmonary hypertension. In this procedure a small catheter is guided into the right side of the heart and the artery going to the lung, measuring the blood pressure along the way.
Another invasive procedure that may be recommended for assistance in diagnosis with pulmonary hypertension or other lung problem is a surgical lung biopsy. This procedure is performed by a thoracic surgeon and is recommended under special circumstances by pulmonologists. The procedure requires the patient to be under general anesthesia. As Dr. Kaner explained, a Surgical Lung Biopsy consists of three holes made in your side, where the surgeon can easily biopsy areas of the lung to gain a better understanding of the cause of the lung problem.
Overall, Dr. Kaner conveyed, people with SLE must work with their rheumatologist and pulmonologist in order to come to the appropriate diagnosis. Afterwards, together with your rheumatologist, your pulmonologist can decide on an appropriate form of treatment.
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Robert J. Kaner, MD
Associate Attending Physician, NY Presbyterian Hospital
Associate Professor of Clinical Medicine, Weill Cornell Medical College
Associate Professor of Genetic Medicine, Weill Cornell Medical College
Medical Director, Ventilator Management Team, NY Presbyterian Hospital
James P. Smith, M.D. Clinical Scholar, Weill Cornell Medical College
Summary completed by Jill Orrock, Masters of Social Work intern and SLE Workshop Coordinator.