Lupus and Pleurisy and Other Lung Conditions

How Systemic Lupus Erythematosis (SLE) Affects the Lungs

X-ray image of lungs with infectious pleurisy
Lupus and Pleurisy. BSIP/UIG / Getty Images

Lupus is an autoimmune condition which can cause pleurisy, but is implicated in several other lung conditions as well. What are the symptoms, how are they diagnosed, and what treatments are available for lupus-associated pleurisy? What other lung diseases occur more commonly among people with lupus?

Systemic Lupus Erythematosis (SLE) or Lupus -

Systemic lupus erythematosus (SLE), an autoimmune disease, is truly a disease of symptoms.

It's attacks on the body’s immune system and can affect everything from the skin and joints to internal organs.

Lupus is the most common connective tissue disease to have pulmonary (lung) symptoms. Most people with lupus develop some lung conditions, and these are important in the prognosis of the disease.

Lupus and Pleurisy

Pleurisy (pleuritic chest pain) is the most common pulmonary (lung) manifestation of lupus. Pleurisy is a descriptive diagnosis which refers to pain in the chest that occurs during breathing (sometimes only during a deep breath or during coughing.)

Underlying this pain is inflammation of the two membranes which line the lungs (the pleura.) The pleura are separated by a small cavity referred to as the pleural cavity, which ordinarily contains three to four teaspoons of pleural fluid. Together, the pleura act as a cushion, reducing friction on the lungs when you breathe in and out.

With lupus, autoantibodies (antibodies directed at your own tissues) target the pleura, creating inflammation. This inflammation can lead to pain that occurs when you take a breath or let it out - anything which causes the pleura to move. Sometimes the inflammation is mild and discomfort only occurs with a deep breath, coughing, sneezing, or with a forced expiration.

At other times the inflammation may be more severe, resulting in pain with even normal quiet breathing.

As a another complication of this process, inflammation may result in a build-up in fluid between the pleura. The resultant pleural effusion may vary in symptoms from none (if only a small amount of excess fluid is present) to severe shortness of breath and pain with a large pleural effusion which compresses the lungs.

Let's take a careful look at the symptoms, diagnosis, and treatment of pleurisy, followed by a brief review of other lung diseases which may be associated with lupus.

Symptoms of Pleurisy with Lupus

Symptoms of pleurisy with lupus may include:

  • Pain with breathing, coughing, or movement of the chest (pleuritic chest pain)
  • Pain in the shoulder (due to inflammation of nerves to the shoulder which pass through the diaphragm
  • Shortness of breath (when a pleural effusion is present, or due to avoiding deep breaths due to discomfort)
  • Rapid respiratory rate
  • Cyanosis (blueness of the skin from oxygen deprivation) - Cyanosis only occurs when a large amount of pleural fluid accumulates

Diagnosis of Pleurisy Related to Lupus

The diagnosis of pleurisy related to lupus is usually made by using a combination of a careful history and physical, plus lab tests and imaging studies.

A history of lupus and pleuritic type chest pain may be all that is needed to make the diagnosis when symptoms are mild. The pleura is smooth, but when affected by pleuritis, it becomes rough, rubs together, and often produces a rough, grating sound. This sound is audible through a stethoscope, and sometimes by simply putting an ear to the chest.

Since lupus is associated with other lung conditions as well, other tests are often done. Some of these may include:

  • Blood work - Blood tests for lupus (if these have not already been done)
  • A complete blood count - A CBC can help rule out other causes of pleurisy such as bacterial or viral infections.
  • A chest x-ray or CT scan.
  • If a pleural effusion is present, thoracentesis is often done. In this procedure, a fine needle is inserted through the chest into the pleural space to withdraw fluid. In addition to reducing the size of a pleural effusion (if shortness of breath is present), this fluid can be analyzed in the lab to rule out other possible causes of an effusion.

Treatment of Pleurisy Related to Lupus

There isn't usually a specific treatment for pleurisy related to pleurisy, unless a bacterial or viral infection is responsible instead of autoimmune-induced inflammation. Since the autoimmune nature of lupus is responsible for symptoms most of the time, whatever treatments are being used to treat lupus in general will be directed at the pleurisy as well. Pleurisy caused by lupus must usually run its course in time, and pain can be managed with anti-inflammatory drugs until it resolves.

Other Ways in Which Lupus May Affect Your Lungs

Some type of lung condition affects up to 50 percent of people with lupus. It can do so by affecting many different parts and structures of the lungs including the:

  • Pleura (as in pleurisy discussed above)
  • Lung tissue itself (the connective tissue within the lungs)
  • Alveoli (the small air sacs in the lungs responsible for the exchange of oxygen and carbon dioxide)
  • Bronchi and bronchioles (the large and small airways of the lungs)
  • Blood vessels in the lungs
  • Diaphragm (as in shrinking lung syndrome discussed below)

Lung Related Medical Conditions Associated with Lupus

In addition to pleurisy, there are other lung diseases which occur more commonly in people with lupus. Many of these conditions have symptoms of a cough, shortness of breath, and sometimes coughing up blood, and therefore a careful evaluation is needed to determine the precise cause. Lung conditions (in addition to pleurisy) which are associated with lupus include:

  • Lupus pneumonitis (both acute and chronic) - Lupus pneumonitis refers to inflammation of lung tissue. When is is acute it can cause a cough, shortness of breath, and pain with breathing. Steroids are usually used, and high doses may be required.
  • Pulmonary hypertension - Pulmonary hypertension is a condition in which the blood pressure inside the pulmonary artery (the artery which travels from the right ventricle of the heart to the lungs) is elevated, and occurs in roughly 10 percent of people with lupus.
  • Shrinking lung syndrome - Damage and inflammation of the diaphragm may result in a condition referred to as shrinking lung syndrome. This uncommon condition results in elevation of the diaphragm, restricting the area of lung tissue which can be used for gas exchange.
  • Interstitial lung diseases such as pulmonary fibrosis - Interstitial diseases are restrictive lung diseases (in contrast to conditions such as asthma and emphysema which are obstructive lung diseases.) The inflammation followed by scarring of the lungs prevents the lungs from fully expanding with a breath, limiting the function of the lungs.
  • Other lung conditions - Conditions such as pulmonary embolism, pneumothorax, recurrent pleural effusion, and asthma are also more common in people with lupus In addition to these condition, the risk of lung cancer, especially squamous cell carcinoma of the lungs, is higher in people who have been diagnosed with lupus.

Bottom Line on Pleurisy and Other Lung Diseases with Lupus

Lung diseases, as noted, are common with lupus, affecting more than half of people with lupus at some time. The most common condition is pleurisy, an inflammation of the membranes lining the lungs due to the inflammatory process which is widespread in lupus. While there is no specific treatment, it's important to make sure one of the other lung diseases commonly found in people with lupus is not present. Measures to reduce pain, such as medications, controlling cough, and avoiding strenuous exercise which leads to deep breathing, can help people feel more comfortable until the disease runs its course.

Sources:

Borrell, H., Narvaez, J., Alegre, J. et al. Shrinking Lung Syndrome in Systemic Lupus Erythematosus: A Case Series and Review of the Literature. Medicine (Baltimore). 2016. 95(33):e4626.

Mittoo, S., and C. Fell. Pulmonary Manifestations of Systemic Lupus Erythematosis. Seminars in Respiratory and Critical Care Medicine. 2014. 35(2):249-54.

Rosenberger, A., Sohns, M., Friedrichs, S. et al. Gene-Set Meta-Analysis of Lung Cancer Identifies Pathway Related to Systemic Lupus Erythematosus. PLoS One. 2017. 12(3):e0173339.

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