About 50% of people with SLE will experience lung involvement during the course of their disease. Five main lung problems occur in lupus: pleuritis, acute lupus pneumonitis, chronic (fibrotic) lupus pneumonitis, pulmonary hypertension, and “shrinking lung” syndrome.
Pleuritis, also known as pleurisy, is characterized by inflammation of the pleura, the two-layered membrane surrounding the lungs. If fluid forms and escapes the membrane, the condition is referred to as pleural effusion. Since inflammation of the pleura puts pressure on the lungs, people with pleuritis often experience pain when taking deep breaths. A pleural effusion is detectable on an x-ray, and pleurisy or a pleural effusion can be treated with NSAIDs or steroid medications. Antimalarials like Plaquenil may also be effective in preventing or fending off the condition.
Acute Lupus Pneumonitis
Acute lupus pneumonitis is a serious condition that affects anywhere from 1-10% of lupus patients. The condition is characterized by chest pain, shortness of breath, and a dry cough that may bring up blood. Acute lupus pneumonitis requires immediate treatment with high dose steroids and possibly immunosuppressive medications. Yet, even with successful treatment, some people develop lung scarring. Thus, it is very important that you speak with your physician if you experience any unfamiliar lung pains or symptoms.
Chronic (Fibrotic) Lupus Pneumonitis
As the name suggests, chronic lupus pneumonitis is the creeping cousin of acute lupus pneumonitis. Whereas the acute form comes on quickly, chronic lupus pneumonitis accumulates subtly over years. Some people with chronic lupus pneumonitis may have had acute lupus pneumonitis as some point during their lives, while others have not. Often the disease takes so long to develop that people do not realize or complain of the initial symptoms. While current inflammation can be treated with steroid medications, any scarring that occurs over time is permanent. Thus, it is very important that you remember to tell your doctor about any chest, lung, or breathing pain during regular visits. If caught early, chronic lupus pneumonitis can be treated.
In order for the organs in your body to work, they must receive oxygen. The right side of your heart is responsible for pumping blood from your veins back to your lungs to receive oxygen; the oxygenated blood then returns to the left side of the heart, where it is pumped to the rest of the body for use. Pulmonary hypertension is a unique form of hypertension (high blood pressure) in which the blood vessel that carries blood from the heart to the lungs thickens, reducing the amount of blood that can move through it in a given amount of time. In order to counter this resistance, your heart must pump harder and faster. When the heart fails to keep up, the rest of the body suffers by receiving less and less oxygen (a condition called hypoxia).
Pulmonary hypertension occurs in about 10% of people with lupus. On rare occasions pulmonary hypertension responds to corticosteroids and immunosuppressive treatment. However, if it is not reversible (meaning, if it is not due to active lupus), then your doctor can discuss with you one of the FDA-approved medications for pulmonary hypertension.
“Shrinking Lung” Syndrome
“Shrinking lung” syndrome is another rare lupus complication. People with the condition feel a sense of breathlessness and reduced chest expansion. Some people with shrinking lung syndrome improve with corticosteroid or immunosuppressive therapy.
- D’Cruz, D, Khamashta, MA, Hughes, G. Pulmonary Manifestations of Systemic Lupus Erythematosus. In: Wallace, DJ, Hahn, BH, eds. Dubois’ Lupus Erythematosus. 7th ed. Philadelphia, Pa: Lippincott Williams & Wilkins; 2007:678-699.
- Medsger, Thomas A. “Pulmonary Manifestations.” The Clinical Management of Systemic Lupus Erythematosus. Ed. Peter H. Schur. 2nd ed. Philadelphia: Lippincott-Raven, 1996. 87-94.
- “Pulmonary hypertension.” American Heart Association. 1 July 2009. <http://www.americanheart.org/presenter.jhtml?identifier=11076>.
- Wallace, Daniel J. “Pants and Pulses: The Lungs and Heart.” The Lupus Book: A Guide for Patients and Their Families. 1st ed. New York: Oxford University Press, 1995. 81-98.