Respiratory Complications and Lupus

Roughly half of the people with lupus will experience some sort of lung complication at some time in their lives.¹ Symptoms range from very mild to severe.² Coughing and chest pains when taking a deep breath are often the first signs that lupus may be affecting the lungs.

A study in the United Kingdom reported that 12 percent of its patients experienced some type of permanent lung damage 10 years after being diagnosed with lupus.²

What are the most common lung complications in lupus?

The five most common lung issues associated with lupus are:

Pleuritis (pleurisy or pleural effusion)

Pleurisy is an inflammation of the pleura, the sac that surrounds the lungs. This puts pressure on the lungs, resulting in pain when taking deep breaths.

Acute lupus pneumonitis

Acute lupus pneumonitis is a serious condition that impacts up to 10 percent of those with lupus. Signs of this complication include sudden chest pain, shortness of breath, and a dry cough that brings up blood. Pneumonitis requires quick treatment because it can cause lung scarring.

Chronic (fibrotic) lupus pneumonitis

Chronic (fibrotic) lupus pneumonitis is a slower version of acute lupus pneumonitis. The acute form comes on quickly, while the chronic form gradually gets worse and causes scarring over the years. People with the chronic form often do not notice or complain of symptoms until the lung scarring is permanent. Early treatment helps prevent long-term damage to the lungs.

Pulmonary hypertension

Pulmonary hypertension is a type of high blood pressure that occurs in the blood vessel that carries blood from the heart to the lungs. This condition reduces the amount of oxygenated blood that can move through the lungs, causing the heart to work harder and harder. It occurs in about 10 percent of people with lupus.

Pulmonary hemorrhage or diffuse alveolar hemorrhage

Pulmonary hemorrhage or diffuse alveolar hemorrhage (DAH) is a rare complication that can be life-threatening. Signs include shortness of breath and coughing up blood. Treatment involves high doses of immunosuppressants.

“Shrinking lung” syndrome

Shrinking lung syndrome is rare and usually occurs only in late-stage lupus. Signs include breathlessness, chest pain, and an inability to breathe deeply.^1,5

How are lung complications of lupus diagnosed?

If you are coughing or feel pain in the chest when breathing deeply, your doctor may suspect lung complications. However, since infections are also common in people with lupus, your doctor will also want to rule out a bacterial or viral infection.³

People with lupus are at higher risk of developing pulmonary embolism or blood clots in the lungs which can have some of the same symptoms.

Tests commonly used to diagnose heart and lung conditions include blood work, chest x-ray, echocardiogram, electrocardiogram (ECG), right heart catheterization, magnetic resonance imaging (MRI), computerized tomography (CT) scan, pulmonary function test, bronchoscopy, ventilation/perfusion (V/Q) scan and lung biopsy. The type of test your doctor orders will depend on the type of lung complication you may have.⁴

How are lung complications of lupus treated?

The type of treatment you will need depends on what sort of lung complication you have.

Pleurisy is treated with NSAIDs (pain relievers that reduce inflammation) or steroids. Antimalarials may help prevent pleurisy.¹

Acute lupus pneumonitis is treated with high doses of steroids, and sometimes immunosuppressants. Chronic lupus pneumonitis is treated with lower doses of steroids.¹

If caused by active lupus, pulmonary hypertension is treated with steroids and immunosuppressants. However, most pulmonary hypertension must be treated one or more of the following type of drugs: vasodilator, endothelin receptor antagonist, sildenafil, tadalafil, high-dose calcium channel blocker, soluble guanylate cyclase (SGC) stimulator, anticoagulant, digoxin, diuretic, or oxygen.⁴

Shrinking lung syndrome is treated with steroids and immunosuppressants.⁵

Compared to other autoimmune diseases, lung complications in people with lupus tend to be milder and are more often successfully treated.²