Lupus: Support and Survival
Pulmonary (Lung) Involvement
Pleuritis / Pleurisy
Chronic diffuse interstitial lung disease
BOOP = Bronchiolitis Obliterans Organizing Pneumonia
Lupus can involve the lung in many ways. Pleuritis (pleurisy) is the most common pulmonary manifestation of SLE. The pleura is a membrane covering the outside of the lung and the inside of the chest cavity. It produces a small amount of fluid to lubricate the space between the lung and the chest wall. When this membrane is attacked by autoantibodies and becomes inflamed, it is called pleuritis.
Sometimes, an excess amount of fluid can accumulate in the pleural space. This is called a pleural effusion and occurs less often than pleuritis. If the effusion is large enough, it can be seen on a chest x-ray. Since pleural effusions can be caused by infection or conditions other than lupus, the physician may need to take a sample of the fluid and perform tests to determine the cause. Symptoms of pleuritis include severe, often sharp, stabbing pain that may be pin-pointed to a specific area or areas of the chest. The pain is often made worse by taking a deep breath, coughing, sneezing or laughing. Analgesics, non-steroidal anti-inflammatory drugs, and/or corticosteroids may be used to treat pleuritis. Pleural effusions will usually respond to these medications or clear by themselves with time.
Pneumonitis is inflammation within the lung tissue, which may be caused by an infection or by lupus. Infection is the most common cause of pneumonitis in people with lupus. Bacteria, viruses, fungi, or protozoa are organisms that can cause infection in the lung. Sometimes pneumonitis may occur without infection and is then called non-infectious pneumonitis. Since both forms of pneumonitis have the same symptoms; fever, chest pain, shortness of breath and cough, the patient is assumed to have an infection until proven otherwise. The diagnosis of pneumonitis requires blood tests, sputum tests and x-rays. Bronchoscopy and/or lung biopsy may also be necessary to determine if infection is the cause of the pneumonitis.
Treatment of pneumonitis initially includes a course of antibiotics. If laboratory and other diagnostic tests show no proof of infection, then the diagnosis is likely lupus pneumonitis. This non-infectious pneumonitis is treated with high doses of corticosteroids. Immunosuppressive drugs such as azathioprine (Imuran) may be added if the inflammation is not controlled with steroids.
Chronic diffuse interstitial lung disease is a relatively uncommon disorder in SLE. It is a chronic form of lupus pneumonitis and affects a relatively small number of people. The symptoms include a gradual onset of a chronic, non-productive cough; pleuritis-like chest pains; and difficulty breathing during physical activity. Diagnosis requires the exclusion of infection as a possible cause. Besides lupus, there are other causes of chronic diffuse interstitial lung disease. To determine the cause, special procedures such as bronchoscopy (visual inspection of the inside of the lungs) and/or lung biopsy are required. Correct identification of the cause is required to accurately select the proper treatment. Chronic lupus pneumonitis scars the lung and decreases the lungs' ability to deliver oxygen to the blood. The scarred lung tissue acts as a barrier to the oxygen which normally moves easily (diffuses) from the lung into the blood.
The severity and activity of this chronic disease can be measured and followed with pulmonary function testing (breathing tests). The diffusion capacity of the lung is a measurement of how readily oxygen moves through the lung and into the blood stream. It is usually reduced in chronic lupus pneumonitis. Periodic measurements of the diffusion capacity can indicate the response to treatment and enable the physician to follow the course of the disease. Chronic lupus pneumonitis is primarily treated with corticosteroids and patients will often have a variable response. The course of the disease also varies; some patients may slowly improve, stabilize, or deteriorate over time.
Occasionally, people with lupus develop pulmonary hypertension or high blood pressure in the blood vessels within the lung. If severe, it can be life-threatening and there tends to be little chance for improvement. There is no successful medical treatment for pulmonary hypertension. Heart-lung transplants may be an option for some patients with pulmonary hypertension caused by SLE.
BOOP = Bronchiolitis Obliterans Organizing Pneumonia is a disease whereby plugs of connective tissue extend into the air sacs obstructing the airway. Most patients have a history suggestive of a slowly resolving viral pneumonia spanning weeks or a few months. The most common symptom is a persistent, nonproductive cough, with some patients reporting flu-like symptoms with a fever, sore throat, and fatigue. Shortness of breath is usually a significant symptom. On physical exam of the lungs, crackles or a “Velcro” sound may be present and wheezes rarely are present.
Early recognition and therapy of bronchiolitis obliterans is important, because treatment is often ineffective when the disease has reached the late, scarred stage. Inhaled medication to open up airways are usually given for smooth muscle contraction and symptomatic relief. Corticosteroids, if given early, may significantly alter the disease process. Corticosteroids are considered the treatment of choice for BOOP. Although spontaneous improvement has been noted in some patients treatment with corticosteroids for patients with moderate to severe BOOP is recommended.
Pulmonary involvement in lupus is not uncommon. Pleurisy and infection are the most common conditions involving the lung. The most common cause of pneumonitis in lupus patients is infection. Bacterial or viral pneumonitis are also common and all people with lupus who have a sudden onset of cough, fever or pleuritic chest pain should notify their physician.
Generally, the cardiopulmonary problems associated with lupus respond rapidly to treatment. But treatment must be tailored for each patient and problem. Again, the early and accurate diagnosis of problems and aggressive treatment to reduce potential organ damage are crucial to the successful management of cardiopulmonary disease in lupus.
Contact the Lupus Foundation of America or the local Chapter that serves your area for more information about lupus, or the programs and services the LFA offers including support group information and physician referral.
Lupus Foundation of America., Inc.
1300 Piccard Drive, Suite 200
Rockville, MD 20850-4303
Information gathered from :
Information on BOOP and Pulmonary topics gathered from :
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