by Nathan Wei, MD, FACP, FACR
Nathan Wei is a nationally known board-certified rheumatologist and author of the Second Opinion Arthritis Treatment Kit. It's available exclusively at this website... not available in stores.
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Information in part from the American College of Rheumatology
Systemic lupus erythematosus (SLE) can affect multiple organ systems.
The lungs and their membranous lining (pleura) may be more frequently involved in lupus than in any other arthritic condition, occurring in forty to ninety percent of cases. Lung involvement may occur either as the presenting symptom of the disease or as a later manifestation after the diagnosis of lupus is well established.
Because the lungs contain an extensive connective tissue and blood supply, lupus may affect the lungs in multiple compartments. These include the airways (bronchi and bronchioles), the air sacs (alveoli), the blood vessels (arteries, capillaries and veins), the pleural lining and the diaphragm and other muscles of breathing.
Both upper and lower airways can develop inflammation, ranging from epiglottis to vocal cord edema and tracheal involvement. Inflammation of the large lower airways (bronchitis) is rare, although smaller lower airways are more often affected (bronchiolitis), with or without associated inflammation of air sacs (alveolitis). Symptoms of airway involvement include hoarseness, stridor (inspiratory whoop), shortness of breath and chest tightness.
In acute lupus pneumonitis, the alveoli and interstium are flooded with inflammatory cells and fluid, and in extreme cases with blood. Some cases progress to diffuse interstitial disease, in which inflammation leads to permanent scarring of lung tissue (fibrosis or honeycombing). Symptoms of pneumonitis vary in onset, but include shortness of breath, rapid shallow breathing, exertional shortness of breath, cough, cyanosis of the lips and fingers, coughing up of blood.
Infectious pneumonia commonly affects lupus patients. This predilection for infection is due to the immunosuppression caused by lupus itself, as well as the medications used to treat it, most commonly corticosteroids. Symptoms of pneumonia and pneumonitis often overlap, although the former more often involves a productive cough. For this reason, when diagnosing acute or chronic pneumonitis, a physician must first exclude infection by obtaining sputum samples for culture.
In lupus, blood clotting abnormalities leading to thromboembolism is common and can affect up to twenty-five percent of patients. Clots can develop suddenly or insidiously and lead to destruction of lung tissue, pulmonary hypertension, and right-sided heart failure. Blood clots in lupus are associated with the presence of antiphospholipid antibodies (lupus anticoagulant and anti-cardiolipin). Symptoms of venous thromboembolism include pain on deep breathing (pleurisy), coughing up blood (hemoptysis), shortness of breath, rapid shallow breathing and fever.
The pleural membranes that line the other surface of lungs and the inner walls of the chest cavity are a prime site of inflammation in lupus. Pleural disease occurs in over ninety percent of patients, although only fifty percent are symptomatic with pleurisy or fever. The pleural space fills with fluid, in which antinuclear and anti- DNA antibodies may be found. Even the muscles lining the chest cavity, including the diaphragm, may be inflamed and weakened in lupus. This results in the “shrinking lung syndrome,” in which the lungs literally lose volume over time, leading to shortness of breath and reduced exercise capacity.
Detection of the variety of lupus-related lung diseases relies on traditional diagnostic studies including chest x-rays, CT-scans and lung perfusions scans, and sampling of sputum and pleural fluid for chemical and microbiologic testing. Treatment varies with the specific disease, with corticosteroids and other immunosuppressives playing a dominant role. Anti-coagulants, non-steroidal drugs and theophylline play specific roles in thromboembolism, pleural disease and diaphragmatic dysfunction respectively. The prognosis of lupus lung disease has improved over the last decade, particularly for the more life threatening conditions such as pulmonary hemorrhage and venous thromboembolism.
Another potential cause of pneumonia in SLE is drug therapy including methotrexate and cyclophosphamide.
1: Nihon Kokyuki Gakkai Zasshi. 1998 Jul;36(7):638-43.
[SLE with interstitial pneumonia during cyclophosphamide pulse therapy]
[Article in Japanese]
Kawamoto S, Kuroda H, Saito M, Azuma Y, Masuoka S, Watanabe S, Ikeda M, Yano H, Kobayashi M.
Department of General Internal Medicine, Jikei University Hospital Kashiwa, Chiba, Japan.
A 49 year-old man was admitted for edema and renal impairment due to SLE. Since he did not improve with predonisolone and methylprednisolone pulse therapy, cyclophosphamide pulse therapy (300 mg div.) was administered. The patient subsequently developed a fever, dyspnea and cough, and interstitial regions of the lungs exhibited shadows on X-ray and CT. The patient also suffered hypoxemia and poor lung function. Since several culture tests and viral antibody tests were negative for infection, antibiotics were not effective, and TBLB indicated interstitial pneumonia, which we speculated was induced by cyclophosphamide. However, this was such a severe case of interstitial pneumonia that it could not be cured merely by discontinuing the cyclophosphamide, but it did improve immediately after starting methylprednisolone pulse therapy. The incidence of cyclophosphamide-induced interstitial pneumonia is very low, but the mortality rate is high. Since cyclophosphamide pulse therapy is often used to treat SLE, attention should be focused on the incidence of interstitial pneumonia.
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