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The lung in autoimmune rheumatic disorders 

The lung in autoimmune rheumatic disorders

The lung in autoimmune rheumatic disorders

A.U. Wells

and H.R. Branley

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date: 23 April 2017

Lung complications occur in all rheumatological disorders, but their frequency and type vary strikingly between different systemic diseases. Greater routine use of high-resolution CT and echocardiography means that interstitial lung disease and pulmonary vascular disease are increasingly recognized, which can create clinical difficulty in distinguishing between subclinical involvement and significant disease.

Particular autoimmune disorders

Systemic sclerosis—pulmonary function is abnormal in up to 90% of cases. Lung disease usually consists of nonspecific interstitial pneumonia (NSIP) and is present on chest radiography at some stage in most patients. Both isolated pulmonary vascular disease and secondary pulmonary hypertension occur. Lung cancer is increased in prevalence. Lung and pulmonary vascular disease are now the main cause of morbidity and mortality.

Polymyositis/dermatomyositis—interstitial lung disease, usually with organizing pneumonia or NSIP, is the commonest pulmonary complication. Aspiration pneumonia is a frequent feature of advanced disease and a common cause of death.

Rheumatoid arthritis—is associated with a wide range of pleuropulmonary complications including interstitial lung disease (with usual interstitial pneumonia and NSIP of equal prevalence), organizing pneumonia, bronchiolitis obliterans, bronchiectasis, pleural effusion, pulmonary vasculitis (rarely), and pulmonary rheumatoid nodules.

Sjögren’s syndrome—interstitial lung disease takes the form of fibrotic NSIP or lymphocytic interstitial pneumonia. Tracheobronchial disease can be in the form of loss of mucus secretion in the trachea (xerotrachea), bronchi and bronchioles, or (less frequently) lymphocytic bronchiolitis.

Systemic lupus erythematosus—clinically significant interstitial lung disease affects about 10% of patients, with NSIP the usual form. Acute lupus pneumonitis is an uncommon life-threatening disorder. Diffuse alveolar hemorrhage due to capillaritis can occur. The ‘shrinking lung syndrome’ is thought to be due to respiratory muscle weakness. Pulmonary hypertension is increasing recognized. Pleural disease is common, affecting 50% of patients at some time.


Is treatment required on account of lung disease?—it is critical that high-resolution CT findings and lung function tests are reconciled, with clear definition of all complications and deconstruction of the functional defect. Most clinicians regard DLco levels below 65% of predicted normal as indicative of clinically significant disease. Maximal exercise testing is often useful in marginal cases, when careful monitoring with regular repetition of pulmonary function tests is wise.

Introduction of treatment for lung disease—the threshold for introducing therapy is reduced by three considerations: (1) the risk of progression of lung disease appears to be greatest early in the course of systemic disease; (2) severe functional impairment has consistently been associated with a higher mortality because it is indicative of a previously progressive course and an increased likelihood of future disease progression, also because loss of pulmonary reserve implies that the symptomatic consequences of a further preventable loss of lung function may be substantial; and (3) observed disease progression is a major indication for treatment.

Therapeutic options—when inflammatory disease predominates, as in organizing pneumonia or lymphocytic interstitial pneumonia, it is appropriate to treat with high-dose steroid therapy, or with intense immunosuppressive therapy in refractory cases. Treatment decisions are less straightforward in predominantly fibrotic disease.

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