Elsevier

Clinics in Chest Medicine

Volume 31, Issue 3, September 2010, Pages 451-478
Clinics in Chest Medicine

Pulmonary Manifestations of Rheumatoid Arthritis

https://doi.org/10.1016/j.ccm.2010.04.003Get rights and content

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Epidemiology

The association between interstitial lung disease and RA was first described in 1948.10 Additional reports of RA patients with ILD, many presenting with cough, dyspnea, and chest radiograph abnormalities, supported this association.10, 11, 12 Early series, using chest radiograph alone, found fewer than 5% of patients with RA to have RA-associated ILD (RA-ILD).13 Subsequent studies using measurements of pulmonary function, particularly diffusing capacity of the lung for carbon monoxide (DLCO),

Epidemiology

Pleural involvement in RA includes pleurisy, pleural effusions, pleural thickening, and pneumothorax.85, 86, 87, 88 Micronodular involvement of the pleura has been described and is one cause of pleural effusions in RA.89 Other potential causes include (1) prior infection, (2) local production of immune complexes, (3) chronic inflammation with cytokine release and activation of fibrosis, (4) impaired resorption of fluid due to its high protein content, and (5) the rupture of subpleural nodules

Upper Airway Disease

Upper airway involvement by RA can result in both disabling chronic disease and life-threatening airway emergencies. Close attention to the larynx should be paid, particularly in the perioperative setting. The most common form of upper airway RA is cricoarytenoid arthritis.

The cricoarytenoid joints function to abduct and adduct the vocal cords during speech. RA involving these joints was first described in 1955.131 Early studies estimated an incidence of 26%.132 In a group of 22 unselected RA

Rheumatoid nodules

Rheumatoid nodules, also called necrobiotic nodules, are common in patients with RA. Subcutaneous nodules are detected in approximately 20% of patients, although some reports estimate a frequency as high as 53%.177, 178, 179 Rheumatoid nodules are more common in males than in females and are closely associated with RF seropositivity.178 Most nodules are clinically asymptomatic, but subcutaneous nodules on pressure points or adherent to periosteum or tendons may become painful.177 Nodules

Drug-induced lung disease

Many of the drugs used to treat RA can cause pulmonary toxicity, but diagnosis of drug-induced lung disease in RA is complex. Distinguishing diagnostically between infection, drug reaction, and underlying RA-ILD can be problematic. The clinical features, pathology, and temporal correlation with drug initiation may be useful, but the diagnosis often remains in question. The manifestations of drug-induced lung disease are comprehensively reviewed in the article by Meyer and colleagues elsewhere

Infections

Patients with RA are at increased risk for infections, which are often severe.238, 239, 240 The sites of highest risk for infection in RA are the joints, bone (osteomyelitis), and soft tissue.241 The lung is also a common site for infection, with RA patients having nearly twice the rate of pneumonia as the general population.241 Some portion of the increased mortality rates in RA is directly attributable to serious infections.242, 243 It is unclear how much of the increased frequency and

Pulmonary vascular disease

Diffuse alveolar hemorrhage (DAH) due to pulmonary capillaritis has been described in association with RA but is extremely rare.263 Some milder cases of alveolar hemorrhage may go unrecognized because presenting signs and symptoms are nonspecific. Affected patients may present with shortness of breath, cough, and fever.264 Hemoptysis may also be present. HRCT features include diffuse ground-glass opacities and alveolar opacities, which are relatively nonspecific findings and can appear similar

Malignancy

Many patients with RA are middle aged, a typical time of onset for cancer, making the diagnosis of a malignancy in a patient with RA relatively common.268 Beyond this baseline risk, patients with RA appear to have increased risk for both lung cancer and lymphoma.196, 269 Whether this elevated risk is related to smoking history, chronic inflammation due to RA itself, RA therapy, or increased detection of malignancy due to higher interaction with the medical system is not clear.270, 271 In

Summary

Pleuropulmonary involvement in RA is common. Although pulmonary disease is often asymptomatic, progressive disease is an important cause of morbidity and mortality in RA. The diagnostic assessment of respiratory abnormalities is complicated by underlying risk for infection, the use of drugs with known pulmonary toxicity, and the frequency of lung disease related to RA itself. Nevertheless, thorough evaluation should be undertaken because early intervention may be life-saving.

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      While a prospective cohort would have been ideal, considering the prevalence and incidence of ILD in this population and the fact that it is not clear at what moment in the evolution of RA ILD develops, a prospective cohort study is difficult to conduct. In this scenario, we believe a case–control study is an acceptable initial approach to build a risk indicator score.2 Our definition of the absence of ILD in control patients may not have enough sensitivity as any ILD below the level of detection with a CXR would have been misclassified.

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