Rheumatoid arthritis-associated interstitial lung disease

Abstract

Rheumatoid arthritis (RA) is a systemic inflammatory disorder affecting 1% of the US population. Patients can have extra-articular manifestations of their disease and the lungs are commonly involved. RA can affect any compartment of the respiratory system and high resolution computed tomography (HRCT) of the lung is abnormal in over half of these patients. Interstitial lung disease is a dreaded complication of RA. It is more prevalent in smokers, males, and those with high antibody titers. The pathogenesis is unknown but data suggest an environmental insult in the setting of a genetic predisposition. Smoking may play a role in the pathogenesis of disease through citrullination of protein in the lung leading to the development of autoimmunity. Patients usually present in middle age with cough and dyspnea. Pulmonary function testing most commonly shows reduced diffusion capacity for carbon monoxide and HRCT reveals a combination of reticulation and ground glass abnormalities. The most common pattern on HRCT and histopathology is usual interstitial pneumonia (UIP), with nonspecific interstitial pneumonia seen less frequently. There are no large-scale well-controlled treatment trials. In severe or progressive cases, treatment usually consists of corticosteroids with or without a cytotoxic agent for 6 months or longer. RA interstitial lung disease is progressive; over half of patients show radiographic progression within 2 years. Patients with a UIP pattern on biopsy have a survival similar to idiopathic pulmonary fibrosis.

Keywords: anti-CCP; interstitial lung disease; nonspecific interstitial pneumonia; rheumatoid arthritis; usual interstitial pneumonia.

Figure 1

High resolution computed tomography (HRCT) of RA-ILD with an NSIP pattern. An HRCT of a patient with RA showing peripheral reticulation and ground glass abnormalities with no notable honeycombing. Abbreviations: RA, rheumatoid arthritis; ILD, interstitial lung disease; NSIP, nonspecific interstitial pneumonia.

Figure 2

High resolution computed tomography (HRCT) of RA-ILD with a UIP pattern. An HRCT of a patient with RA showing reticulation and significant honeycombing in a basilar and peripheral distribution with an absence of significant ground glass abnormality. Abbreviations: RA, rheumatoid arthritis; ILD, interstitial lung disease; UIP, usual interstitial pneumonia.

Figure 3

Histopathology in a patient with RA-ILD associated NSIP. A surgical biopsy specimen in a patient with RA showing cellular interstitial infiltrates and fibrosis in a temporally uniform distribution. Abbreviations: RA, rheumatoid arthritis; ILD, interstitial lung disease; NSIP, nonspecific interstitial pneumonia.

Figure 4

Histopathology in a patient with RA-ILD associated UIP. A surgical biopsy specimen in a patient with RA showing patchy interstitial fibrosis that starts in the peripheral acinar regions and is in close proximity to unaffected lung tissue. Abbreviations: RA, rheumatoid arthritis; ILD, interstitial lung disease; UIP, usual interstitial pneumonia.