Rheumatoid arthritis (pulmonary manifestations)

Changed by Andrew Murphy, 7 Feb 2023
Disclosures - updated 4 Sep 2022: Nothing to disclose

Updates to Article Attributes

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Definition: Rheumatoid lung is a type of interstitial lung disease (ILD) that occurs as a complication of rheumatoid arthritis (RA). It is characterised by progressive fibrosis and nodular infiltration of the lungs.

Terminology: Rheumatoid lung disease is also referred to as rheumatoid pneumonitis, rheumatoid arthritis-associated interstitial lung disease (RA-ILD), and rheumatoid pleuritis.

Pulmonary manifestations are relatively common in rheumatoid arthritis, and like many of its non-articular manifestations, tend to develop later in the disease.

Please refer to the related articles for a general discussion of rheumatoid arthritis, and for the specific discussion of its musculoskeletal manifestations.

Epidemiology

Although rheumatoid arthritis is more common in women, respiratory disease more commonly develops in men 6.

Clinical presentation

Respiratory symptoms are often absent, or non-specific such as dyspnoea, and chronic cough and chest pain.

Pathology

Pathophysiology: Rheumatoid lung is thought to be a result of chronic inflammation secondary to RA, which causes fibrotic changes in the lung tissue. The lung damage is thought to be caused by a combination of direct lung injury and systemic factors, including systemic inflammation and the release of cytokines.

Gross anatomical pathology: Gross examination of rheumatoid lung may reveal a nodular pattern of infiltration and fibrotic changes in the lung parenchyma.

Associations

Radiographic features

Patterns of lung involvement include interstitial, airway and pleural disease.

Pleural involvement is a common manifestation of rheumatoid arthritis, although usually asymptomatic. 

  • pleural thickening: is seen more commonly than pleural effusions

  • pleural effusions: occur late in the disease, are often unilateral and associated with pericarditis and subcutaneous nodules 5

Other manifestations include:

Plain radiograph

On chest radiographs, rheumatoid lungChest radiograph may present as diffuse interstitial infiltration with reticulation, nodules, and cystic changes. The findings may resemble other forms of ILD.show: 

On chest radiographs, rheumatoid lung necrobiotic nodules may appear as well-defined, round or oval opacities with smooth margins. They may be associated with the presence of pleural effusions, interstitial changes, or fibrotic bands.

CT

High-resolution computed tomography (HRCT) is the preferred imaging modality for the evaluation of rheumatoid lung.

HRCT findings may include diffuse interstitial infiltrates, reticulation, nodules, and cystic changes. The HRCT findings are consistent with fibrotic changes in the lung parenchyma.

Chest CT or HRCT features include:

Treatment and Prognosis:

Treatment for rheumatoid lung typically involves aggressive management of RA, including the use of disease-modifying antirheumatic drugs (DMARDs) and biologic agents. Corticosteroids may be used in severe cases. The prognosis for patients with rheumatoid lung is variable and depends on the severity of lung involvement, the degree of fibrosis, and the response to treatment.

History and etymology:

Rheumatoid lung disease was first described in the early 20th century. The term "rheumatoid lung" is derived from the underlying autoimmune condition, rheumatoid arthritis.

The term "necrobiotic" refers to the process of necrosis, or tissue death, and is used to describe the findings seen in these lung nodules. The term "rheumatoid lung nodules" reflects the association between these nodules and rheumatoid arthritis.

Differential diagnosis:

Differential diagnoses for rheumatoid lung include other forms of ILD, such as idiopathic pulmonary fibrosis, sarcoidosis, and lymphangitic carcinomatosis. The differential diagnosis should also consider infections, such as tuberculosis, and neoplastic diseases, such as lung cancer.

See See also

  • -<p><strong>Definition</strong>: Rheumatoid lung is a type of interstitial lung disease (ILD) that occurs as a complication of rheumatoid arthritis (RA). It is characterised by progressive fibrosis and nodular infiltration of the lungs.</p><p><strong>Terminology</strong>: Rheumatoid lung disease is also referred to as rheumatoid pneumonitis, rheumatoid arthritis-associated interstitial lung disease (RA-ILD), and rheumatoid pleuritis.</p><p><strong>Pulmonary manifestations </strong>are relatively common in<strong> rheumatoid arthritis,</strong> and like many of its non-articular manifestations, tend to develop later in the disease.</p><p>Please refer to the related articles for a <a href="/articles/rheumatoid-arthritis">general discussion of rheumatoid arthritis</a>, and for the specific discussion of its <a href="/articles/rheumatoid-arthritis-musculoskeletal-manifestations-2">musculoskeletal manifestations</a>.</p><h4>Epidemiology</h4><p>Although rheumatoid arthritis is more common in women, respiratory disease more commonly develops in men <sup>6</sup>.</p><h4>Clinical presentation</h4><p>Respiratory symptoms are often absent, or non-specific such as dyspnoea, chronic cough and chest pain.</p><h4>Pathology</h4><p><strong>Pathophysiology</strong>: Rheumatoid lung is thought to be a result of chronic inflammation secondary to RA, which causes fibrotic changes in the lung tissue. The lung damage is thought to be caused by a combination of direct lung injury and systemic factors, including systemic inflammation and the release of cytokines.</p><p><strong>Gross anatomical pathology</strong>: Gross examination of rheumatoid lung may reveal a nodular pattern of infiltration and fibrotic changes in the lung parenchyma.</p><h5>Associations</h5><ul><li><p><a href="/articles/bronchocentric-granulomatosis">bronchocentric granulomatosis</a>: rare <sup>7</sup></p></li></ul><h4>Radiographic features</h4><p>Patterns of lung involvement include interstitial, airway and pleural disease.</p><p>Pleural involvement is a common manifestation of rheumatoid arthritis, although usually asymptomatic. </p><ul>
  • -<li><p><a href="/articles/pleural-thickening">pleural thickening</a>: is seen more commonly than pleural effusions</p></li>
  • -<li><p><a href="/articles/pleural-effusion">pleural effusions</a>: occur late in the disease, are often unilateral and associated with pericarditis and subcutaneous nodules <sup>5</sup></p></li>
  • +<p><strong>Pulmonary manifestations </strong>are relatively common in<strong> rheumatoid arthritis,</strong> and like many of its non-articular manifestations, tend to develop later in the disease.</p><p>Please refer to the related articles for a <a href="/articles/rheumatoid-arthritis">general discussion of rheumatoid arthritis</a>, and for the specific discussion of its <a href="/articles/rheumatoid-arthritis-musculoskeletal-manifestations-2">musculoskeletal manifestations</a>.</p><h4>Epidemiology</h4><p>Although rheumatoid arthritis is more common in women, respiratory disease more commonly develops in men <sup>6</sup>.</p><h4>Clinical presentation</h4><p>Respiratory symptoms are often absent, or non-specific such as dyspnoea and chronic cough. </p><h4>Pathology</h4><h5>Associations</h5><ul><li>
  • +<a href="/articles/bronchocentric-granulomatosis">bronchocentric granulomatosis</a>: rare <sup>7</sup>
  • +</li></ul><h4>Radiographic features</h4><p>Patterns of lung involvement include interstitial, airway and pleural disease.</p><p>Pleural involvement is a common manifestation of rheumatoid arthritis, although usually asymptomatic. </p><ul>
  • +<li>
  • +<a href="/articles/pleural-thickening">pleural thickening</a>: is seen more commonly than pleural effusions</li>
  • +<li>
  • +<a href="/articles/pleural-effusion">pleural effusions</a>: occur late in the disease, are often unilateral and associated with pericarditis and subcutaneous nodules <sup>5</sup>
  • +</li>
  • -<li><p><a href="/articles/pulmonary-fibrosis">pulmonary fibrosis</a></p></li>
  • -<li><p><a href="/articles/peribronchiolar-metaplasia" title="peribronchiolar metaplasia">peribronchiolar metaplasia</a> </p></li>
  • -<li><p><a href="/articles/rheumatoid-pulmonary-nodule-1">rheumatoid lung nodules</a></p></li>
  • -</ul><h5>Plain radiograph</h5><p>On chest radiographs, rheumatoid lung may present as diffuse interstitial infiltration with reticulation, nodules, and cystic changes. The findings may resemble other forms of ILD.</p><ul>
  • -<li><p>pleural effusion</p></li>
  • -<li><p>lower zone predominant reticular or reticulonodular pattern</p></li>
  • -<li><p>volume loss in advanced disease</p></li>
  • -<li><p><a href="/articles/rheumatoid-arthritis-musculoskeletal-manifestations-2">skeletal changes</a>, e.g. <a href="/articles/distal-clavicular-erosion-differential">erosion of clavicles</a>, glenohumeral erosive arthropathy, <a href="/articles/rib-notching">superior rib notching</a></p></li>
  • -</ul><p>On chest radiographs, rheumatoid lung necrobiotic nodules may appear as well-defined, round or oval opacities with smooth margins. They may be associated with the presence of pleural effusions, interstitial changes, or fibrotic bands.</p><h5>CT</h5><p>High-resolution computed tomography (HRCT) is the preferred imaging modality for the evaluation of rheumatoid lung.</p><p>HRCT findings may include diffuse interstitial infiltrates, reticulation, nodules, and cystic changes. The HRCT findings are consistent with fibrotic changes in the lung parenchyma.</p><p>Chest CT or HRCT features include:</p><ul>
  • -<li><p>pleural thickening or effusion</p></li>
  • -<li><p><a href="/articles/ground-glass-densities">ground-glass densities</a> <sup>8</sup></p></li>
  • -<li>
  • -<p><a href="/articles/pulmonary-fibrosis">interstitial fibrosis</a> (<a href="/articles/rheumatoid-arthritis-associated-interstitial-lung-disease">rheumatoid arthritis associated interstitial lung disease</a>)</p>
  • -<ul>
  • -<li><p>reticulation</p></li>
  • -<li><p>occurs in ~10% of RA patients <sup>3</sup></p></li>
  • -<li><p>can have either a <a href="/articles/uip">UIP</a> (slightly more common <sup>8</sup>) or <a href="/articles/non-specific-interstitial-pneumonia-1">NSIP</a> pattern</p></li>
  • +<li><a href="/articles/pulmonary-fibrosis">pulmonary fibrosis</a></li>
  • +<li>
  • +<a title="peribronchiolar metaplasia" href="/articles/peribronchiolar-metaplasia">peribronchiolar metaplasia</a> </li>
  • +<li><a href="/articles/rheumatoid-pulmonary-nodule-1">rheumatoid lung nodules</a></li>
  • +</ul><h5>Plain radiograph</h5><p>Chest radiograph may show: </p><ul>
  • +<li>pleural effusion</li>
  • +<li>lower zone predominant reticular or reticulonodular pattern</li>
  • +<li>volume loss in advanced disease</li>
  • +<li>
  • +<a href="/articles/rheumatoid-arthritis-musculoskeletal-manifestations-2">skeletal changes</a>, e.g. <a href="/articles/distal-clavicular-erosion-differential">erosion of clavicles</a>, glenohumeral erosive arthropathy, <a href="/articles/rib-notching">superior rib notching</a>
  • +</li>
  • +</ul><h5>CT</h5><p>Chest CT or HRCT features include:</p><ul>
  • +<li>pleural thickening or effusion</li>
  • +<li>
  • +<a href="/articles/ground-glass-densities">ground-glass densities</a> <sup>8</sup>
  • +</li>
  • +<li>
  • +<a href="/articles/pulmonary-fibrosis">interstitial fibrosis</a> (<a href="/articles/rheumatoid-arthritis-associated-interstitial-lung-disease">rheumatoid arthritis associated interstitial lung disease</a>)<ul>
  • +<li>reticulation</li>
  • +<li>occurs in ~10% of RA patients <sup>3</sup>
  • +</li>
  • +<li>can have either a <a href="/articles/uip">UIP</a> (slightly more common <sup>8</sup>) or <a href="/articles/non-specific-interstitial-pneumonia-1">NSIP</a> pattern</li>
  • -<li><p><a href="/articles/organising-pneumonia">organising pneumonia</a></p></li>
  • -<li><p><a href="/articles/bronchiectasis">bronchiectasis</a>. Occationally bronchiectasis without fibrosis is noted.</p></li>
  • -<li><p><a href="/articles/obliterative-bronchiolitis">bronchiolitis obliterans</a></p></li>
  • -<li>
  • -<p>large <a href="/articles/rheumatoid-nodules">rheumatoid nodules</a>. </p>
  • -<ul>
  • -<li><p>single or multiple</p></li>
  • -<li><p>tend to be based peripherally</p></li>
  • -<li><p>may cavitate (<a href="/articles/necrobiotic-lung-nodules">necrobiotic lung nodules</a>)</p></li>
  • -<li><p>cavitation of a peripheral nodule can lead to <a href="/articles/pneumothorax">pneumothorax</a> or <a href="/articles/haemopneumothorax">haemopneumothorax</a></p></li>
  • -<li><p>On HRCT, rheumatoid lung necrobiotic nodules are typically well-defined, round or oval, with smooth margins and a homogenous, dense appearance. They may appear more prominent than surrounding lung tissue, and may be associated with interstitial changes, pleural effusions, or fibrotic bands.</p></li>
  • +<li><a href="/articles/organising-pneumonia">organising pneumonia</a></li>
  • +<li><a href="/articles/bronchiectasis">bronchiectasis</a></li>
  • +<li><a href="/articles/obliterative-bronchiolitis">bronchiolitis obliterans</a></li>
  • +<li>large <a href="/articles/rheumatoid-nodules">rheumatoid nodules </a><ul>
  • +<li>single or multiple</li>
  • +<li>tend to be based peripherally</li>
  • +<li>may cavitate (<a href="/articles/necrobiotic-lung-nodules">necrobiotic lung nodules</a>)</li>
  • +<li>cavitation of a peripheral nodule can lead to <a href="/articles/pneumothorax">pneumothorax</a> or <a href="/articles/haemopneumothorax">haemopneumothorax</a>
  • +</li>
  • -<p><a href="/articles/follicular-bronchiolitis">follicular bronchiolitis</a>: rare</p>
  • -<ul><li><p>small centrilobular nodules or <a href="/articles/tree-in-bud-sign-lung">tree-in-bud</a></p></li></ul>
  • +<a href="/articles/follicular-bronchiolitis">follicular bronchiolitis</a>: rare<ul><li>small centrilobular nodules or <a href="/articles/tree-in-bud-sign-lung">tree-in-bud</a>
  • +</li></ul>
  • -<li><p><a href="/articles/caplan-syndrome">Caplan syndrome</a></p></li>
  • -</ul><h5>
  • -<strong>Treatment and Prognosis</strong>:</h5><p>Treatment for rheumatoid lung typically involves aggressive management of RA, including the use of disease-modifying antirheumatic drugs (DMARDs) and biologic agents. Corticosteroids may be used in severe cases. The prognosis for patients with rheumatoid lung is variable and depends on the severity of lung involvement, the degree of fibrosis, and the response to treatment.</p><h5>History and etymology:</h5><p>Rheumatoid lung disease was first described in the early 20th century. The term "rheumatoid lung" is derived from the underlying autoimmune condition, rheumatoid arthritis.</p><p>The term "necrobiotic" refers to the process of necrosis, or tissue death, and is used to describe the findings seen in these lung nodules. The term "rheumatoid lung nodules" reflects the association between these nodules and rheumatoid arthritis.</p><h5>Differential diagnosis:</h5><p>Differential diagnoses for rheumatoid lung include other forms of ILD, such as idiopathic pulmonary fibrosis, sarcoidosis, and lymphangitic carcinomatosis. The differential diagnosis should also consider infections, such as tuberculosis, and neoplastic diseases, such as lung cancer.</p><h4>See also</h4><ul>
  • +<li><a href="/articles/caplan-syndrome">Caplan syndrome</a></li>
  • +</ul><h4> See also</h4><ul>
  • -<p><a href="/articles/rheumatoid-arthritis">rheumatoid arthritis</a> (general article)</p>
  • -<ul><li><p><a href="/articles/rheumatoid-arthritis-musculoskeletal-manifestations-2">musculoskeletal manifestations of rheumatoid arthritis</a></p></li></ul>
  • +<a href="/articles/rheumatoid-arthritis">rheumatoid arthritis</a> (general article)<ul><li><a href="/articles/rheumatoid-arthritis-musculoskeletal-manifestations-2">musculoskeletal manifestations of rheumatoid arthritis</a></li></ul>
  • -<li><p><a href="/articles/rheumatoid-pulmonary-vasculitis">rheumatoid pulmonary vasculitis</a></p></li>
  • +<li><a href="/articles/rheumatoid-pulmonary-vasculitis">rheumatoid pulmonary vasculitis</a></li>

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