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Rheumatoid Lung Disease

Rheumatoid lung disease, as its name suggests, is associated with rheumatoid arthritis. Of the 1.3 million Americans who have rheumatoid arthritis, nearly half may have some abnormal lung function. Up to one-fourth develop rheumatoid lung disease.

Symptoms of Rheumatoid Lung Disease

Symptoms associated with rheumatoid lung disease which occur in addition to arthritic symptoms of joint pain, joint swelling, joint stiffness, and nodules include:

shortness of breath cough chest pain fever crackle sounds when listening to lungs with stethoscope (but decreased breath sounds or normal breath sounds are also possible)

Diagnosis and Treatment of Rheumatoid Lung Disease

Procedures used to help diagnose rheumatoid lung disease include:

chest x-rays computed tomography (CT) scan of the chest echocardiogram thoracentesis bronchoscopy

Currently there are no effective treatments for rheumatoid lung disease. Corticosteroids and immunosuppressive therapies help treat the complications. In rheumatoid lung disease, the air sacs or alveoli of the lungs and their supporting structures become scarred by inflammation, resulting in impaired lung function.

The Respiratory System (Image) Lung Anatomy (Image) The Lungs (Image)

Thoracic and Pulmonary Abnormalities Associated With Rheumatoid Lung Disease

The thoracic and pulmonary abnormalities associated with rheumatoid lung disease include:

Pleural effusion

Pleural thickening Pulmonary fibrosis Necrobiotic nodules Bronchiolitis obliterans organizing pneumonia (BOOP) Bronchiectasis Bronchiolitis obliterans Interstitial pneumonitis Pulmonary hypertension

Interstitial Lung Disease and Rheumatoid Arthritis

The most common manifestation of pulmonary disease in rheumatoid arthritis is interstitial lung disease (ILD). Patients with severe rheumatoid arthritis or patients who smoke are more likely to develop rheumatoid arthritis associated interstitial lung disease. A report by Dr. Jeffrey T. Chapman from the Cleveland Clinic Foundation details the prevalence, signs and symptoms, diagnosis, and treatment for ILD. Findings reported in January 2005 by Mayo Clinic researchers suggest that rheumatoid lung disease may be "fundamentally different from other forms of lung disease", and possibly should be treated differently. Through advancements in computer-assisted image analysis, it may be possible to diagnose rheumatoid lung disease earlier and treat it aggressively as a disease of the immune system. The Mayo Clinic research revealed an abundance of T cells known as CD4 and CD3 cells in rheumatoid lung disease tissue samples. These findings may impact the development of new, more effective drugs to treat rheumatoid lung disease. Drugs designed to block T cell action may ultimately allow for treatment of rheumatoid lung disease in its early stages -- possibly even prolonging lives -- important because Mayo Clinic research indicated rheumatoid arthritis which spreads beyond the joints to the lungs is more likely to be fatal.

Rheumatoid lung disease

Rheumatoid lung disease is a group of lung problems related to rheumatoid arthritis. The condition can include:

Blockage of the small airways (bronchiolitis obliterans) Fluid in the chest (pleural effusions) High blood pressure in the lungs (pulmonary hypertension)

Lumps in the lungs (nodules) Scarring (pulmonary fibrosis)

Pulmonary abnormalities are common in rheumatoid arthritis, but they often cause no symptoms. The causes of lung disease associated with rheumatoid arthritis are unknown. Sometimes the medicines used to treat rheumatoid arthritis, especially methotrexate, may result in lung disease.


Chest pain Cough Fever Shortness of breath

Other symptoms that may occur with this disease include:

Joint pain Joint stiffness Joint swelling Skin nodules

Exams and Tests

The doctor may hear crackles (rales) when listening to the lungs with a stethoscope. Or, the patient may have decreased breath sounds, wheezing, a rubbing sound, or normal breath sounds. The following tests may show signs of rheumatoid lung disease:

Chest x-ray CT scan of the chest Echocardiogram (may show pulmonary hypertension) Lung biopsy (bronchoscopic, video-assisted, or open) Lung function tests Needle inserted into the fluid around the lung (thoracentesis)

Many people with this condition have no symptoms. Treatments are aimed at the underlying disorder and the complications caused by the disorder. Corticosteroids or other medicines that suppress the immune system are sometimes useful.

Outlook (Prognosis)
The outcome is related to the underlying disorder and the type and severity of lung disease.

Possible Complications

Pneumothorax Pulmonary hypertension

Rheumatoid Arthritis and the Lung

This PatientPlus article is written for healthcare professionals so the language may be more technical than the condition leaflets. You may find the abbreviations list helpful. Pulmonary involvement is one of the most frequent extra-articular manifestation of rheumatoid arthritis.1 Most common lung diseases associated with rheumatoid arthritis are interstitial lung diseases (ILD) and pleural effusions. The range of pulmonary problems includes:

Rheumatoid nodules: o The only pulmonary manifestation specific to rheumatoid arthritis o Typically benign but can lead to pleural effusion, pneumothorax, haemoptysis, secondary infection, and bronchopulmonary fistula Caplan's syndrome: o The combination of rheumatoid arthritis with pneumoconiosis related to mining dust o Look for rapid development of multiple basal peripheral nodules in the rheumatoid arthritis patient who has a history of exposure to mining dusts. o This can progress to severe pulmonary fibrosis Interstitial lung disease: o Radiographic findings of ILD occur in 2-5% of patients, while diffusion capacity abnormalities occur in 40%. o High resolution CT scan and histology have shown even higher rates, but clinically significant disease probably occurs in 5-10% of rheumatoid patients. Bronchiolitis:

Bronchiolitis obliterans with organising pneumonia: bilateral parenchymal opacities, often with preserved lung volumes. Typically presents as a relapsing, non-resolving pneumonia that does not respond to antibiotics. Steroids can be curative. o Obliterative bronchiolitis: rare, usually fatal condition. Associated with penicillamine, gold, and sulfasalazine treatment. Presents with rapidonset dyspnoea and dry cough. Fever is uncommon. Bronchiectasis: o 10% of patients may show radiographic signs of bronchiectasis; it may occur in the absence of ILD. o Rheumatoid arthritis patients that get this are more likely to be heterozygous for the CTFR mutation seen in cystic fibrosis. Arteritis:

Arteritis of the pulmonary artery and lung is rare; signs of systemic vasculitis are usually present.

Infection: o Respiratory infections account for 15 to 20% of deaths in rheumatoid patients. Drug toxicity: o Acute interstitial pneumonitis may occur in 1-5% of patients treated with methotrexate (see below). o Penicillamine and gold may also cause pulmonary complications.2 Pleural effusions: o Common in RA; they are exudative and have a low glucose o Occasionally an empyema may develop Lung cancer is more common in rheumatoid arthritis patients than in normal control subjects. Other diseases: o RA patients can get apical fibro-bullous disease (apical fibrotic cavity lesions similar to ankylosing spondylitis). o Thoracic cage immobility causing restrictive lung disease o Primary pulmonary hypertension (rare); secondary pulmonary hypertension (due to ILD) is more common.

Methotrexate-associated lung disease in rheumatoid arthritis

Methotrexate pneumonitis is an unpredictable and life-threatening side effect of methotrexate therapy. Presentation is often subacute with symptoms often present for several weeks or months before diagnosis. Presents most often with cough, dyspnoea and fever. May progress rapidly to respiratory failure. Early diagnosis, cessation of methotrexate, and treatment with corticosteroids and/or cyclophosphamide are important in management. There is a high rate of recurrence of lung injury after re-challenge with methotrexate.


Although rheumatoid arthritis is more common in women, rheumatoid lung disease occurs more frequently in men who have long-standing rheumatoid disease, positive rheumatoid factor and subcutaneous nodules.3 Approximately 30% to 40% of patients with rheumatoid arthritis demonstrate either radiological or pulmonary function abnormalities indicative of interstitial fibrosis or restrictive lung disease.4 Although rheumatoid arthritis disease activity is important, smoking has been shown to be the most consistent independent predictor of radiological and physiological abnormalities suggestive of ILD in rheumatoid arthritis.5

Differential diagnosis
The association of rheumatoid arthritis with lung disease may be due to:

Rheumatoid-associated lung disease Drug-related lung disease secondary to drugs used to treat rheumatoid arthritis Infection secondary to immunosuppression Coexistent medical conditions


Blood tests for evaluation of rheumatoid arthritis, including serology Respiratory function tests Chest x-ray Aspiration of pleural fluid CT or MRI scan Lung biopsy

There have been new guidelines and NICE guidance published recently.6,7,8,9 These are important guidelines for improvement of the management of RA. They do not include details of the management of lung disease in RA.

The majority of patients with progressive pulmonary symptomatology, when treated with corticosteroids, will have equivocal results.4 Some patients appear to respond to immunosuppressive or cytotoxic medications but responses are often disappointing.4, 10 Tumour necrosis factor blockade with infliximab has shown promising results but has also been implicated in causing serious lung toxicity.11

Survival rates in patients with coexisting RA and pulmonary fibrosis are similar to those of patients with idiopathic pulmonary fibrosis.3