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Rheumatoid lung disease

Definition

Rheumatoid lung disease is a group of lung problems related to rheumatoid arthritis (RA). 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)

Causes

Lung problems are common in people with RA. They often cause no symptoms, but for some people they are the main symptom of their RA.

The cause of lung disease associated with RA is unknown. Sometimes, the medicines used to treat RA, especially methotrexate, may result in lung disease.

Symptoms

Symptoms may include any of the following:

• Chest pain

• Cough

• Fever

• Shortness of breath

• Joint pain, stiffness, and swelling

• Skin nodules

Exams and Tests

Your health care provider will perform a physical exam and ask about your symptoms.

Symptoms depend on the type of lung disease RA is causing in the lungs.

When listening to the lungs with a stethoscope, your provider may hear normal breath sounds or:

• Crackles (rales)

• Decreased breath sounds

• Wheezing

• A rubbing sound

When listening to the heart, there may be abnormal heart 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)

• Blood tests for rheumatoid arthritis

Treatment

Many people with this condition have no symptoms. Treatment is usually aimed at the RA causing the lung problem and the complications caused by the disorder. Corticosteroids or other medicines that suppress the immune system are sometimes useful.

There is emerging evidence that pirfenidone and nintedanib may work for people with fibrosis due to rheumatoid lung disease.

Outlook (Prognosis)

The outcome is related to the underlying disorder and the type and severity of lung disease. In severe cases, lung transplantation can be considered. This is more common in cases of bronchiolitis obliterans, pulmonary fibrosis, or pulmonary hypertension.

Possible Complications

Rheumatoid lung disease may lead to:

• Collapsed lung (pneumothorax)

• Pulmonary hypertension

When to Contact a Medical Professional

Contact your provider right away if you have rheumatoid arthritis and you develop unexplained breathing difficulties or worsening of baseline breathing difficulties.

References

Corte TJ, Wells AU. Connective tissue diseases. In: Broaddus VC, Ernst JD, King TE, et al, eds. Murray & Nadel's Textbook of Respiratory Medicine. 7th ed. Philadelphia, PA: Elsevier; 2022:chap 92.

Johnson SR, Bernstein EJ, Bolster MB, et al. 2023 American College of Rheumatology (ACR)/American College of Chest Physicians (CHEST) guideline for the treatment of interstitial lung disease in people with systemic autoimmune rheumatic diseases. Arthritis Care Res (Hoboken). 2024;76(8):1051-1069. PMID: 38973731 pubmed.ncbi.nlm.nih.gov/38973731/.

Raghu G, Martinez FJ. Interstitial lung disease. In: Goldman L, Cooney KA, eds. Goldman-Cecil Medicine. 27th ed. Philadelphia, PA: Elsevier; 2024:chap 80.

Review Date: 4/10/2025

Reviewed By: Allen J. Blaivas, DO, Division of Pulmonary, Critical Care, and Sleep Medicine, VA New Jersey Health Care System, Clinical Assistant Professor, Rutgers New Jersey Medical School, East Orange, NJ. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.