Rheumatoid Lung Disease

What is Rheumatoid lung disease? Rheumatoid lung disease comprises a group of lung problems related to rheumatoid arthritis (RA). Rheumatoid lung disease can present with one or more abnormalities of the lung, including:

RA-Lung

  • Pleuritis (inflammation of the lung lining) with or without pleural effusion (fluid in the chest cavity)
  • Empyema (pus in the chest cavity)
  • Pulmonary fibrosis (scarring of the lung tissue)
  • Necrobiotic nodules (lumps), including Caplan’s Syndrome (associated with Coal Workers’ Pneumoconiosis or Silicosis)
  • Bronchiolitis Obliterans Organizing Pneumonia (BOOP)
  • Pulmonary hypertension (high blood pressure in the lungs)
  • Drug-induced lung disease (including penacillamine, methotrexate and gold)
  • Respiratory infection, including tuberculosis
  • Bronchiectasis (destruction of the airway)

Rheumatoid lung disease is quite common and x-ray findings may be seen in 20% of patients with RA. Pleural disease has been reported in over 70% of some series. Although RA is more common amongst women, pulmonary disease in RA is more common in men and most common in those with late-onset RA. In many cases, the cause(s) of lung disease associated with rheumatoid arthritis are unknown. Sometimes the medicines used to treat rheumatoid arthritis may result in lung disease. Patients with severe rheumatoid arthritis or patients who smoke are more likely to develop rheumatoid arthritis associated interstitial lung disease.

What are the symptoms of Rheumatoid lung disease? In many cases, there may be little or no symptoms. Commonly occurring symptoms include shortness of breath, cough, chest pain, and fever. The symptoms depend upon the underlying lung process caused by the RA.

How will the doctor diagnose this? On physical examination, the doctor might be able to detect fibrosis (crackles heard when listening to the lungs with a stethoscope), pleural effusion (decreased breath sounds and a dull thud when percussing), pleuritis (hearing a rubbing sound), or bronchiectasis (harsh breath sounds). Often, however, the lungs seem normal.

What tests might the doctor do if he suspects Rheumatoid lung disease? To evaluate for Rheumatoid lung disease, the doctor will usually begin by taking a plain, two-view chest x-ray. Chest CT scanning is more expensive but will provide more detailed information.

Other tests might include:

  • Echocardiogram (may show pulmonary hypertension)
  • Thoracentesis (Needle inserted into the fluid around the lung)
  • Bronchoscopy (insertion of a flexible tube into the lungs)
  • Pulmonary function testing
  • Blood testing, including rheumatoid serologic tests

How is this treated? Many patients with this condition have no symptoms. Therapies are usually aimed at treating the underlying condition and to prevent complications. Corticosteroids or other immunosuppressive therapies are sometimes useful.

What is the prognosis? The outcome is related to the underlying disorder and the type and severity of lung disease. Possible complications include pneumothorax, pulmonary hypertension, chronic hypoxia (low oxygen), and respiratory insufficiency/failure.

When to Contact a Medical Professional? Call your health care provider PROMPTLY if you have rheumatoid arthritis and you develop unexplained breathing difficulties.

Where can I read more?

National Institutes of Health
du Bois RM, et al. Rheumatoid arthritis. In: Mason RJ, et al. Murray and Nadel’s Textbook of Respiratory Medicine. 4th ed. Philadelphia, Pa.: Saunders Elsevier; 2005.
Strange C, Highland KB. Interstitial lung disease in the patient who has connective tissue disease. Clin Chest Med. September 2004;25:549-559.