Breathless with Rheumatoid Arthritis: A Case of ILD

Shafei R, Goalby R, Hutchinson J. Endgames: Case Review: A case of breathlessness. BMJ.2015;351:h3515 doi: http://dx.doi.org/10.1136/bmj.h3515 (16 July 2015)

This case discusses a patient with rheumatoid arthritis (RA) controlled by methotrexate, who developed progressive shortness of breath, which was diagnosed as interstitial lung disease (ILD).

She was first treated for pneumonia with intravenous antibiotics, but continued to deteriorate and was given a working diagnosis of ILD. Other possibilities would have been pulmonary emboli or edema.

RA is associated with infectious complications. There is debate about whether the predisposition to infection is caused by methotrexate and other drugs, or by the underlying disease. Many clinicians avoid methotrexate and use an alternative drug in this situation.

ILD is one of several pulmonary complications of RA, with a 7.7% lifetime risk. Other complications are nodules, bronchiectasis, pleural disease, and airflow obstruction.

High resolution computed tomography (CT) is important to characterize ILD. But CT imaging may mimic other idiopathic interstitial lung diseases, such as usual interstitial pneumonia, idiopathic pulmonary fibrosis, or non-specific interstitial pneumonia.

The evidence base for managing acute exacerbations of ILD is poor. Supportive care with oxygen and close monitoring are essential.

British Thoracic Society guidelines recommends pulsed intravenous methylprednisolone for 3 days, followed by maintinenace therapy with prednisolone. Second line agents, such as cyclophosphamide or azathioprine, can be considered if the response is poor.

Standard practice includes oral corticosteroids. Serial monitoring of lung function is essential.

ILD in RA has a poor prognosis, with a median survival of 3 years in one cohort. Lung transplantation may be considered. But patients with limited disease have a better prognosis, and may not undergo clinical progression.

This patient was placed on ventilatory support and high dose immunsuppression, then stepped down to the ward and discharged with long term oxygen therapy and a tapering dose of oral steroids.