Precise and integrated diagnostics avert need for lung transplant in a patient with rheumatoid arthritis
A 78-year-old male patient diagnosed with Idiopathic Pulmonary Fibrosis (IPF) came to the Temple Lung Center hoping to be considered for lung transplant listing. He was already listed for transplant at a high-volume New York City transplant center, where he had received his workup and diagnosis several years previously. Since then, he had consulted four other pulmonologists and his disease had worsened markedly, such that he required 8 liters of oxygen in order to walk. He was a former smoker, and had acid reflux disease with Barrett’s esophagus.
In the patient’s transplant workup at his New York City transplant center, he tested negative for aspiration; coronary or other heart disease remaining after prior stenting; common cancers; rheumatologic diseases including ANA, RF, SCL-70, and SSA/SSB; and other significant diseases such as diabetes or hypertension. He underwent Video-Assisted Thorascopic Surgery (VATS) with biopsy of multiple lobes, revealing features suggestive of Usual Interstitial Pneumonia (UIP).
At the Temple Lung Center:
• Lung Function Testing revealed an FVC of 56% of predicted, no obstruction was present. Total lung capacity was 62% of predicted. DLCO, which was of greatest concern, was only 44%, even after adjusting for alveolar volume.
• In addition to bibasilar crackles, his physical examination revealed a warm, tender right wrist with limited range of motion and active synovitis.
• Non-enhanced High-Resolution CT of the thorax was performed, demonstrating emphysematous changes, traction bronchiectasis, honeycombing, and bibasilar and peripheral predominant reticular changes and ground glass opacities.
Diagnosis and Treatment
Given physical exam findings, a repeat panel of serologies was obtained and consultation made to a rheumatologist. Patient was diagnosed with rheumatoid arthritis (RA) with associated interstitial lung disease and started on treatment with mycophenolate mofetil titrated as tolerated. In addition, his rheumatologist prescribed hydroxychloroquine for his joint symptoms.
The patient made an impressive clinical recovery. He now ambulates without oxygen and was deemed too healthy for transplant. CT performed one year later showed dramatic improvement of the pulmonary interstitial disease with improvement of ground glass opacities. Patient continues to follow closely with his local center and with Temple in case re-listing is needed.
A significant proportion of patients with rheumatic disease will develop parenchymal lung disease, which prognostically and clinically mirrors idiopathic pulmonary fibrosis (IPF). Although 10% of patients with RA have clinical ILD, 30% have subclinical ILD, and this may develop before the onset of joint disease. Further complicating the picture, the UIP pattern predominates in this population. In RA, swollen joints in the hands (especially the wrists and MCP joints), knees, or ankles may identify patients in need of further evaluation and screening, even when other tests do not.
Erin Narewski, DO
Assistant Professor, Thoracic Medicine and Surgery
Lewis Katz School of Medicine at Temple University