Case 2
A 51-year-old woman with a five-year history of recurrent pneumonia and heavy tobacco use was referred for evaluation of CTD-ILD after surgical lung biopsy showed usual interstitial pneumonia (UIP) vs. diffuse interstitial pneumonia. She was initially diagnosed with idiopathic pulmonary fibrosis (IPF) but couldn’t tolerate treatment with pirfenidone. Two years ago, the patient developed joint pain and stiffness. Dyspnea worsened to the point she required supplemental oxygen.
An examination revealed active inflammatory arthritis. Laboratory tests were notable for positive anti-cyclic citrullinated peptide (anti-CCP) antibodies. CT chest imaging showed UIP. Radiographs revealed erosive disease of the small joints of the hands and feet. Her clinical picture was most consistent with seropositive rheumatoid arthritis (RA).
“Remember, lung disease precedes articular symptoms in about 10 to 15% of ILD, so it’s important to check serologies,” Dr. Wilfong said.
A consensus guideline on which serologies should be checked has yet to be established, but she recommended immunofluorescent anti-nuclear antibody (ANA), anti-neutrophil cytoplasmic antibody (ANCA), rheumatoid factor (RF), anti-CCP antibody and an extended myositis antibody panel.1,2
When it comes to the treatment of RA-ILD, there are no specific therapeutic recommendations. “If there is articular disease, it’s really just important to treat it with something. Options [may] include a tumor necrosis factor inhibitor, tocilizumab, abatacept or rituximab,” she said. “Of note, methotrexate is not absolutely contraindicated in mild ILD. I use a maximum dose of 20 mg of methotrexate weekly and watch closely for signs and symptoms of methotrexate toxicity.”
This patient had marked improvement of articular symptoms with etanercept until suffering a secondary loss of efficacy, at which time she was switched to adalimumab. She declined alternative agents, such as disease-modifying anti-rheumatic drugs or nintedanib, due to concerns about side effects. Unfortunately, the patient’s pulmonary function continued to decline, and she wasn’t a transplant candidate. She was transitioned to hospice.
‘ILD imaging is all about learning by doing. Talk to your radiologists & review interesting scans together.’—Dr. Wilfong
Case 3
A 46-year-old woman with a diagnosis of seronegative RA organizing pneumonia was referred for a second opinion. She had been treated with prednisone at doses greater than or equal to 20 mg daily for the past year, without prophylaxis against Pneumocystis jiroveci pneumonia. She now had a worsening cough and intermittent fevers. The examination was notable for Gottron’s papules, inflammatory arthritis and mechanic’s hands. Laboratory studies showed an elevated creatinine kinase level.
