The ACR’s State-of-the-Art Clinical Symposium: Patients with Scleroderma, Lung Disease May Benefit from Aggressive Therapy

Ranked highest are methotrexate and TNF-alpha inhibitors—at five stars. They’re followed by rituximab at four; azathioprine, cyclophosphamide, D-penicillamine, gold, leflunomide and sulfasalazine at three; cyclosporine, hydroxychloroquine, mycophenolate and tofacitinib at two; and anakinra with one star. Abatacept is not yet listed in the database and may be the least toxic option based on what’s known now, Dr. Highland said.

Despite methotrexate’s potential for toxicity, there is no evidence that patients with preexisting pulmonary disease are at increased risk for further deterioration of lung function, Dr. Highland said.

Dr. Highland

“I believe that, if methotrexate is working to treat the joint disease, having interstitial lung disease is not a contraindication to continuing therapy,” Dr. Highland said.

As for TNF-alpha inhibitors, the other class with the worst rank, whether or not they remain a risk factor for ILD remains debatable, she said.

An important consideration when determining whether a drug has become toxic to the lung is ruling out a possible infection. Shortness of breath, unproductive cough and fever are all signs of both drug toxicity and infection.

Also, prophylaxis treatment for pneumocystis jiroveci pneumonia—the first line treatment is Bactrim—should be considered, Dr. Highland said.

“There is no such thing as a free lunch, and we have to really [weigh] the risks and benefits of these therapies in our patients,” she said. She also added a word of caution: “I think you can expect that with time we’ll start to see even more drug toxicities with some of the drugs that maybe aren’t on the radar screen yet.”

Thomas R. Collins is a freelance medical writer based in Florida.

References

1. Pulido T, Adzerikho I, Channick RN, et al. Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med. 2013 Aug 29;369(9):809–818.
2. Park JH, Kim DS, Park IN, et al. Prognosis of fibrotic interstitial pneumonia: Idiopathic versus collagen vascular disease-related subtypes. Am J Respir Crit Care Med. 2007 Apr 1;175(7):705–711.
3. Beyeler C, Jordi B, Gerber NJ, et al. Pulmonary function in rheumatoid arthritis treated with low-dose methotrexate: A longitudinal study. Br J Rheumatol. 1996 May;35(5):446–452.