drugs in I.L.D. in sclerderma

Question

My mother aged 47 years, is a known case of hypertension for last 5 years. Currently taking Losartan 50 mg twice a day, Ditide 12.5 mg twice a day, Amlodipine 10 mg once a day and Prazosin 5 mg once a day. She is also a follow through case of hypothyroidism, taking Eltroxin 150 microgram per day. She is also a follow through case of CREST syndrome. For past 2 years she is having dyspnea on exertion and cough. She was recently done CT thorax demonstrating early changes of interstitial lung disease. On PFT there is restrictive lung pattern. I am an MD physician and wants to know whether I should start Cyclophosphamide and Predinosolone or I should go for less toxic drug like Mycophenolate mofetil. What are other alternatives?

Answer

Unfortunately, there is no clear answer. Dr. Wigley in the Johns Hopkins Scleroderma Center is part of a multicenter study looking at cyclophosphamide in scleroderma interstitial lung disease. Some earlier studies have suggested that cyclophosphamide is useful when there is active inflammation in the lung diagnosed with high resolution CT scan showing alveolitis, bronchoalveolar lavage, or rapidly declining PFTs. But other agents such as azathioprine (Imuran) have also been used. There is no clear answer as of yet.

Alan Matsumoto, M.D.

About Alan Matsumoto, M.D.

Part-time Faculty, Division of Rheumatology
Johns Hopkins University