Scleroderma - 2001 National Meeting Reports (cont.)

Below are perspectives on key reports presented at the recent national meeting of the American College of Rheumatology:

Lung Disease

Lung scarring (pulmonary fibrosis) did not progress in most patients with systemic sclerosis, even without cyclophosphamide (Cytoxan), over 6 years. 

Dr. Shiel's Perspective: Recognizing that lung scarring conditions can seriously damage the lung, and that Cytoxan can improve many lung scarring conditions, it is now difficult to recommend this potentially toxic drug for the scarring form of scleroderma lung (pulmonary fibrosis). This form of lung disease must be distinguished from inflammation of the lungs' tiny air sacs (alveolitis), as in the next report below.

Inflammation of the lungs' tiny air sacs (alveolitis) stabilized or improved in scleroderma patients treated with cyclophosphamide (Cytoxan) and prednisone

Dr. Shiel's Perspective: This form of lung disease (as compared to the one described in the report above) actually did respond to Cytoxan. The key element seems to be inflammation. If inflammation is present in the lungs, it may be helped by Cytoxan treatments (typically given as a monthly intravenous infusion). The exact form of lung disease requires a lung biopsy for a precise diagnosis.

Specific genes were found responsible for why some scleroderma patients develop lung disease while others do not. 

Dr. Shiel's Perspective: This has always been a perplexing phenomenon with autoimmune rheumatic diseases - why they present so differently from patient to patient. It may be that environmental differences account for some of this, but it seems that there are also subtle differences genetically in the inherited material of each patient that may be playing a significant role.

Viagra (sildenafil) seemed to help function in scleroderma patients with severe pulmonary hypertension (elevated blood pressure in the artery to the lungs).

Dr. Shiel's Perspective: Because Viagra has an effect on the smooth muscle of the blood vessels, it was tried in these very ill patients who had failed the traditional prostacyclin intravenous infusions. Although Viagra did show favorable results, these are very small preliminary studies. Incidentally, it might be expected that there would be some benefit on the Raynaud's phenomenon in patients treated with this drug, and that is exactly what the researchers saw in all patients. Further research studies are now needed to really determine the exact role and safety of Viagra in patients with scleroderma.

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