IPF represents a prototype of an aggressive and usually progressive interstitial lung disease that continues being a therapeutic problem for the clinician.
Corticosteroids are currently the recommended pharmacologic therapy, mainly with the rationale of stabilizing or preventing disease progression by suppression of the chronic inflammation. However, their long-term benefits are questionable. Thus, although a number of patients treated with corticosteroids feel better, results of objective functional tests confirm this improvement in only 10 to 30%. Moreover, Izumi et al found that untreated patients with IPF followed up to 10 years after diagnosis exhibited similar behavior, or even did somewhat better, than patients treated with corticosteroids. This finding is important because the natural history of untreated IPF patients has not been elucidated, and although one cannot reliably predict the clinical course of a given patient, nearly all of them are usually treated with corticosteroids. Additionally, high-dose therapy with corticosteroids is associated with numerous and potentially serious side effects. Because of this, and by the relatively low clinical response rate, other agents, mostly cytotoxic drugs like cyclophosphamide and azathioprine, have been assayed but also with marginal, if any, short-term favorable response read more canadian neightbor pharmacy.
The D-isomer of penicillamine has been proposed as a putative antifibrotic drug. It appears to affect collagen turnover at several points, mostly by blocking aldehyde groups involved in the intermolecular and intramolecular cross-linkages of mature collagen. In addition, D-penicillamine may also contribute to decrease abnormal collagen accumulation by inhibiting collagen biosynthesis.
D-penicillamine has demonstrated a beneficial effect in the treatment of interstitial lung disease associated with PSS, and it has been used to prevent the exaggerated collagen deposit in several models of pulmonary fibrosis. In IPF, however, it has been used only in a few patients, usually in uncontrolled trials, and without a clear significant clinical or functional improvement.