Chest
Volume 110, Issue 4, October 1996, Pages 1058-1067
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Reviews
Corticosteroids and the Treatment of Idiopathic Pulmonary Fibrosis: Past, Present, and Future

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Why are corticosteroids used in patients with IPF? There is evidence that corticosteroid therapy is associated with clinical improvement in only a small percentage of IPF patients. This improvement is seen primarily in patients with a cellular histologic pattern on lung biopsy specimens, but no clinical, physiologic, or pathologic finding has been consistently associated with corticosteroid response. For IPF patients with a fibrotic histologic pattern on lung biopsy specimens, there is little evidence that corticosteroids are of any benefit, and available reports suggest that the cost of therapy in terms of both morbidity and mortality may be high. Little is known about the effect of corticosteroids on long-term clinical outcomes, and even less is known about their effects on the overall quality of life. Although current treatment regimens are based on the rationale that IPF is due to alveolar inflammation, which is probably an accurate assumption, the treatment to suppress the alveolitis should have an outcome that patients consider favorable. Data from the New Mexico ILD Registry and from other researchers suggest that the paucity of useful clinical data in IPF has resulted in a hodgepodge of diagnostic approaches and therapeutic criteria, as well as a fatalistic approach to treatment.

Medical history is replete with examples of failed therapies. Analogies can be drawn between the history of IPF treatment and other catastrophic illnesses.67 The common threads in all poorly treatable diseases are a lack of understanding about the cause and pathophysiologic state of the illness, an emphasis on indirect encouraging evidence about therapy instead of direct discouraging evidence, and the physician's ever-present compulsion to “do something.” What we should learn from these examples is that when clearly effective therapies are not available, we are ethically obligated to give patients full information about the faults and merits of what is available and to assist them in choosing what is best for them. Medicine has advanced in the past only when assumptions about diseases and treatments were examined critically; we must continue to challenge our assumptions about IPF and corticosteroid therapy.

Key words

corticosteroids
idiopathic pulmonary fibrosis
interstitial lung disease

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Supported by National Research Service Award 5T32H207733 (Dr. Mapel) and by NIH-NHLBI grant R01-HL40587.

revision accepted May 2.