What are the Outcomes?

Prognosis

Some people will have their disease stabilized by medication and may have to remain on medication for life.  Others may be able to be weaned off medication after their disease stabilizes.  However, some patients will not respond to medication and, despite the best efforts of their doctors, will continue to develop more and more scarring in the lungs and become totally disabled.  There is no way currently to tell ahead of time who will respond to medication and who will not.   However, it is generally agreed that treatment early in the course of the disease is more likely to benefit the patient.  The type of IPF seen on biopsy is particularly predictive of prognosis.  

UIP is the most common form of IPF and, in general, has a poor prognosis.  Mortality rates range from 50 to 70%, and the average duration from diagnosis to death is about 2 to 4 years (above figure).  Spontaneous remissions do not occur.  Better survival has been associated with younger age, female gender, earlier stage of disease, and a beneficial response to corticosteroids.  Lower survival has been associated with male gender, more advanced disease (worse pulmonary function tests and X-rays), and possibly certain cellular changes on bronchoalveolar lavage.  In addition, more rapid progression of disease is associated with cigarette smoking. 

Smoking and IPF

AIP has an equally poor prognosis with a mortality rate of about 60%.  Most deaths occur in just 1 to 2 months.  The remaining forms of IPF all have a relatively better prognosis.  DIP has a mortality rate of about 28% and an average survival of 12 years.  No deaths have ever been attributed to RBILD. Finally, NSIP generally has a good prognosis with a mortality rate of just 11%.  Of the remaining cases of NSIP, 45% have been reported to recover completely and 42% remained stable or improved.

Panos. Am J Med 1990; 88:396

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References

• Gay SE, Kazerooni EA, Toews GB, Lynch J, Gross BH, Cascade PN, Spizarny DL, Flint A, Schork A, Whyte RI, Popovich J, Hyzy R, Martinez FJ. Idiopathic Pulmonary Fibrosis: predicting response to therapy and survival. American Journal of Respiratory and Critical Care Medicine. 157:1063-1072, 1998.

• Hubbard R, Johnston I, Britton J. Survival in patients with cryptogenic fibrosing alveolitis: a population-based cohort study. Chest. 113:396-400, 1998.

• Johnston IDA, Prescott RJ, Chalmers JC, Rudd RM for the Fibrosing Alveolitis Subcommittee of the Research Committee of the British Thoracic Society. British Thoracic Society study of cryptogenic fibrosing alveolitis: current presentation and initial management. Thorax. 52:38-44, 1997.

• Katzenstein A and Myers J. Idiopathic Pulmonary Fibrosis: Clinical Relevance of Pathologic Classification. American Journal of Respiratory and Critical Care Medicine. 157: 1301-1315, 1998.

• Mapel DW, Hunt WC, Utton R, Baumgartner KB, Samet JM, Coultas DB. Idiopathic pulmonary fibrosis: survival in population based and hospital based cohorts. Thorax. 53:469-476, 1998.

• Rudd RM, Haslam PL, Turner-Warwick M. Cryptogenic fibrosing alveolitis relationships of pulmonary physiology and bronchoalveolar lavage to treatment and prognosis. American Review of Respiratory Disease. 124:1-8, 1981.

• Schwartz DA, Helmers RA, Galvin JR, et al. Determinants of Survival in Idiopathic Pulmonary Fibrosis. American Journal of Respiratory and Critical Care Medicine. 149: 450-454, 1994.

• Schwartz DA, Van Fossen DS, Davis CS, et al. Determinants of Progression in Idiopathic Pulmonary Fibrosis. American Journal of Respiratory and Critical Care Medicine. 149(2 Pt 1): 444-449, 1994.

• Turner-Warwick M, Burrows B, Johnson A. Crytpogenic fibrosing alveolitis: clinical features and their influence on survival. Thorax. 35:171-180, 1980.

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