Management of Idiopathic Pulmonary Fibrosis Cases at a Tertiary Care Hospital

Authors

  • Mahesh NS Assistant Professor, Department of TB and Chest Diseases, Akash Institute of Medical Sciences and Research Centre, Bangalore, Karnataka
  • Sadanand C D Associate Professor, Department of Medicine, Akash Institute of Medical Sciences and Research Centre, Bangalore, Karnataka
  • Durga Lawande Professor, Department of Pulmonary Medicine, Goa Medical college, Goa.

DOI:

https://doi.org/10.48165/cf21p159

Keywords:

Idiopathic Pulmonary Fibrosis, Pirfenidone, Outcome

Abstract

Background: The clinical history of the disease is quite variable; there is usually a slow physiological deterioration, but in some patients there  is a faster decline in lung function and death occurs within 6-12 months after diagnosis. Others experience an acute exacerbation during the  course of the disease with a sudden worsening of respiratory symptoms, hypoxemia and the appearance of new radiological infiltrates without  an identifiable cause. Despite different types of clinical course, IPF is inevitably associated with a poor prognosis, with a median survival of 2- 3 years. Subjects and Methods: Age, sex, occupation, address (including phone number) was noted down. A detailed occupational history  was sought, enquiring about the age at start of the occupation, exact nature of work, any history of exposure to fumes, gases, dust, etc, any  other person in the work place suffering from similar complaints etc were obtained. Results: Majority of the patients were treated with steroids  alone 22(62.8%). 10(28.5%) patients were treated with steroids with azathioprine. 3(8.5%) patients were treated with pirfenidone. Conclusion: Majority of the patients, 17(48.5%) out of 35 patients, expired during the study period. 5(14.2%) patients were lost to follow up  and the outcome was not available. 10(28.5%) patients remained stable, while 3(8.5%) patients worsened. None of the patients improved during the study period. 

References

1. Kim DS, Collard HR, King Jr TE. Classification and natural history of idiopathic interstitial pneumonias. Proc Am Thorac Soc. 2006; 3:285-92.

2. American Thoracic Society, European Respiratory Society. American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. Am J RespirCrit Care Med. 2002;165:277- 304.

3. Raghu G, Collard HR, Egan JJ, Martinez FJ, Behr J, Brown KK, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J RespirCrit Care Med. 2011;183: 788-824.

4. Fernández Pérez ER, Daniels CE, Schroeder DR, St Sauver J, Hartman TE, Bartholmai BJ, et al. Incidence, prevalence and clinical course of idiopathic pulmonary fibrosis. Chest. 2010;137:129-37.

5. Maheshwari U, Gupta D, Aggarwal A.N, Jindal S.K. Spectrum and diagnosis of Idiopathic pulmonary fibrosis. Indian T.Chestdis Allied Sci. 2004; 46: 23-26.

6. S Raniga, S Sharma, AArora, Y Khalasi, Pa Vora. Utility of High Resolution Computed Tomography (HRCT) in diagnosis and management of Idiopathic Pulmonary Fibrosis - A study of 10 cases". Ind J RadiolImag. 2006;16:4:841-46.

7. Wells, A. U., P. Cullinan, D. M. Hansell, M. B. Rubens, C. M. Black, A. J. Newman-Taylor, and R. M. Du Bois. Fibrosingalveolitis associated with systemic sclerosis has a better prognosis than lone cryptogenic fibrosingalveolitis..Am J. Respir. Crit. Care Med.1994;149:1583-90.

8. Cherniack, R. M., T. V. Colby, A. Flint, W. M. Thurlbeck, J. A. Waldron, Jr., L. Ackerson, M. I. Schwarz, and T. E. King, Jr. Correlation of structure and function in idiopathic pulmonary fibrosis. Am.J. Respir. Crit. Care Med.1995;151:1180–88.

9. F. SoaresPires, P. Caetano Mota, N. Melo, D. Costa, J.M. Jesus, R. Cunha, S. Guimarães, C. Souto-Moura, A. Morais . Idiopathic Pulmonary Fibrosis-Clinical presentation, outcome and baseline prognostic factors in a Portuguese cohort. Rev Port Pneumol. 2013;19(1):19-27.

10. Katzenstein, A.-L. A., J. L. Myers, W. D. Prophet, L. S. Corley, III, and M. S. Shin. Bronchiolitis obliterans and usual interstitial pneumonia: a comparative clinicopathologic study. Am. J. Surg. Pathol. 1986;106: 373-81.

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Published

2019-11-30

How to Cite

Management of Idiopathic Pulmonary Fibrosis Cases at a Tertiary Care Hospital . (2019). Academia Journal of Medicine, 2(2), 90–95. https://doi.org/10.48165/cf21p159