Idiopathic interstitial pneumonia medical therapy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ahmed Zaghw, M.D. [2];

Overview

As IIPs are heterogenous group of unknown interstitial lung disease with different natural history and clinical course, their management should be based on the clinical subtype. Optimal therapy for IPF is controversial as all currently available medications for IPF are severely limited by the lack of clear understanding of the natural history of IPF, the presence of various forms of study designs; heterogeneous patient groups, disputable diagnostic certainty; variable study duration; differences in medication formulation, dosage, route of administration, and duration of treatment; lack of placebo controls; variable intervals between evaluations and differing types of non quantitative assessment criteria. To date, most of treatment strategies have been based on eliminating or suppressing the inflammatory component. As no pharmacological therapy has been proven a clinical efficacy in altering or reversing the inflammatory process of IPF. The clinical trials over the past decades are hopefully held to clear the controversial dilemmas regarding which patients should be treated? When should therapy be started? What is the best treatment and how it should delivered and maintained? and how can be the treatment monitored especially that majority of patients are suffering comorbidities related functional limitations.

Medical Response of Clinical Subtype

The following points are a general summary for the responsiveness of each subtype of IIPs

Chronic Fibrosing IIPs

Idiopathic Pulmonary Fibrosis
  • poor response to corticosteroids and cytotoxic drugs
Idiopathic Nonspecific Interstitial Pneumonia
  • good response to corticosteroids

Acute/Subacute IIPs

Cryptogenic Organizing Pneumonia
  • good response to corticosteroids
Acute Interstitial Pneumonia (Hamman-Rich Syndrome)]
  • unknown response to corticosteroids

Smoking-related IIPs

Respiratory Bronchiolitis-Interstitial Lung Disease
  • good response to smoking cessation but unknown response to corticosteroids
Desquamative Interstitial Pneumonia
  • good response to smoking cessation but unknown response to corticosteroids

Outline of Medical Therapy in Acute IIPs

The main treatment for acute interstitial pneumonia (AIP) is supportive care and corticosteroids.Supportive care with Noninvasive or invasive mechanical ventilation is usually required, since most patients develop respiratory failure also the prevention of complications as venous thromboembolism, gastrointestinal bleeding and nosocomial pneumonia. The optimum dosing of glucocorticoids and its clinical benefit remains unclear, However these are widely used.
  • Glucocorticoids: Once the diagnosis of AIP is made, high dose systemic glucocorticoids ( methylprednisolone 2 gm per day intravenously in divided doses) are given[1] High dose glucocorticoid therapy are just supported by small case series with widely varying results [2][3][4][5][6]
  • Antibiotics: Empiric broad-spectrum antibiotics are given to cover any infections.

Outline of Medical Therapy in Chronic Fibrosing IIPs

Establishment of the diagnosis is the first critical step in management as misdiagnosis can lead to inappropriate initial therapy. The second step is severity stage to guide treatment choices. Finally, a disease management plan is tailored to the disease severity and patient’s preferences. Follow up assessment is needed to refine treatment options with the disease progression. As no therapy has been proven to be efficacious in this disease, management generally includes some combination of supportive care such as supplemental oxygen and pulmonary rehabilitation, consideration for participation in clinical trials, referral for lung transplant evaluation if possible and early detection and management of comorbidities.[7][8]

General Approach

Supportive Care: The most important components of supportive care for patients with IPF are provision of supplemental oxygen (when needed), education, pulmonary rehabilitation, and vaccination against Streptococcus pneumoniae and influenza.

Supplemental oxygen: All IPF patients will at the end require supplemental oxygen, initially with exertion and then continuously. Oxygen therapy should be prescribed to enable the patients to maintain normal activity and possibly to prevent or delay the onset of secondary pulmonary hypertension in hypoxemic patients.

Education: Improved education and communication about the diagnosis, management of IPF, end of life issues and advanced directives are needed to optimize the plan of care. [9][10]

Pulmonary rehabilitation: Significant reduction in dyspnea and improvement in six-minute walk distance were reported after a pulmonary rehabilitation program [11][12][13][14][15][16]

Vaccination: Pulmonary infections are poorly tolerated in patients with interstitial lung disease, so Influenza and pneumococcal polysaccharide vaccine should be offered to patients with IPF.

Comorbidities: Prevention of gastroesophageal reflux and recurrent microaspiration and cotrolling other comorbidities may slow the progression and have an additional treatment benefit [7][17]

Gastroesophageal reflux and chronic microaspiration: GERD is an important risk factor for the development and progression of IPF,[18][19][20][21] especially that 90% of IPF patients have GERD. [22] Studies reported that use of anti-GERD medications were associated with decreased radiographic fibrosis scores on chest computed tomography and was an independent predictor of longer survival time.[23] Those reports suggesting that abnormal acid gastroesophageal reflux contributes to disease progression. However, controlled clinical trials of acid reflux treatments in IPF are needed.

Medical agents such as pirfenidone show promise, but there is insufficient evidence to recommend their general use at this time. In the past colchicine, cyclophosphamide, endothelin receptor antagonists, interferon gamma, methotrexate,cyclosporine, penicillamine) have been in case series or clinical trials. Recently there is an evidence against their routine use due to their doubtful benefit and intolerable toxicity.

  • Pirfenidone: Antifibrotic agents
Most case series and randomized trials have shown a modest beneficial effect of pirfenidone in slowing the progression of IPF [24][25][26][27][28][29]
  • CAPACITY 004 and 006 trials (Clinical studies Assessing Pirfenidone in idiopathic pulmonary fibrosis, showed that the higher dose of pirfenidone significantly reduced the decline in the 6 minutes walk test distance (MWTD).
  • A randomized trial of pirfenidone versus placebo [30] suggested that there may be greater benefit in patients whose disease is less severe.
  • Dosage and administration : 40 mg/kg per day in three divided doses. It is approved for use in patients with mild-to-moderate IPF in Japan, Europe, and Canada, but not in the United States.
  • Phosphodiesterase Inhibitors: The IPF-related pulmonary hypertension may be treated with a phosphodiesterase inhibitor that might improve exercise tolerance, as in idiopathic pulmonary hypertension. [25][31][32] A trial of sildenafil may be a good option in patients with a DLCO <35 percent, echocardiographic evidence of right ventricular dysfunction, and no contraindications to sildenafil ( unstable angina, use of nitrates). Longer duration trials are needed to assess the safety and efficacy of sildenafil in the treatment of IPF.

References

  1. ↑ Vourlekis, JS. (2004). "Acute interstitial pneumonia". Clin Chest Med. 25 (4): 739–47, vii. doi:10.1016/j.ccm.2004.07.001. PMID 15564019. Unknown parameter |month= ignored (help)
  2. ↑ Olson, J.; Colby, TV.; Elliott, CG. (1990). "Hamman-Rich syndrome revisited". Mayo Clin Proc. 65 (12): 1538–48. PMID 2255216. Unknown parameter |month= ignored (help)
  3. ↑ Vourlekis, JS.; Brown, KK.; Cool, CD.; Young, DA.; Cherniack, RM.; King, TE.; Schwarz, MI. (2000). "Acute interstitial pneumonitis. Case series and review of the literature". Medicine (Baltimore). 79 (6): 369–78. PMID 11144035. Unknown parameter |month= ignored (help)
  4. ↑ Suh, GY.; Kang, EH.; Chung, MP.; Lee, KS.; Han, J.; Kitaichi, M.; Kwon, OJ. (2006). "Early intervention can improve clinical outcome of acute interstitial pneumonia". Chest. 129 (3): 753–61. doi:10.1378/chest.129.3.753. PMID 16537878. Unknown parameter |month= ignored (help)
  5. ↑ Avnon, LS.; Pikovsky, O.; Sion-Vardy, N.; Almog, Y. (2009). "Acute interstitial pneumonia-Hamman-Rich syndrome: clinical characteristics and diagnostic and therapeutic considerations". Anesth Analg. 108 (1): 232–7. doi:10.1213/ane.0b013e318188af7a. PMID 19095855. Unknown parameter |month= ignored (help)
  6. ↑ Quefatieh, A.; Stone, CH.; DiGiovine, B.; Toews, GB.; Hyzy, RC. (2003). "Low hospital mortality in patients with acute interstitial pneumonia". Chest. 124 (2): 554–9. PMID 12907542. Unknown parameter |month= ignored (help)
  7. ↑ 7.0 7.1 Raghu, G.; Collard, HR.; Egan, JJ.; Martinez, FJ.; Behr, J.; Brown, KK.; Colby, TV.; Cordier, JF.; Flaherty, KR. (2011). "An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management". Am J Respir Crit Care Med. 183 (6): 788–824. doi:10.1164/rccm.2009-040GL. PMID 21471066. Unknown parameter |month= ignored (help)
  8. ↑ Walter, N.; Collard, HR.; King, TE. (2006). "Current perspectives on the treatment of idiopathic pulmonary fibrosis". Proc Am Thorac Soc. 3 (4): 330–8. doi:10.1513/pats.200602-016TK. PMID 16738197. Unknown parameter |month= ignored (help)
  9. ↑ Collard, HR.; Tino, G.; Noble, PW.; Shreve, MA.; Michaels, M.; Carlson, B.; Schwarz, MI. (2007). "Patient experiences with pulmonary fibrosis". Respir Med. 101 (6): 1350–4. doi:10.1016/j.rmed.2006.10.002. PMID 17107778. Unknown parameter |month= ignored (help)
  10. ↑ Daniels, CE.; Ryu, JH. (2006). "Treatment of idiopathic pulmonary fibrosis". Semin Respir Crit Care Med. 27 (6): 668–76. doi:10.1055/s-2006-957338. PMID 17195143. Unknown parameter |month= ignored (help)
  11. ↑ Holland, AE.; Hill, CJ.; Conron, M.; Munro, P.; McDonald, CF. (2008). "Short term improvement in exercise capacity and symptoms following exercise training in interstitial lung disease". Thorax. 63 (6): 549–54. doi:10.1136/thx.2007.088070. PMID 18245143. Unknown parameter |month= ignored (help)
  12. ↑ Ferreira, A.; Garvey, C.; Connors, GL.; Hilling, L.; Rigler, J.; Farrell, S.; Cayou, C.; Shariat, C.; Collard, HR. (2009). "Pulmonary rehabilitation in interstitial lung disease: benefits and predictors of response". Chest. 135 (2): 442–7. doi:10.1378/chest.08-1458. PMID 18849399. Unknown parameter |month= ignored (help)
  13. ↑ Nishiyama, O.; Kondoh, Y.; Kimura, T.; Kato, K.; Kataoka, K.; Ogawa, T.; Watanabe, F.; Arizono, S.; Nishimura, K. (2008). "Effects of pulmonary rehabilitation in patients with idiopathic pulmonary fibrosis". Respirology. 13 (3): 394–9. doi:10.1111/j.1440-1843.2007.01205.x. PMID 18399862. Unknown parameter |month= ignored (help)
  14. ↑ Salhi, B.; Troosters, T.; Behaegel, M.; Joos, G.; Derom, E. (2010). "Effects of pulmonary rehabilitation in patients with restrictive lung diseases". Chest. 137 (2): 273–9. doi:10.1378/chest.09-0241. PMID 19858229. Unknown parameter |month= ignored (help)
  15. ↑ Kozu, R.; Senjyu, H.; Jenkins, SC.; Mukae, H.; Sakamoto, N.; Kohno, S. (2011). "Differences in response to pulmonary rehabilitation in idiopathic pulmonary fibrosis and chronic obstructive pulmonary disease". Respiration. 81 (3): 196–205. doi:10.1159/000315475. PMID 20516666.
  16. ↑ Huppmann, P.; Sczepanski, B.; Boensch, M.; Winterkamp, S.; Schönheit-Kenn, U.; Neurohr, C.; Behr, J.; Kenn, K. (2013). "Effects of inpatient pulmonary rehabilitation in patients with interstitial lung disease". Eur Respir J. 42 (2): 444–53. doi:10.1183/09031936.00081512. PMID 23100507. Unknown parameter |month= ignored (help)
  17. ↑ Lancaster, LH.; Mason, WR.; Parnell, JA.; Rice, TW.; Loyd, JE.; Milstone, AP.; Collard, HR.; Malow, BA. (2009). "Obstructive sleep apnea is common in idiopathic pulmonary fibrosis". Chest. 136 (3): 772–8. doi:10.1378/chest.08-2776. PMID 19567497. Unknown parameter |month= ignored (help)
  18. ↑ Schachter, LM.; Dixon, J.; Pierce, RJ.; O'Brien, P. (2003). "Severe gastroesophageal reflux is associated with reduced carbon monoxide diffusing capacity". Chest. 123 (6): 1932–8. PMID 12796170. Unknown parameter |month= ignored (help)
  19. ↑ Lee, JS.; Collard, HR.; Raghu, G.; Sweet, MP.; Hays, SR.; Campos, GM.; Golden, JA.; King, TE. (2010). "Does chronic microaspiration cause idiopathic pulmonary fibrosis?". Am J Med. 123 (4): 304–11. doi:10.1016/j.amjmed.2009.07.033. PMID 20362747. Unknown parameter |month= ignored (help)
  20. ↑ Pashinsky, YY.; Jaffin, BW.; Litle, VR. (2009). "Gastroesophageal reflux disease and idiopathic pulmonary fibrosis". Mt Sinai J Med. 76 (1): 24–9. doi:10.1002/msj.20088. PMID 19170215. Unknown parameter |month= ignored (help)
  21. ↑ Raghu, G.; Yang, ST.; Spada, C.; Hayes, J.; Pellegrini, CA. (2006). "Sole treatment of acid gastroesophageal reflux in idiopathic pulmonary fibrosis: a case series". Chest. 129 (3): 794–800. doi:10.1378/chest.129.3.794. PMID 16537884. Unknown parameter |month= ignored (help)
  22. ↑ Sweet, MP.; Hoopes, C.; Golden, J.; Hays, S.; Leard, L.; Patti, M. (2006). "Prevalence of delayed gastric emptying and gastroesophageal reflux in patients with end-stage lung disease". Ann Thorac Surg. 82 (4): 1570, author reply 1570-1. doi:10.1016/j.athoracsur.2005.11.018. PMID 16996990. Unknown parameter |month= ignored (help)
  23. ↑ Lee, JS.; Ryu, JH.; Elicker, BM.; Lydell, CP.; Jones, KD.; Wolters, PJ.; King, TE.; Collard, HR. (2011). "Gastroesophageal reflux therapy is associated with longer survival in patients with idiopathic pulmonary fibrosis". Am J Respir Crit Care Med. 184 (12): 1390–4. doi:10.1164/rccm.201101-0138OC. PMID 21700909. Unknown parameter |month= ignored (help)
  24. ↑ Raghu, G.; Johnson, WC.; Lockhart, D.; Mageto, Y. (1999). "Treatment of idiopathic pulmonary fibrosis with a new antifibrotic agent, pirfenidone: results of a prospective, open-label Phase II study". Am J Respir Crit Care Med. 159 (4 Pt 1): 1061–9. doi:10.1164/ajrccm.159.4.9805017. PMID 10194146. Unknown parameter |month= ignored (help)
  25. ↑ 25.0 25.1 Azuma, A.; Nukiwa, T.; Tsuboi, E.; Suga, M.; Abe, S.; Nakata, K.; Taguchi, Y.; Nagai, S.; Itoh, H. (2005). "Double-blind, placebo-controlled trial of pirfenidone in patients with idiopathic pulmonary fibrosis". Am J Respir Crit Care Med. 171 (9): 1040–7. doi:10.1164/rccm.200404-571OC. PMID 15665326. Unknown parameter |month= ignored (help)
  26. ↑ Taniguchi, H.; Ebina, M.; Kondoh, Y.; Ogura, T.; Azuma, A.; Suga, M.; Taguchi, Y.; Takahashi, H.; Nakata, K. (2010). "Pirfenidone in idiopathic pulmonary fibrosis". Eur Respir J. 35 (4): 821–9. doi:10.1183/09031936.00005209. PMID 19996196. Unknown parameter |month= ignored (help)
  27. ↑ Noble, PW.; Albera, C.; Bradford, WZ.; Costabel, U.; Glassberg, MK.; Kardatzke, D.; King, TE.; Lancaster, L.; Sahn, SA. (2011). "Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials". Lancet. 377 (9779): 1760–9. doi:10.1016/S0140-6736(11)60405-4. PMID 21571362. Unknown parameter |month= ignored (help)
  28. ↑ Spagnolo, P.; Del Giovane, C.; Luppi, F.; Cerri, S.; Balduzzi, S.; Walters, EH.; D'Amico, R.; Richeldi, L. (2010). "Non-steroid agents for idiopathic pulmonary fibrosis". Cochrane Database Syst Rev (9): CD003134. doi:10.1002/14651858.CD003134.pub2. PMID 20824834.
  29. ↑ Okuda, R.; Hagiwara, E.; Baba, T.; Kitamura, H.; Kato, T.; Ogura, T. (2013). "Safety and efficacy of pirfenidone in idiopathic pulmonary fibrosis in clinical practice". Respir Med. 107 (9): 1431–7. doi:10.1016/j.rmed.2013.06.011. PMID 23849626. Unknown parameter |month= ignored (help)
  30. ↑ Azuma, A.; Nukiwa, T.; Tsuboi, E.; Suga, M.; Abe, S.; Nakata, K.; Taguchi, Y.; Nagai, S.; Itoh, H. (2005). "Double-blind, placebo-controlled trial of pirfenidone in patients with idiopathic pulmonary fibrosis". Am J Respir Crit Care Med. 171 (9): 1040–7. doi:10.1164/rccm.200404-571OC. PMID 15665326. Unknown parameter |month= ignored (help)
  31. ↑ Jackson, RM.; Glassberg, MK.; Ramos, CF.; Bejarano, PA.; Butrous, G.; Gómez-Marín, O. (2010). "Sildenafil therapy and exercise tolerance in idiopathic pulmonary fibrosis". Lung. 188 (2): 115–23. doi:10.1007/s00408-009-9209-8. PMID 20012639. Unknown parameter |month= ignored (help)
  32. ↑ Zisman, DA.; Schwarz, M.; Anstrom, KJ.; Collard, HR.; Flaherty, KR.; Hunninghake, GW.; de Andrade, J.; Anstrom, KJ.; Collard, HR. (2010). "A controlled trial of sildenafil in advanced idiopathic pulmonary fibrosis". N Engl J Med. 363 (7): 620–8. doi:10.1056/NEJMoa1002110. PMID 20484178. Unknown parameter |month= ignored (help)

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