A Pilot Trial of Herpesvirus Treatment in Idiopathic Pulmonary Fibrosis (IPF)

Inclusion Criteria:

1. age >21 and <80 years

2. ability to provided informed consent

3. diagnosis of probable or definite IPF according to American Thoracic Society (ATS)
criteria

4. tolerance of full-dose (2403 mg/day) pirfenidone

5. Positive serology for EBV or CMV

Exclusion Criteria:

1. FVC < 40% predicted

2. Diffusing capacity for carbon monoxide (DLCO) < 35% predicted (Crapo)

3. Forced expiratory volume (FEV)1/FVC <0.7

4. Significant centrilobular emphysema (>40% by HRCT)

5. Active tobacco use (cigarette or cigar smoking)

6. Resting oxygen saturation (SpO2) on room air <89%

7. Listed for lung transplantation defined as being assigned a lung allocation score

8. environmental exposure (occupational, environmental, drug, etc.) felt by the principal
investigator (PI) to be the etiology of the interstitial disease

9. diagnosis of collagen-vascular conditions (according to the published American College
of Rheumatology criteria)

10. history of unstable or deteriorating cardiac disease

11. acute coronary syndrome, coronary artery bypass, or angioplasty within 3 months of
screening

12. uncontrolled arrhythmia

13. uncontrolled hypertension

14. known HIV or hepatitis C

15. known cirrhosis or chronic active hepatitis

16. active substance or alcohol abuse

17. pregnancy or lactation

18. Women of childbearing potential who are not using a medically approved means of
contraception. Subjects will be considered of childbearing potential if they are not
surgically sterile or have not been postmenopausal for at least 2 years [any subject
who is postmenopausal for < 2 years will be required to have a follicle-stimulating
hormone (FSH) level to assess her potential to become pregnant

19. clinically relevant lab abnormalities (obtained within 30 days before enrollment),
including:

1. creatinine > 2 x upper limit of normal (ULN)

2. hematology outside of specified limits: white blood cells (WBCs) < 3,500/mm3;
hematocrit < 25% or > 59%; platelets < 100,000/mm3;

3. total bilirubin > 2 x ULN

4. Aspartate (AST) or alanine aminotransferases (ALT)/ serum glutamic-oxaloacetic;
transaminase (SGOT), or serum glutamic pyruvic transaminase (SGPT) > 2.0 x ULN

5. alkaline phosphatase > 3 x ULN

6. albumin < 3.0 mg/dL at screening

20. known hypersensitivity to study medication

21. any condition that, in the judgment of the PI, might cause participation in this study
to be detrimental to the subject or that the PI deems makes the subject a poor
candidate

22. any therapy with immunosuppressants such as prednisone, azathioprine, or mycophenolate
currently or anticipated to be needed during the study period (subjects on these drugs
prior to the study will require a 30-day washout period before randomization)

23. participation in another IPF clinical treatment trial during the study period (if
completing another IPF clinical treatment trial, then a 30-day washout period is
required before randomization)

24. requirement for chronic suppressive therapy with valacyclovir for recurrent herpes
virus infection

25. History of myelodysplasia, aplastic anemia, refractory anemia, or multiple myeloma.