Safety and PK Study of BIBF 1120 in Japanese Patients With IPF

• Incidence and intensity of adverse events [ Time Frame: 2 weeks for cohort 1& 2 and 4 weeks for cohort 3 ] [ Designated as safety issue: No ]
• Withdrawal due to adverse events [ Time Frame: 2 weeks for cohort 1& 2 and 4 weeks for cohort 3 ] [ Designated as safety issue: No ]

• Laboratory tests (Hematology, Biochemistry, Urine, Stool). Data will be analysed both quantitatively as well as qualitatively. [ Time Frame: 2 weeks for cohort 1& 2 and 4 weeks for cohort 3 ] [ Designated as safety issue: No ]
• Lung function measurement: Spirometry: Forced vital capacity (FVC), Forced expiratory volume in 1second (FEV1) [ Time Frame: 2 weeks for cohort 1& 2 and 4 weeks for cohort 3 ] [ Designated as safety issue: No ]
• Lung function measurement: Diffusing capacity for carbon monoxide (DLCO) [ Time Frame: 2 weeks for cohort 1& 2 and 4 weeks for cohort 3 ] [ Designated as safety issue: No ]
• Vital signs (systolic and diastolic blood pressure and pulse rate). Data will be assessed with regard to possible changes compared to findings before start of treatment. [ Time Frame: 2 weeks for cohort 1& 2 and 4 weeks for cohort 3 ] [ Designated as safety issue: No ]

• Laboratory tests (Hematology, Biochemistry, Urine, Stool). Data will be analysed both quantitatively as well as qualitatively. [ Time Frame: 2 weeks for cohort 1& 2 and 4 weeks for cohort 3 ] [ Designated as safety issue: No ]
• Lung function measurement: Spirometry: Forced vital capacity (FVC), Foced expiratory volume in 1second (FEV), Diffusing capacity for carbon monoxide(DLco). [ Time Frame: 2 weeks for cohort 1& 2 and 4 weeks for cohort 3 ] [ Designated as safety issue: No ]
• Vital signs (systolic and diastolic blood pressure and pulse rate). Data will be assessed with regard to possible changes compared to findings before start of treatment. [ Time Frame: 2 weeks for cohort 1& 2 and 4 weeks for cohort 3 ] [ Designated as safety issue: No ]

To investigate safety of BIBF 1120 in Japanese patients with idiopathic pulmonary fibrosis (IPF), with and without pirfenidone background treatment.

To assess pharmacokinetics of BIBF 1120 in Japanese patients, with and without pirfenidone background treatment.

To assess pharmacokinetics of pirfenidone in Japanese patients, alone and in combination with BIBF 1120 treatment.

• Drug: Placebo
  Placebo BID for cohort 1,2,3
• Drug: BIBF 1120
  50 mg, 100 mg, 150 mg BID will be used for Cohort 1, 2, and 3 respectively

• Experimental: BIBF 1120 50 mg
  Low dose for cohort 1
  Intervention: Drug: BIBF 1120
• Experimental: BIBF 1120 100 mg
  Middle dose for cohort 2
  Intervention: Drug: BIBF 1120
• Experimental: BIBF 1120 150 mg
  High dose for cohort 3
  Intervention: Drug: BIBF 1120
• Placebo Comparator: Placebo
  Placebo for cohort 1,2,3
  Intervention: Drug: Placebo

Inclusion criteria:

1. Diagnosis of idiopathic pulmonary fibrosis (IPF) according to American Thoracic Society (ATS) /European Respiratory Society (ERS) guideline
2. Forced vital capacity (FVC) 50-90%
3. Diffusing capacity for carbon monoxide (DLCO) 30-79%
4. For patients on pirfenidone, have been on a steady dose for at least 3 months

Exclusion criteria:

1. Aspartate aminotransferase (AST), alanine aminotransferase (ALT) > 1.5 x upper limit of normal range (ULN) at screening.
2. Bilirubin > 1.5 x ULN at screening.
3. Relevant airways obstruction (i.e. pre-bronchodilator FEV1/FVC <0.7) at screening.
4. Continuous oxygen supplementation.
5. Active infection at screening or randomisation.

6. Being treated with any of the following concomitant medications.

   • Oral corticosteroid medication at unstable dose
   • ketoconazole or atazanavir
7. Patients who are expected to go on to lung transplantation, have rapidly deteriorating disease, or have a life expectancy less than 3 months from screening