Small molecule FIEL1 inhibitor in inflammatory and fibrotic lung injury

1. A method of treating a disease associated with an activated inflammatory pathway selected from a NF-kB, TGFβ and JAK/STAT pathway, the disease selected from pulmonary fibrosis, inflammatory bowel disease, and colitis, the method comprising administering to a subject in need thereof a therapeutically effective amount of a compound represented by Formula (I):

wherein:

R is

and

wherein:

W is selected from the group consisting of H, optionally substituted alkyl, optionally substituted alkoxy, optionally substituted aryl, optionally substituted cycloalkyl, optionally substituted heterocyclic, halogen, amino, and hydroxy;

X is selected from the group consisting of H, optionally substituted alkyl, optionally substituted alkoxy, optionally substituted aryl, optionally substituted cycloalkyl, optionally substituted heterocyclic, halogen, amino, and hydroxy;

Y is selected from the group consisting of H, optionally substituted alkyl, optionally substituted aryl, optionally substituted cycloalkyl, and optionally substituted heterocyclic;

Z is selected from the group consisting of H, optionally substituted alkyl, optionally substituted aryl, optionally substituted cycloalkyl, and optionally substituted heterocyclic, and wherein Y and Z optionally bind together to form a ring;

R′ is selected from the group consisting of H, optionally substituted alkyl, optionally substituted aryl, optionally substituted cycloalkyl, and optionally substituted heterocyclic;

R″ is independently selected from the group consisting of H, optionally substituted alkyl, optionally substituted aryl, optionally substituted cycloalkyl, and optionally substituted heterocyclic; and

wherein one or more of the alkyl, cycloalkyl, heterocycloalkyl, aryl or heteroaryl may be substituted by one or more C 1 -C 6 alkoxy, halogen or deuterium,

or a pharmaceutically acceptable salt thereof,

wherein the compound is capable of inhibiting NF-kB, TGFβ and/or JAK/STAT pathways, and

wherein the compound binds to FIEL1.

2. The method of claim 1 , wherein the therapeutically effective amount is about 0.1 to about 20 mg/kg/d.

3. The method of claim 1 , wherein the administration is oral, inhalation, intravenous, or intramuscular.

4. The method of claim 1 , wherein:

(a) W is selected from the group consisting of H, optionally substituted alkyl, and optionally substituted aryl;

(b) X is selected from the group consisting of H, optionally substituted alkyl, and optionally substituted aryl, and

(c) W and X cannot both be H.

5. The method of claim 1 , wherein:

(a) Y is selected from the group consisting of H, optionally substituted alkyl, and optionally substituted aryl,

(b) Z is selected from the group consisting of H, optionally substituted alkyl, and optionally substituted aryl, and

(c) Y and Z cannot both be H.

6. The method of claim 1 , wherein R′ is H.

7. The method of claim 1 , wherein R″ is H.

8. The method of claim 1 , wherein R is: