Method of Treating Pemphigus by Administering (R)-2-[3-[4-Amino-3-(2-Fluoro-4-Phenoxy-Phenyl)Pyrazolo[3,4-D]Pyrimidin-1-YL]Piperidine-1-Carbonyl]-4-Methyl-4-[4-(Oxetan-3-YL)Piperazin-1-YL]Pent-2-Enenitrile
Disclosed herein are methods for treating pemphigus in a human patient in need thereof comprising administering to the human patient a dose of at least 400 mg of (R)-2-[3-[4-amino-3-(2-fluoro-4-phenoxy-phenyl)pyrazolo[3,4-d]pyrimidin-1-yl]piperidine-1-carbonyl]-4-methyl-4-[4-(oxetan-3-yl)piperazin-1-yl]pent-2-enenitrile (PRN1008) once a day (QD) or twice a day (BID).
1 - 17 . (canceled)
18 . A method of treating pemphigus in a human patient in need thereof comprising administering to the human patient a dose of 400 mg of (R)-2-[3-[4-amino-3-(2-fluoro-4-phenoxy-phenyl)pyrazolo[3,4-d]pyrimidin-1-yl]piperidine-1-carbonyl]-4-methyl-4-[4-(oxetan-3-yl)piperazin-1-yl]pent-2-enenitrile (PRN1008) twice a day (BID) for at least 14 days, wherein the human patient is characterized by relapsing pemphigus prior to the administration of PRN1008.
19 . The method of claim 18 , comprising administering to the human patient a dose of 400 mg of PRN1008 BID for 14 to 84 days.
20 - 42 . (canceled)
43 . The method of claim 18 , comprising administering PRN1008 in combination with a first corticosteroid at a dose of less than or equal to 0.5 mg/kg/day.
44 . The method of claim 43 , wherein the first corticosteroid is chosen from prednisone, prednisolone, and methylprednisolone.
45 - 47 . (canceled)
48 . The method of claim 18 , wherein the human patient is characterized by a pemphigus disease activity index (PDAI) skin score from 8 points to 60 points prior to the administration of PRN1008.
49 . The method of claim 18 , wherein the human patient is characterized by a maintenance dose of less than or equal to 0.5 mg/kg/day of a second corticosteroid prior to the administration of PRN1008.
50 - 52 . (canceled)
53 . The method of claim 18 , wherein PRN1008 comprises the (E) isomer of (R)-2-[3-[4-amino-3-(2-fluoro-4-phenoxy-phenyl)pyrazolo[3,4-d]pyrimidin-1-yl]piperidine-1-carbonyl]-4-methyl-4-[4-(oxetan-3-yl)piperazin-1-yl]pent-2-enenitrile.
54 . The method of claim 18 , wherein PRN1008 comprises the (Z) isomer of (R)-2-[3-[4-amino-3-(2-fluoro-4-phenoxy-phenyl)pyrazolo[3,4-d]pyrimidin-1-yl]piperidine-1-carbonyl]-4-methyl-4-[4-(oxetan-3-yl)piperazin-1-yl]pent-2-enenitrile.
55 . The method of claim 18 , wherein PRN1008 comprises a mixture of (E) and (Z) isomers of (R)-2-[3-[4-amino-3-(2-fluoro-4-phenoxyphenyl)pyrazolo[3,4-d]pyrimidin-1-yl]piperidine-1-carbonyl]-4-methyl-4-[4-(oxetan-3-yl)piperazin-1 -yl]pent-2-enenitrile.
56 . The method of claim 18 , comprising treating pemphigus vulgaris.
57 . The method of claim 18 , comprising treating pemphigus foliaceus.
58 . The method of claim 18 , comprising reducing average daily corticosteroid usage by the human patient.
59 - 61 . (canceled)
62 . The method of claim 18 , comprising healing at least 50% of established pemphigus lesions.
63 . The method of claim 18 , comprising preventing new pemphigus lesion formation.
64 . The method of claim 18 , comprising reducing a pemphigus disease activity index (PDAI) skin score.
65 . (canceled)
66 . The method of claim 18 , wherein the human patient is characterized by a pemphigus disease activity index (PDAI) skin score of 0 or 1 following the administration of PRN1008.
67 - 68 . (canceled)
69 . The method of claim 18 , further comprising achieving a control of disease activity of pemphigus.
70 . The method of claim 18 , further comprising achieving an end of consolidation phase of pemphigus.
71 - 72 . (canceled)
73 . The method of claim 18 , wherein PRN1008 is orally administered to the human patient.
74 - 77 . (canceled)