# US 10,946,008 B2 · Treatment of pemphigus > Agents: this data is available as JSON from the patentgrep API and MCP server — see https://patentgrep.com/llms.txt - Number: US 10,946,008 B2 - Document ID: US10946008B2 - Status: Granted - Kind: B2 - Application number: 16/593,352 - Filed: 2019-10-04 - Published: 2020-04-02 - Granted: 2021-03-16 - Earliest claimed: 2014-12-18 - Assignees: Principia Biopharma Inc. - Applicants: Principia Biopharma Inc. - Inventors: Steven Gourlay - Web page: https://patentgrep.com/patent/US10946008B2 ## CPC - A61K 31/4545: containing a six-membered ring with nitrogen as a ring hetero atom, e.g. pipamperone, anabasine - A61K 31/4985: Pyrazines or piperazines ortho- or peri-condensed with heterocyclic ring systems - A61K 31/505: Pyrimidines; Hydrogenated pyrimidines, e.g. trimethoprim - A61K 31/519: ortho- or peri-condensed with heterocyclic rings - A61K 45/06: Mixtures of active ingredients without chemical characterisation, e.g. antiphlogistics and cardiaca - A61P 29/00: Non-central analgesic, antipyretic or antiinflammatory agents, e.g. antirheumatic agents; Non-steroidal antiinflammatory drugs [NSAID] - A61P 37/00: Drugs for immunological or allergic disorders - A61P 37/06: Immunosuppressants, e.g. drugs for graft rejection - A61K: PREPARATIONS FOR MEDICAL, DENTAL OR TOILETRY PURPOSES (devices or methods specially adapted for bringing pharmaceutical products into particular physical or administering forms A61J3/00; chemical aspects of, or use of materials for deodorisation of air, for disinfection or sterilisation, or for bandages, dressings, absorbent pads or surgical articles A61L; soap compositions C11D) - A61P: SPECIFIC THERAPEUTIC ACTIVITY OF CHEMICAL COMPOUNDS OR MEDICINAL PREPARATIONS ## Abstract The present disclosure provides methods of treating with a BTK inhibitor a blistering disease, in particular pemphigus vulgaris or pemphigus folliaceous in a mammal, use of a BTK inhibitor as a replacement therapy for corticosteroid therapy for diseases treatable with a corticosteroid, such as autoimmune or inflammatory disease and in particular where corticosteroids are used as first or second line therapy, and pharmaceutical formulations comprising the same. ## Claims ### Claim 1 A method of treating pemphigus vulgaris or pemphigus foliaceus in a mammal in need thereof comprising administering to the mammal a therapeutically effective amount of a BTK inhibitor (R,E)-2-(3-(4-amino-3-(2-fluoro-4-phenoxyphenyl)-1H-pyrazolo[3,4-d]-pyrimidin-1-yl)piperidine-1-carbonyl)-4,4-dimethylpent-2-enenitrile, wherein the mammal is not a human. ### Claim 2 (depends on 1) The method of claim 1, wherein the mammal is a dog. ### Claim 3 (depends on 1) The method of claim 1, wherein the mammal is a cat. ### Claim 4 (depends on 1) The method of claim 1, further comprising administering the BTK inhibitor in combination with a noncorticosteroidal immunosuppressive and/or anti-inflammatory agent. ### Claim 5 (depends on 4) The method of claim 4, wherein the noncorticosteroidal immunosuppressive and/or anti-inflammatory agent is chosen from anti-CD20 antibody, anti-TNF antibody, anti-IL6 agent, anti-IL17 agent, and abatacept. ### Claim 6 (depends on 4) The method of claim 4, wherein the noncorticosteroidal immunosuppressive and/or anti-inflammatory agent is chosen from rituximab, tositumomab, veltuzumab, obinutuzumab, Enbrel, and ofatumumab, or a biosimilar version thereof. ### Claim 7 A method of eliminating or reducing a therapeutic dose of a corticosteroid therapy used in the treatment of pemphigus vulgaris or pemphigus foliaceus in a mammal in need thereof, wherein the mammal is not a human and the corticosteroid therapy is used as the first or second line in chronic maintenance therapy, comprising administering to the mammal a therapeutically effective amount of a BTK inhibitor (R,E)-2-(3-(4-amino-3-(2-fluoro-4-phenoxyphenyl)-1H-pyrazolo[3,4-d]-pyrimidin-1-yl)piperidine-1-carbonyl)-4,4-dimethylpent-2-enenitrile. ### Claim 8 (depends on 7) The method of claim 7, wherein the mammal is a dog. ### Claim 9 (depends on 7) The method of claim 7, wherein the mammal is a cat. ### Claim 10 (depends on 7) The method of claim 7, further comprising administering the BTK inhibitor in combination with a noncorticosteroidal immunosuppressive and/or anti-inflammatory agent. ### Claim 11 (depends on 10) The method of claim 10, wherein the noncorticosteroidal immunosuppressive and/or anti-inflammatory agent is chosen from anti-CD20 antibody, anti-TNF antibody, anti-IL6 agent, anti-IL17 agent, and abatacept. ### Claim 12 (depends on 10) The method of claim 10, wherein the noncorticosteroidal immunosuppressive and/or anti-inflammatory agent is chosen from rituximab, tositumomab, veltuzumab, obinutuzumab, Enbrel, and ofatumumab, or a biosimilar version thereof. ### Claim 13 (depends on 7) The method of claim 7, wherein the BTK inhibitor is used in place of the corticosteroid therapy. ### Claim 14 (depends on 7) The method of claim 7, wherein the BTK inhibitor is used in combination with the corticosteroid therapy. ### Claim 15 A method of treating acute flares of pemphigus vulgaris or pemphigus foliaceus in a mammal in need thereof, wherein the mammal is not a human and a corticosteroid therapy is used as the first or second line in chronic maintenance therapy, comprising administering to the mammal a therapeutically effective amount of a BTK inhibitor (R,E)-2-(3-(4-amino-3-(2-fluoro-4-phenoxyphenyl)-1H-pyrazolo[3,4-d]-pyrimidin-1-yl)piperidine-1-carbonyl)-4,4-dimethylpent-2-enenitrile. ### Claim 16 (depends on 15) The method of claim 15, wherein the mammal is a dog. ### Claim 17 (depends on 15) The method of claim 15, wherein the mammal is a cat. ### Claim 18 (depends on 15) The method of claim 15, further comprising administering the BTK inhibitor in combination with a noncorticosteroidal immunosuppressive and/or anti-inflammatory agent. ### Claim 19 (depends on 18) The method of claim 18, wherein the noncorticosteroidal immunosuppressive and/or anti-inflammatory agent is chosen from anti-CD20 antibody, anti-TNF antibody, anti-IL6 agent, anti-IL17 agent, and abatacept. ### Claim 20 (depends on 18) The method of claim 18, wherein the noncorticosteroidal immunosuppressive and/or anti-inflammatory agent is chosen from rituximab, tositumomab, veltuzumab, obinutuzumab, Enbrel, and ofatumumab, or a biosimilar version thereof. ### Claim 21 (depends on 15) The method of claim 15, wherein the BTK inhibitor is used in place of the corticosteroid therapy. ### Claim 22 (depends on 15) The method of claim 15, wherein the BTK inhibitor is used in combination with the corticosteroid therapy.