# US 2025/0084167 A1 · METHODS AND COMPOSITIONS FOR TREATING IgG4-RELATED DISEASES

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- Number: US 2025/0084167 A1
- Document ID: US20250084167A1
- Status: Published application
- Kind: A1
- Application number: 18/960,561
- Filed: 2024-11-26
- Published: 2025-03-13
- Earliest claimed: 2022-06-03
- Assignees: Zenas BioPharma, Inc.
- Applicants: Zenas BioPharma, Inc.
- Inventors: Allen POMA; Shauna QUINN; Xiaodong WANG
- Web page: https://patentgrep.com/patent/US20250084167A1

## CPC

- C07K 16/2803: {against the immunoglobulin superfamily}
- A61K 39/395: Antibodies (agglutinins A61K38/36 {; as drug carriers A61K47/50}); Immunoglobulins; Immune serum, e.g. antilymphocytic serum
- A61P 37/02: Immunomodulators
- A61P 37/06: Immunosuppressants, e.g. drugs for graft rejection
- A61K 2039/505: {comprising antibodies}
- A61K 2039/54: {characterised by the route of administration}
- A61K 2039/545: {characterised by the dose, timing or administration schedule}
- C07K: PEPTIDES (peptides in foodstuffs A23; obtaining protein compositions for foodstuffs, working-up proteins for foodstuffs A23J; preparations for medicinal purposes A61K; peptides containing beta-lactam rings C07D; cyclic dipeptides not having in their molecule any other peptide link than those which form their ring, e.g. piperazine-2,5-diones, C07D; ergot alkaloids of the cyclic peptide type C07D519/02; macromolecular compounds having statistically distributed amino acid units in their molecules, i.e. when the preparation does not provide for a specific; but for a random sequence of the amino acid units, homopolyamides and block copolyamides derived from amino acids C08G69/00; macromolecular products derived from proteins C08H1/00; preparation of glue or gelatine C09H; single cell proteins, enzymes C12N; genetic engineering processes for obtaining peptides C12N15/00; compositions for measuring or testing processes involving enzymes C12Q; investigation or analysis of biological material G01N33/00)
- 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 invention provides compositions and methods of treating and improving the symptoms of IgG4-RD using an antibody or antigen-binding fragment thereof that specifically binds human CD19.

## Claims

### Claim 1

A method of treating IgG4-related disease (IgG4-RD), comprising administering obexelimab subcutaneously to a human patient at least 18 years of age at a dose of 250 mg once a week.

### Claim 2 (depends on 1)

The method of claim 1, wherein the obexelimab is administered as 2×1 mL injections or 1×2 mL injection.

### Claim 3 (depends on 1, 2)

The method of claim 1 or 2, wherein the obexelimab is administered in a liquid formulation comprising 125 mg/mL obexelimab, 2.35 mg/mL sodium acetate trihydrate, 0.17 mg/mL acetic acid, 30 mg/mL L-proline, 0.1 mg/mL polysorbate 80 at pH 5.5.

### Claim 4

The method of any one of the preceding claims, wherein the human patient has received glucocorticoid (GC) therapy.

### Claim 5 (depends on 4)

The method of claim 4, wherein the GC therapy is administered at a dose of 20-60 mg/day prednisone or equivalent.

### Claim 6 (depends on 4, 5)

The method of claim 4 or 5, wherein the GC therapy continues during the treatment of obexelimab.

### Claim 7 (depends on 4, 5)

The method of claim 4 or 5, wherein the GC therapy is tapered.

### Claim 8 (depends on 7)

The method of claim 7, wherein the GC therapy is tapered to complete discontinuation.

### Claim 9 (depends on 1, 2, 3)

The method of any one of claims 1-3, wherein the obexelimab is administered in combination with a GC therapy.

### Claim 10

The method of claim any one of the preceding claims, wherein obexelimab is administered for a time period sufficient to improve, stabilize or reduce one or more symptoms of IgG4-RD relative to a control.

### Claim 11 (depends on 10)

The method of claim 10, wherein the at least one symptom is exhibited in an organ selected from lymph nodes, submandibular glands, parotid glands, lacrimal glands, kidney, heart, pericardium, orbit, nasal cavity, lungs, liver or bile ducts, salivary glands, and pancreas.

### Claim 12

The method of any one of the proceeding claims, wherein obexelimab is administered to the patient to improve clinical symptoms.

### Claim 13

The method of any one of the preceding claims, wherein obexelimab is administered to the human patient during a recurrence-free period to prevent relapse.

### Claim 14 (depends on 13)

The method of claim 13, wherein the recurrence-free period is determined by the days to disease flare.

### Claim 15 (depends on 14)

The method of claim 14, wherein the recurrence-free period is at least 60 days, 70 days, 80 days, or 90 days from the first administration of obexelimab.

### Claim 16 (depends on 15)

The method of claim 15, wherein the recurrence-free period is up to 40 weeks, up to 45 weeks, up to 50 weeks, up to 52 weeks from the first administration of obexelimab.

### Claim 17

The method of any one of the proceeding claims, wherein obexelimab is administered to the patient until the patient has active IgG4-RD disease flare that requires initiation of rescue therapy.

### Claim 18 (depends on 17)

The method of claim 17, wherein the rescue therapy comprises administration of GC therapy.

### Claim 19

The method of any of the preceding claims, wherein the human patient is relapsed or refractory to a previous treatment for IgG4-RD.

### Claim 20 (depends on 19)

The method of claim 19, wherein the human patient is relapsed or refractory to rituximab.

### Claim 21

The method of any of one of the preceding claims, wherein the human patient is assessed for disease activity using the IgG4-RD responder index (RI).

### Claim 22

The method of any of one of the preceding claims, wherein the human patient achieves a ≥2 points decrease in IgG4-RD RI from day 1 of administration with obexelimab.

### Claim 23

The method of any of one of the preceding claims, wherein the human patient has American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) classification criteria for IgG4-RD score (IgG4-RD score) ≥20 prior to administration of obexelimab.

### Claim 24

The method of any one of the preceding claims, wherein the human patient presents with IgG4-RD manifestation selected from the group consisting of IgG4-related sialadenitis (chronic sclerosing sialadenitis, Kuttner's tumour, Mikulicz's disease), IgG4-related dacryoadenitis (Mikulicz's disease), IgG4-related ophthalmic disease (idiopathic orbital inflammatory disease, orbital pseudotumor), chronic sinusitis IgG4-related hypophysitis (IgG4-related panhypophysitis, IgG4-related adenohypophysitis, IgG4-related infundibuloneurohypophysitis, autoimmune hypophysitis), IgG4-related pachymeningitis, IgG4-related leptomeningitis (idiopathic hypertrophic pachymeningitis), IgG4-related pancreatitis (Type 1 autoimmune pancreatitis, IgG4-related AIP, lymphoplasmacytic sclerosing pancreatitis, chronic pancreatitis with diffuse irregular narrowing of the main pancreatic duct), IgG4-related lung disease (Pulmonary inflammatory pseudotumour), IgG4-related pleuritis, IgG4-related hepatopathy, IgG4-related sclerosing cholangitis, IgG4-related cholecystitis, IgG4-related aortitis (inflammatory aortic aneurysm), IgG4-related periaortitis (chronic periaortitis), IgG4-related periarteritis, IgG4-related pericarditis, IgG4-related mediastinitis (fibrosing mediastinitis), IgG4-related retroperitoneal fibrosis (retroperitoneal fibrosis, Albarran-Ormond syndrome, Ormond's disease (retroperitoneal fibrosis), perirenal fasciitis, Gerota's fasciitis/syndrome, periureteritis fibrosa, sclerosing lipogranuloma, sclerosing retroperitoneal granuloma, non-specific retroperitoneal inflammation, sclerosing retroperitonitis, retroperitoneal vasculitis with perivascular fibrosis), IgG4-related mesenteritis (subtypes are: mesenteric panniculitis, mesenteric lipodystrophy and retractile mesenteritis) (sclerosing mesenteritis, systemic nodular panniculitis, liposclerosis mesenteritis, mesenteric Weber-Christian disease, mesenteric lipogranuloma, xanthogranulomatous mesenteritis), IgG4-related mastitis (sclerosing mastitis), IgG4-related kidney disease (IgG4-RKD), IgG4-related tubulointerstitial nephritis (IgG4-TIN), IgG4-related membranous glomerulonephritis (idiopathic tubulointerstitial nephritis), IgG4-related prostatitis, IgG4-related perivasal fibrosis (chronic orchialgia), IgG4-related paratesticular pseudotumor, IgG4-related epididymo-orchitis (paratesticular fibrous pseudotumor, inflammatory pseudotumor of the spermatic cord, pseudosarcomatous myofibroblastic proliferations of the spermatic cord, proliferative funiculitis, chronic proliferative periorchitis, fibromatous periorchitis, nodular periorchitis, reactive periorchitis, fibrous mesothelioma), IgG4-related lymphadenopathy, IgG4-related skin disease (angiolymphoid hyperplasia with eosinophilia, cutaneous pseudolymphoma), IgG4-related perineural disease, and IgG4-related thyroid disease (Reidel's thyroiditis), inflammatory pseudotumour, and multifocal fibrosclerosis.

### Claim 25

The method of anyone of the preceding claims, wherein the human patient presents an IgG4-RD manifestation selected from the group consisting of autoimmune pancreatitis (lymphoplasmacytic scleorising pancreatitis), eosinophilic angiocentric fibrosis (affecting the orbits and upper respiratory tract), fibrosing mediastinitis, idiopathic hypertrophic pachymeningitis, idiopathic tubulointerstitial nephritis, inflammatory pseudotumour, Kuttner's tumour, Mikulicz's disease, fibrosclerosis, periaortitis, periarteritis, inflammatory aortic multifocal aneurysm, Ormond's disease (tetroperitoneal fibrosis), Riedel's thyroiditis, and sclerosing mesenteritis.