Pemphigus

Disease ID:1237
Name:Pemphigus
Associated with:1 target
1 immuno-relevant target
2 immuno-relevant ligands
Description
A group of rare, chronic autoimmune diseases of the skin and connective tissue which are characterized by severe blistering of the skin and mucous membranes. The most common subtype is pemphigus vulgaris (PV).
Database Links
Disease Ontology: DOID:9182

Targets

CD20 (membrane-spanning 4-domains, subfamily A, member 1)
Comments:  CD20 is the molecular target of the antibody veltuzumab.
Ligand interactions: 
Ligand Comments
veltuzumab
FDA orphan drug for pemphigus.
rituximab
Rituximab is the first biologic therapy approved for pemphigus vulgaris (PV), and represents the first major PV therapeutic advance in more than 60 ye ...

Ligands

Key to terms and symbols Click ligand name to view ligand summary Click column headers to sort
Ligand References Clinical and Disease comments
veltuzumab
Immuno Disease Comments: FDA orphan drug for pemphigus.
Clinical Use: The EMA and FDA have granted veltuzumab orphan designation for the treatment of the rare disease chronic lymphocytic leukemia (CLL). The FDA also includes treatment of pemphigus (a group of autoimmune diseases causing severe blistering of the skin and mucous membranes) in its orphan drug designation. Phase II clinical trials for non-Hodgkin's lymphoma, CLL and autoimmune thrombocytopenic purpura are ongoing. | View clinical data
rituximab
Immuno Disease Comments: Rituximab is the first biologic therapy approved for pemphigus vulgaris (PV), and represents the first major PV therapeutic advance in more than 60 years.
Clinical Use: Used to treat CD20-positive non-Hodgkins lymphoma, chronic lymphocytic leukemia, and rheumatoid arthritis. Rituximab is also used to suppress antibody-mediated rejection in living-donor kidney recipients prior to an ABO-incompatible transplant [3-4]. A modified formulation containing rituximab + human hyaluronidase (Rituxan Hycela) that can be delivered subcutaneously (the original rituximab only formulation was administered intravenously) was FDA approved in June 2017 for the treatment of previously untreated and relapsed or refractory follicular lymphoma, previously untreated diffuse large B-cell lymphoma (DLBCL), and previously untreated and previously treated chronic lymphocytic leukemia (CLL). In June 2018, the FDA approved the use of rituximab as a much needed treatment for the potentially life-threatening skin disease pemphigus vulgaris that can be used for adults patients with moderate to severe disease. | View clinical data
Bioactivity Comments: The patents covering rituximab do not contain any data regarding antibody-antigen affinity [1-2], but a dissociation constant is provided by Stein et al (2004) [5]. | View biological activity

References

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1. Anderson DR, Nabil H, Leonard JE, Newman RA, Reff ME, Rastetter WH. (1998) Therapeutic application of chimeric and radiolabeled antibodies to human B lymphocyte restricted differentiation antigen for treatment of B cell lymphoma. Patent number: US5843439. Assignee: Anderson DR, Nabil H, Leonard JE, Newman RA, Reff ME, Rastetter WH. Priority date: 13/11/1992. Publication date: 01/12/1998.

2. Anderson DR, Rastetter WH, Hanna N, Leonard JE, Newman RA, Reff ME. (1994) Therapeutic application of chimeric and radiolabeled antibodies to human b lymphocyte restricted differentiation antigen for treatment of b cell lymphoma. Patent number: WO1994011026. Assignee: Idec Pharma Corp. Priority date: 26/02/2015. Publication date: 26/05/1994.

3. Fehr T, Stussi G. (2012) ABO-incompatible kidney transplantation. Curr Opin Organ Transplant, 17 (4): 376-85. [PMID:22790072]

4. Macklin PS, Morris PJ, Knight SR. (2015) A systematic review of the use of rituximab as induction therapy in renal transplantation. Transplant Rev (Orlando), 29 (2): 103-8. [PMID:25555541]

5. Stein R, Qu Z, Chen S, Rosario A, Shi V, Hayes M, Horak ID, Hansen HJ, Goldenberg DM. (2004) Characterization of a new humanized anti-CD20 monoclonal antibody, IMMU-106, and Its use in combination with the humanized anti-CD22 antibody, epratuzumab, for the therapy of non-Hodgkin's lymphoma. Clin. Cancer Res., 10 (8): 2868-78. [PMID:15102696]