Wegener's Granulomatosis, or GPA, is an autoimmune disorder marked by the vasculitis inflammation of small to medium-sized blood vessels. Protheragen, a leading provider of research services, specializes in the development of therapeutics for complex autoimmune ophthalmic diseases like GPA, leveraging advanced scientific techniques and extensive industry experience.
Overview of Wegener's Granulomatosis
Wegener's Granulomatosis, which occurs as a manifestation of Granulomatosis with Polyangiitis (GPA), an autoimmune disorder is marked by inflammation of the blood vessels of small to medium diameter, including the eyes and the surrounding structures. GPA, in the past, referred to as Wegener's Granulomatosis, rest is classified as a systemic necrotizing vasculitis which primarily affects the upper and lower respiratory tracts along with the kidneys, although other organs such as the eyes may also be involved. Involvement of the eyes in GPA can cause some symptoms that are as mild as mere discomfort, or as severe as loss of vision and life-threatening complications.
Fig.1 A case analysis of diagnostic test results for Wegener's granulomatosis. (Mirescu A. E.,
et al., 2022)
Diagnostics Development for Wegener's Granulomatosis
- Laboratory Diagnostics: For GPA, the laboratory diagnostics procedures include testing for ANCA which is fundamental in the diagnostics processes. Generally, two forms of ANCA are tested; these are c-ANCA (cytoplasmic pattern, usually PR3) and p-ANCA (perinuclear pattern, usually MPO). Common techniques include enzyme linked immunosorbent assays (ELISA) and indirect immunofluorescence. Other laboratory tests include complete blood count, tests of renal function, and urinalysis for evidence of glomerulonephritis.
- Imaging Studies: Radiological imaging using CT scans can show signs like bony destruction of the nasal cavity, maxillary sinuses, and mastoid air cells along with thickening of mucosal layers and sclerosing osteitis.
Therapeutics Development for Wegener's Granulomatosis
- Induction Therapy
High-dose immunosuppression to control disease activity is employed during induction therapy for Granulomatosis with Polyangiitis (GPA). Such therapy commonly uses cyclophosphamide, an alkylating agent used since the 1970s, and rituximab, a monoclonal antibody selective for CD20 on B cells. GPA also less commonly employs methotrexate, an antimetabolite, in cases of less severe or limited disease.
- Maintenance Therapy
The purpose of maintenance therapy is to sustain remission and avert relapses. Usual maintenance therapies consist of using the less harmful immunosuppressant, azathioprine, in comparison to long-term cyclophosphamide, as well as methotrexate which is administered after cyclophosphamide induction. Rituximab is under investigation for use in maintenance therapy and has been effective in reducing relapse rates.
Our Services
For Protheragen, the development of diagnostics and therapeutics for GPA encompasses a complete set of customizable services. This includes, but is not limited to, laboratory testing, imaging studies, histopathological evaluations, and preclinical evaluations of proprietary drugs and novel therapies.
Diagnostics Development
- Karyotype Analysis Service
- Omics Analysis Service
- Biomarker Development Service
- Artificial Intelligence Service
Therapeutic Development
- Small Molecule Drug
- Cell Therapy
- Gene Therapy
- Therapeutic Antibody
- Therapeutic Peptide
- Therapeutic Protein
Preclinical Research
- Pharmacodynamics Study Services
- Pharmacokinetics Study Services
- Drug Safety Evaluation Services
Disease Models
- Myeloperoxidase Knockout Mouse Models
- Proteinase-3 (PR3) Transgenic Mouse Models
- Adoptive Transfer Models
- Zebrafish Models
- Non-Human Primate Models
Protheragen's preclinical research services are designed to support the development of new therapeutics for GPA. Our services include preclinical testing of novel induction and maintenance therapies, evaluation of adjuvant therapies, and development of diagnostic assays. If you are interested in our services, please feel free to contact us.
References
- Mirescu, Andrada-Elena, et al. "Wegener's granulomatosis with orbital involvement: Case report and literature review." Romanian Journal of Ophthalmology 65.1 (2021): 93.
- Erickson, Vanessa R., and Peter H. Hwang. "Wegener's granulomatosis: current trends in diagnosis and management." Current opinion in otolaryngology & head and neck surgery 15.3 (2007): 170-176.
- Lutalo, Pamela MK, and David P. D'Cruz. "Diagnosis and classification of granulomatosis with polyangiitis (aka Wegener's granulomatosis)." Journal of autoimmunity 48 (2014): 94-98.
