Bay Biosciences provides high quality, clinical grade bio-samples, biopsy samples, cryogenically preserved sera (serum), plasma and peripheral blood mononuclear cells (PBMC) biofluid specimens from patients diagnosed with Granulomatosis with Polyangiitis (GPA) disease.
The sera (serum), plasma and peripheral blood mononuclear cells (PBMC) biofluid samples are processed from patient’s peripheral whole-blood using customized collection and processing protocols.
The Granulomatosis with Polyangiitis (GPA) disease bio-specimens are collected from unique patients diagnosed with Granulomatosis with polyangiitis (GPA) disease and are provided to a valued pharmaceutical customer for translational research, genomics, proteomics and biomarker research, drug discovery and development.
Granulomatosis with Polyangiitis (GPA) Disease Overview
Granulomatosis with Polyangiitis (GPA), formerly known as Wegener’s granulomatosis (WG), is a very rare autoimmune disorder that causes the inflammation of blood vessels in different parts of the body. Granulomatosis with Polyangiitis (GPA) is a long-term systemic disorder that involves the formation of granulomas and inflammation of blood vessels (vasculitis). It is a form of vasculitis that affects small- and medium-size vessels in many organs but most commonly affects the upper respiratory tract, lungs and kidneys. The signs and symptoms of GPA are highly varied and reflect which organs are supplied by the affected blood vessels. Typical signs and symptoms include nosebleed, stuffy nose and crustiness of nasal secretions, and inflammation of the uveal layer of the year. Damage to the heart, lungs and kidneys can be fatal. The cause of GPA is unknown, genetics have been found to play a role in GPA though the risk of inheritance appears to be low.
Granulomatosis with Polyangiitis (GPA) disease treatment depends on the severity of the disease. Severe disease is typically treated with a combination of immunosuppressive medications and high-dose corticosteroids to control the symptoms of the disease to keep the disease under control. Plasma exchange is also used in severe cases with damage to the lungs, kidneys, or intestines.
Granulomatosis with Polyangiitis (GPA) Disease Causes
The cause of Granulomatosis with Polyangiitis (GPA) is unknown, although microbes, such as bacteria and viruses, as well as genetics have been implicated in its pathogenesis. As with all autoimmune disorders, Granulomatosis with Polyangiitis (GPA) is characterized by an immune system gone awry. For reasons unknown, the body will mistakenly identify normal tissue in blood vessels as foreign. In order to contain the perceived threat, immune cells will surround the cells and form a hardened nodule known as a granuloma.
The formation of granulomas can lead to the development of chronic inflammation in the affected blood vessels a condition known as vasculitis. Over time, this can structurally weaken the vessels and cause them to burst, usually at the site of the granulomatous growths. It can also cause the blood vessels to harden and narrow, cutting the blood supply to key parts of the body. Granulomatosis with Polyangiitis (GPA) mainly affects small- to medium-sized blood vessels. While the respiratory tract, lungs, and kidneys are the main targets of attacks, GPA can also cause damage to the skin, joints, and nervous system. The heart, brain, and gastrointestinal tract are rarely affected. GPA affects men and women equally, primarily between the ages of 40 and 60.
Signs and Symptoms of Granulomatosis with Polyangiitis (GPA) Disease
Initial signs and symptoms of Granulomatosis with Polyangiitis (GPA) disease are highly variable, and diagnosis can be severely delayed due to the nonspecific nature of the symptoms. In general, irritation and inflammation of the nose are the first signs in most patients. Involvement of the upper respiratory tract, such as the nose and sinuses, is seen in nearly all people with Granulomatosis with Polyangiitis (GPA) disease. Typical signs and symptoms of nose or sinus involvement include crusting around the nose, stuffiness, nosebleeds, runny nose and saddle-nose deformity due to a hole in the septum of the nose. Inflammation of the outer layers of the eye, Scleritis, Episcleritis and Conjunctivitis are the most common signs of GPA in the eye; involvement of the eyes is common and occurs in slightly more than half of the patients with the GPA disease.
Signs and symptoms of Granulomatosis with Polyangiitis (GPA) as the disease progresses, other, more serious symptoms may develop, including:
The generalized nature of these symptoms can often make the diagnosis difficult. It is not uncommon, for example, for Granulomatosis with Polyangiitis (GPA) disease to be misdiagnosed and treated as a respiratory infection. It only when doctors can find no evidence of a viral or bacterial cause that further investigations may be ordered, particularly when there is evidence of vasculitis.
Systemic Symptoms
As a systemic disease, Granulomatosis with Polyangiitis (GPA) can cause injury one or several organ systems at once. While the location of symptoms can vary, the underlying cause (vasculitis) can usually point the doctor in the direction of an autoimmune diagnosis if multiple organs are involved.
Systemic symptoms of Granulomatosis with Polyangiitis (GPA) disease may include:
- The collapse of the nasal bridge due to a perforated septum (also known as the “saddle nose” deformity similar to that seen with long-term cocaine use)
- Tooth loss due to underlying bone destruction
- Sensorineural hearing loss caused by damage to the inner ear
- The development of granulomatous growths in parts of the eye
- Voice changes due to the narrowing of the trachea
- Blood in urine (hematuria)
- The rapid loss of kidney function leading to kidney failure
- A cough with bloody phlegm due to the formation of granulomatous lesions and cavities in the lungs
- Arthritis (often initially diagnosed as rheumatoid arthritis)
- The development of red or purple patches on the skin (purpura)
- Numbness, tingling, or burning sensations caused by nerve damage (neuropathy)
Granulomatosis with Polyangiitis (GPA) Disease Diagnosis
The diagnosis of Granulomatosis with Polyangiitis (GPA) disease is typically only made after several, unrelated symptoms go unexplained for a long period of time. While there are blood tests available to identify the specific autoantibodies associated with the disease, the presence (or lack) of antibodies is not enough to confirm (or reject) a diagnosis.
Instead, diagnoses are made based on the combination of symptoms, lab tests, X-rays, and the results of a physical examination. Other tools may be needed to support a diagnosis, including a biopsy of the affected tissue. A lung biopsy is usually the best place to start even if there are no respiratory symptoms. Biopsies of the upper respiratory tract, by contrast, tend to be the least helpful since 50 percent will show no signs of granulomas or tissue damage. Similarly, a chest X-ray or CT scan can often reveal lung abnormalities in persons with otherwise normal lung function. Together, the combination of tests and symptoms may be enough to support a Granulomatosis with Polyangiitis (GPA) disease diagnosis.
Detailed clinical data, ANCA positive test, biopsy elevated biomarker levels, genetic and metabolic information, histopathological findings, annotations associated with the Granulomatosis with Polyangiitis (GPA) disease patient’s specimens is provided to a valued customer for research, development and drug discovery. The Granulomatosis with Polyangiitis (GPA) disease sera (serum), plasma and PBMC biofluid samples are processed from patients peripheral whole-blood using customized collection and processing protocols provided by the researcher.
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