Telitacicept combo works to treat severe GPA in man, 64: Report

Triple therapy rapidly controlled patient's autoimmune and kidney diseases

Andrea Lobo, PhD avatar

by Andrea Lobo, PhD |

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A 64-year-old man with severe granulomatosis with polyangiitis (GPA) saw his condition improve with the use of a triple combination of standard cyclophosphamide and glucocorticoids along with the experimental therapy telitacicept, according to a case report from China.

RemeGen’s telitacicept is approved in China under the brand name Tai’ai for another autoimmune disease called lupus. It’s not approved in the U.S. for any indication, and this case is the first to test its use as a combination treatment for severe GPA.

In their report, the researchers suggest that telitacicept may be beneficial for people with severe GPA and other types of ANCA-associated vasculitis (AAV) when combined with standard treatment.

“However, more clinical studies are needed to confirm the therapeutic effect of telitacicept,” the team wrote.

The case report, “Combination treatment with telitacicept, cyclophosphamide and glucocorticoids for severe Granulomatous polyangiitis: a case report and literature review,” was published in the journal Frontiers in Immunology.

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Telitacicept thought to reduce levels of ANCA antibodies

GPA is a type of AAV, a group of autoimmune conditions characterized by the inflammation of small blood vessels in various organs and tissues that leads to damage.

AAV typically occurs when self-reactive antibodies, called ANCAs, attach to a type of immune cells called neutrophils, overactivating them and ultimately causing blood vessel inflammation.

This disease type typically affects the upper respiratory tract, specifically the ears, nose, and trachea, and the lungs. It some patients, it also can involve the kidneys — particularly in cases of glomerulonephritis, a condition in which inflammation affects the tiny filters, called glomeruli, inside these organs, which remove waste products from the blood and produce urine.

Standard AAV treatment consists mainly of immunosuppressive agents such as glucocorticoids and cyclophosphamide (sold under the brand name Cytoxan in the U.S. and with generics available).

Telitacicept is a lab-made, fully-human protein that blocks key proteins involved in the survival and maturation of B-cells, the type of immune cell that produces antibodies, including ANCAs. Therefore, the therapy may reduce ANCA levels, easing AAV symptoms.

Here, researchers in Shenzhen detailed the case of an older man with GPA and severe kidney involvement, whose condition was rapidly controlled with a treatment combination of telitacicept and conventional medications.

The man was admitted to the hospital due to hearing loss that developed three months prior, cough for the previous two months, and an increase in the levels of creatinine — an indicator of kidney damage — two weeks before. He also had lost 10 kg (about 22 lbs) in the previous two months, and was a heavy smoker for 30 years.

Upon admission, he was found to be overweight, but had normal vital signs. A cardiopulmonary examination showed no obvious abnormalities.

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Case suggests triple combo may be ‘effective and safe’ for severe GPA

Blood work showed the man had higher than normal levels of immune cells and low levels of hemoglobin, which is the protein in red blood cells that carries oxygen in the blood. A urine analysis revealed excessive protein levels, as well as high urea and creatinine levels, which are indicative of kidney disease.

Further evaluation revealed nose inflammation, an ear infection, and the presence of lung nodules and kidney lesions, all indicating glomerulonephritis. The man also was positive for ANCAs targeting the myeloperoxidase protein, one of the two main targets of these self-reactive antibodies.

These findings indicated a diagnosis of GPA with progressive glomerulonephritis. The man was initially treated with 500 mg of intravenous or into-the-vein methylprednisolone, a glucocorticoid, once daily. This was combined with the intravenous administration of healthy antibodies that are used to neutralize self-reactive antibodies.

After three days, the glucocorticoid was changed to 50 mg of oral prednisone once daily, which was reduced by 5 mg per week. He also received cyclophosphamide at a cumulative monthly dose of 1.2 g, and telitacicept at 160 mg once a week.

The patient’s hearing gradually returned to normal in the three following months. Also, his kidney function improved, as shown by a creatinine level reduction. Markers of inflammation also were reduced, as were the man’s lung lesions.

Our case report suggests that telitacicept in combination with [cyclophosphamide] and [glucocorticoids] may be an effective and safe treatment for severe GPA.

In the first month of treatment, he developed an upper respiratory tract infection that responded to proper medication.

“Based on our review of the available literature, this is the first case reported in the literature in which telitacicept was applied in combination with [cyclophosphamide] and [glucocorticoids] for the treatment of severe GPA,” the researchers wrote.

“Our case report suggests that telitacicept in combination with [cyclophosphamide] and [glucocorticoids] may be an effective and safe treatment for severe GPA,” they concluded.