What is the Eosinophilic Granulomatosis with Polyangiitis (EGPA) condition of Kris Aquino?

Kris Aquino has a rare auto-immune disease called Eosinophilic Granulomatosis with Polyangiitis (EGPA). Kris traveled to the United States in June. She stated in 2018 that she had been diagnosed with an autoimmune condition, and since then, she has been through different medical exams and therapies. 

What is this disease all about?

According to the website Allergology International,

https://www.jstage.jst.go.jp/article/allergolint/68/4/68_430/_article/-char/ja/

A rare form of anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis known as eosinophilic granulomatosis with polyangiitis (EGPA) (previously Churg-Strauss syndrome) is characterized by eosinophil-rich granulomatous inflammation and small to medium-size vessel vasculitis linked to bronchial asthma and eosinophilia. Research on EGPA has lagged behind other types of ANCA-associated vasculitis for a number of years due to its rarity and distinctive characteristics, such as eosinophilic inflammation.

Although EGPA patients often respond well to glucocorticoids, they frequently relapse. Currently, there isn't a proven treatment for EGPA, according to the results of numerous clinical trials. Mepolizumab was recently licensed for the treatment of EGPA after a randomized controlled trial demonstrated its therapeutic efficacy. Additionally, a number of fresh medications are being tested. Future research will be required to determine the best way to use these medications and to address unmet requirements, like relapse prevention.

Explain:

There is no proven treatment for EGPA, there is one licensed treatment Mepolizumab and it can be therapeutic after a controlled trial.  Steroids can be used but there is a relapse. 

The condition can harm key organs and can cause morbidity and mortality, This condition is bad for Kris, it can lead to mortality.

I hope Kris can survive her health problems. 

Let us pray for Kris recovery.

What is ANCA?

Antineutrophil cytoplasmic antibodies (ANCA) are antibodies that attack cytoplasmic components of neutrophils and monocytes (Lai, Leung, Rifkin and Lockwood, 1994). These antibodies are of major importance in detecting and classifying systemic vasculitides (Sinclair and Stevens, 2007),  like  eosinophilic granulomatosis with polyangiitis (eGPA, previously named Churg-Strauss Syndrome (CSS) (Sable-Fourtassou, Cohen, Mahr, Pagnoux, Mouthon, Jayne and French (2005). This is the condition of Kris Aquino. 

Different systemic forms of these vasculitides may harm key organs and cause significant morbidity and mortality as a result.

References: 

Lai, K. N., Leung, J. C., Rifkin, I., & Lockwood, C. M. (1994). Effect of anti-neutrophil cytoplasm autoantibodies on the intracellular calcium concentration of human neutrophils. Laboratory Investigation; a Journal of Technical Methods and Pathology, 70(2), 152-162.

Sinclair, D., & Stevens, J. M. (2007). Role of antineutrophil cytoplasmic antibodies and glomerular basement membrane antibodies in the diagnosis and monitoring of systemic vasculitides. Annals of clinical biochemistry, 44(5), 432-442.

Sablé-Fourtassou, R., Cohen, P., Mahr, A., Pagnoux, C., Mouthon, L., Jayne, D., ... & French Vasculitis Study Group*. (2005). Antineutrophil cytoplasmic antibodies and the Churg–Strauss syndrome. Annals of internal medicine, 143(9), 632-638.