EXPLAINER: Kris Aquino’s medical condition, EGPA

·6 min read
Kris Aquino (DO NOT USE)
The Philippines's "Queen of All Media" is currently in the United States as she undergoes treatment for her autoimmune disorders. She previously shared that she was diagnosed with chronic spontaneous urticaria, autoimmune thyroiditis, and Eosinophilic Granulomatosis with Polyangiitis. (Photo: Kris Aquino/Instagram)

The Philippines's "Queen of All Media", Kris Aquino, shared with her 4.8 million followers on Instagram on September 7 that she was diagnosed with a fourth autoimmune condition – with "physical manifestations [that] are pointing to a possible 5th."

She said in the caption of her post, "There have been times I wanted to give up because of fatigue and being forever bedridden; the bruises all over my body that suddenly appear; my inability (since February) to tolerate solid food; headaches; bone-deep pain in my spine, knees, and joints in my fingers; and my constant flares, especially in my face that just keeps getting worse.”

"I am grateful to be blessed to have the means for us to move to another state and have more tests done & go to other specialists, and finally start my immunosuppressant therapy,” she added. Her post has since received a lot of good wishes from her colleagues in the entertainment industry as well as supporters and fans.

She also shared that, soon, she may experience the effects of chemotherapy, such as hair loss. "I was warned that the safest form of chemotherapy that can be used for my autoimmune conditions will make me lose my hair. Hair will eventually grow back but permanently damaged organs won't."

Aquino is currently in Houston, Texas in the United States for her treatment and medication. Last April, she shared on social media that she was then diagnosed with late-stage 3 of Churg Strauss Syndrome, now known as EGPA or Eosinophilic Granulomatosis with Polyangiitis. Her two other autoimmune diseases at that time were autoimmune thyroiditis and chronic spontaneous urticaria. Her EGPA diagnosis came after her third skin biopsy was read by a pathologist from the Philippines and the US.

Since Aquino shared her diagnosis, various reports described EGPA to be a rare autoimmune illness, as it is. But not much has been said about what it is, what causes it, and just how rare it is exactly.

Yahoo Philippines sought the expert insights of Dr. Noriezel V. Arcigal-Trinidad, the Rheumatology Section Head of the Medical City in Ortigas, Pasig City, to know more about Aquino's autoimmune illness. As EGPA is not exactly a common medical condition, given its rarity, Dr. Trinidad’s expert insights may help shed light on this serious disorder.

Yahoo Philippines: What, exactly, is Eosinophilic Granulomatosis with Polyangiitis?

Dr. Trinidad: EGPA, formerly known as Churg-Strauss syndrome, is an extremely rare disorder marked by blood vessel inflammation that can restrict blood flow and, sometimes, cause permanent damage to organs and tissues.

Yahoo: How common or how rare is this condition?

Dr. Trinidad: EGPA is a very rare condition. It affects men and women equally at any age, though it is usually diagnosed between the ages of 35 and 50. The estimated global incidence of EGPA is 2.5 cases per 100,000 adults yearly. The incidence of EGPA in the Philippines is unknown since only a few cases were reported.

Yahoo: To which more common/popular medical condition can we relate EGPA, and how do they differ from each other?

Dr. Trinidad: EGPA can be related to a more common medical condition such as Systemic Lupus Erythematosus or Lupus. They are both autoimmune diseases that are caused when the body's natural defenses against viruses and bacteria begin to attack healthy tissue, causing widespread inflammation. Both disorders may affect many organs of the body; they have no cure and usually require lifelong treatment to manage symptoms.

They differ because Lupus occurs when the body's immune system attacks its tissues and organs, while EGPA is caused by an abnormal accumulation of a large number of antibodies, increased numbers of eosinophils indicating inflammatory response, inflammation of small and medium-sized vessels, and the development of inflammatory nodular lesions or granulomatosis within specific tissues and the walls of blood vessels.

Yahoo: What are the common factors that may lead to EGPA? Is this hereditary?

Dr. Trinidad: People diagnosed with EGPA usually have a history of asthma, chronic sinusitis, or allergies. It is not hereditary. The cause of EGPA is unknown, but it is classified as an autoimmune disease. Researchers believe that genetic and environmental factors, such as allergens and some medications, play a role in triggering an overactive immune system response.

Yahoo: What are the symptoms?

Dr. Trinidad: EGPA can affect blood vessels of different organs, so symptoms can vary from mild to severe life-threatening manifestations, depending on the affected organs. Almost all patients diagnosed with EGPA have asthma, chronic sinusitis, and a high level of white blood cells called "eosinophils."

Many patients diagnosed with EGPA may experience fever, fatigue, weight loss, muscle, and joint pains, skin rashes, numbness or tingling sensation of the hands and feet, chest pains, palpitations, cough, shortness of breath, abdominal pain, bloody stools or kidney disease. People who experience these symptoms should consult their doctors right away. Early detection and prompt treatment improve the chances of a favorable outcome.

Yahoo: What are the common treatments for EGPA? And how long does treatment take?

Dr. Trinidad: EGPA has no cure, and treatment help manage symptoms and prevent complications. EGPA is usually treated with corticosteroids, such as prednisone, to reduce inflammation. For more severe cases, corticosteroids are combined with immunosuppressive medications such as methotrexate, azathioprine, mycophenolate mofetil, and cyclophosphamide. These immunosuppressive drugs turn down the immune system's activity. Biologic medications, rituximab, benralizumab, and mepolizumab, are also used in treating EGPA. (Biologics are complex proteins from living organisms that target specific parts of the immune system to control inflammation.) Mepolizumab is currently the only drug approved by the U.S.

Food and Drug Administration to treat EGPA. It reduces the eosinophils in the body to control inflammation. (Eosinophils can help protect the body against infection, but overproduction can cause inflammation and damage organs and tissues). The goal of therapy is to stop all damage from EGPA, which is a condition called "remission."

The duration of maintenance of immunosuppressive drugs depends from person to person. It is usually given for one to two years before the doctor considers slowly tapering the dose of the medications and eventually stopping it.

Yahoo: If not treated, what could be the possible scenarios for the patient?

Dr. Trinidad: EGPA is a serious condition that could lead to organ damage and can be fatal if not treated. Complications depend on the organs involved, which include peripheral nerve damage, leading to numbness and burning sensation in the hands and feet, heart disease, which includes inflammation of the heart, heart attack, and heart failure and kidney damage.

Juju Z. Baluyot is a Manila-based writer who writes in-depth special reports, news features, and opinion-editorial pieces for a wide range of publications. He covers cultures, media, and gender.

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