Autoimmune hemolytic anemia (AIHA) is a rare autoimmune disorder that occurs when the body makes antibodies against its own red blood cells (RBCs). This results in fewer RBCs circulating in the body, causing anemia, which reduces blood’s ability to carry oxygen around the body. If your heart needs more oxygen than your blood can provide, you may experience chest pain, sometimes known as angina.

How Does AIHA Cause Chest Pain?

People living with AIHA may experience a wide range of symptoms, including chest pain. If you have chest pain, you may experience a feeling of tightness, heaviness, or pressure in the chest or, perhaps, feel like you have indigestion.

When the body isn’t getting enough oxygen, the heart tries to make up for it by working harder. If anemia prevents your blood from carrying enough oxygen, you may experience heart-related symptoms such as:

• Chest pain
• Rapid heartbeat
• Irregular heartbeat
• Palpitations (racing, pounding, or fluttering heartbeat)
• Heart failure

To understand how AIHA can cause chest pain, it helps to know about the types of AIHA.

What Is AIHA?

AIHA is an autoimmune disease. If you have AIHA, your body makes autoantibodies to red blood cells. Autoantibodies are antibodies — proteins made by the immune system — that recognize your own tissue instead of foreign invaders. In AIHA, the autoantibodies destroy RBCs in a process called hemolysis.

RBCs carry oxygen to all the tissues in your body through your bloodstream using a protein called hemoglobin. AIHA can cause low oxygen in the blood, also known as hypoxemia, by attacking RBCs.

Common symptoms of AIHA are related to anemia and low oxygen in the blood. They include:

• Weakness or tiredness
• Shortness of breath
• Pale skin
• Jaundice (yellowing of the skin or eyes)
• Dark urine
• Headaches
• Nausea and vomiting
• Diarrhea

Primary AIHA vs. Secondary AIHA

Symptoms can vary based on the cause and type of AIHA. If there is no identifiable cause, the condition is known as idiopathic or primary AIHA. If AIHA is associated with a related condition, such as blood cancer or a viral illness, it is known as secondary AIHA.

Types of AIHA

There are two types of AIHA — warm AIHA and cold AIHA. Warm AIHA, the most common type, involves antibodies known as immunoglobulin (Ig) G antibodies that identify RBCs for destruction at normal body temperatures.

Cold AIHA is less common than warm AIHA, affecting about 10 percent to 20 percent of people with AIHA. As the name suggests, autoantibodies in people with cold AIHA target RBCs only at colder temperatures. There are two types of cold AIHA — cold agglutinin disease and paroxysmal cold hemoglobinuria. Cold agglutinin disease involves a different antibody, IgM, which attaches to RBCs at cold temperatures, forming what is known as cold agglutinins and causing hemolysis (destruction of red blood cells). In people with paroxysmal cold hemoglobinuria, IgG antibodies bind to RBCs at cold temperatures and then cause hemolysis at warmer temperatures.

Paroxysmal nocturnal hemoglobinuria is a type of immune hemolytic anemia in which the bone marrow makes RBCs that are more likely to be destroyed by the immune system.

People with any type of AIHA can experience chest pain. This symptom is most commonly associated with cold AIHA, but in a small study, 9 percent of people with primary warm AIHA and 5 percent of those with secondary warm AIHA experienced chest pain.

Conditions Associated With AIHA That May Cause Chest Pain

Evidence shows that anemia can worsen existing heart conditions that may cause chest pain.

The additional stress placed on the heart from working harder to pump oxygen may make heart conditions (such as congestive heart failure) worse. People with severe anemia may retain more sodium and fluid, which also occurs with heart failure. Low hemoglobin in people with heart failure is associated with an increased risk of hospitalization and death.

AIHA can also raise the risk of developing blood clots. Studies show that people with AIHA have a 2.6-fold higher risk of developing a blood clot in the veins (venous thromboembolism) than those without AIHA. Additionally, almost half of all people with paroxysmal nocturnal hemoglobinuria experience at least one blood clotting event. If the blood clot moves to the lung or heart, it can cause chest pain.

Managing Chest Pain

A common medication used to treat chest pain due to reduced blood flow to the heart is nitroglycerin (Nitrostat). Nitroglycerin relaxes the blood vessels around the heart, allowing blood to flow more easily but should not be used in people with severe anemia.

When chest pain is a symptom of AIHA, it’s important to manage AIHA — the underlying cause. When anemia is improved, the heart muscle will get the oxygen it needs, and chest pain should get better.

AIHA Treatment

Treatment options for AIHA include immunosuppressive therapy, removal of the spleen (splenectomy), and blood transfusion.

The first-line therapy for AIHA is corticosteroids. These immunosuppressants reduce the activity of the immune system to limit the damage caused by autoantibodies. If corticosteroids aren’t effective, your doctor may recommend a splenectomy or a medication called rituximab (Rituxan). In severe cases, a blood transfusion may be needed.

Talk With Your Doctor About Chest Pain

It’s important to uncover the cause of chest pain. Talk to your health care provider if you experience this symptom. Chest pain can be caused by many conditions other than AIHA, including:

• Heart disease
• Digestive issues
• Muscle or bone disorders
• Lung disease
• Panic attacks

Chest pain can be life-threatening when it is a sign of a heart attack. Call 911 and go to the emergency department as soon as possible if you experience chest pain that lasts longer than five minutes or doesn’t get better with rest or medication. Other signs of a heart attack include:

• Sweating
• Nausea or vomiting
• Shortness of breath
• Overwhelming anxiety
• Lightheadedness or dizziness
• Pain spreading from your chest to another part of the body such as the arm, jaw, or neck

Talk With Others Who Understand

On myAIHAteam, the social network for people with AIHA and their loved ones, members come together to ask questions, give advice, and share their stories with others who understand life with AIHA.

Have you experienced chest pain with AIHA? Have you found a way to manage this symptom? Share your experience in the comments below, or start a conversation by posting on your Activities page.