Many people with autoimmune hemolytic anemia (AIHA) develop Raynaud’s phenomenon, which occurs when blood doesn’t properly flow to all your body’s tissues. This symptom can particularly affect people living with cold agglutinin disease (CAD), a subtype of cold AIHA that is triggered by cold temperatures.

Raynaud’s phenomenon is common for people with CAD. If you have been diagnosed with cold AIHA, talk to your doctor about keeping your blood vessels healthy and ask whether treatments to prevent Raynaud’s phenomenon may be helpful.

What Is Raynaud’s Phenomenon?

Your body contains many blood vessels, which supply blood, oxygen, and nutrients to all your cells. Typically, when your body gets cold, these blood vessels get smaller, which pushes your warm blood toward bigger, deeper blood vessels and helps you warm up. This process is an important part of staying healthy, but Raynaud’s phenomenon takes it to an extreme.

During Raynaud’s phenomenon, triggers such as cold or stress lead the tiny vessels within certain tissues to narrow, making it harder for blood to reach those areas. Raynaud’s phenomenon is most likely to affect your:

• Fingers
• Toes
• Ears
• Nose
• Nipples
• Knees

When Raynaud’s strikes, tissues in these parts of your body may feel cold. The skin may turn pale, white, or blue, although the discoloration may be hard to see, depending on your skin tone. When you try to warm up, the affected areas may redden, swell, and feel painful or numb. It could take up to 15 minutes for blood to start flowing normally again once your tissues warm up.

Over time, Raynaud’s phenomenon may lead to ongoing damage. Fatty deposits may build up in your blood vessels, making it even harder for blood to pass through. Sores may develop on your skin. Rarely, Raynaud’s phenomenon can lead to gangrene, in which the tissue dies because of lack of blood. Gangrene can cause severe pain, shiny skin, or additional color changes.

Raynaud’s Phenomenon and AIHA

AIHA is an autoimmune disease that causes your immune system to make autoantibodies — proteins that destroy healthy red blood cells in a process known as hemolysis. Because of the reduced number of red blood cells, too little oxygen gets carried to the rest of the body’s tissues. For people who have CAD, cold temperatures trigger not only their condition but also other problems with the blood and blood vessels, including Raynaud’s phenomenon.

When Raynaud’s phenomenon occurs because of AIHA or another underlying condition, such as rheumatoid arthritis or systemic lupus erythematosus, it’s called secondary Raynaud’s phenomenon.

Diagnosing Raynaud’s Phenomenon

Your doctor may do a few tests to diagnose Raynaud’s phenomenon and its cause, including AIHA. These tests include:

• Nailfold capillaroscopy — Looks for problems with blood vessels at the base of your fingernail
• Antinuclear antibody (ANA) test — Identifies antibodies in blood that may be attacking your red cells
• Erythrocyte sedimentation rate — Signals whether an autoimmune disorder is causing ongoing inflammation in your body
• Pulse volume recording — Measures how well blood flows through your veins

Preventing Raynaud’s Phenomenon

You can avoid symptoms of Raynaud’s phenomenon by keeping tabs on your body temperature and making sure your face, hands, and feet stay warm.

Stay out of the cold if you can, and avoid going outside as temperatures drop. When that’s not possible and you know you’ll be exposed to cold temperatures, take steps like these to keep warm:

• Layer your clothing, starting with thermal underwear.
• Wear socks and shoes that will keep your feet dry and warm.
• Bundle up with appropriate clothing and outerwear.
• Add a hat, scarf, or face mask and multiple pairs of gloves.
• Limit your time outside so you can warm up again as soon as possible.

Hotter weather or a cranked-up thermostat won’t necessarily offer a reprieve from symptoms. To keep Raynaud’s phenomenon from striking, you also might need to:

• Adjust your air conditioner to prevent blasts of cold air from triggering symptoms.
• Pull on gloves or oven mitts before rummaging in the freezer or handling cold items.
• Use insulated cups to protect your hands when holding a cold beverage.

You may also want to avoid other risk factors that can lead to Raynaud’s phenomenon. For example, smoking increases your risk of this condition, so it may help to give up cigarettes if you have AIHA.

Being more physically active can improve blood flow throughout your body, although it may be best to stick to indoor workouts when it’s cold out if you have an increased risk of developing Raynaud’s phenomenon. Exercise also provides a healthy way to manage high stress levels, another trigger for this condition.

Treating Raynaud’s Phenomenon

Raynaud’s phenomenon can’t be cured, but you can manage the symptoms and protect your tissues from damage. You may need to try different treatments based on how severe your symptoms are and any other health issues you may have.

Lifestyle Changes

When you have an attack, do your best to warm up right away. Wiggle and shake your fingers and toes, and gently rub your feet and hands. Holding the affected area under comfortably warm (not hot) running water can also help.

If you develop sores, ulcers, or irritated patches of skin, cover these areas with finger guards to protect them from further damage. You can also apply a topical medication that contains nitroglycerin to help heal sores.

Medications and Procedures

During colder seasons, your doctor may recommend blood pressure medications such as vasodilators or calcium channel blockers. These drugs can help widen your blood vessels and maintain normal blood flow.

Some treatments focus on the small nerves that play a role in the narrowing of blood vessels. Blocking these nerves can sometimes help keep the arteries and veins open. This can be accomplished by injecting medications that numb or block the nerves near the affected area or by surgically cutting the nerves. However, these treatment options are usually only recommended only for people with severe cases of Raynaud’s phenomenon.

It’s important to work with your health care team to find a treatment plan that keeps your AIHA under control. For example, if your cold AIHA was caused by an infection or another disease, treating the first condition can help prevent AIHA symptoms such as tiredness, lightheadedness, breathing difficulties, jaundice (yellow appearance of skin and eyes), and Raynaud’s phenomenon.

You may also be able to manage CAD with medications like rituximab (Rituxan) or sutimlimab-jome (Enjaymo), IV drugs that protect your red blood cells from damage by your immune system. Blood transfusions or plasma exchanges can also help boost your red blood cell levels or get rid of your autoantibodies.

Talk With Others Who Understand

At myAIHAteam, people with autoimmune hemolytic anemia and their loved ones come together to ask questions, give advice, and share their stories with others who understand life with this rare disease.

Have you been diagnosed with AIHA and Raynaud’s phenomenon? Do you have any tips for avoiding symptoms brought on by cold temperatures? Share your experience in the comments below, or start a conversation by posting on myAIHAteam.