Pulmonary Langerhans Cell Histiocytosis

Last Updated 05/08/2020

Authors:Daniel Dilling, MD, FCCP; Tejaswini Kulkarni, MD, MPH; Jay Peters, MD, FCCP

About Pulmonary Langerhans Cell Histiocytosis

Key facts about Pulmonary Langerhans Cell Histiocytosis
  • Pulmonary Langerhans cell histiocytosis (PLCH) can cause long-term ill health, with long-lasting shortness of breath and cough.
  • PLCH may improve if you stop smoking.
  • If you keep smoking, PLCH can cause lung scarring.
  • You can’t undo lung scarring once it has started.

Pulmonary Langerhans cell histiocytosis (PLCH) is a lung disease that creates air-filled spaces (called cysts) in the lungs. It occurs most often in young adults if they smoke and may improve if they stop.

How Pulmonary Langerhans Cell Histiocytosis affects your body

At first, you may not notice symptoms or they will be mild, such as cough or shortness of breath. Eventually, your lungs will develop infections and may collapse (a condition called pneumothorax).

As the disease gets worse, your lungs may scar. You’ll find it difficult to breathe and do simple activities.

How serious is Pulmonary Langerhans Cell Histiocytosis?

PLCH is not common. Usually younger people who smoke can get PLCH. If they don’t stop smoking, PLCH can be serious, with the following symptoms:

  • Shortness of breath
  • Bad cough
  • Scarred lung tissue
  • Collapsed lung
  • Lung infection
  • Lung cancer
  • Lung pressure
  • Lung transplant

Symptoms of Pulmonary Langerhans Cell Histiocytosis

PLCH is a lung disease that at first has no symptoms—or only mild ones. You may be short of breath or have a cough. Some people may have many lung infections or a collapsed lung. As the disease continues, it becomes harder to breathe, even when resting.

The most common symptoms of PLCH are:

  • Shortness of breath when you exercise;
  • Dry cough;
  • Chest pain with deep breathing;
  • Infections;
  • Fevers and night sweats;
  • Tiredness;
  • Gradual weight loss;
  • Coughing up blood; and
  • Bone pain or fractures.

What causes Pulmonary Langerhans Cell Histiocytosis?

There are proteins, or antigens, in cigarette smoke that cause Langerhans cells to enter the lungs. PLCH occurs when the lungs have many of these cells.

What are risk factors for Pulmonary Langerhans Cell Histiocytosis?

The biggest risk for PLCH is smoking cigarettes.

Diagnosing Pulmonary Langerhans Cell Histiocytosis

It can be hard for health care providers to diagnose PLCH because people have different symptoms. Provider can use the following tests to see if you have PLCH:

  • High-resolution chest computed tomography (CT) scan. Your provider may order a CT scan (a type of X-ray) of your chest. This scan can show air-filled spaces (called cysts) or scar tissue in your lungs.
  • Breathing test. A lung function test can help your provider tell how well your lungs are working. For this test, you breathe in and out of a piece of equipment.
  • Lung biopsy. Your doctor may to do a surgical procedure, called a lung biopsy, to take a small piece of tissue from your lung to look at under a microscope.

Treating Pulmonary Langerhans Cell Histiocytosis

The best way to stop or lessen PLCH is to quit smoking. If it’s hard for you to stop, your provider may have ways to help.

No medications have been approved to treat PLCH, but some of the following may help manage your symptoms:

  • Corticosteroids (prednisone)
  • Chemotherapeutic drugs:
    • Cladribine
    • Cyclophosphamide
    • Vinblastine
    • Methotrexate
    • Etoposide

Other treatments your provider may try if your PLCH is serious include the following:

  • Pleurodesis. A procedure to fix a collapsed lung
  • Supportive care. Therapy to increase the amount of oxygen in your blood so that you’re not short of breath and can sleep better
  • Pulmonary rehabilitation. A combination of exercise, education, and counseling so that you can be more active and better manage your PLCH
  • Lung transplant. If your PLCH becomes serious and nothing else helps

Managing Pulmonary Langerhans Cell Histiocytosis

It’s important to work with your health care provider and team to learn about PLCH and manage your symptoms:

  • If you smoke, quit. Ask your provider about programs to help you quit smoking. Stay away from secondhand smoke.
  • Take the medications your provider prescribes.
  • Use oxygen therapy, if your provider prescribes it.
  • Stay active, and eat healthy foods.
  • Ask your provider about pulmonary rehabilitation programs.
  • Ask your provider about vaccinations. Get a flu shot every year.
  • Join support groups to help you cope with PLCH.
  • If your symptoms change, call your provider or health care team immediately.

Every person with PLCH may have different treatments. Many who quit smoking can lessen their symptoms or stop the disease from getting worse.

Preventing Pulmonary Langerhans Cell Histiocytosis

The most important thing to do to avoid PLCH is to quit smoking. To help protect your lungs, wear a mask over your mouth and nose if you’re using paint, paint remover, or varnish.

Other ways to help prevent PLCH include:

  • Washing your hands often to remove any germs; and
  • Getting a flu shot every year.

Ask your provider if you should get a pneumonia shot, especially if you’re 60 years of age or older.

Resources

The American Lung Association recommends that patients and caregivers join its Living with Lung Disease support community to connect with others facing this disease. To talk to a trained lung professional, call the American Lung Association’s Lung Helpline at 1-800-LUNGUSA. They can help answer your questions and connect you with additional support.

Consider joining an online support group:

Questions to ask your health care provider

Making notes before your visit and taking along a trusted family member or friend can help you through the first appointment with your provider. Here are a few frequently asked questions and the answers to them:

  • What causes PLCH?

    Antigens in cigarette smoke cause a process that results in spread of cells called Langerhans cells into lung tissue to cause PLCH.

  • How can I quit smoking?

    Your provider can discuss various tobacco-cessation interventions, including behavioral counseling and medications to help you quit smoking.

  • What are the symptoms of PLCH?

    You may not have any symptoms in early in the disease. The most common symptoms are shortness of breath, dry cough, chest pain, and recurrent infections. You may also have sudden shortness of breath if you have a lung collapse.

  • What can I do to maintain a good quality of life with PLCH?

    Stay active, and ask your provider about pulmonary rehabilitation programs. These programs include exercise, education, and counseling. You can also meet other individuals with chronic lung disease, which may help you cope with your disease better.

  • Which vaccinations do I need?

    When you have a chronic lung disease, you are at a higher risk for developing serious lung infections, such as flu. Get vaccinated against flu and pneumonia, and take precautions to avoid infections.