Pulmonary Langerhans Cell Histiocytosis

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:

• The Histiocytosis Association: LCH in Adults
• National Organization for Rare Disorders: Langerhans Cell Histiocytosis
• National Center for Advancing Translational Sciences: Langerhans Cell Histiocytosis
• Histiocytosis Association of Canada

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: