What is Pulmonary Langerhans Cell Histiocytosis?
Pulmonary Langerhans Cell Histiocytosis (PLCH), also called eosinophilic granuloma of the lung, pulmonary Langerhans cell granulomatosis, or pulmonary histiocytosis X, is an interstitial lung disease that occurs most often in young adults. PLCH can be one manifestation of Langerhans cell histiocytosis, a multisystem disease characterized by infiltration of cells called histiocytes. Cigarette smoking is a strong risk factor for the development of this condition (seen in over 90% of cases).
- PLCH can cause long-term disability with chronic shortness of breath and cough.
- The disease may stabilize or regress with smoking cessation.
- Continued cigarette smoking can result in progression to scar tissue formation in the lung. For this reason, quitting smoking is the most important part of therapy.
- Once the lung tissue becomes scarred, there are no treatment options to reverse this damage.
What Is Pulmonary Langerhans Cell Histiocytosis?
Pulmonary Langerhans cell histiocytosis is a chronic lung disease that results in the formation of multiple spherical or irregularly shaped, thin-walled, air-filled spaces within the lung parenchyma. It is strongly related to cigarette smoking. There may be regression of the disease and improvement in symptoms with smoking cessation but with continued smoking, lung tissue can become thick and scarred.
How Does Pulmonary Langerhans Cell Histiocytosis Affect Your Body?
In early disease, the majority of patients can remain asymptomatic or have mild symptoms, such as cough and shortness of breath. The thin-walled, air-filled spaces in the lung, called cysts, can lead to several complications, including pneumothorax (spontaneous lung collapse) and lung infections.
As the disease progresses, lung tissue becomes thick and stiff with scarring. When the lung tissue becomes scarred, it affects the person’s ability to breathe and reduces the amount of inhaled oxygen from reaching their blood through the walls of the air sacs. This results in shortness of breath, even during everyday activities, thus limiting the person’s ability to work, perform household chores, and engage in social activities.
How Serious is the Disease?
PLCH is a very uncommon chronic lung disease, and the precise occurrence of this condition is not known. It most commonly affects younger individuals, with peak incidence at 20 to 40 years of age. Smoking cessation and avoidance of second-hand smoke results in disease regression in some people and may prevent progression of disease in most people with this condition. If you are diagnosed with PLCH, smoking cessation is very strongly encouraged.
As the disease progresses, symptoms such as shortness of breath and cough worsen over time. There is no proven cure for this disease once the lung tissue is scarred. PLCH is associated with complications such as collapsed lung, lung infections and occasionally lung cancer. Advanced PLCH can lead to a buildup of pressure in the blood vessels in the lungs (called pulmonary hypertension) and respiratory failure. In advanced cases, lung transplantation may be the only remaining therapy.
In many cases, PLCH can be prevented, and it is important to remember that smoking cessation is the key treatment for this condition.
Symptoms, Causes, and Risk Factors
Pulmonary Langerhans cell histiocytosis is a chronic lung disease that presents with none or mild symptoms, such as shortness of breath or cough in early disease. Some individuals may even present with sudden shortness of breath associated with spontaneous lung collapse or recurrent lung infections. As the disease advances, breathing symptoms become worse and, in few people, shortness of breath can occur even at rest.
What are the symptoms of pulmonary Langerhans cell histiocytosis?
- Shortness of breath, particularly during exertion
- Dry cough
- Chest pain, particularly with deep breathing
- Recurrent infections
- Fevers and night sweats
- Gradual weight loss
- Coughing up blood
- Bone pain or fractures
What causes pulmonary Langerhans cell histiocytosis?
Proteins, called antigens, present in cigarette smoke, stimulate a reactive process that results in proliferation of specific cells called Langerhans cells and their infiltration into the lung tissue to cause PLCH.
What are the risk factors for pulmonary Langerhans cell histiocytosis?
Cigarette smoking is the single most known risk factor for development of PLCH.
When to see your doctor?
If you develop any symptoms listed above, talk to your doctor.
Diagnosis and Treatment
Your doctor will ask you questions regarding your symptoms, smoking history, exposure to particles or chemicals in the air at home or your job, other medical conditions, medications (current and past) and history of lung diseases in your family members. A history of recurrent lung collapse and/or infections can be helpful in making the diagnosis. Your doctor will also perform a physical exam. It is important to work with your doctor and a pulmonary specialist to make the right diagnosis of PLCH.
How is pulmonary Langerhans cell histiocytosis diagnosed?
The symptoms and signs on physical exam of PLCH are nonspecific. Several individuals may present with abnormal chest x-ray performed for other reasons. Other tests as below may be obtained by your doctor to make the diagnosis of PLCH. Sometimes the diagnosis can be determined by washing part of the lung or taking small pieces of tissue during an outpatient procedure called bronchoscopy. However, obtaining a larger piece of lung tissue (by surgical biopsy) is often required.
High resolution Chest CT scan
If PLCH is suspected, your doctor will perform a high-resolution computed tomography (HRCT) scan of your chest. The presence of multiple nodules or spherical or irregularly shaped, thin-walled, air-filled spaces (cysts) within the lung tissue is classically seen in PLCH and is confirmatory of the disease in the right clinical setting. In advanced disease, scar tissue can be seen in lung tissue.
A pulmonary function test is a simple test to determine how well your lungs take in and breathe out air and estimate how efficiently your lungs transfer oxygen from the inhaled air across air sacs into the bloodstream. The information obtained from these tests will help your doctor diagnose your condition and find out how severe the disease is.
If a diagnosis cannot be made by HRCT scan, lung biopsy may be necessary. Small pieces of lung tissue are obtained by a surgical procedure.
How is pulmonary Langerhans cell histiocytosis treated?
In individuals with minimally symptomatic PLCH, resolution of symptoms may occur with smoking cessation. However, some patients demonstrate a slow progression of disease with lung scarring over time in spite of smoking cessation. Serial breathing tests to measure impairment of lung function will help in deciding treatment options.
Smoking cessation is the main focus of treatment in individuals with PLCH. Ask your doctor about tobacco cessation interventions including behavioral counseling and pharmacologic treatments.
No large clinical trials have compared the benefit of various medications in treatment of PLCH. Your doctor may prescribe a corticosteroid (prednisone) to reduce inflammation in the lung that has been reported to be helpful in some patients. Your doctor may discuss chemotherapeutic medications such as cladribine, cyclophosphamide, vinblastine, methotrexate and etoposide if you have progressive disease that does not improve with corticosteroids, even though their efficacy has not been proven.
Management of Complications
If you have lung collapse, your doctor may suggest a procedure called pleurodesis in which the pleural space is artificially obliterated. This may be offered with the first episode of collapse since recurrence may be as high as 50%. Many centers recommend pleurodesis with the first PTX since the risk of recurrence rate is very high. You may develop pulmonary hypertension in advanced disease, which may respond to therapy.
Your doctor may prescribe supplemental oxygen therapy if the oxygen level in your blood is low. Supplemental oxygen therapy helps in maintaining normal body function, reducing your shortness of breath, and improving sleep and physical strength.
This is a program designed to help individuals with advanced lung diseases improve strength and activity levels and learn how to manage your disease. These programs are a combination of exercise training, education, and counseling.
Evaluation of lung transplantation is recommended if there is a decline in your lung function or you develop severe pulmonary hypertension with severe disability.
Living With the Disease
Every individual diagnosed with PLCH may have variable clinical course. With smoking cessation, a majority of the patients may have resolution of symptoms or stabilization of lung disease. Some individuals may experience progressive decline in lung function over time.
How to Manage Pulmonary Langerhans Cell Histiocytosis
It is very important to work closely with your doctor and care team to understand your disease and manage your symptoms effectively.
- If you smoke, QUIT! Ask your doctor about programs to help you quit smoking. Avoid second-hand smoke.
- Take medications as prescribed by your doctor.
- Use oxygen as prescribed by your doctor.
- Maintain an active lifestyle and eat a nutritious diet.
- Ask your doctor about pulmonary rehabilitation programs.
- Ask your doctor about vaccinations. Get a flu shot every year.
- Join support groups to help you with cope with PLCH.
- If you have sudden change in symptoms, call your doctor or healthcare team immediately.
How to Find Support
Online support groups:
1) The Histiocytosis Association: https://www.histio.org/page.aspx?pid=383 – .WEsi0WURLpA
Questions to Ask Your Doctor About Pulmonary Langerhans Cell Histiocytosis
What Is pulmonary Langerhans cell histiocytosis?
PLCH is a very uncommon type of interstitial lung disease that occurs most often in young adults, and cigarette smoking is a strong risk factor for the development of this condition. It is characterized by multiple nodules and spherical or irregularly shaped, thin-walled, air-filled spaces within the lung parenchyma in early disease. If the disease progresses, scar tissue is formed in the lungs; this damage is irreversible and results in shortness of breath.
How can I quit smoking?
Smoking cessation is the single most important intervention in the treatment of PLCH. Your doctor can discuss various tobacco cessation interventions, including behavioral counseling and pharmacologic treatments to help you increase the chances of smoking cessation.
What are the symptoms of pulmonary Langerhans cell histiocytosis?
You may not have any symptoms in early 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 doctor about participating in pulmonary rehabilitation programs. These programs include exercise regimen, education, and counseling. You can also meet other individuals with chronic lung disease and could help you cope with your disease better.
What vaccinations do I need?
When you have a chronic lung disease, you are at a higher risk of developing respiratory failure with infections such as flu. Get vaccinated against flu and pneumonia, and take precautions to avoid infections.
Daniel Dilling, MD, FCCP
Loyola University Medical Center
Tejaswini Kulkarni, MD, MPH
University of Alabama at Birmingham School of Medicine
Jay Peters, MD, FCCP
South Texas Veterans Healthcare System
Date Last Reviewed