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Pulmonary Langerhans Cell Histiocytosis

by Harold R. Collard, MD

Pulmonary Langerhans cell histiocytosis (granulomatosis, or eosinophilic granuloma) is a disorder in which cells called histiocytes and eosinophils proliferate in the lungs, often causing scarring.

  • People may have no symptoms or may cough and have difficulty breathing.

  • Diagnosis requires computed tomography and sometimes analysis of a sample of lung tissue (biopsy).

  • Whether and which treatments help are unknown, but stopping smoking may help.

Pulmonary Langerhans cell histiocytosis (granulomatosis) is one form of Langerhans cell histiocytosis, which can affect other organs (such as the pituitary gland, bones, and the lymph nodes) as well as the lungs. The cause is unknown, and the disorder is rare. It occurs almost exclusively in whites aged 20 to 40 who smoke cigarettes. It starts with infiltration of the lung by histiocytes, which are cells that scavenge for foreign materials, and to a lesser extent by eosinophils, which are cells that are normally involved in allergic reactions.

Symptoms

About 15% of people have no symptoms and the disorder is first recognized when an imaging study of the chest is done for another reason. The remainder develop coughing, shortness of breath, fever, chest pain worsened by deep breathing, fatigue, and weight loss. Pneumothorax (a collapsed lung) is a common complication due to rupture of a lung cyst. It occurs in 10 to 25% of people with the disorder and may be the cause of the first symptoms that develop. Scarring makes the lungs stiff and impairs their ability to transfer oxygen into and out of the blood. A few people cough up blood (hemoptysis).

Some people have pain in certain parts of a bone or a pathologic bone fracture (a fracture that occurs after only a minor injury because the bone has been thinned by a disorder). A few people develop central diabetes insipidus (see Central Diabetes Insipidus) when histiocytes also affect the pituitary gland in the brain. The person makes excessive amounts of urine that is dilute. People with diabetes insipidus probably have a worse prognosis than those who do not.

Diagnosis

Chest x-rays show nodules, small thick-walled lung cysts (called honeycombing because they resemble the cells of a beehive), and other changes that are typical of this disorder. Computed tomography (CT) may show these changes in enough detail to establish the diagnosis. X-rays may also show that the bones are affected. Pulmonary function tests (see Pulmonary Function Testing (PFT)) show that the amount of air the lungs can hold is below normal. If CT is not diagnostic, biopsy is required. Biopsy can usually be done during bronchoscopy (see Bronchoscopy).

Prognosis and Treatment

Half of people are alive more than 12 years after diagnosis. Death usually results from respiratory failure or cor pulmonale (see ). When people stop smoking, improvement occurs in about one third of cases.

The disorder may be treated with corticosteroids and immunosuppressants such as cyclophosphamide, although no therapy is clearly beneficial.

Drugs Mentioned In This Article

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  • CYTOXAN (LYOPHILIZED)