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Histiocytosis X

Histiocytosis from Langerhans cells is a group of diseases manifested by the accumulation of Langerhans cells in various organs and tissues with the formation of foci of inflammation – granulomas.

The pulmonary variant of histiocytosis X in adults is a rare interstitial lung disease.

The course of the disease varies – from asymptomatic forms to severe lung damage with the development of respiratory failure.

The disease is most common in young and middle-aged patients. Men are more likely to get sick At the heart of the development of the disease is the pathology of the cellular immunity system.

Among people suffering from this disease, smokers account for 96.5%. The most significant is not the smoking experience, but the number of cigarettes smoked per day.

Nosology may be accompanied by damage to other organs:cystic lesion of bones, skin

  • diabetes insipidus, which develops due to damage to the posterior lobe of the pituitary gland.

Pathology can be suspected if the following symptoms occur:shortness of breath during physical exertion

  • cough, more often dry
  • changes detected during chest X-ray
  • recurrent pneumothorax
  • rarely hemoptysis
  • Weakness
  • weight loss
  • Fever
  • the development of diabetes insipidus, pulmonary hypertension
  • cystic changes in bones, skin manifestations
  • enlargement of the lymph nodes of the neck, marking, spleen

If one or more of these signs are detected, an examination is recommended. A specialist doctor will assess the situation and prescribe a follow-up examination.

To diagnose lung histiocytosis,:

  • Laboratory tests: general and biochemical blood tests, examination of arterial blood gases.
  • Instrumental studies: multispiral computed tomography, diagnostics of the function of external respiration (bodyplethysmography with the study of the diffusion capacity of the lungs). In order to establish the presence of bone lesions (extrapulmonary), radiography and bone scintigraphy are used. To clarify the involvement of other organs, an MRI scan is possible. Bronchoscopy with bronchial lavage examination and biopsy make it possible to identify Langerhans cells and morphologically confirm the diagnosis.

Treatment of lung histiocytosis has not yet been developed. The primary method of treatment is smoking cessation, there is evidence of stabilization of the disease against the background of taking systemic glucocorticosteroids. In the treatment of patients with multiple organ progression of the disease, chemotherapy is used. With the rapid progression of pulmonary histiocytosis, lung transplantation is the option of choice.

Prognosis: in almost half of cases, the clinical and radiological picture of the disease remains stable, spontaneous remission is observed in 25% of patients, and in 25% there is an increase in cystic changes with the development of severe respiratory failure.

In the Moscow Clinical Scientific and Practical Center named after A.S. Loginov, the treatment and diagnosis of lung histiocytosis is carried out by the efforts of pulmonologists, endoscopists, morphologists, doctors of radiation and functional diagnostics using modern diagnostic methods. Do not delay your visit to our specialists. This will help to identify the disease in a timely manner and choose the right treatment tactics. Doctors of GBUZ MCSC named after A.S. Loginov DZM are always ready to help you.

GBUZ Moscow Clinical Scientific Center named after Loginov MHD