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Lymphoid interstitial pneumonia
Last reviewed: 23.04.2024
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Lymphoid interstitial pneumonia (lymphocytic interstitial pneumonitis) is characterized by lymphocytic infiltration of interstitium of alveoli and air spaces.
The cause of lymphoid interstitial pneumonia is unknown. Most often occurs in children with HIV infection and people of any age who suffer from autoimmune diseases. Symptoms of lymphoid interstitial pneumonia include coughing, progressive dyspnoea and wet wheezes. The diagnosis is established when analyzing the history, physical examination, radiation studies, lung function and histological examination of the lung biopsy. Treatment of lymphoid interstitial pneumonia involves the use of glucocorticoids and / or cytotoxic agents, although its effectiveness is not known. Five-year survival rate is from 50 to 66%.
Lymphoid interstitial pneumonia is a rare disease characterized by infiltration of alveoli and alveolar septa with small lymphocytes and a different number of plasma cells. There may be noncaseating granulomas, but they are usually rare and inconspicuous.
Lymphoid interstitial pneumonia is the most common cause of lung disease after pneumocystis infection in HIV-positive children and a pathology that indicates the development of AIDS in about half of them. Less than 1% of cases of lymphoid interstitial pneumonia are seen in adults who can be both HIV-infected and not suffer from this disease. Women are sick more often.
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What causes lymphoid interstitial pneumonia?
The cause of the disease is an autoimmune process or a nonspecific reaction to infection with Epstein-Barr viruses , HIV or others. Evidence of autoimmune etiology is the frequent association with Sjogren's syndrome (25% of cases) and other systemic processes (eg, systemic lupus erythematosus, rheumatoid arthritis, Hashimoto's disease - 14% of cases). Indirect evidence of viral etiology is the frequent association with immunodeficiency states (HIV / AIDS, combined variable immunodeficiency, agammaglobulinemia, which is observed in 14% of cases) and detection of the DNA of the Epstein-Barr virus and HIV RNA in lung tissue in patients with lymphoid interstitial pneumonia. According to this theory, lymphoid interstitial pneumonia is an extremely pronounced manifestation of the normal capacity of the lung lymphoid tissue to respond to inhaled and circulating antigens.
Symptoms of lymphoid interstitial pneumonia
In adults, lymphoid interstitial pneumonia causes the development of progressive dyspnea and cough. These symptoms of lymphoid interstitial pneumonia progress for several months or, in some cases, several years; the average age of the sick is 54 years. Also there are, but less often, weight loss, increased temperature, arthralgia and night sweats.
In children, lymphoid interstitial pneumonia leads to the development of bronchospasm, cough and / or respiratory distress syndrome and developmental disorders, usually occurring between the ages of 2 and 3 years.
Physical examination allows you to identify wet rales. Manifestations in the form of hepatosplenomegaly, arthritis and lymphadenopathy are rare and suggest a concomitant or alternative diagnosis.
Diagnosis of lymphoid interstitial pneumonia
The diagnosis is established when analyzing the history, physical examination, radiation studies and lung function and is confirmed by the results of a histological examination of the biopsy material.
Radiography of chest organs reveals linear or focal blackouts, as well as increased pulmonary pattern, mainly in the basal areas, as well as nonspecific changes seen in other lung infections. Alveolar darkening and / or cellular pattern can be detected in more advanced cases of the disease. CT scan of high resolution helps to determine the prevalence of lesions, assess the anatomical structure of the lung root and identify pleural lesions. Typical changes are centrilobular and subpleural nodules, thickened bronchoconstrictive cords, darkening by the type of frosted glass and, in rare cases, diffuse cystic changes.
Lung function tests reveal changes of a restrictive type with a decrease in pulmonary volumes and diffusivity for carbon monoxide (DL ^) while maintaining speed characteristics. Expressed hypoxemia can be detected. Broncho alveolar lavage should be performed to exclude infection and may show an increase in the number of lymphocytes.
Approximately 80% of patients have changes in the protein composition of blood serum, usually in the form of polyclonal gammopathy and, especially in children, hypogammaglobulinemia, but the significance of these changes is unknown. These findings are usually enough to confirm the diagnosis in HIV-positive children. In adults, for diagnosis, it is necessary to detect expansion of alveolar septa with infiltration of lymphocytes and other immunocompetent cells (plasma cells, immunoblasts and histiocytes). Germinal centers and multinucleated giant cells with noncaseating granulomas can also be detected. Sometimes infiltrates develop along the course of bronchi and vessels, but usually infiltration extends along the alveolar septa. Immunohistochemical staining and flow cytometry should be performed for the purpose of differential diagnosis of lymphoid interstitial pneumonia and primary lymphomas; with lymphoid interstitial pneumonia, infiltration is a polyclonal (B- and T-cell), whereas a lymphomatous infiltrate is monoclonal.
Treatment of lymphoid interstitial pneumonia
Treatment of lymphoid interstitial pneumonia involves the use of glucocorticoids and / or cytotoxic agents, similar to many other variants of IBLARB, but the effectiveness of this approach is not known.
What is the prognosis of lymphoid interstitial pneumonia?
Lymphoid interstitial pneumonia little studied lung disease, as well as current and prognosis. The prognosis may be associated with the severity of the changes in radiography, which may correlate with greater severity of the immune response. There may be a spontaneous resolution, stopping on the background of therapy with glucocorticoids or other immunosuppressive agents, progression with the development of lymphoma or pulmonary fibrosis and respiratory failure. The five-year survival rate ranges from 50 to 66%. Frequent causes of death are infection, development of malignant lymphomas (5%) and progressive fibrosis.