Lymphoid Interstitial Pneumonia

(Lymphocytic interstitial pneumonitis)

In the lymphoid interstitial pneumonitis (LIP), the alveolar interstitium and the air spaces of lymphocyte infiltrated. The cause is unknown. The symptoms consists in cough, dyspnoea and increasing wet RG. Diagnosis is based on medical history, physical examination findings, imaging and lung biopsies. is treated with corticosteroids and / or immunosuppressants, although the effectiveness is unclear. The 5-year survival rate is 50-66%.

LIP is a rare idiopathic interstitial pneumonia, which is characterized by the infiltration of small lymphocytes and different amounts of plasma cells in the alveoli and alveolar walls. Nichtnekrotisierende weakly formed granulomas may be present, but are usually rare and inconspicuous.

In the lymphoid interstitial pneumonitis (LIP), the alveolar interstitium and the air spaces of lymphocyte infiltrated. The cause is unknown. The symptoms consists in cough, dyspnoea and increasing wet RG. Diagnosis is based on medical history, physical examination findings, imaging and lung biopsies. is treated with corticosteroids and / or immunosuppressants, although the effectiveness is unclear. The 5-year survival rate is 50-66%. LIP is a rare idiopathic interstitial pneumonia, which is characterized by the infiltration of small lymphocytes and different amounts of plasma cells in the alveoli and alveolar walls. Nichtnekrotisierende weakly formed granulomas may be present, but are usually rare and inconspicuous. The LIP is after Pneumocystis infection is the most common lung disease in HIV-positive children and up to half of HIV-positive children, the AIDS-defining illness. The LIP concerns <1% of adults with and without HIV infection. Women and girls are often affected. The cause is to be an autoimmune disease or a non-specific immune response to infection with EBV, HIV or other viruses. Evidence for the autoimmune etiology include the often simultaneous occurrence with Sjögren's syndrome (25% of cases of LIP) and other diseases (such as SLE, RA, Hashimoto's thyroiditis -. 14% of cases). Evidence for an indirect Virusätiologie are the frequent occurrence of immunosuppression (HIV / AIDS, combined variable immune deficiency, agammaglobulinemia - 14% of the cases) and the detection of EBV DNA and HIV RNA in the lung tissue of patients with LIP. According to this theory, the LIP is an extreme expression of the normal ability of pulmonary lymphoid tissues to respond to inhaled and circulating antigens. Symptoms and ailments in adults caused the LIP increasing dyspnea and cough. These symptoms take months or - in some cases - for years to and occurs at an average age of 54 years. Weight loss, fever, arthralgia and night sweats also occur, but are rare. When investigating wet RG can be heard. Findings such as hepatosplenomegaly, arthritis, and lymphadenopathy are unusual and suggest the presence of concomitant disease or other close. Diagnosis High-resolution CT (HRCT) biopsy to confirm the diagnosis is usually placed in high-risk patients with compatible symptoms. Are carried out imaging and sometimes lung biopsy. In the chest X-ray on both sides basal linear reticular or nodular drawings nonspecific findings, which appear in numerous pneumonia show. Shadows of the alveoli and / or cysts may be present in advanced disease. A HRCT of the thorax makes it possible to assess the extent of disease Hilusanatomie and a possible pleural. However, HRCT findings vary considerably. Typical findings are centrilobular and pleuranahe nodules, thickened bronchovascular vascular, nodular opacities and frosted glass cystic structures. A significant hypoxemia may occur. A BAL should be performed to rule out infections. Here, there are occasionally increased lymphocyte numbers. Routine laboratory tests and serum protein electrophoresis (SPEP) are carried out because about 80% of patients show abnormal serum proteins, most commonly a polyclonal gammopathy and hypogammaglobulinemia whose significance is unclear. Lung biopsy, which reveals infiltration of the alveolar walls due to lymphocytes and other immune cells (plasma cells, immunoblastic, histiocytes) is required for diagnosis in adults. The infiltrates appear occasionally arranged along the bronchi and blood vessels, but most often along the alveolar septa. Immunohistochemical staining and flow cytometry must occur on the tissue in order to differentiate LIP of primary lymphoma. In a LIP is the infiltrate polyclonal (T- and B-cells), while others lymphomas produce monoclonal infiltrates. Other common findings are germinal centers and polynuclear giant cells with nichtverkäsenden granulomas. Forecasting natural development and progress of the LIP are poorly understood. Spontaneous healing, healing after treatment with corticosteroids or other immunosuppressants, lymphoma progression to or development of lung fibrosis with respiratory failure may occur. The 5-year survival rate is 50-66%. Common causes of death are infections, development of a malignant lymphoma (5%) and increased fibrosis. Therapy corticosteroids or cytostatics Treatment is with corticosteroids, immunosuppressants or both. The efficacy is unknown how many interstitial lung diseases other etiology. Conclusion LIP is rare overall, but is one of the most common lung diseases in HIV-positive children. The symptoms and complaints are rather non-specific. By HRCT should be performed and sometimes a lung biopsy, a bronchoalveolar lavage. Patients are treated with corticosteroids, cytotoxic drugs or both.

Health Life Media Team

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