Lymphangioleiomyomatosis

Lymphangioleiomyomatosis (LAM) refers to the low, progressive growth of smooth muscle cells in the area of ??the entire lung, the pulmonary blood vessels, the lymphatic system and the pleura. They rarely and exclusively in young women occurs. The symptoms include dyspnea, cough, chest pain and hemoptysis; a spontaneous pneumothorax is common. The diagnosis is suspected based on the symptoms and chest X-ray findings and confirmed by HRCT. The prognosis is uncertain. However, the disease is slowly progressive and leads over the course of years, often to respiratory failure and death. Treatment is with sirolimus or lung transplantation.

LAM is no interstitial lung disease, but is in the patients they sometimes misdiagnosed (as well as asthma or COPD).

Lymphangioleiomyomatosis (LAM) refers to the low, progressive growth of smooth muscle cells in the area of ??the entire lung, the pulmonary blood vessels, the lymphatic system and the pleura. They rarely and exclusively in young women occurs. The symptoms include dyspnea, cough, chest pain and hemoptysis; a spontaneous pneumothorax is common. The diagnosis is suspected based on the symptoms and chest X-ray findings and confirmed by HRCT. The prognosis is uncertain. However, the disease is slowly progressive and leads over the course of years, often to respiratory failure and death. Treatment is with sirolimus or lung transplantation. LAM is no interstitial lung disease, but is in the patients they sometimes misdiagnosed (as well as asthma or COPD). LAM is a rare disease that occurs exclusively in women, usually between 20 and 40 years. Among whites, the risk of disease is highest. The LAM affects <1 of 1 million people. It is characterized by a proliferation of atypical smooth muscle throughout the thorax, including the lung parenchyma, blood vessels, lymphatic system and pleura, which, cystic emphysema and progressive deterioration of lung function leads to changes in the lung architecture. Etiology The cause of LAM is unknown. The tantalizing hypothesis that female sex hormones play a pathogenetic role is not proven. The disease usually occurs spontaneously, but has many similarities with the lung findings in tuberous sclerosis (TS); LAM occurs in some patients with TS and is considered by some a "forme fruste" TS held. Mutations in tuberous sclerosis complex-2 gene (TSC-2) were described in LAM cells and angiomyolipoma (benign renal hamartomas that arise from smooth muscle, blood vessels and adipose tissues). Angiomyolipoma occur in up to 50% of patients with LAM. These observations 1 of 2 options near: Somatic mosaic of TSC-2 mutations in lung and kidney leads to disease sites that occur in addition against a background of normal cells in these tissues (though many outbreaks could be expected) LAM is an inferior, destructive, metastatic tumor, possibly leading to the uterus, which spreads through the lymphatic system symptoms and complaints the first symptoms are dyspnea and rarely cough, chest pain, and hemoptysis. There are few objectified examination findings. Some women have wet RG and wheezing. Many patients present with a spontaneous pneumothorax. You can also watch with signs of obstruction of the lymphatic vessels incl. Chylothorax, chylous ascites and imagine chyluria. Symptoms seem to worsen during pregnancy. Renal angiomyolipoma, although they are usually asymptomatic, cause bleeding when they are very extensive (eg. B.> 4 cm), which manifest themselves typically in hematuria or flank pain. Diagnostic chest X-ray and high-resolution CT (HRCT) lung biopsy when HRCT does not lead to diagnosis. The diagnosis is suspected in young women with dyspnea and interstitial changes with normal or increased lung volumes on the chest x-ray image, spontaneous pneumothorax or chylous pleural effusion. HRCT is performed in all patients in whom one suspects the disease; Findings of several small, distributed diffusely cysts are pathognomonic for LAM usually. A biopsy (surgical) is only indicated if the HRCT findings does not allow diagnosis. The histological findings of a pathological proliferation of smooth muscle cells (LAM cells) associated with cystic changes confirmed the diagnosis. Pulmonary function tests support the diagnosis and are particularly useful for monitoring the course. Typical findings include obstruction or a mixed obstructive and restrictive lung disease. The lungs are normally overinflated with increase in the total lung capacity (TLC) and the intrathoracic gas volume. A hyperinflation (increase of the residual volume [RV] and the RV / TLC ratio) is common. PaO2 and the CO diffusing capacity (DLco) are often reduced. Exercise capacity is reduced in most patients. Prognosis The prognosis is unclear, because the disease occurs so rarely, and the clinical course is variable. In general, a LAM runs slowly progressive, leading over time to respiratory failure and death. However, the time of death varies greatly between individual case reports. The median survival time is probably in> 8 years from diagnosis. The lung function takes two to three times faster than in healthy people. Women should be advised that the disease may progress rapidly during pregnancy. Therapy sirolimus or lung transplantation The standard therapy is in a lung transplantation. However, the disease can occur in the transplanted lung back. Recent data suggest that sirolimus (an mTOR inhibitor) in patients with moderate impairment of lung (forced volume in one second [FEV1] determined <70%) can help stabilize lung function or slow its decline. Alternative therapies such as hormone treatment with progestin, tamoxifen and ovariectomy proved on the whole to be ineffective. Pneumothorax can be difficult to treat because it often recurs, occur on both sides and less well respond to the usual treatment measures. A recurrent pneumothorax may require Pleuraabrasio or pleurodesis with talc or other substances or pleurectomy. Embolization to prevent bleeding should be considered in angiomyolipoma> 4 cm into consideration. Air travel are well tolerated by most patients. (D Note Übers .: in Germany from the LAM-self-help Germany under www.lam-info.de The LAM Foundation..) In the US, patients can education and psychological help from the LAM Foundation received. Conclusion LAM can mimic interstitial lung disease, but is actually a rare, slowly progressive growth of smooth muscle cells in various organs. The diagnosis should be suspected in young women with unexplained dyspnea and interstitial changes with normal or increased lung volumes on the chest x-ray image, spontaneous pneumothorax or chylous pleural effusion. A HRCT should be performed, and if the results are not conclusive, a biopsy. In LAM with progressively decreasing lung function, treatment with sirolimus should be considered.

Health Life Media Team

Leave a Reply