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A Rare Combination of Lymphangioleiomyomatosis with Hypersensitivity Pneumonia
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A1695 - A Rare Combination of Lymphangioleiomyomatosis with Hypersensitivity Pneumonia
Author Block: R. B. Filho1, M. Rodrigues de Oliveira1, A. F. Amaral2, E. Nascimento3, F. E. Arimura4, B. G. Baldi5, R. A. Kairalla6, C. R. Carvalho7; 1Pulmonary Medicine, Pulmonary Division, Heart Institute (InCor), Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo, Brazil., Sao Paulo, Brazil, 2Respiratory and Critical Care Medicina, Pulmonary Division, Heart Institute (InCor), Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo, Brazil., São Paulo, Brazil, 3University of Sao Paulo, Sao Paulo, Brazil, 4Pulmonology, Pulmonary Division, Heart Institute (InCor), Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo, Brazil., Sao Paulo, Brazil, 5Pulmonary Division, Heart Institute (InCor), University of Sao Paulo, Sao Paulo, Brazil, 6Univ Sao Paulo, Sao Paulo, Brazil, 7Pulmonary Division, Heart Institute (InCor), Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo, Brazil., Sao Paulo, Brazil.
Introduction: Lymphangioleiomyomatosis (LAM) is a rare neoplastic disease of smooth muscle like-cells affecting predominantly women in reproductive age. Hypersensitivity pneumonia (HP) is defined as an immunologically mediated inflammation of the lung parenchyma in response to inhalation of a wide variety of antigens. This is a report of a rare combination of LAM and HP in a single patient.
Objectives: Report a case of lymphangioleiomyomatosis with hypersensitivity pneumonia.
Method: Review of medical records.
Case Report: An asymptomatic 51 year-old woman presented diffuse thin-walled cysts on chest CT performed due to a car accident. The patient had no rheumatological or constitutional symptoms but reported daily long term contact with birds. Physical examination was unremarkable and there was any other characteristic suggestive of LAM. Then, an open lung biopsy was performed and hispathological analysis revealed chronic interstitial inflammation with multiple small loose granulomas and lymphocytic infiltrate associated with cysts. There were also nodules of atypical muscle cells in non-granulomatous areas with positivity to smooth-muscle actin and HMB-45 antibodies. The multidisciplinary discussion concluded that it was compatible with the combination of HP and LAM.
Conclusions: We report a rare case of a patient with LAM and HP. Other differential diagnosis should be included in cases of unexplained dyspnea in patients with LAM.
Author Block: R. B. Filho1, M. Rodrigues de Oliveira1, A. F. Amaral2, E. Nascimento3, F. E. Arimura4, B. G. Baldi5, R. A. Kairalla6, C. R. Carvalho7; 1Pulmonary Medicine, Pulmonary Division, Heart Institute (InCor), Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo, Brazil., Sao Paulo, Brazil, 2Respiratory and Critical Care Medicina, Pulmonary Division, Heart Institute (InCor), Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo, Brazil., São Paulo, Brazil, 3University of Sao Paulo, Sao Paulo, Brazil, 4Pulmonology, Pulmonary Division, Heart Institute (InCor), Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo, Brazil., Sao Paulo, Brazil, 5Pulmonary Division, Heart Institute (InCor), University of Sao Paulo, Sao Paulo, Brazil, 6Univ Sao Paulo, Sao Paulo, Brazil, 7Pulmonary Division, Heart Institute (InCor), Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo, Brazil., Sao Paulo, Brazil.
Introduction: Lymphangioleiomyomatosis (LAM) is a rare neoplastic disease of smooth muscle like-cells affecting predominantly women in reproductive age. Hypersensitivity pneumonia (HP) is defined as an immunologically mediated inflammation of the lung parenchyma in response to inhalation of a wide variety of antigens. This is a report of a rare combination of LAM and HP in a single patient.
Objectives: Report a case of lymphangioleiomyomatosis with hypersensitivity pneumonia.
Method: Review of medical records.
Case Report: An asymptomatic 51 year-old woman presented diffuse thin-walled cysts on chest CT performed due to a car accident. The patient had no rheumatological or constitutional symptoms but reported daily long term contact with birds. Physical examination was unremarkable and there was any other characteristic suggestive of LAM. Then, an open lung biopsy was performed and hispathological analysis revealed chronic interstitial inflammation with multiple small loose granulomas and lymphocytic infiltrate associated with cysts. There were also nodules of atypical muscle cells in non-granulomatous areas with positivity to smooth-muscle actin and HMB-45 antibodies. The multidisciplinary discussion concluded that it was compatible with the combination of HP and LAM.
Conclusions: We report a rare case of a patient with LAM and HP. Other differential diagnosis should be included in cases of unexplained dyspnea in patients with LAM.
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