Diagnosis of the case presented in the previous edition

RADIOLOGICAL DIAGNOSIS

Diagnosis of the case presented in the previous edition

J Bras Pneumol 2004;30(2):187

LYMPHANGIOLEIOMYOMATOSIS

Comments

Lymphangioleiomyomatosis (LAM) is an idiopathic disease that mainly affects women of childbearing age. It is characterized by non-neoplastic and atypical proliferation of smooth muscle cells, especially in the pulmonary parenchyma, resulting in vascular and airway obstruction, pulmonary cysts and progressively greater impairment of respiratory function. In approximately half of all cases, LAM may involve concomitant renal angiomyolipomas, appearing sporadically or as part of the tuberous sclerosis complex (TSC), which also includes mental retardation, convulsions and skin lesions.

Symptoms of LAM commonly include dyspnea, cough and thoracic pain. In many cases, the initial manifestation is pneumothorax. According to some authors, the prevalence of pneumothorax may be as high as 68%, compared with 29% for chylothorax.

In virtually all cases, simple radiographs present alteration in the form of a diffuse reticular pattern, and larger cysts may also be seen. Pneumothorax and pleural effusion may occasionally be observed. Lung volume is maintained or increased.

The imaging method of choice is computed tomography, preferably high-resolution computed tomography, which will show air-filled thin-walled cysts measuring 0.2 to 5.0 cm in diameter with diffuse distribution. Lymph nodes may be enlarged, and pleural effusion, pneumothorax and pulmonary opacities with ground-glass patterns may be present. The abdominal CT may reveal renal angiomyolipomas and enlargement of additional lymph nodes.

The main differential diagnoses are those that lead to pulmonary hypertransparencies. In most cases, pulmonary emphysema can easily be distinguished from LAM since emphysema does not present the walled cysts seen in LAM and is predominantly found in the upper lobes. Histiocytosis of Langerhans cells is the main differential diagnosis, although also found mainly in the upper lobes and rarely involves the costophrenic sulci, producing cysts that are usually irregular and nodular. In addition, histiocytosis almost exclusively affects smokers. Lymphoid interstitial pneumonia with follicular bronchiolitis represents another differential diagnosis, although it presents fewer cysts and it is usually related to collagenosis or AIDS.

References

1. Avila NA, Chen CC, Chu SC: Pulmonary lymphangioleiomyomatosis: correlation of ventilation-perfusion scintigraphy, chest radiography, and CT with pulmonary function tests. Radiology 2000; 214::441-6

2. Sullivan EJ: Lymphangioleiomyomatosis: a review. Chest 1998; 114:1689-703

3. Lim KE, Tsai YH, Hsu YIY, Hsu WW. Pulmonary lymphangioleiomyomatosis: high-resolution CT findings in 11 patients and compared with the literature. Clin Imaging 2004; 28:1-5

4. Paciocco G, Uslenghi E, Bianchi A. Diffuse cystic lung diseases: correlation between radiologic and functional status.Chest 2004;125:135-42.

5. Koyama M, Johkoh T, Honda O. Chronic cystic lung disease: diagnostic accuracy of high-resolution CT in 92 patients. AJR 2003;180:827-35.

Acertadores do caso de Março/Abril 2004

Ahmad Abduny Rahal - Clinar, Pouso Alegre - MG

André L. S. Becker, Hospital Fátima - Caxias do Sul RS

Angelica Vianna - Universidade Federal do Rio de Janeiro, RJ

Angelo Fernandez - Faculdade de Medicina da Universidade de São Paulo- SP

Antonio Delfino de Oliveira Junior - Santa Casa de Sao Carlos, Sao Carlos - SP

Carlos Leonardo Carvalho Pessôa - Universidade Federal do Rio de Janeiro, Niterói RJ

Carminda Maria Campanate Ribeiro - Hospital Cardoso Fontes, Rio de Janeiro - RJ

Célio de Deus Simões - Policlinica Mara, Patos de Minas - MG

Evandro Monteiro de Sá Magalhaes - Faculdade de Medicina de Alfenas MG

Giancarlo Sanches - Universidade Estadual de Maringá, Maringá - PR

Guilherme de Carvalho Ribeiro - Universidade Federal de Minas Gerais - MG

Hugo Hyung Bok Yoo - Hospital das Clínicas de Botucatu-UNESP, Botucatu/SP

Juliana Cardozo Fernandes - Pavilhão Pereira Filho, Porto Alegre RS

Leandro Almeida Streher - Hospital de Caridade Dr. Astrogildo de Azevedo, Santa Maria - RS

Luiz Carlos Corrêa da Silva - Pavilhão Pereira Filho - Santa Casa, Porto Alegre - RS

Marcelo Coelho Machado - Centro Médico Itamaraty, Vitoria da Conquista - BA

Marcelo Zanchetin - Hospital São Roque, Carlos Barbosa, RS

Marilia de Castro Lima Varella - Universidade de Mogi das Cruzes, Mogi das Cruzes, SP

Marlon Molina - Belo Horizonte - MG

Marta de Andrade Lima Coêlho - Hospital Octávio de Freitas, Recife-PE

Mauro Zamboni - Instituto Nacional do Cancer, Rio de Janeiro, RJ

Miguel Abidon Aidé - Universidade Federal Fluminense, RJ

Nivaldo Santos Gribel - Hospital de Cataguases, Cataguases MG

Oreste Pedro Maia Andrade - Hospital Uniclínicas, Chapecó - SC

Pedro Mello - Votuporanga - SP

Ricardo Delduque - Catanduva SP

Rodrigo Bortoli - Hospital Santo Ângelo e Hospital de Guarnição de Santo ângelo, Santo

Rogério de Mattos Bártholo - Hospital Municipal Cardoso Fontes, Rio de Janeiro - RJ

Rubens Altair Amaral de Padua - Hospital Vaz Monteiro, Lavras, MG

Saulo Maia d`Avila Melo - Hospital São Lucas, Aracaju - SE

Shirley Coletty dos Santos - DEMED da Câmara dos Deputados, Brasília/DF

Suzete Varela Mayo - Hospital Heliópolis, São Paulo/SP

Virgílio Alexandre Nunes de Aguiar - Faculdade de Medicina da UNIMES, Santos-SP

Publication Dates

• Publication in this collection
  02 Sept 2004
• Date of issue
  June 2004