Diffuse pulmonary lymphatic disease presenting as interstitial lung disease in adulthood: report of 3 cases.

Oct 2012

Boland JM, Tazelaar HD, Colby TV, Leslie KO, Hartman TE, Yi ES.

Source

Departments of *Laboratory Medicine and Pathology ‡Radiology, Mayo Clinic, Rochester, MN †Mayo Clinic, Scottsdale, AZ.

Abstract

Diffuse pulmonary lymphatic diseases are typically diagnosed shortly after birth or in childhood, but rarely may become evident in adulthood. We report 3 adult patients who presented with diffuse interstitial lung disease clinically and radiologically but on biopsy were found to have diffuse pulmonary lymphatic disease (2 cases of pulmonary lymphangiectasis and 1 case of pulmonary lymphangiomatosis). These patients presented with the insidious onset of symptoms including shortness of breath and cough. Imaging studies of the chest showed diffuse pulmonary interstitial opacities, often with a perilymphatic distribution. The clinical differential diagnostic considerations before surgical lung biopsy included infection, neoplasm, and interstitial lung disease. The histopathologic features included abnormal vessels and associated fibrosis following lymphatic routes, namely visceral pleura, bronchovascular bundles, and interlobular septa. Lymphangiectasis was characterized by dilation of normally distributed lymphatic spaces, whereas lymphangiomatosis showed a complex anastamosing proliferation of lymphatic vascular spaces without significant dilatation. The dilated lymphatic spaces often had undergone muscularization, which could easily lead to misclassification as veins. Immunohistochemical staining for the lymphatic endothelial marker D2-40 was helpful in correctly classifying these lesions. Diffuse pulmonary lymphatic disease can rarely present in adulthood, wherein the histologic findings can be subtle and could be overlooked as nonspecific reactive changes or misdiagnosed as an idiopathic interstitial lung disease. Recognition of the characteristic lymphangitic distribution of abnormally dilated or reduplicated lymphatic spaces is key to the correct diagnosis.

Surgical Pathology

Chronic interstitial lung disease in children

Chronic interstitial lung disease in children
J Pediatr (Rio J). 2007 May/June

Paiva MA, Amaral SM.

Hospital dos Servidores do Estado, Rio de Janeiro, RJ, Brazil. mariaaparecida.paiva@gmail.com.

OBJECTIVES: To describe clinical and diagnostic features and the results of therapeutic conduct in a group of pediatric patients with chronic interstitial lung disease.

METHODS: A retrospective study of 25 immunocompetent patients, aged 2 months to 17 years, with chronic interstitial lung disease, admitted to the Pediatric Pulmonary Section, Department of Pediatrics, Hospital dos Servidores do Estado, over a 20-year period (1984-2004). A routine protocol for persistent chronic pneumonias was used and the patients with interstitial lung disease were selected. Clinical, laboratory and imaging data were analyzed.

RESULTS: Twenty-five patients were diagnosed with chronic interstitial lung disease, 13 were aged less than 2 years and 17 were male. Diagnoses were made based on history, physical examination and routine tests in one case, based on more complex tests in three cases and based on the results of invasive tests in 21 cases (20 by lung biopsy and one by bronchoalveolar lavage). Except for one patient with pulmonary lymphangiectasia, the long-term treatment (1 to 7 years) consisted of corticosteroid, in six cases associated with hydroxychloroquine. Four patients required home oxygen therapy. The authors followed the patients in the outpatient department (6 a 8 visits/year). Patient outcome was: good (15); regular, with mild sequelae (4); and poor, with severe sequelae (3). One patient was lost in the follow-up period and two died.

CONCLUSIONS: Chronic interstitial lung diseases in children are a group of rare pulmonary disorders, but a relevant one because of the possible progression to pulmonary fibrosis. Early diagnosis and a long-term, specialized treatment and follow-up are important for the patient outcome. Pediatricians should be aware of these diseases because in many cases diagnosis and treatment are overlooked.

PMID: 17551654 [PubMed - as supplied by publisher]