TY - JOUR T1 - Pulmonary lymphangitic carcinomatosis presenting as severe interstitial lung disease in a 15-year-old female JF - European Respiratory Review JO - EUROPEAN RESPIRATORY REVIEW SP - 208 LP - 210 DO - 10.1183/09059180.00000911 VL - 20 IS - 121 AU - F.J. Gilchrist AU - H. Alton AU - M-A Brundler AU - L. Edwards AU - A. Plunkett AU - S. Rao Y1 - 2011/09/01 UR - http://err.ersjournals.com/content/20/121/208.abstract N2 - To the Editor:Pulmonary lymphangitic carcinomatosis (PLC) is a metastatic lung disease characterised by the diffuse infiltration and obstruction of the pulmonary parenchymal lymphatic system by tumour cells [1]. It is an extremely rare diagnosis in paediatrics and we believe that this is the first reported case in a child, presenting as interstitial lung disease without a known primary tumour.A 15-yr-old Caucasian female was transferred to our hospital (Birmingham Children's Hospital, Birmingham, UK) for further assessment and management of respiratory failure, secondary to presumed interstitial lung disease. 8 weeks previously she had presented to her local hospital with a 5-week history of cough and shortness of breath associated with lethargy, poor appetite and early satiety. On examination she was hypoxic with bilateral wheeze and crackles. Bilateral perihilar shadows and increased bronchovascular markings were seen on the chest radiograph. Atypical pneumonia was diagnosed and she received oxygen, oral erythromycin and inhaled salbutamol. She recovered but after discharge required two courses of oral prednisolone and salbutamol for wheeze and shortness of breath.She deteriorated while visiting relatives in the Birmingham area of the UK and required admission for cough, shortness of breath and haemoptysis. On examination she was tachypnoeic, tachycardic and hypoxic but not clubbed. There was generalised decreased air entry and bilateral inspiratory crackles on ausultation. Her blood tests were unremarkable apart from elevated C-reactive protein (54 mg·L−1). A chest radiograph and subsequent computed tomography (CT) scan of her chest showed extensive, bilateral interstitial changes (fig. 1). A diagnosis of presumed interstitial lung disease was made. The differential diagnoses are … ER -