ERS 2019 – Lungs on Fire: Interstitial lung diseases? (1st part)

  • Eliana Mesa
  • Conference Reports
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  • A 56-year-old smoker woman presented with lung functional impairment and basal bilateral crackles. Chest CT showed patchy ground glass and basal crazy paving bilateral, which was dominant on the left side. Infection was ruled out. The patient had been frequently using nasal lipid cream and following 3 months of prednisone treatment, the illness progressed. A surgical lung biopsy found focal suggestion of lipoid pneumonia. After 8 weeks of avoiding cream and prednisone treatment, her clinical and radiological features worsened. The diagnostic was reassessed and a new biopsy revealed histology of diffuse adenocarcinoma with lipidic growth, positive for KRAS and negative for PD-L1 .
  • A 63-year-old ex-smoker male presented with a 6-month cough and mild exertional dyspnoea but no other known risk factor. He had a respiratory pattern of mild restrictive lung disease. Chest X-ray showed mild hyperlucency at the upper zones and mild left diaphragm elevation. Thoracic high-resolution CT (HRCT) showed bilateral, multiple and uniform thin-walled cysts. The diagnosis was oriented to Langerhans cell histiocytosis or lymphangioleiomyomatosis. Surgical biopsy by thoracoscopy or video-assisted thoracoscopic surgery (VATS) was suggested. Histopathology revealed areas of emphysema and nodular lesions. A diagnosis of diffuse pulmonary meningotheliomatosis should be contemplated. Considering the good prognosis, the treatment should be symptomatic with a close follow-up.
  • A 33-year-old male presented with a 3-month history of progressive exertional dyspnoea, requiring oxygen therapy. He had arthritis in his hands and knees for the previous 5 years. With the exception of allergic rhinitis and professional exposure to wood dust, no other significant features were reported. Serological findings showed autoimmune features. Treatment with prednisolone and azathioprine improved his arthritis but his respiratory condition deteriorated further. Interestingly, his 6-year-old daughter was also suffering from mild arthritic symptoms. Patchy ground glass, with no evidence of nodules, consolidation or honeycombing was visible on a CT-scan. Histological evaluation revealed follicular bronchiolitis with interstitial fibrosis and organised pneumonia. Bronchoalveolar lavage (BAL) showed an inflammatory reaction (26% lymphocytes). The suggested diagnosis is interstitial lung disease (ILD) caused by connective tissue disease (CTD). Mutations in the COPA gene were identified following a genetic test of the daughter and, subsequently, the patient. COPA-syndrome should be considered in young patients with ILD and extrapulmonary features with familial predisposition. The treatment of choice is canakinumab.