ERS 2019 – Lungs on Fire: Interstitial lung diseases (2nd part)

  • Eliana Mesa
  • Conference Reports
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  • First case reports on a 19-year-old male, with a 5-year history of diabetes insipidus, and a 2-year history of smoking, until one month ago, but no other known risk factors. He presented with exertional dyspnoea, dry cough and fever (primarily nocturnal). Upon physical examination he had fever, a SpO2 of 88% and bilateral decreased breathing sound. The chest X-ray revealed bilateral infiltrates. The first diagnostic approach was tuberculosis, although infectious disease was discarded. A CT scan showed images of emphysema and cysts, suggested to be pulmonary Langerhans Cell Histiocytosis. To reach a diagnosis, bronchoscopy with bronchoalveolar lavage (BAL) or transbronchial lavage was proposed. All investigations were negative with the exception of Stenotrophomonas maltophilia in bacteriological cultures. In spite of wide-spectrum antibiotics, clinical symptoms persisted and an increase of infiltration was observed on chest ray. A lung biopsy revealed an infiltration of CD-1a positive Langerhans cells in the lung parenchyma and granulomas. Antibiotics against tuberculosis was initiated. After 40 days of treatment, pneumothorax was presented and resolved. In a follow-up evaluation, the consolidations and infiltrations had been cleared.
  • The second case was that of a 49-year old female with a 2-week history of dry cough, dyspnoea, fever and fatigue. She presented with fever, bilateral crackles, an oxygen saturation of 84%, blood eosinophilia Blastocystis hominis in her stool. The fact that she had pets at home was potentially relevant. A CT revealed bilateral areas of consolidation and ground glass opacities in both upper and lower lobes. Prednisone, antibiotics and oxygen therapy was initiated which gave rapid improvement. Subsequent anatomopathological findings were a chronic granulomatous inflammatory process with numerous eosinophils, interstitial fibrosis and vascular alterations without fibrosis. Following the exclusion of other diagnoses, a parasitic pulmonary infection was diagnosed. The patient was IgG positive for Toxocara canis. Treatment with albendazole was initiated and the patient recovered. A follow-up chest CT was proposed to assess changes in lung lesions.
  • A 76-year-old woman with no relevant respiratory history complained of fatigue, dry cough and wheezing. She had clinically moderate restrictive syndrome in lung function tests. Thoracic CT revealed alterations compatible with interstitial lung disease (ILD). Thoracic high-resolution CT (HRCT) showed areas of ground glass, consolidation areas with air bronchogram, generalised fibrosis and emphysematous bullae. There were no other significant findings. Features seen on the lung biopsy were compatible with organised pneumonia; centrilobular and paraseptal fibrosis, and proliferation of fibrosis within the alveolar sacs and ducts, without identifiable cause. After one month of prednisolone, the patient developed pleuritic chest pain and shortness of breath. A Chest X-ray revealed hydropneumothorax (drained compatible with pyopneumothorax). Ceftriaxone was initiated and corticoid therapy continued. After one month, the previously negative bacterial culture gave a positive result for Nocardia Cyriacigeorgica. Co-trimoxazole was prescribed. In spite of clinical improvement, a new aggravation of the pneumothorax worsened the respiratory insufficiency and the patient died.