A clinical case of late diagnosis of PAP with good response to whole lung lavage


Submitted: 4 May 2020
Accepted: 30 November 2020
Published: 26 January 2021
Abstract Views: 509
PDF: 119
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Authors

  • Maria Cardone Respiratory subintensive care unit, Monaldi Hospital, Naples, Italy.
  • Ludovica Capitelli Department of Clinical Medicine and Surgery, Section of Respiratory Diseases, University of Naples Federico II, Naples, Italy.
  • Francesco Squillante Respiratory subintensive care unit, Monaldi Hospital, Naples, Italy.
  • Filippo Ricciardiello Department of otolaryngology/head and neck surgery, AORN Antonio Cardarelli, Naples, Italy.
  • Marco Bocchetti Department of Precision Medicine, University of Campania “Luigi Vanvitelli”, Naples; Biogem Scarl, Institute of Genetic Research, Laboratory of Molecular and Precision Oncology, Ariano Irpino, Italy.
  • Angela Irene Mirizzi Department of Clinical Medicine and Surgery, Section of Respiratory Diseases, University of Naples Federico II, Naples, Italy.
  • Giuseppe Russo Department of otolaryngology/head and neck surgery, AORN Antonio Cardarelli, Naples, Italy.
  • Francesco Perna Department of Clinical Medicine and Surgery, Section of Respiratory Diseases, University of Naples Federico II, Naples, Italy.

Pulmonary Alveolar Proteinosis (PAP) is a rare syndrome characterized by the accumulation of surfactant in alveoli and terminal airways resulting in respiratory failure. PAP comprises part of a spectrum of disorders of surfactant homeostasis (clearance and production). We describe the case of a 54 years-old woman hospitalized in March 2017 with diagnosis of acute hypoxemic respiratory failure secondary to pulmonary interstitial disease. In our case, the patient arrived at our observation after developing a severe respiratory insufficiency although a long term history of respiratory symptoms. This diagnostic delay suggests that alveolar proteinosis should always be considered as a possible diagnosis in patients with dyspnea, cough and crazy paving at HRTC. In spite of this the disease resolved with a cycle of total lung lavage and the symptomatology has improved with the resolution of the radiological picture without any other therapeutic approach.


Cardone, M., Capitelli, L., Squillante, F., Ricciardiello, F., Bocchetti, M., Mirizzi, A. I., Russo, G., & Perna, F. (2021). A clinical case of late diagnosis of PAP with good response to whole lung lavage. Translational Medicine Reports, 4(1). https://doi.org/10.4081/tmr.9081

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