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Pulmonary alveolar proteinosis: A single center retrospective analysis of 14 cases

Ning Zhang1, Zhilong Jiang2, Changzhou Shao2

  • 1Department of Pulmonary Medicine, Xiamen Branch, Zhongshan Hospital, Fudan University, Xiamen 361000, China; Shanghai Institute of Respiratory Diseases, Department of Pulmonary Medicine, Zhongshan Hospital, Fudan University, Shanghai 200032, China.

Medicina Clinica
|August 29, 2020
PubMed
Abstract

Insights

Pulmonary alveolar proteinosis (PAP) is a rare lung disease. This study suggests lactate dehydrogenase (LDH) may indicate disease severity, and bronchoscopy is reliable for diagnosis, with lung lavage effective for treatment.

Area of Science:

  • Pulmonology
  • Rare Diseases
  • Respiratory Medicine

Background:

  • Pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by abnormal alveolar accumulation of foamy macrophages and PAS-positive material.
  • Current knowledge regarding PAP characteristics remains limited, necessitating further clinical understanding.

Observation:

  • A retrospective analysis of 14 adult PAP patients was conducted.
  • Serum lactate dehydrogenase (LDH) levels were correlated with arterial partial pressure of oxygen (PaO2) and diffusion capacity for carbon monoxide (DLCO).
  • Transbronchial lung biopsy (TBLB) achieved a 100% diagnostic positive rate.

Findings:

  • LDH levels may correlate with PAP disease severity.
  • TBLB is a highly sensitive method for definitive PAP diagnosis.
  • Whole lung lavage (WLL) resulted in varying degrees of remission for treated patients.
  • Supportive care and observation were sufficient for mild cases, maintaining stability.

Implications:

  • LDH may serve as a biomarker for PAP severity.
  • Bronchoscopy is a reliable diagnostic tool for PAP.
  • Bilateral WLL is an effective treatment for indicated PAP patients.
  • Unilateral WLL or observation may be suitable alternatives for select patients.
Keywords:
Enfermedad pulmonar intersticialEnfermedad pulmonar raraInterstitial lung diseaseProteinosis alveolar pulmonarPulmonary alveolar proteinosisRare lung disease

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