Abstract

BackgroundThe differential diagnosis of diffuse cystic lung disease (DCLD) is a clinical challenge. We wish to analyze the distribution of the etiology of DCLD based on data from a single lymphangioleiomyomatosis (LAM) clinic.MethodsAll DCLD patients at the LAM Clinic of Peking Union Medical College Hospital between January 2006 and December 2019 were analyzed. Information on the demographic, clinical, radiological, and pathological features was collected.ResultsA total of 1010 patients with DCLD on CT scan were evaluated. A sum of 711(70.4%) patients were diagnosed with definite or probable LAM. Other diagnoses included Birt–Hogg–Dubé syndrome (46), Sjogren's syndrome (38), pulmonary Langerhans cell histiocytosis (14), lung tumors (3), Castleman disease (2), antineutrophil cytoplasmic antibody-associated vasculitis (2), systemic lupus erythematosus (1), Marfan syndrome (1), amyloidosis (1), congenital cystic adenomatoid malformation of the lung (1), and pleuroparenchymal fibroelastosis (1). In the 38 patients diagnosed with Sjogren's syndrome, 2 were diagnosed with light-chain deposition disease, 2 were diagnosed with amyloidosis and 1 was diagnosed with lymphocytic interstitial pneumonia. One hundred and eighty-nine patients (18.7%) were undiagnosed. Lung biopsy results were available in 27 patients in the undiagnosed DCLD group but did not provide a diagnosis.ConclusionApproximately 70% of DCLD patients in our LAM clinic had LAM. The common differential diagnoses included Birt–Hogg–Dubé syndrome, Sjogren’s syndrome, and pulmonary Langerhans cell histiocytosis. Detailed clinical information and laboratory, genetic, and pathological investigations provide correct diagnoses in most patients with DCLD.

Highlights

  • Diffuse cystic lung disease (DCLD) refers the presence of multiple air-filled spaces with a wall thickness less than 2 mm and clear boundaries with the lung tissues [1]

  • Full list of author information is available at the end of the article

  • The aim of this study was to analyze the clinical data of diffuse cystic lung disease (DCLD) patients registered in the Lymphangioleiomyomatosis (LAM) clinic of Peking Union Medical College Hospital (PUMCH) in recent years

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Summary

Introduction

Diffuse cystic lung disease (DCLD) refers the presence of multiple air-filled spaces with a wall thickness less than 2 mm and clear boundaries with the lung tissues [1]. An increasing number of diseases labeled DCLD make the differential diagnosis of this condition difficult [2]. The aim of this study was to analyze the clinical data of DCLD patients registered in the Lymphangioleiomyomatosis (LAM) clinic of Peking Union Medical College Hospital (PUMCH) in recent years. We summarized the distribution of their etiologies and proposed a stepwise diagnostic procedure to improve the diagnosis of DCLD. The differential diagnosis of diffuse cystic lung disease (DCLD) is a clinical challenge. We wish to ana‐ lyze the distribution of the etiology of DCLD based on data from a single lymphangioleiomyomatosis (LAM) clinic

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