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Cystic lung disease

Van Holden, M.D.
Pulmonary and Critical Care Medicine Fellow
University of Maryland Medical Center, Baltimore, MD

Case:

A 33 year-old woman with a history of uterine fibroids and iron deficiency anemia presented with progressively worsening dyspnea on exertion for 2 years. She reported shortness of breath with going up a flight of stairs. She had an associated dry cough and intermittent sharp right-sided chest pain.

Social History: Smokes 3-5 cigarettes per day for 15 years. Drinks alcohol occasionally. Prior history of smoking marijuana. Has had 1 sexual partner.

Vital signs and cardiopulmonary exam were normal. Basic labs and EKG were also normal. Due to an elevated D-dimer of 570 ng/mL, she had a CTA chest done.

CXR and CT chest

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What is the most likely etiology of her diffuse cystic lung disease?

  1. Pulmonary Langerhans cell histiocytosis
  2. Lymphoid interstitial pneumonia
  3. Lymphangioleiomyomatosis
  4. Emphysema

Correct Answer 2

Work-up revealed HIV positivity. Subsequently, pathology from transbronchial biopsies correlated to lymphoid interstitial pneumonia.  

The Fleischner Society defines a cyst as a "round parenchymal lucency or low-attenuating area with a well-defined interface with normal lung and a wall thickness < 2mm," as opposed to a cavity which has a wall thickness > 2 mm. Other cyst mimics include emphysema, pneumatoceles, bronchiectasis, and honeycombing.

Differential of Cystic Lung Disease

Pulmonary Langerhans cell histiocytosis
Lymphoid interstitial pneumonia
Lymphangioleiomyomatosis
Birt-Hogg-Dubé syndrome
Erdheim Chester disease
Follicular bronchitis
Sarcomas
Pleuropulmonary blastoma
Light chain deposition disease
Amyloidosis
Sjogren syndrome
Pneumocystis jiroveci
Recurrent respiratory papillomatosis
Desquamative interstitial pneumonia
Post-traumatic pseudocysts

We review the 3 answer choices here.

 Pulmonary Langerhans cell histiocytosisLymphoid interstitial pneumoniaLymphangioleiomyomatosis
 
Clinical characteristics 80-90% of patients are current or former smokers. Presents in young patients, M>F. Commonly affects middle-aged women. Associated with connective tissue disorders and immunodeficiency states. Young women. Can be sporadic or associated with tuberous sclerosis. Extrathoracic features include hepatic and renal angiomyolipomas and chylous ascites.
Presentation About 2/3 present with cough or dyspnea. 15% present with a pneumothorax. Cough or dyspnea 40-50% present with a pneumothorax
Histology Peribronchiolar infiltration by the Langerhans cells, leading to stellate nodule formation Diffuse interstitial inflammatory infiltrate, usually around lymphatics Smooth muscle cell infiltration of the lung parenchyma, airways, lymphatics, and blood vessels
Imaging

Thick or thin-walled bizarre-shaped cysts in the upper lung that spare the costophrenic angles

Cysts are usually few in number and distributed diffusely in both lungs or primarily in the lower lung

Diffuse thin-walled cysts that are round, ovoid, or polygonal

  image 4 image 5 image 6

References:

  1. Gupta N, Vassallo R, Wikenheiser-Brokamp KA, et al. Diffuse cystic lung disease part I. Am J Respir Crit Care Med 2015;191(12):1354-1366.

  2. Gupta N, Vassallo R, Wikenheiser-Brokamp KA, et al. Diffuse cystic lung disease part II. Am J Respir Crit Care Med 2015;192(1):17-29.

  3. Hansell DM, Bankier AA, MacMahon H, et al. Fleischner Society: Glossary of terms for thoracic imaging. Radiology 2008;246:697-722.

  4. Jawad H, Walker CM, Wu CC, et al. Cystic interstitial lung diseases: recognizing the common and uncommon entities. Curr Probl Diagn Radiol 2014;43:115-127.

Last Reviewed: February 2016