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Rare disease
CASE REPORT
Middle lobe syndrome: an intriguing presentation of tracheobronchial amyloidosis
  1. Shekhar Kunal1,
  2. Shashi Dhawan2,
  3. Arvind Kumar3,
  4. Ashok Shah1
  1. 1Department of Pulmonary Medicine, Vallabhbhai Patel Chest Institute, University of Delhi, Delhi, India
  2. 2Department of Pathology, Histopathology Unit, Sir Ganga Ram Hospital, New Delhi, India
  3. 3Institute of Robotic Surgery, Sir Ganga Ram Hospital, New Delhi, India
  1. Correspondence to Professor Ashok Shah, ashokshah99{at}yahoo.com

Summary

Pulmonary involvement in amyloidosis is a distinct rarity. This clinical entity usually presents as tracheobronchial amyloidosis (TBA). A 32-year-old, never-smoker man presented with episodic dyspnoea and wheezing along with cough and mucoid sputum. The chest radiograph was suggestive of a middle lobe syndrome (MLS). High-resolution CT (HRCT) of the chest confirmed the presence of MLS. In addition, HRCT showed circumferential thickening of the trachea and the main bronchi, with thickening of the posterior membranous wall of trachea. Fibrebronchoscopy, done to evaluate MLS, visualised multiple small polypoidal lesions in the lower part of trachea and carina. Endobronchial biopsies showed homogeneous, acellular amorphous deposit in the subepithelial region, which was congophilic in nature. A diagnosis of TBA presenting as MLS was made. To the best of our knowledge, this is the first detailed report of MLS as a presentation of TBA in the English literature.

  • Asthma
  • Radiology

https://doi.org/10.1136/bcr-2017-219480

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    Footnotes

    • Contributors SK, AK and AS collected the clinical data and reviewed the literature. SD reviewed the pathological aspects. SK, SD, AK and AS drafted the manuscript. AS worked on the concept and is responsible for the genuineness of the data and is also the guarantor of the paper. All the authors have read and approved the final manuscript.

    • Competing interests None declared.

    • Patient consent Obtained.

    • Provenance and peer review Not commissioned; externally peer reviewed.