IS Case 300: Langerhans cell histiocytosis, pulmonary

Gunvir Gill, MD

Imaging Sciences URMC 2009
Publication Date: 2009-08-05

History

Patient is a 51-year-old female with history of Crohn's disease, 40-pack-year smoking history, as well as toxic pulmonary exposure while working as a furniture polisher. She now presents with dyspnea on exertion, cough productive of clear whitish sputum, and increasing oxygen requirement.

Findings

Multiple pulmonary nodules of varying sizes with upper lobe predominance, as well as a reticulonodular pattern on chest plain films.

Diagnosis

Langerhans cell histiocytosis, pulmonary

Discussion

The differential for diffuse reticulonodular pattern on chest x-ray as well as multiple bilateral pulmonary nodules and lymphadenopathy in a patient with the above history would be broad, including infection (TB, varicella and histoplasmosis), autoimmune diseases (rheumatoid arthritis), metastasis, hypersensitivity pneumonitis, pneumonia, sarcoidosis, and Langerhans cell histiocytosis.

This patient underwent a video-assisted thoracoscopic surgery (VATS) with left lung biopsy, pathology from which demonstrated Langerhans cell histiocytosis.

The most common pulmonary findings on CT are nodular and cystic changes, which occur predominantly in the middle and upper lobes. The abnormality most discussed in the literature is the presence of lung cysts, which are often (but not always) less than 20 mm in diameter and typically have a thin wall (1 mm or less) that can be seen on high-resolution CT. The frequency of cystic change reflects the timing of imaging during the course of the disease. In the early stages, the most common finding is nodular change, whereas in the later stages, cystic change and fibrosis predominate.

Interestingly, smoking has been associated with Langerhans cell histiocytosis, though the pathophysiology is still unclear. However in a substantial portion of patients this is the only intervention required for stabilization of symptoms, and a few c

References

  1. Brauner MW, Grenier P, Tijani K, Battesti JP, Valeyre D. Pulmonary Langerhans cell histiocytosis: evolution of lesions on CT scans. Radiology. 1997 Aug;204(2):497-502. PMID: 9240543 [PubMed]
  2. Mogulkoc N, Veral A, Bishop PW, Bayindir U, Pickering CA, Egan JJ. Pulmonary Langerhans' cell histiocytosis: radiologic resolution following smoking cessation. Chest. 1999 May;115(5):1452-5. PMID: 10334170 [PubMed]
  3. Vassallo R, Ryu JH, Colby TV, Hartman T, Limper AH. Pulmonary Langerhans'-cell histiocytosis. N Engl J Med. 2000 Jun 29;342(26):1969-78. PMID: 10877650 [PubMed]

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