Figure 2: CT virtual bronchoscopy (A-left) and bronchography (B-right) demonstrating narrowed left main stem bronchus with progression of wall thickening.
Figure 3: Non-contrast CT showing anteroposterior collapse of the tracheal cartilage. Note relative sparing of posterior wall.
in the literature for any paper describing the clinical and radiological evolution of this entity. The disease typically responds to steroids, but other immunosuppressants such as Methotrexate have also been used to provide relief. Current therapy also involves bronchoscopic/surgical palliation, and radiological imaging is indispensable for monitoring therapy to achieve the best quality of life for patients with this currently incurable disease. Several radiological features have been associated with the disease, but only one is pathognomonic; namely, inflam- mation of the anterior and lateral tracheal wall, sparing the posterior wall. 5.6 Since this disease shares common features Figure 4: Non-contrast CT showing airspace disease with consolidation in the left lower lobe. An endobronchial stent is seen in the left main stem bronchus.
more common in Caucasians, and there is consequently very little data on non-Caucasian patients. Several attempts have been made to identify the etiology, and some progress made includes identification of its autoimmune nature and the discovery of antibodies to cartilage-specific collagen. 1 Pathology demonstrates inflammation and subsequent fibrosis in affected cartilage. The constellation of clinical features, which characterizes the condition, is currently the mainstay of diagnosis. 2,3 Radiology, especially CT, is essen- tial to management, as it provides confirmatory evidence of clinical features and can be used safely for follow-up. 4 To the best of our knowledge, there is no current evidence
J La State Med Soc VOL 166 March/April 2014 57
Made with FlippingBook - Online catalogs