Journal of the Louisiana State Medical Society
Radiological Evolution of Relapsing Polychondritis
Oluwayemisi Ojemakinde, MD; Threta Reddy, MD; Carlos Previgliano, MD; Guillermo Sangster, MD; Adam Wellikoff, MD; Eduardo Gonzalez-Toledo, MD
Relapsing Polychondritis (RP) is a systemic condition characterized by chronic, episodic inflammation, es- pecially of cartilaginous and proteoglycan-rich structures. The etiology of this rare autoimmune disease is unknown, and so far, there is very little data available for non-Caucasians. RP presents with a constellation of non-specific inflammation, which sometimes appear in characteristic locations. Radiology is important in supporting the diagnosis, and this paper presents a case of a non-Caucasian patient monitored radiologically from early onset to the terminal stages.
CASE REPORT A 29-year-old African-American female initially pre- sented during a pregnancy with scleritis, nasal and aural chondritis, inflammatory arthritis, and axonal neuropathy. A clinical diagnosis of Relapsing Polychondritis (RP) was made, and management commenced with steroids with good response. Her initial chest radiograph and baseline chest computed tomography (CT) showed very minimal abnormalities. A year later, she developed symptoms concerning for obstructive sleep apnea, tracheobronchomalacia, and
tracheal stenosis. Chest CT at this time showed smooth anterior and lateral wall thickening with sparing of the posterior wall of the trachea, a typical finding which is almost pathognomonic 1 for RP (Figure 1). Her symptoms became progressive over a period of 18 months, despite use of BIPAP and steroid treatment. Attempts at bronchoscopy resulted in cardiac arrest from which she was resuscitated. However, volume-rendered CT bronchography and virtual bronchoscopy immaculately demonstrated the progressive airway narrowing (Figure 2A & B). She had a long stent placed via tracheostomy to bypass the tracheal stenosis, and a left main stem stent was placed to alleviate continuing respiratory distress (Figure 3). Over the course of 32 months and despite the use of Methotrexate, this patient developed progressive sensorineural hearing loss and cataracts. By the 40th month of her disease, she developed recalcitrant pneumonia (Figure 4) and pleural effusions on the left. The onset of progressive dysphagia in this patient prompted an esophagogram, which revealed a left tracheosophageal fis- tula that was repaired endoscopically (Figure 5). The patient terminally hadwhole lung collapse on the left (Figure 6), and she opted for home hospice. She had other complications of therapy, including acute kidney injury, Mallory Weis tear, osteoporosis, and obesity. The patient succumbed to respiratory compromise soon after. DISCUSSION RP is a rare disease with only 3.5 cases per million popu- lation reported in the United States. It has been recognized as a disease entity as far back as 1923 with 600 cases reported by 1997. 1 Although this disease has been found in all races, it is
Figure 1: Non-contrast chest CT showing smooth anterior and lateral wall thickening with sparing of posterior wall.
56 J La State Med Soc VOL 166 March/April 2014
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