Author(s)

Mitropoulou M, Tavernaraki A, Skarpalezos D, Stasis A, Stassinopoulou M

Patient

female, 58 year(s)

A 58-year-old greek woman presented with a long history of respiratory symptoms and ocular, auricular and nasal inflammation, which she had been suffering from since 1981.

A 58-year-old greek woman had a long history of respiratory symptoms and ocular, auricular and nasal inflammation since 1981. Initially, she had recurrent cough, conjuctivitis, episcleritis, and increased C- reactive protein. Later, she had recurrent inflammation of the pinnae and nose deformity. In 1989 the diagnosis of relapsing polychondritis was made and was treated with steroids. Soon after, the cough became intense and more frequent. A chemotherapeutic treatment with Endoxan (Cyclophosphamide) was initiated. The year before, the patient was admitted to our hospital with persistent wheezing and inspiratory dyspnea. She was suggested to undergo a CT scan of the thorax, in order to evaluate the tracheobronchial tree. The results showed an alteration of the normal morphology and marked stenosis of the distal trachea and main bronchi (Figs. 1a, 2a, 3a). A CT scan of the paranasal (Fig. 4a) sinuses was also performed, which showed a collapse of the nasal cartilage. The last CT scan of the thorax (Fig. 5a) showed infiltration of the right lung due to food aspiration.

Relapsing polychondritis (RPC) is a rare, chronic, multisystem inflammatory disorder, which mainly affects the cartilaginous tissues, and which is difficult to be diagnosed at an early stage. It is frequently associated with rheumatoid arthritis, systemic vasculitis, connective tissue diseases, and haematologic disorders. The tissues involved are the ears, joints, nose, larynx, trachea, eyes, heart valves, kidneys,and the skin. Airway complications–tracheobronchial narrowing and obstruction–are the most serious manifestations and can be fatal. The diagnosis of RPC is made on the basis of Damiani's criteria and imaging findings [1], [2]. CT is the most useful imaging modality to identify laryngeal, tracheal, bronchial and nasal cartilage involvement. CT findings include tracheobronchial wall thickening, lumen stenosis (due to oedema and inflammatory granulomas) and wall calcification, and, later, collapse of the lumen (caused by the destruction of the cartilage and by fibrous scarring of the tracheal walls). Collapse of the cartilage of the nose and calcification in the cartilages of the ears can also be clearly demonstrated by CT [4], [5]. The early diagnosis of RPC is crucial, as appropriate pharmacological treatment may prevent life-threatening airway obstruction. Expandable metallic stents can be placed using a flexible bronchoscope at a later stage, of tracheobronchial stenosis [1], [3].