Relapsing polychondritis (RP) is a rare multisystem disease characterised by progressive inflammation and destruction of cartilaginous structures. Airway involvement in RP occurs in approximately 50% of cases. We present a 70-year-old woman with a diagnosis of early adult onset asthma. She required multiple hospital admissions for recurrent episodes of acute severe dyspnoea for 1 year. These were treated as asthma. Months later she developed saddle nose deformity and hoarseness of voice. CT revealed tracheal/bronchial wall thickening and luminal narrowing. Based on these findings, RP was diagnosed. Insertion of a tracheobronchial stent was necessary due to severe tracheobronchomalacia. This intervention improved the patient's dyspnoea immediately. This case is reported to raise awareness of airway involvement in RP and discuss its current management. Early diagnosis of RP is essential to allow prompt treatment and to decrease the risk of life-threatening airway collapse.
We present a case of a patient with sarcoidosis and who subsequently developed dysphagia for solids, and some difficulty in swallowing liquids. High-resolution manometry of the oesophagus showed absent peristalsis in the oesophageal body and incomplete relaxation of the lower oesophageal sphincter. The diagnosis of sarcoidosis with oesophageal involvement was made and treatment with prednisolone 30 mg OD initiated. The patient improved symptomatically and high-resolution manometry was repeated showing complete recovery of oesophageal peristalsis and a deeper relaxation of the lower oesophageal sphincter. This is thus the first description of high-resolution manometry in sarcoidosis-induced changes of the oesophagus and of the effect treatment has on these motility changes. Oesophageal involvement of sarcoidosis is extremely rare and only a few cases have been reported. The symptoms and manometric pattern of this disorder mimics that of achalasia. However, we show that treatment with prednisolone results in a completely disappearance of the symptoms of dysphagia and subsequently lead to a large improvement of oesophageal motility.
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