Abstract
IntroductionRelapsing polychondritis is a rare multisystem autoimmune disease of uncertain etiology characterized by recurrent episodes of inflammation and progressive destruction of cartilaginous tissues. Its respiratory, cardiovascular, renal and neurological complications are life-threatening, and it is thus important to recognize the disease and its complications early. Relapsing polychondritis may follow a slowly evolving or rapidly progressive course.Case presentationThe case of a 39-years-old Caucasian woman with a three-year history of recurrent bilateral chondritis of the auricles, nasal chondritis, seronegative polyarthritis and dermatitis is reported. She had an associated bilateral stapedial fixation and one side was successfully operated on. She also had a large septal perforation involving both the cartilaginous and bony parts. The patient first presented with severe cutaneous inflammation when she was only one month old, and so this is an illustrative case of relapsing polychondritis that slowly evolved over many years.ConclusionsRelapsing polychondritis is still a relatively uncommon condition, which explains why there is often a delayed diagnosis of the disease. It is usually difficult to examine tympanic membranes in cases of relapsing polychondritis, and, therefore stapes fixation should also be suspected when there is an associated conductive hearing loss.
Highlights
Introduction: Relapsing polychondritis is a rare multisystem autoimmune disease of uncertain etiology characterized by recurrent episodes of inflammation and progressive destruction of cartilaginous tissues
The patient first presented with severe cutaneous inflammation when she was only one month old, and so this is an illustrative case of relapsing polychondritis that slowly evolved over many years
Relapsing polychondritis is an uncommon, chronic multisystem disorder characterized by recurrent episodes of inflammation of the cartilaginous tissues that often leads to progressive destruction
Summary
Relapsing polychondritis (RP) is an uncommon connective tissue disease characterized by recurrent episodes of inflammation and progressive destruction of cartilaginous tissues. Biopsies of the patient’s left auricular cartilage, nasal septal cartilage and the lateral walls of the nose revealed a chronic non-specific inflammation. Based on her clinical features at presentation and her medical history, a clinical diagnosis of relapsing polychondritis was made, the patient having exhibited the following features: 1) recurrent bilateral auricular chondritis; 2) non-erosive seronegative polyarthritis; 3) chondritis of nasal cartilage; and 4) cutaneous inflammation. She was initially treated with high dose prednisolone and cyclophosphamide and is on maintenance doses of prednisolone and azathioprine
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