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A Unique Case of Relapsing Polychondritis Presenting with Acute Pericarditis

DOI: 10.1155/2013/287592

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Abstract:

Relapsing polychondritis (RP) is an inflammatory disease of the cartilaginous tissue primarily affecting the cartilaginous structures of the ear, nose, joints, and the respiratory system. Cardiovascular complications of RP are associated with high morbidity and mortality and occur most commonly as valvular disease. Pericarditis is a less common complication, occurring in 4% of patients with RP and has not previously been described at presentation. We describe a case of relapsing polychondritis with acute pericarditis at presentation. 1. Introduction Relapsing polychondritis (RP) is an autoimmune disorder of unknown etiology primarily affecting the cartilaginous structures of the body. It may also involve other noncartilaginous, proteoglycan rich organs [1]. It is a relatively rare disease with no animal model, making investigation into the mechanism of disease more difficult. Due to this, RP is often difficult to diagnose and treat, with life threatening consequences, if proper diagnosis is not made [2]. The disease is episodic and progressive, with a heterogenous phenotype [3]. The most common presenting features include auricular chondritis, seronegative arthritis, nasal chondritis, ocular inflammation, and laryngotracheal symptoms [1, 3]. Infection and respiratory problems are the most common cause of death, but cardiac complications are the next most common cause of mortality [4]. Valvular involvement is the most common cardiac cause of both morbidity and mortality. Other less common cardiac complications include conduction disturbances, pericarditis, vasculitis, and vascular disease such as aortic aneurysm or dissection [5]. Cardiovascular complications almost universally present later in the disease course with a mean interval of six years after presentation [5]. Only AV nodal conduction abnormalities, presenting as a third degree heart block, have been reported in the literature at presentation of RP [6]. Acute pericarditis is a relatively rare complication of RP, present in only 4% of patients during the course of the disease, and has never been reported at the time of presentation [5]. We present the case of a patient diagnosed with RP who was found to have acute pericarditis on presentation. 2. Case Report A 31-year-old woman presented to the emergency department with chest and facial pain. She had no significant past medical history. She was in her usual state of health until 6 weeks prior to presentation when she developed rhinitis and cough. She had been treated with antibiotics with no relief. She then developed pain and swelling on the

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