|LETTER TO THE EDITOR
|Year : 2016 | Volume
| Issue : 2 | Page : 136-137
Arulprakash Sarangapani, Sahil Rasane, Vikas D Kohli
MIOT Advanced Center for GI and Liver Diseases, MIOT International, Chennai, Tamil Nadu, India
|Date of Web Publication||9-Mar-2016|
Plot No. 119 A, First Main Road, Second Cross Street, Lakshmi Nagar Extension, Porur, Chennai - 600 116, Tamil Nadu
|How to cite this article:|
Sarangapani A, Rasane S, Kohli VD. Relapsing polychondritis. Saudi J Med Med Sci 2016;4:136-7
Relapsing polychondritis (RP) is an autoimmune disorder of unknown etiology, primarily affecting the cartilaginous structures of the body, such as the ears, nose, laryngotracheal tree, and joints. It also involves the noncartilaginous proteoglycan-rich structures, including the eye, heart, blood vessels, and inner ear. This relatively rare disease is episodic and progressive, with a heterogeneous phenotype. The common presenting features include auricular chondritis, seronegative arthritis, nasal chondritis, ocular inflammation, and laryngotracheal symptoms. 
A 70-year-old male presented with a history of pain and swelling of the right ear lobe for a week's duration. The symptoms preceded a transient fleeting type of joint pain involving both small and large joints for the past 6 months, which was temporarily relieved with nonsteroidal anti-inflammatory drugs (NSAIDS). Symptoms worsened when he had a postcataract iridocyclitis, which was steroid responsive. He had one episode of vertigo, which was extensively evaluated by a neurologist, who diagnosed the patient with labyrinthitis and provided with appropriate treatment. Over the past 5 years, he had been evaluated by ENT surgeons for repeated throat clearing and discomfort, which was treated as gastroesophageal reflux disease with proton pump inhibitors. An upper gastrointestinal endoscopy revealed no abnormalities. The patient is a known hypertensive and diabetic, for which he has been receiving treatment for the past 15 years.
On physical examination, he was noted to have a swollen exquisitely tender erythematous upper cartilaginous part of the right pinna with sparing of the ear lobule [Figure 1] with no tracheal tenderness. No joint swelling or redness of eyes was noted. Initial laboratory data were remarkable revealing a sedimentation rate of 36 mm/hr, C-reactive protein of 40, and hemoglobin of 10.4 g/dL. Antineutrophil cytoplasmic antibodies, antinuclear antibodies, rheumatoid factor, and creatinine were all unremarkable. Clinical diagnosis of relapsing polychondritis was made based on McAdams criteria.  He was started on oral prednisolone, colchicine, and methotrexate. On a 2-month follow-up visit, steroids were tapered, and remission was sustained with methotrexate.
|Figure 1: Typical violaceous hue, involving the cartilaginous part of pinna with classical sparing of ear lobule: Hallmark of relapsing polychondritis.|
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RP is characterized by widespread, inflammatory, destructive, and degenerative lesions. No laboratory test is pathognomonic for this disease. Disease manifestations vary with the severity and depend on organ involvement. Apart from fever, the common myalgia manifestations include auricular chondritis (60-100%); nonerosive non inflammatory arthritis, mostly proximal interphalangeal joints (50-80%); episcleritis and scleritis (50-60%); nasal chondritis (50%); and laryngotracheal involvement (20%). Other organ involvement includes cardiac (valvular lesions and arrhythmias), renal, dermatological, and neurological manifestations. As no laboratory test is pathognomic for RP, diagnosis is based on Damiani and Levine modification of McAdam's criteria.  Treatment depends on the severity of the illness with mild disease managed by NSAIDS. Severe illness may require steroids, immunosuppressants like cyclophosphamide, azathioprine, cyclosporine, and methotrexate. Biological agents are found to be useful in those patients who are difficult to treat.
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| References|| |
Kent PD, Michet CJ Jr, Luthra HS. Relapsing polychondritis. Curr Opin Rheumatol 2004;16:56-61.
McAdam LP, O'Hanlan MA, Bluestone R, Pearson CM. Relapsing polychondritis: Prospective study of 23 patients and a review of the literature. Medicine (Baltimore) 1976;55:193-215.
Damiani JM, Levine HL. Relapsing polychondritis - report of ten cases. Laryngoscope 1979;89(6 Pt 1):929-46.