Relapsing polychondritis—case series from South India

Abstract

Relapsing polychondritis (RP) is a rare recurring inflammatory disorder with variable clinical course. It has been described mainly in Caucasian population. Reports from other ethnic groups are few. We report seven cases of relapsing polychondritis in south Indian population. In between 1995 and 2008, seven patients fulfilling the McAdam-Damiani-Levine criteria for diagnosis of relapsing polychondritis were identified. Records pertaining to these patients were studied and clinical presentation, course, and treatment offered were analyzed retrospectively. The female-to-male ratio in our series was 2.5:1. The age of onset of symptoms ranged from 28 to 54 years, with a mean of 40.2 years. An average of 20 months, ranging from 3 months to 6 years, elapsed before the patient presented to us seeking a diagnosis. Various structural involvement in our series were as follows: pinna in four (57%), nasal cartilage in five (71%), joints in three (43%), eyes in three (43%), laryngotracheal tree in three (43%), inner ear in one (14.3%), skin in one (14.3%), and heart in one (14.3%). Associated autoimmune diseases were present in four (57%) patients in the form of one of the following in each: vasculitis, autoimmune hemolytic anemia, hypothyroidism, and rheumatoid arthritis. All seven patients received prednisolone with three of them requiring additional immunosuppressants. There was no mortality amongst the four patients who had remained on follow-up at the time of this report. Although RP is an uncommon disorder, clinicians should be aware of the manifestations so as to initiate prompt treatment and prevent complications. Our series reports less frequent auricular cartilage and skin involvement and an exceptional case of basal cell carcinoma, although the other manifestations were similar to that seen in Caucasian and other Asian populations.

Relapsing polychondritis: a review

“…In a case series conducted in an Oriental population [7], it was observed that the disease course was similar to that in the Caucasian population except that cutaneous, renal or nervous system involvement was not seen and the incidence of airway complications was more. Another series from North India [8] reported that the clinical features were similar, but laryngotracheal involvement was less frequent in these patients, whereas that from Southern India [9] inferred that auricular and skin involvement were less common.…”

Relapsing polychondritis: clinical presentations, disease activity and outcomes

“…Case series from western nations defined its clinical spectrum [1-3]. Smaller series from south-east Asia and India have, however, depicted clinical presentations at variance from those in Caucasian patients [4-6] (see Table 1). To document these differences, we present the largest single centre experience from the Indian subcontinent.…”

Relapsing Polychondritis: Systemic and Ocular Manifestations, Differential Diagnosis, Management, and Prognosis

“…10,11 Some authors report no significant gender predilection, 1,3,4,11,12 while others find a female preponderance with a female-to-male ratio of 2:1 to 3:1. 1,13,14 The peak age of onset is the fourth or fifth decade of life; 1,4,8,14,15 two large studies describe patients ranging between the age of 3 to 87 years. 8,16 …”