Mr A was a 58-year old accountant who presented with painful ears. He had been aware of sensitivity and tenderness in the upper part of his ears three months previously, but over the preceding six weeks his ears had become very red and sore. He had also been aware of some ringing and a feeling of air block in his ears.
Around a month earlier, he had attended the local eye hospital with red eyes and was diagnosed then as having episcleritis. He was treated with steroid eye drops, which improved his symptoms. On further questioning, he told me that he had generally felt more tired than usual and also had intermittent pains in his joints, mainly his wrists, finger joints and knees.
He had mild asthma but is otherwise fit and well and was not taking any regular medication.
On examination Mr A had very red and swollen pinnae bilaterally. Examination of his respiratory, gastrointestinal tract and joints was unremarkable.
On referral, the local rheumatologist confirmed my suspected diagnosis of relapsing polychondritis. He was started on high dose prednisolone (80mg a day) and is going to start methotrexate soon. He has had an ECG, chest x-ray, CT chest, lung function tests and echocardiogram which have all been normal.
Relapsing polychondritis (RP) is a rare systemic disease characterised by recurrent, widespread chondritis of the auricular, nasal and tracheal cartilages. Additional clinical features include audiovestibular dysfunction, ocular inflammation, vasculitis, myocarditis, and nonerosive arthritis. Although the cause remains unknown, the etiology is suspected to be autoimmune.1 A concomitant autoimmune disease is present in around a third of patients with RP.2
RP usually presents in the fourth and fifth decade and affects men and women equally.
The spectrum of clinical presentations is large and may vary from intermittent episodes of painful and disfiguring auricular and nasal chondritis or polyarthritis to severe progressive multi-organ damage3.
The presence of auricular inflammation is characteristic of RP and is an important diagnostic feature. The inflammation typically spares the auricular lobe as it is devoid of cartilage and this sign can aid in its differentiation from other causes of auricular inflammation4.
Involvement of nasal cartilage can be seen in around half of cases, and saddle nose deformity occurs in around 10% of patients.5
Severe inflammatory changes affecting ocular tissues can lead to permanent damage and loss of vision. Any part of the eye can be affected. Scleritis can be particularly challenging to manage and may lead to globe perforation. Keratitis may also occur, leading to corneal perforation.
Involvement of the tracheobronchial tree can occur in around half of patients and renal involvement in around 25% of patients.6
Diagnosis of RP
There is no single diagnostic test for RP. The diagnosis of RP is largely based on the clinical features and the role of laboratory and imaging investigations is purely supportive, to rule out other related or associated systemic diseases.1
Management of RP
Relapsing polychondritis often has a fluctuating course and disease progression can be difficult to predict. The treatment of RP is symptomatic and should be tailored to each individual patient based on disease activity and severity. The mainstay of therapy revolves around control of inflammation and suppression of autoimmunity. Glucocorticoids are usually given to most patients. However, less severe symptoms can be treated with non-steroidal anti-inflammatory drugs.
The choice of therapy is dependent on the severity of symptoms and the extent of disease. Methotrexate is often effective.2 Emerging treatment options include the use of immunomodulatory agents.7,8
Although RP is a rare condition, it may be associated with significant morbidity and mortality especially if associated with respiratory tract or cardiovascular involvement. With earlier recognition of this disease and optimal treatment, the prognosis has improved over recent years. The most common causes of death in these patients include infection secondary to corticosteroid treatment or respiratory compromise, systemic vasculitis and malignancy unrelated to relapsing polychondritis.
- Sosada B, Loza K, Bialo-Wojcicka E. Relapsing polychondritis. Case Rep Dermatol Med. 2014;2014:791951
- Puéchal X, Terrier B, Mouthon L, Costedoat-Chalumeau N, Guillevin L, Le Jeunne C. Relapsing polychondritis. Joint Bone Spine. 2014 Mar;81(2):118-24.
- Cantarini L, Vitale A, Brizi MG, Caso F, et al. Diagnosis and classification of relapsing polychondritis. J Autoimmun. 2014 Feb-Mar;48-49:53-9.
- Sharma A, Law A, Bambery P, Sagar V, et al. Relapsing polychondritis: clinical presentations, disease activity and outcomes. Orphanet J Rare Dis. 2014 Dec 20;9(1):198
- Zeuner M1, Straub RH, Rauh G, Albert ED, Schölmerich J, Lang B. Relapsing polychondritis: clinical and immunogenetic analysis of 62 patients. J Rheumatol. 1997 Jan;24(1):96-101.
- Gorard C, Kadri S. Critical airway involvement in relapsing polychondritis. BMJ Case Rep. 2014 Sep 11;2014.
- Navarrete VM, Lebron CV, de Miera FJ, Candau CP, Sarabia FN. Sustained remission of pediatric relapsing polychondritis with adalimumab. J Clin Rheumatol. 2014;20:45–46
- Peng SL, Rodriguez D. Abatacept in relapsing polychondritis. Ann Rheum Dis. 2013;72:1427–1429.