Academic Rheumatology Unit and
1 Department of Radiology, University Hospital Aintree, Liverpool, UK
SIR, the outcome of relapsing polychondritis (RP) depends on remission of the underlying inflammatory process. Most patients experience a fluctuant inflammatory course, but the disease is generally progressive. Ten per cent of deaths in RP are due to respiratory complications. The optimal therapeutic approach to patients with RP is poorly understood. Corticosteroids are able to suppress disease activity but do not stop disease progression and are associated with side-effects. Various disease-modifying anti-rheumatic drugs have been used in an attempt to achieve a lower dose of steroid or for refractory cases. Infliximab is a monoclonal antibody that binds and inactivates tumour necrosis factor (TNF-
) and has been successfully used in the management of TNF-
-mediated diseases, such as Crohn's disease and RA. Infliximab is not licensed for the treatment of RP, but because TNF-
has a potential pathological role in this condition, it may be of therapeutic benefit, especially after failure of conventional therapeutic approaches. We report here a patient with RP who developed recalcitrant and life-threatening respiratory tract complications, in whom treatment with infliximab led to resolution of disease activity.
A 51-yr-old female presented at the age of 49 yr with a 1-yr history of sudden intermittent bilateral redness, pain and swelling of the ears. She later developed nasal stuffiness, dysphonia and throat pain. On examination, both cartilaginous parts of the ears showed a dusky erythema and were swollen and tender. The rest of the examination was normal. Renal function, liver function, echocardiogram and all serological investigations [anticardiolipin, antineutrophil cytoplasmic antibody (ANCA), antinuclear antibody (ANA), Borrelia burgdorferi] were all normal. Computed tomography (CT) scanning showed circumferential deformity and oedema of the proximal tracheal rings and sclerosis of the left arytenoid cartilage, with normal signal in the cricoid cartilage and vocal cords. A diagnosis of RP was made using the diagnostic criteria of McAdam et al. [1]. She was treated with intravenous methyl prednisolone pulses followed by oral prednisolone 30 mg daily in a reducing course, which cleared her symptoms. However, when steroid was reduced she relapsed. She developed methaemoglobinaemia 3 days after a trial of dapsone and continued on prednisolone 7.5 mg daily with azathioprine 200 mg daily added.
During the following year she experienced several relapses of ear involvement and associated hoarse voice, which improved with high doses of steroid and substitution of methotrexate 25 mg once a week for azathioprine. Five months later, she developed a hoarse voice and throat pain. On examination she was tender over the anterior cervical trachea and larynx. Her spirometry showed non-reversible obstructive ventilatory impairment with reductions in expiratory flow rates and maximal voluntary ventilation. Her diffusion capacity was normal. Bronchoscopy showed inflammation of the laryngeal cartilages. CT scan showed pronounced soft-tissue thickening affecting almost the entire cervical trachea with sclerosis of the cricoid and arytenoid cartilages (Fig. 1). Following counselling on the benefit and possible risks of infliximab therapy, she consented to commence on infliximab 5 mg/kg 0, 2, 4, 8 weekly. She tolerated this well and no immediate adverse effects were noted. She is now symptom-free and in remission. Repeat CT scanning showed essentially a marked improvement in the tracheal thickening (Fig. 2
).
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Infliximab treatment resulted in a rapid and complete clinical response in potentially life-threatening RP that had not responded to conventional immunosuppression. RP is so rare that controlled therapeutic trials have not been carried out. Multicentre studies are required, with careful examination of treatment benefits. This may result in unique insights into the mechanism of action of infliximab in RP and increase our knowledge of the pathophysiology of RP. This case highlights the usefulness of anti TNF- therapy in patients with refractory RP.
Notes
Correspondence to: S. Mpofu, Academic Rheumatology Unit, University Hospital Aintree, Lower Lane, Liverpool L9 7AL, UK. E-mail: smpofu{at}liv.ac.uk
References