Article Text

Diplopia as the first manifestation of relapsing polychondritis
  1. I MARIE,
  2. H LEVESQUE,
  3. N CAILLEUX,
  4. H COURTOIS
  1. Département de Médecine Interne, CHU Rouen-Boisguillaume, 76031 Rouen Cedex, France
  2. Sevice de Neurologie, CHU Rouen-Boisguillaume, Rouen, France
  1. Dr I Marie.
  1. B MIHOUT,
  2. L IASCI
  1. Département de Médecine Interne, CHU Rouen-Boisguillaume, 76031 Rouen Cedex, France
  2. Sevice de Neurologie, CHU Rouen-Boisguillaume, Rouen, France
  1. Dr I Marie.

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Relapsing polychondritis (RP) is an uncommon systemic disorder, which is characterised by recurrent inflammation and destruction of cartilage structures.1 Auricular, nasal, ocular, articular, tracheobronchial, and cardiac impairment are well recognised manifestations of RP.1-4 Central nervous system involvement is considered to be rare in RP, usually occurring in the course of the disease.1-4 We have observed a new case, which is of particular interest, as the patient developed diplopia revealing RP.

A 69 year old man presented with an uveitis of the right eye in November 1997. The patient had no previous medical history, and he was successfully treated with both corticosteroid and antibiotic ointments. He was admitted to our hospital in February 1998 with a two day history of diplopia. Physical examination showed a right VI nerve palsy. The patient had no fever and general physical examination was otherwise normal. Laboratory findings were: erythrocyte sedimentation rate 60 mm 1st h, C reactive protein 20 mg/l (normal <5), haemoglobin 7.3 mmol/l, white cell count 4.7 × 109/l, and platelets 580 × 109/l. Glycaemia, liver, and renal tests were normal. Cerebral computed tomography was within normal limits. Cebrospinal fluid analysis revealed no abnormality, and notably IgG index was normal without oligoclonal bands; both Gram staining and bacterial cultures remained negative. Other investigations, including chest radiographs, electrocardiogram, transoesophageal echocardiography and cerebral arterial Doppler were also normal. Because of the previous history of uveitis of the right eye, an accessory salivary gland histological examination was carried out, which was normal without evidence of granulomas or vasculitis. Both thoracic computed tomography and abdominal ultrasound were also normal, and notably hetatosplenomegaly and lymphadenopathy were absent. On the ninth day, the patient developed both conjunctivitis and uveitis of the left eye, and then bilateral auricle chondritis and hoarseness of voice within one week. Autoantibody screening tests—that is, complement profile, rheumatoid factors, antinuclear antibodies, anticardiolipin and antiphospholipid antibodies, lupus-like anticoagulant, antineutrophil cytoplasmic antibodies, anticartilage antibodies, and cryoglobulinaemia, were negative. Otorhinolanryngeal examination showed no abnormality. The diagnosis of RP was made because of the following manifestations: (a) recurrent bilateral uveitis, (b) bilateral auricle chondritis, and (c) hoarseness of voice related to laryngeal cartilage inflammation. The patient was successfully treated with prednisone 1 mg/kg daily, resulting in resolution of diplopia and uveitis of the left eye and in improvement of both left auricular chondritis and dysphonia. After a four month follow up, the patient remains free of ocular, auricular, laryngeal, and neurological features with prednisone at a dose of 45 mg daily.

Central nervous system involvement is rare in RP. In a series of 62 patients with RP, Zeuner et al 4 have therefore found that only six patients (9%) had neurological manifestations. However, neurological impairment may lead to life threatening complications in patients with RP, for example, hemiplegia, cerebellar dysfunction, aseptic meningoencephalitis, rhombencephalitis or cerebral arterial aneurysms, and also to cranial nerve palsy, polyneuritis or myelitis.1-10 The pathological mechanisms of neurological impairment are still not clearly understood, but it may be related to vasculitis of both small and medium sized vessels.1-3 Our case is original in that neurological involvement—that is, diplopia related to right VI nerve palsy—revealed a typical RP. Because central nervous system manifestations may precede other systemic signs, we suggest therefore that when unexplained neurological symptoms are noted in patients, a clinical evaluation for an underlying and misdiagnosed RP may be done. Our findings further emphasise the importance of recognising such neurological complications at an early stage in RP, resulting in accurate diagnosis and management, and therefore avoiding the risk of severe sequelae. Finally, we postulate that clinical neurological examination should systematically be carried out in patients with RP, as such an involvement may result in more follow up and aggressive treatment.

Acknowledgments

The authors thank Mr Richard Medeiros for his advice in editing the manuscript.

References

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