Relapsing polychondritis is an uncommon, recurrent, progressive autoimmune inflammatory disorder that affects cartilaginous tissue, predominantly in the ears, nose, and laryngotracheobronchial tree but also in the sclera, the heart valves, and in the peripheral and axial joints.^1,2 Central nervous system (CNS) complications, such as meningitis, myelitis, hemiplegia, and cerebellar signs, are extremely rare.^3-5

Case Presentation

A 47-year-old right-handed male Mexican immigrant was admitted to the hospital with expressive aphasia for the past 2 weeks. Vital signs on admission were: blood pressure, 158/87 mm Hg; regular heart rate, 78 beats/min; respiratory rate, 18 breaths/min; temperature, 98.8ºF. He was able to follow all commands but could not repeat words that were spoken to him. Physical examination was unremarkable, except for mild scleral injection in the right eye, consistent with scleritis. No sensory or motor deficits were evident; his reflexes were normal bilaterally, and a cerebellar examination was normal. He had no significant medical history and denied smoking, alcohol, and intravenous drug use.

Lumbar puncture on admission revealed a clear, colorless fluid; white blood cells, 9 x 10¹²/L; and red blood cells, 324 x 10¹²/L, with 37% neutrophils and 57% lymphocytes, and a negative Gram's stain. Cerebrospinal fluid (CSF) chemistry showed: glucose, 64 mg/dL; total protein, 37 mg/dL. CSF venereal disease research laboratory test and CSF polymerase chain reaction test for herpes simplex virus were negative.

Laboratory results were: hemoglobin, 15.4 g/dL; white blood cell count, 6.8 x 10⁹/L; neutrophils, 62% (normal, 56%); lymphocytes, 24% (normal, 34%); platelet count, 336 x 10⁹/L; sodium, 138 mmol/L; potassium, 4.1 mmol/L; creatinine, 0.8 mg/dL; glucose, 99 mg/dL; calcium, 8.9 mg/dL; antinuclear antibody (positive), 1:40, with speckled pattern. All other test results were negative or normal, including tests for anticardiolipin and antiphospholipid antibodies.

Computed tomography of the head revealed an area of low attenuation in the left posterior temporal lobe, with loss of the gray-white interface and sulcal effacement consistent with a recent stroke. Electrocardiography demonstrated normal sinus rhythm. A transesophageal echocardiogram showed left ventricular ejection fraction of 50% and no valvular or septal defects.

Magnetic resonance imaging (MRI)/magnetic resonance angiography of the brain demonstrated 60% stenosis in the left common carotid artery and 50% stenosis in the left internal carotid artery, with a normal right carotid supply and an occipitotemporal infarct on the left side. MRI also revealed tiny subcortical infarcts in both cerebral hemispheres, with possible small-vessel disease. To further evaluate the carotid stenosis, a carotid angiogram was obtained, which showed only 30% narrowing in the left proximal internal carotid artery and normal Circle of Willis circulation. The presence of minimal atherosclerosis and bilateral tiny infarcts suggested the possibility of an embolic or a vasculitic process.

On hospital day 2, the patient complained of pain in his right ear. Examination showed he had tender right pinna, and the skin overlying his right ear was violaceous, consistent with chondritis (Figure). The external auditory canal of the right ear showed mild erythema; the tympanic membrane was normal, as was the left ear.

Figure—Violaceous skin overlaying the ear consistent with chondritis in a patient with tender pinna.

The development of auricular chondritis and the presence of scleritis were strongly suggestive of relapsing polychondritis as the primary diagnosis. The patient was started on prednisolone, and the inflammation in his ear and eye improved dramatically in a single day; his aphasia resolved within 2 days. He was discharged home with prescriptions for prednisolone, aspirin, simvastatin (Zocor), and metformin HCl (Glucophage) and was scheduled for outpatient follow-up.

Discussion

Relapsing polychondritis can present with a range of clinical features. The most common signs and symptoms are recurrent pain and edema of the external ear (resulting in "cauliflower ear") and nose (resulting in a "saddle-nose" deformity), uveitis, and peripheral joint arthropathy.⁶ In about half of all cases, the cartilage of the tracheolaryngeal tract is affected, which can be fatal if the tracheal rings and bronchi collapse or the airways are obstructed by inflammation.⁷

McAdam and colleagues developed the original diagnostic criteria for relapsing polychondritis in 1976,⁸ which required the presence of 3 or more of the clinical features listed in the Table. Using the modified Damiani and Levine criteria, the diagnosis now requires any one of the following 3 combinations:⁹

• At least 3 McAdam clinical features, with or without histologic confirmation
• At least 1 McAdam criterion, plus positive histologic confirmation
• Chondritis in 2 separate anatomic locations, combined with response to corticosteroid therapy.

Only about 3% of patients with relapsing polychondritis have CNS and peripheral nervous system involvement.¹⁰ The cranial nerves are most frequently involved, but seizures, confusion, cerebral aneurysms,¹¹ and rhomboencephalitis⁴ have all been described, along with psychiatric symptoms and memory impairment.⁴ Autopsy brain biopsies have demonstrated diffuse vasculitis involving medium- and small-sized arteries, as well as small veins, with necrotic white-matter lesions.¹² MRI of the brain may show bilateral multifocal areas of enhancement consistent with cerebral vasculitis.¹³ CSF pleocytosis, although rare, has also been reported in relapsing polychondritis.¹⁴ Even in the absence of classic radiographic findings, unusual calcifications of cartilaginous structures should suggest the diagnosis of relapsing polychondritis.⁶

We acknowledge that it is not yet possible to definitely diagnose CNS vasculitis with current diagnostic modalities and that brain biopsy is the only means of establishing a definitive diagnosis.¹⁵

Auricular chondritis is present in almost all patients with relapsing polychondritis, typically involving the cartilaginous portion of the pinna and sparing the noncartilaginous lobe.

Characteristic histologic findings include a pink coloration on hematoxylin and eosin stain and an infiltrate composed of various inflammatory cells, including lymphocytes, plasma cells, eosinophils, and neutrophils.² Almost half of all patients have ocular symptoms, which are usually reflected as recurrent episcleritis or scleritis, conjunctivitis, keratitis, or uveitis.

Auricular or nasal chondritis and scleritis should alert the physician to the possibility of relapsing polychondritis as the cause of concurrent neurologic symptoms. The response of these symptoms to steroids, and MRI findings of bilateral small infarcts with minimal atherosclerosis, further strengthen the possibility that relapsing polychondritis is the underlying cause.

Conclusion

Relapsing polychondritis is a recurrent disease characterized by inflammation of cartilaginous tissues and proteoglycan-rich structures. There are protean manifestations. Vasculitis should always be considered in the differential diagnosis of any patient with transient or persistent neurologic deficits, especially if the history, physical examination, and laboratory data argue against the likelihood of accelerated atherosclerosis, carotid disorders, or hypercoagulable disorders.

References

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