Relapsing polychondritis— its otolaryngological manifestations By J. R. MOLONEY (London) Introduction RELAPSING polychondritis is a rare condition in which there is recurrent inflammation of cartilage, especially the cartilage of the organs of special sense. It was first described in 1923 by Jacksch-Wartehorst who suggested the name polychondropathis. Pearson, Kline and Newcomer (1960) called it relapsing polychondritis. It is of particular interest to the otolaryngologist as it may present to him first. The condition may occur at any age but is commonest between 20 and 60. The sex incidence is approximately equal. Most patients have been Caucasians but it has been reported in one Negro (Reefe and Sierra, 1970) and 3 Japanese (Johnson, 1963; Yamasaki et ah, 1966; Hainer and Hamilton, 1969). The only familial incidence has been a report of a sufferer giving birth to a child with the condition (Arundell and Hasarick, 1960). Another sufferer who gave birth to a normal child has also been reported (Pearson, Kline and Newcomer, 1960). The aetiology of the condition is unknown, but it is thought that it may be an autoimmune disease, coming within the collagen-vascular group of diseases, or possibly the rheumatic group (Dolan et al., 1966). It is occasionally associated with rheumatoid arthritis, S.L.E., ankylosing spondylitis and Reiter's disease. Histology shows an initial neutrophil infiltration in the acute stage, later followed by plasma cells and lymphocytes in the more established case. The cartilage loses its normal basophilia early in the condition. Eventually atrophic cartilage with cystic spaces containing gelatinous material is seen as the condition burns itself out. Among its clinical features are recurring inflammation of the pinna and nasal cartilage, larynx and trachea; episcleritis or scleritis, iritis, conjunctivitis and keratitis; a variety of arthropathies (which are reported as occurring in 78 per cent of cases); aortic regurgitation, aneurysm formation, vasculitis, glomerulonephritis, and mild liver function abnormalities and fever. The clinical manifestations have been analysed in an excellent paper by Dolan et al. (1966). There is no specific diagnostic test and diagnosis rests on the appearance of recurrent inflammation of two or more cartilaginous sites (at least one I of which involves an organ of special senses), and Dolan et al. (1966)

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suggest that a biopsy with compatible histology should be obtained. Further features are that the ESR is usually raised, except very early in the condition, and a mild to moderate anaemia is usually present. Oto-rhinolaryngological manifestations

The external ear It is of particular interest to the otolaryngologist that the commonest presenting symptom is painful swelling of one or both pinnae. Approximately one third of cases up to 1971 presented in this way—31 cases out of a total of 98—(Hughes et ah, 1972). The whole of the pinna is involved, except the lobule (Fig. 1). The cartilage of the external auditory canal may also be involved, and this can be reduced to a slit (Jensen, 1962; Rabuzzi, 1970). The auricle has a deep

FIG. 1 The pinna in relapsing polychondritis. The lobule is not involved.

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violacious hue, or a bright red cyanotic appearance (Kaye and Sones, 1964; Rabuzzi, 1970; Jensen, 1962). It becomes swollen and may be exquisitely tender (Jensen, 1962; Pearson et al., 1960). The auricular cartilage may be soft and boggy, and later in the condition the pinna may become droopy, and pale greyish brown in appearance (Kaye and Sones, 1964; Jensen, 1962). When the ear becomes painless and soft it can sometimes be compressed into a compact mass, and may even flap up and down as the patient walks (Pearson et al, 1960; Bean et al, 1958). The middle ear Deafness may be caused by serous otitis media which is thought to be due to swelling of the eustachian tube cartilage. Myringotomy and insertion of a grommet has been necessary in some patients (Daly, 1966; Cody and Sones, 1971; Hughes et al, 1972). A patient has also been reported who appears to have developed serous otitis media, and later mastoiditis as the presenting features of his disease. It was necessary for him to undergo a right radical mastoidectomy (Hughes et al, 1972). The inner ear Sensorineural hearing loss and vertigo have been recorded in many patients. In the series reported by Cody and Sones (1971), it was found that the hearing loss developed with varying degrees of rapidity. It may come on suddenly, that is over less than twenty four hours; it may be rapidly progressive, that is developing over 2-3 weeks; or it may be slowly progressive, taking 9 months to 2 years. In one of their cases sudden sensorineural hearing loss was the presenting symptom of the disease. The hearing loss was bilateral in over half the cases. The mean duration of the disease prior to the appearance of auditory or vestibular trouble was 2£ years (range, presentation to 9£ years). It appears From their cases (Cody and Sones, 1971) that the hearing loss is often associated with a recent flare up of auricular cartilage involvement, or a reduction in the steroids after a recent flare up. The hearing loss may fluctuate from day to day. In addition they report that 3 patients had improvement in hearing with steroid treatment. Rabuzzi (1970) also reports a reversal of sensorineural hearing loss with steroids. Most cases with hearing loss have an associated chondritis of the external ear. However, Kaye and Sones (1964) record a patient in.which this was not the case. The available evidence suggests that the hearing loss is of an end-organ type, and Cody and Sones (1971) feel that it is probably caused by involvement of the internal auditory artery. Some of the patients in the Mayo Clinic Series (Cody and Sones, 1971) had vestibular symptoms. However, none had vestibular symptoms without having hearing impairment. Their patients were recorded as having severe unsteadiness; postural vertigo; severe vertigo with vomiting; loud tinnitus

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and hearing loss. Spontaneous nystagmus was seen in only one patient. However, caloric responses were reduced or absent in many ears. Hughes et ah (1972) also report a patient who had a severe attack of vertigo during the course of his disease. The nose The most common way in which the nose appears to be involved is by collapsing painlessly to give a saddle deformity (Rabuzzi, 1970). In addition to this there may be flattening of the tip of the nose (Bean et ah, 1958). The collapse of the nose may be fairly sudden, occurring overnight (Swain and Stroud, 1972). Collapse has been reported even though the patient was on corticotrophin injections (Jensen, 1962). Prior to collapse of the nose, there may be episodes of pain and erythema of the nose, which are likely to be treated as furuncles (Hughes et ah, 1972). Kaye and Sones (1964) record a patient who had surgery to the left side of the nose, for a chronic sinus complaint, which resulted in cartilage collapse and a saddle deformity. A diagnosis of relapsing polychondritis was made later. The nasal septum may become swollen, and crusting and epistaxis may occur (Odkvist, 1970; Hughes et ah, 1972). Kaye and Sones (1964) report the occurrence of rhinorrhoea, and this and lacrimation have been recorded as the presenting symptoms (Bean et ah, 1958). Larynx and trachea Involvement of the laryngeal cartilages may result in hoarseness (Purcelli et ah, 1962; Kaye and Sones, 1964). In addition the larynx may be tender to palpation (Purcelli et ah, 1962) and the thyroid cartilage may feel soft (Mahindraker and Libman, 1970). The laryngeal mucosa may be red and swollen (Mahindraker and Libman, 1970) and the false cords may look enlarged (Kaye and Sones, 1964). Biopsy of the larynx has led to a worsening of the condition post operatively, and the need for tracheostomy (Kaye and Sones, 1964). Stenosis and collapse of the trachea and bronchi occur commonly. Dyspnoea results, and tracheostomy may be required (Purcelli et ah, 1962; Daly, 1966; Kaye and Sones, 1964; Mahindraker and Libman, 1970). Laryngotracheal involvement required permanent tracheostomy in five of the cases reported by Kaye and Sones (1964). Purcelli et ah (1962) report a case who died on the table following a cardiac arrest during tracheostomy. This case had also been reported by Pearson et ah (1962) and the X-rays of the case have been reported by Horns and O'Loughlin (1962). Dyspnoea is not unexpected in the well-established case of relapsing polychondritis, but when it is the presenting symptom it is usually misdiagnosed. Purcelli et ah (1962) report a patient who had dyspnoea, cough, wheezing and mild bilateral chest pains. A diagnosis of status asthmaticus was made. Bronchoscopy showed severe tracheal stenosis. Here also

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biopsy, in this case of the trachea, led to a worsening of the condition and tracheostomy was performed later. An extra long tracheostomy tube was required, so that its end lay near the tracheal bifurcation. The X-rays of this case have also been reported by Horns and O'Loughlin (1962). A further case presenting with dyspnoea was reported by Daly (1966). The patient had an eighteen-month history of increasing dyspnoea and a mass was present in the upper trachea. It was initially thought to be a chondroma. The mass was resected by dividing the thyroid and cricoid cartilages and upper trachea in the mid line. It was removed and a stent was put in position. The patient did well following the removal of the stent. Tracheal tomograms (Kaye and Sones, 1964), tracheal bronchograms (Rabuzzi, 1970) and bronchoscopy (Mahindraker and Libman, 1970; Thould et ah, 1965; Self et al., 1967) have been used to assess the state of the trachea and bronchi. In Mahindraker and Libman's (1970) case, bronchoscopy showed collapse of the cervical trachea. In the case reported by Self et al. (1967), bronchoscopy showed that the trachea tended to collapse on expiration. Both Thould et a/.'s (1965) and Self et a/.'s (1967) cases came to post mortem. Thould et a/.'s (1965) showed that the glottic opening of the larynx was much reduced, apparently due to disappearance of the laryngeal cartilages, with collapse of the soft tissues. The tracheal cartilages had been destroyed and the trachea was soft and compressible. In Self et a/.'s (1967) case, post mortem examination showed that the large bronchi were flaccid and collapsed easily. Costochondral cartilage The costochondral cartilages may sometimes be involved. They may become tender (Rabuzzi, 1970). Softening of the cartilages may cause a flail chest, in which the sternum is drawn in on inspiration, as reported by Hilding (1952). Differential diagnosis The differential diagnosis of the various otolaryngological aspects of the disease includes: erysipelas, perichondritis or gout of the pinna; furuncle of the nose; the various causes of serous otitis media; Meniere's disease; Cogan's syndrome; Wegener's granuloma; chondroma of the cricoid and asthma. Prognosis The mean survival for those twenty-seven patients who died up to 1971 was 5-25 years, but varied from 1 month to 23 years (Hughes et al., 1972). A benign course in the early part of the disease does not exclude a fulminating course later (Kaye and Sones, 1964).

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Treatment Treatment is usually by prednisolone which appears to have been helpful in about 50 per cent of the cases reported (Hughes et al., 1972). High doses are often required and complications may result. Some cases have been treated with azothioprine and this seems to have allowed a reduction in the prednisolone dose (Mahindraker and Libman, 1970; Hughes et al., 1972). Case report Miss M.B., aged 42, a lady of Korean origin presented to the ENT department via casualty. She had woken the same morning with a very painful small red swelling on the upper and inner aspect of her left pinna. It was thought to be either an insect bite, or a developing pustule. The condition was treated with ampicillin and piriton. However, it continued to spread until she had what appeared to be an infective perichondritis involving the whole of the pinna except the lobule (Fig. 1). Cloxacillin was added to the regime and she developed a penicillin rash. Her treatment was then changed to intramuscular and oral tetracycline. The swelling eventually subsided to some degree, and improved further when Betnovate N cream was applied. The ear finally settled after one month's treatment. She reappeared 2 weeks later as the condition had started to flare up again. She was given a further supply of tetracycline. There was no improvement when she was seen one week later. In addition she said she had been very hoarse, though her voice had improved a little. It was difficult to obtain a view of the cords, though the arytenoids were seen to be oedematous. It was thought from the course of the disease that she had relapsing polychondritis. She was given a 10-day reducing course of prednisone, starting with 10 mg. t.d.s. The response was quite dramatic, of both her voice and her ear. When seen four weeks later, the back of the left pinna had become swollen and painful again. Her larynx appeared normal. Prednisone was therefore restarted with good results. General examination at that time revealed no abnormalities apart from a soft mid systolic ejection murmur over the apex. There was no relevant past or family history. There were no significant abnormal findings in blood count, chest X-ray, lung function tests, urea and electrolytes, liver function tests or ECG. In addition rheumatoid latex S.G.A.T. were negative. Immunoglobulin levels were normal. Early in the condition, her ESR was 63, and remained around 30 even when she was maintained on prednisone 5 mg. a day. The patient was referred to the Chest Department where she has been followed up for 2 years. She was maintained on prednisone 5 mg. a day for sixteen months. The steroids were then stopped by reducing the dosage gradually over 12 weeks. She has now been without prednisone for 7 months and remains well at the present time. Summary and conclusions

Relapsing polychondritis is a rare condition which may initially present to the otolaryngologist in a variety of ways. Even if the patient presents to

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another department, it is very likely that a specialist opinion will be sought at some stage during the course of the disease. The aspects of the condition which are of particular interest to the otolaryngologist are discussed and a case is reported. Acknowledgements

I would like to thank Mr. L. N. Dowie, who made the diagnosis in the patient reported, for his permission to pubhsh the case. In addition I would like to thank him and Mr. R. F. McNab Jones, Consultant in charge of the ENT Department, for their help and advice during the preparation of the paper. The Department of Medical Illustration kindly provided the photograph. REFERENCES ARUNDELL, F. W., and HASERICK, J. R. (1960) Archives of Dermatology, 82, 439. BEAN, W. B., DREVETS, C. C , and CHAPMAN, J. S. (1958) Medicine (Baltimore), 37, 353. CODY, D. T. R., and SONES, D. A. (1971) Laryngoscope, 81, 1208.

DALY, J. F. (1966) Archives of Otolaryngology (Chicago), 84, 570. DOLAN, D. L., LEMMON, G. B., and TEITELBAUM, S. L. (1966) American Journal of Medicine, 41

285. HAINER, J. W., and HAMILTON, G. W. (1969) New England Journal of Medicine, 280, 1166. HILDING, A. C. (1952) Archives of Internal Medicine, 89, 445. HORNS, J. W., and O'LOUGHLIN, B. J. (1962) American Journal of Roentgenology, 87, 844. HUGHES, R. A. C , BERRY, C. L., SEIFERT, M., and LESSOF, M. H. (1972) Quarterly Journal of

Medicine, XLI, No. 163, 363. JENSEN, F. (1962) Acta Otolaryngologica, 54, 423. JOHNSON, H. M. (1963) Archives of Dermatology, 88, 651. JOHNSON, T. H., MITAL, N., RODNAN, G. P., and WILSON, R. J. (1973) Radiology, 106, 313.

KAHN, A., and KILBURY, M. J. (1956) Annals of Otolaryngology, 65, 615. KAPLAN, H. L., NORRIS, J. E., FREEMAN, B. S., and BROWN, W. B. (1962) Journal of the American

Medical Association, 180, 164. KAYE, R. L., and SONES, D. A. (1964) Annals of Internal Medicine, 60, 653. MAMNDRAKER, N. H., and LIBMAN, L. J. (1970) Journal of Laryngology and Otology, 84, 337. •• ODKVIST, L. (1970) Acta Otolaryngologica, 70, 448. PEARSON, C. M., KLINE, H. M., and NEWCOMER, V. D. (1960) New England Journal of Medicine,

263, 31. I PURCELLI, M. F., NAHUM, A., and MONELL, C. (1962) Annals of Otolaryngology, 71, 1120.

I RABUZZI, D. D. (1970) Archives of Otolaryngology, 91, 188. _§-' REEFE, W. E., and SIERRA, R. (1970) Medical Annual D.C., 39, 27. SELF, J., HAMMARSTEN, J. F., LYNE, B., and PETERSON, D. A. (1967) Archives ofInternal Medicine,

120, 109. SWAIN, R. E., and STROUD, M. H. (1972) Laryngoscope, 82, 891. THOULD, A. K., STANSFIELD, A. G., and BALME, H. W. (1965) Annals of Rheumatic Diseases,

24, 563. E.N.T. Department, St. Bartholomew's Hospital, West Smithfield, London EC1A 7BE.

Relapsing polychondritis--its otolaryngological manifestations.

Relapsing polychondritis— its otolaryngological manifestations By J. R. MOLONEY (London) Introduction RELAPSING polychondritis is a rare condition in...
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