Annals of the Rheumatic Diseases, 1988; 47, 1021-1026

Case report

Relapsing polychondritis mimicking rheumatoidarthritisP SCHLAPBACH,1 N J GERBER,1 P RAMSER,2 AND F M VAN'T HOOFT2

From the 'Department of Rheumatology, University of Berne, Inselspital, CH-3010 Berne, Switzerland; andthe 2Department of Internal Medicine, Tiefenauspital, CH-3004 Berne, Switzerland

SUMMARY A woman with relapsing polychondritis presented with progressive and deformingpolyarthritis (but always negative for rheumatoid factor) 14 years before the appearance oftypical clinical and histological changes of nasal and auricular cartilage destruction.

Key words: nose deformity, ear deformity.

Case report

Relapsing polychondritis is a rather rare but severesystemic disease, characterised by recurrent epi-sodes of inflammation and destruction mainly ofcartilaginous structures. The principal destructionsoccur in the respiratory tract (larynx, tracheobron-chial cartilage), sensory organs (ears, nose, andeyes), cardiovascular system (leading to aorticinsufficiency, aortic and other arterial aneurysms,vasculitis), and peripheral joints."v Recent reportsshow possible involvement of the kidneys.5We have observed a patient with symmetrical

erosive polyarthritis of 14 years' duration mimickingclassical rheumatoid arthritis, but with persistentabsence of rheumatoid factor or other autoanti-bodies. The purpose of the following case report isto draw attention to the possibility of joint erosionsin relapsing polychondritis.

CASE HISTORYThe patient, a woman born in 1918, had Bell's palsyof the left facial nerve in 1940 with a recurrence in1956. In 1968 and 1972 she had facial 'erysipelas'with swelling of the nose, frontal region, and bothcheeks. The symptoms abated slowly with parenteralpenicillin treatment. Simultaneously with the secondinflammatory episode the patient became hard ofhearing. In the course of 1973 symmetrical synovitisAccepted for publication 23 April 1988.Correspondence to Dr P Schlapbach, Oberarzt, RheumatologischeUniversitatsklinik, Inselspital, CH-3010 Berne, Switzerland.

developed in both knees, anikles, radiocarpal joints,and a few proximal interphalangeal joints of bothhands. The patient also complained of morningstiffness of longer than one hour in the afflictedjoints. Rheumatoid factor negative rheumatoidarthritis was diagnosed, and treatment with chloro-quine and systemic, low dose corticosteroids pro-duced substantial improvement of all symptoms.A few months later radiotherapy of the left

shoulder and right knee was necessary owing tointractable pain. In 1978 pulmonary infarction andpneumonia with bilateral pleural effusions afterdeep venous thrombosis of the left calf occurred.

Until 1984 the polyarthritis showed an undulatingcourse with asymptomatic periods of severalmonths. On clinical examination the peripheraljoints showed persistent bilateral, low grade synovitisof the metacarpophalangeal joints, hyperextensionof the interphalangeal joint of the right thumb, swanneck deformity of various fingers (Figs la and b),reduced wrist motility, extension deficits of bothelbows, and subluxation of all metatarsophalangealjoints (Fig. 2). An x ray examination of the hands(Figs 3a and b) and forefeet (Figs 4a and b) showederosive destructions of the radiocarpal, intercarpal,metacarpophalangeal, isolated proximal interpha-langeal, and metatarsophalangeal joints bilaterally.Rheumatoid factor and antinuclear autoantibodiesremained persistently negative. In 1984 chloroquinewas replaced by aurothiomalate, without substantialeffect. In the same year there was spontaneousappearance of saddle nose deformity (Fig. 5). In

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Fig. la Fig. lbFig. 1 (a) Left and (b) right hand showing synovial swelling of radiocarpal, metacarpophalangeal, and isolated proximalinterphalangeal joints and swan neck deformitv ofseveral fingers bilaterally.

Fig. 2 The right and left forefootshowing spreading, hallux valgusdeformity, and dorsal subluxationof the second to fifthmetatarsophalangeal jointsbilaterally.

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Relapsing polychondritis mimicking RA 1023

Fig. 3a Fig. 3b

Fig. 3 x Rays of(a) the left and (b) the right hand showing juxta-articular osteopenia, erosive destruction of theradiocarpal, intercarpal, metacarpophalangeal, and isolated proximal interphalangeal joints bilaterally.

October 1985 the patient developed leucopenia(3-Ox109/l) and thrombocytopenia (92x109/l), thecause remaining unclear. Both findings persistedafter parenteral gold treatment was stopped. In 1986painful swelling of the tragus of the left earoccurred, preventing further use of the hearing aidand necessitating excision of the tragus. Histologyshowed focal necrosis of the hyaline cartilage,chronic granulating perichondritis, and surroundingscar tissue (Fig. 6). A few months later the patientwas readmitted to hospital because of upper gastro-intestinal haemorrhage due to stomach ulcer causedby non-steroidal anti-inflammatory drugs.

FURTHER INVESTIGATIONSIn Octber 1986 further investigations showed anerythrocyte sedimentation rate of 90 mm/lst h;

haemoglobin 74 g/l; packed cell volume 0*24;normal erythrocyte indices. Leucocytes were2*5 x 109/l with normal differential; thrombocytes45x109/l. Rheumatoid factor (RF) (IgM RF, IgARF, IgE RF, and IgD RF) and antinuclear anti-bodies (including all subgroups) were negative.There were no circulating immune complexes.Normal values were obtained for electrolytes, bloodurea nitrogen, creatinine, serum aspartate transami-nase, serum alanine transaminase, alkaline phos-phatase, glucose, and for serum IgG, IgM, IgA.There was hypoalbuminaemia. No light chains werefound. Urinary sediment was normal. Bone marrowaspiration showed focal increase of polymorphiclymphoid elements, without selective monoclonalincrease in any subpopulation, and without progres-sion in the follow up examination 12 months later.

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Fig. 4a Fig. 4b

Fig. 4 x Rays of (a) the left and (b) the rightforefoot showing diffuse osteopenia, erosive destruction, and subluxation ofall metatarsophalangeal joints.

HLA typing showed positive antigens of the loci A2,A29, Bw44, Bw4, Cw5, DR4, and DR7. Anti-phospholipid antibody was negative (March 1988).

Discussion

Relapsing polychondritis with erosive and deform-ing joint involvement has to our knowledge onlyonce been reported.8 This is rather unusual for a

systemic disorder characterised by episodic in-flammation and destruction of cartilage and itssurroundings. The present case report is intended todraw attention to the possibility that relapsingpolychondritis may mimic rheumatoid arthritis bydeveloping a symmetrical and erosive deformingpolyarthritis of small and large joints. Our patientshowed recurrent inflammation of ears and nose,leading to destruction of the left tragus and saddlenose deformity. She also developed an erosivedeforming arthritis, mainly involving the radiocarpal,metacarpophalangeal, metatarsophalangeal, andisolated proximal interphalangeal joints bilaterally.The arthropathy misled the treating physicians for

many years because of its erosive nature and the lateappearance of auricular and nasal destruction. Herserum was persistently negative for rheumatoidfactor, she never developed subcutaneous nodules,and she showed no evidence of a metabolic diseasedespite extensive investigation. Several unusualsystemic features (leucopenia, thrombocytopenia,and deep venous thrombosis) could imply coexistentsystemic lupus erythematosus. The patient, however,does not fulfil the revised criteria for the classi-fication of sXstemic lupus erythematosus proposedby Tan et al. Antinuclear autoantibodies (includingall subgroups) were persistently undetectable. Inaddition, the antiphospholipid antibody titre wasnegative.

Until now the arthropathy of relapsing poly-chondritis has been considered as migratory, asym-metric, anodular, non-erosive, and rheumatoidfactor negative, affecting large and small peripheraljoints as well as parasternal and sacroiliac articu-lations.3 10 11 The feet have been reported to bespared.3 Our case, therefore, is the first withdefinite involvement of the metatarsophalangeal

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Fig. 5 Saddle nose deformity appearing many years afterdevelopment ofsymmetrical erosive polyarthritis.

joints. The arthritic attacks were episodic, nottemporally related to the extra-articular inflam-mation of nose and ears, and were at times verypainful.

Destructive and erosive joint lesions in patientswith relapsing polychondritis are still a matter fordebate. Braunstein et al and O'Hanlan et al suggestthat erosive joint changes are not a feature of truerelapsing polychondritis, but probably due to coexis-tent rheumatoid arthritis or another inflammatoryarthropathy.'10 1 Such coexistence (with rheuma-toid arthritis or lupus erythematosus) has beenfrequently recorded.' 3 12-1 In contrast, Johnson etal showed erosive articular changes indistinguishablefrom those of classical rheumatoid arthritis in onecase of relapsing polychondritis.8 To conclude, afterexclusion of other conditions we believe that relaps-ing polychondritis is the cause of the symmetricaland erosive lesions in the radiocarpal, metacar-pophalangeal, metatarsophalangeal, and isolatedproximal interphalangeal joints of this patient. She

Fig. 6 Histology ofthe tragus showing focal necrosis ofthehyaline cartilage, chronic granulating perichondritis, andsurrounding scar tissue.

fulfills the diagnostic criteria of relapsing polychond-ritis, as proposed by McAdam et al.3

We thank Miss M Kummer and Mrs E Gerny for the preparation ofthis manuscript.

References

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