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Syncope in a patient with relapsing polychondritis
  1. W R Saliba,
  2. L H Goldstein,
  3. G S Habib,
  4. M Elias
  1. HaemeK Medical Centre, Afula 18101, Israel
  1. Correspondence to:
    Dr W R Saliba
    HaemeK Medical Centre, Afula 18101, Israel Afula, IL; salibussyahoo.com

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Relapsing polychondritis (RP) is a rare disease, characterised by recurrent episodes of inflammation of cartilaginous tissues and other proteoglycan rich structures.1,2 The cardiovascular system is affected in 24–52% according to different series,1,3,4 and typically is not the presenting feature but appears after a mean of six years after the onset of symptoms5; aortic regurgitation is the most common manifestation (4–10%), followed by mitral regurgitation (2–8%), aortic aneurysm (2–7%), pericarditis (0–8%), and conduction disturbances (4–6%).1,3,6,7 Cardiovascular disease mostly affects male patients and is the second most common cause of death in RP.5 All types of atrioventricular conduction defects have been reported in RP.6,7 Godeau described transient complete heart block (CHB) during relapses that required temporary pacing,4 suggesting recurrent acute inflammation of the conduction system during relapses. Fibrosis of the conduction system was detected at necropsy in a patient with CHB.8

CASE REPORT

Here we report a case of RP in which the relapse was preceded by true syncope, caused by suspected sinus node dysfunction (SND). To our knowledge, both relapse preceded by syncope and SND have not been previously reported in RP.

A 43 year old woman with RP of six years’ duration presented to the emergency room with syncope followed by sudden onset of auricular chondritis and right knee arthritis.

On arrival the patient was alert and complained of pain in the inflamed areas. Her blood pressure was 122/73 mm Hg, temperature was 37°C, pulse was regular 33 beats/min (her basal rate was 68 beats/min two months previously). Complete blood count, creatinine, urea, serum electrolytes, liver and thyroid function tests were normal. An electrocardiogram showed a regular rhythm 34 beats/min with normal QRS and PR intervals. The rhythm was not affected by carotid sinus massage and an echocardiogram was normal. Her drugs were prednisone, azathioprine, and thyroxine (Eltroxin), and she did not take any drugs known to cause bradyarrhythmia.

Within hours almost all joints were affected, the large and small joints of the arms, hip, knee, ankle, foot, costochondral, sternomanubrial, sternoclavicular, and finally, sacroiliac joints. The auricular chondritis worsened and the right external auditory canal was rapidly affected with severe inflammation that led to almost total occlusion with a large serous discharge. She also developed hoarseness and wheezing as a manifestation of laryngotracheal involvement.

Prednisone and azathioprine doses were increased and analgesics were added, as needed, to relieve pain. Five days later the pulse rate gradually increased. Pulse rate at discharge was 65 beats/min.

DISCUSSION

The inadequate acceleration in sinus rate in response to severe pain in our patient may indicate SND. Sinoatrial block might also be the cause of this bradycardia and invasive intracardiac recordings are required to differentiate it from sinus bradycardia.

The tachybradia syndrome is also a possibility, although the patient did not complain of palpitations. Vagal response that can cause sinus bradycardia and syncope is less likely in this case because the pain was preceded by syncope, and the heart rate did not recover with pain relief.

The sinus node, similarly to the atrioventricular conduction system, may be affected in RP by acute inflammation that eventually may lead to fibrosis. In conclusion, we have described a patient with RP who had a rapid progressive course. Syncope may be the first manifestation of an RP flare, thus doctors should be aware of this symptom in these patients. Finally, SND in RP warrants further investigation.

REFERENCES

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