Abstract
Introduction
Juvenile rheumatoid arthritis (JRA) is one of the most common rheumatic diseases of childhood and a major cause of chronic disability. Most standard paediatric textbooks and review articles about this condition do not mention cricoarytenoid joint fixation as a presenting feature or complication. 1 There are only a few cases reported worldwide, primarily in emergency medicine or anaesthesia journals. 2 Early recognition of this potentially life-threatening condition can prevent the need for tracheostomy.
Case report
A 3-year-old girl presented with a 2-day history of pyrexia and inability to walk. Examination revealed swelling of feet, wrists and small joints of both hands, and painful restriction of finger movements. She was also noted to have a non-pruritic erythematous rash over the trunk, arms and face. Initial investigations showed raised acute phase reactants and rheumatoid factor was negative. Other investigations included immunoglobulins, complement, liver, bone and renal profiles, all of which were within normal limits. Echocardiogram showed a structurally and functionally normal heart.
In view of the quotidian nature of fever, rash correlating with pyrexia and involvement of several joints, a diagnosis of polyarticular juvenile rheumatoid arthritis was suspected. This was confirmed by the rheumatologist who discharged her home on an optimal dose of Ibuprofen with follow- up in place.
She presented soon after discharge, late in the evening, with a 24-hour history of noisy breathing and barking cough. She was found to havebiphasic stridor on exertion and moderate respiratory distress. A diagnosis of croup was made and an initial dose of oral Dexamethasone (0.15 mg/kg) was given. As symptoms persisted she was given an additional dose of oral Dexamethasone and nebulized Budesonide with minimal improvement. Investigations at this point showed persistently elevated inflammatory markers. However, over the next two days her stridor became less noticeable and following complete resolution of respiratory symptoms, she was discharged home. In the interim her joint symptoms, which initially appeared quiescent, gradually worsened over the next three weeks and she was started on high-dose oral Prednisolone by the rheumatologist.
A month after her first presentation with stridor, but within a week of starting steroids, she was readmitted with a 3-day history of shortness of breath and noisy breathing. This was preceded by viral upper respiratory infection. On examination she was found to have biphasic stridor at rest, grunting and significantly increased work of breathing. Her oxygen saturations were 95% in air. She was given oral Dexamethasone followed by nebulized Budesonide without any benefit. An ENT consultation was requested in view of persistent severe stridor and respiratory distress. Brochochoscopy done under general anaesthetic showed fixed non-mobile vocal cords in the midline with a very narrow opening and normal sub glottis. These findings were consistent with cricoarytenoid dysfunction associated with juvenile rheumatoid arthritis and a tracheostomy was performed. In addition, pulsed Methylprednisolone was started on account of the above findings.
She subsequently made a good recovery with uneventful healing of the tracheostomy.
Discussion
Cricoarytenoid (CA) arthritis is a rare complication of JRA. This is in striking contrast to rheumatoid arthritis in adults where the incidence of cricoarytenoid arthritis is reported to be as high as 25–30%. 3 In adults symptoms mainly consist of hoarseness of voice, and pain on talking and swallowing. Other symptoms that should alert one to the possibility of CA arthritis include foreign body sensation in the throat and pain radiating to one or both ears. Upper airway obstruction is relatively uncommon. 4, 5
Croup is very common in infants and young children and therefore the likely diagnosis in any child presenting with stridor and respiratory distress. However, atypical cases which respond poorly to steroids and show recurrence should prompt reviewing the possible differential diagnosis. In a child with suspected or confirmed JRA a possibility of cricoarytenoid arthritis should not be overlooked. Careful history-taking and examination along with a high index of suspicion are vital in the early detection of CA joint dysfunction. Definitive diagnosis is established only by direct visualization of the vocal cords which is often difficult in children and in almost all cases involves examination under anaesthesia.
Cricoarytenoid arthritis is a life-threatening condition and prompt recognition is essential to achieve a good outcome. Early institution of corticosteroids is reported to be the treatment of choice 6, 7 and may avoid the need for tracheostomy. However, the role of steroids, particularly with regards to the timing, remains controversial. Most of the reported cases successfully treated with systemic steroids have been adults with chronic rheumatoid arthritis. In a few children with JRA complicated by cricoarytenoid joint fixation, the use of systemic high-dose steroids has prevented tracheostomy. There are also reports in adults, on local intra-articular injection of steroids which have avoided the need for surgical airway intervention. There are no such reports in the paediatric population.
In our case although high-dose oral Prednisolone was started, it must be borne in mind that symptoms recurred with severity within a week. This may suggest that the cricoarytenoid fixation was far too advanced to respond to oral medication. This perhaps was the most important contributory factor which influenced the decision to carry out a tracheostomy. On balance, a tracheostomy is far less deleterious than a prolonged intubation, and in those developing stridor and severe respiratory distress tracheostomy remains the treatment of choice. 8
Our case serves as a useful reminder of a rare but potentially fatal condition which requires prompt recognition and management to ensure a good outcome.
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Acknowledgements
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