Medical ophthalmology: Case one

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Figure 1   Figure 2

Figure 3

This 26 year-old man was followed up in the eye clinic for a right scleritis. He was also under the chest physician because of respiratory problem (figure 2).

a. What is the differential diagnosis?

The pictures show saddle nose (better shown in figure 3) and a tracheostomy scar. The two conditions to be considered are:

• relapsing polychondritis
• Wegener's granulomatosis

b. He also had a history of recurrent earache. What is the most
likely diagnosis?

Recurrent earache suggests chondritis of the pinna and favours the diagnosis of relapsing polychondritis.

Relapsing polychondritis is a rare auto-immune disease with episodic progressive inflammation. The cartilagenous structures throughout the body are involved including the larynx, trachea, auricular, eyes etc.
Associated systemic vasculitis and glomerulonephritis increases the morbidity and mortality.

c. How do you diagnose this condition?

The diagnosis can be made with the McAdam's criteria (1976) in which three or more of the following are present:

• bilateral auricular chondritis
• non-erosive, seronegative inflammatory polychondritis
• nasal chondritis with saddle nose
• ocular inflammation (keratitis, scleritis, episcleritis and uveitis)
• respiratory tract chondritis (laryngeal and/or tracheal cartilage)
• cochlear and/ or vestibular dysfunction (neurosensory hearing loss, tinnitus and / or vertigo)
• cartilage biopsy confirmation of a compatible histologic picture

d. How do you manage this condition?

In mild cases, the treatment is with non-steroidal anti-inflammatory medication and low dose steroid. In severe cases, intravenous prednisolone followed by high dose steroid is usually given.
The condition has a varied response to treatment.