Other features

Gastrointestinal Manifestations

Nausea, vomiting, haemorrhage, abdominal pain and malaise occur in around 20% of cases; the patient may have an enlarged liver and/ or spleen, and this is usually associated with haemolytic anaemia. Around 10% suffer from dysphagia (problems with swallowing). Pancreatitis is a rare complication.

Eye (Ocular) manifestations

The most common eye manifestations are conjunctivitis (inflammation of the conjunctiva, the part that lines the surface of the eye and inner surface of the eyelid) and episcleritis (inflammation of the superficial layer of the white of the eye), which occur in around 15% of cases.

Dry eyes (sicca symptoms) may occur with or without association of secondary Sjogren’s syndrome (the association of a connective tissue disease with reduced production of tears, causing dry itchy eyes or reduced saliva production causing a dry mouth).

Rarely, retinitis (inflammation of the blood vessels at the back of the eye) can occur and can lead to blindness. Rapid treatment must be implemented as blindness can develop over a few days.

As well as the above clinical features, systemic features which may begin suddenly or insidiously are -:

• Fever, which commonly is one of the first presenting symptoms or which occurs at the time of a bad flare - the person (77%) often has “flu- like” symptoms.
• Tiredness or fatigue.
• Malaise (feeling unwell).

Widespread enlargement of the lymph nodes is common.

Amended November 2008