A 53-year-old woman was referred to the immunology clinic for further assessment after an episode of serotype Y meningococcal meningitis. She had no history of exposure to meningococcal infection or of foreign travel. Otherwise, she had no history of infections. Six months before the admission with the meningitis, she had an episode of pleurisy, which did not respond to antibiotics but responded to a 5 day course of prednisolone. Apart from this short course of steroids 6 months before the meningitis she had not received any immunosuppressive drugs.

She had had persistent non-productive cough with breathlessness since the age of 47 years. A chest CT scan and spirometry, at this stage, were normal.

From the age of 48, she had been experiencing urticaria, mainly on her arms and legs, almost every day. The wheals took about 12 hours to develop and lasted for at least 24 hours, leaving bruise-like marks. In addition, she had episodes of angioedema affecting the face, cheeks, lips and eyelids. Because of the atypical features of her chronic urticaria, which were suggestive of urticarial vasculitis, a skin biopsy was done. This showed non-specific changes of dermal oedema and little perivascular inflammatory cell infiltration. Treatment with antihistamines had little benefit.

She had had recurrent episodes of aches and painful swelling of her knees, ankles, wrists and fingers, lasting for more than a week from the age of 50, but no morning stiffness or deformity. Most recently, she had been diagnosed with anterior uveitis.

On physical examination, she had non-scarring urticaria on legs and arms, angioedema of the upper lip and red left eye. Both knees and both ankles were tender and swollen with synovitis. There was no organomegaly. Her blood pressure was 159/87 mm Hg. Urinalysis …