Case Number 24: Scalp necrosis in giant cell arteritis
Authors: F H Khattak
Journal: Annals of the Rheumatic Diseases (2002)

A 30 year old woman with systemic lupus erythematosus (SLE) was admitted to hospital complaining of shortness of breath, orthopnoea, swelling of ankles, and increased weight over the preceding eight days.She otherwise felt well, and reported that her lupus was inactive.She denied any associated chest pain, cough, or fever.A similar presentation had occurred three months previously, when she went to another hospital with the same symptoms, associated with a raised temperature (38.4°C).Investigations performed at that time included a complete blood picture, cardiac enzymes, and the measurement of antibodies against double stranded DNA (dsDNA).Blood and urine cultures were normal and a chest x ray examination was normal.All blood tests were normal with the exception of a raised troponin-t level of 0.62 µg/l (normal <0.1 µg/l).An echocardiogram and high resolution computed tomography (HRCT) chest scan were also done.She was discharged from hospital and given oral diuretics without a definite diagnosis being made.Two weeks later, her case was reviewed in our hospital and in view of the recent history, a repeat chest x ray examination, echocardiogram, and HRCT scan of the chest were performed.The only abnormality was seen on echocardiography, which showed a hypokinetic inferior, inferoposterior, and inferoapical left ventricle wall, with some mild to moderate left ventricular dilatation and systolic dysfunction.There was a small pericardial effusion with no valvular abnormalities and the ejection fraction of the left ventricle was estimated at 43%.Treatment was not altered and she remained stable until the current presentation.The patient's SLE had been diagnosed 10 years previously, with initial symptoms including alopecia, Raynaud's syndrome, arthralgia, and a photosensitive skin rash.Six years after diagnosis she was diagnosed with WHO grade 4 lupus nephritis (diffuse proliferative and necrotising glomerulonephritis).Serum creatinine remained normal and she received no specific treatment for the glomerulonephritis.Two years before the current presentation she developed a vasculitic rash with painful leg ulcers, and treatment with pulse methylprednisolone (1 g) and intravenous cyclophosphamide (500 mg) monthly was started and continued for six months, then every three months for two years.Treatment was complicated by genital herpes requiring long term treatment with famciclovir.Other drugs included hydroxychloroquine 200 mg daily, prednisolone 5 mg daily, and calcitriol 0.25 µg twice daily.She was seen to have labile hypertension during this period, with blood pressure readings varying between 130/70 and 170/100 on repeated measurements.On physical examination she was afebrile, hypertensive (blood pressure 180/100), with a 2 cm increase in the jugular venous pressure, a gallop rhythm 100/min, dual heart sounds with no murmurs, and mild pitting oedema of the ankles.Chest examination was clear without basal crepitations, there was no ascites, and there was no evidence of vasculitic rash or leg ulcers.Investigations showed urea 14.6 mmol/l (normal 3-8) and creatinine 0.145 mmol/l (normal 0.045-0.090),nor-