British Journal of Rheumatology 1992;31:619-622


SERONEGATIVE SYSTEMIC LUPUS ERYTHEMATOSUS BY P. CREAMER AND J. KIRWAN Rheumatology Unit, University Department of Medicine, Bristol Royal Infirmary, Bristol BS2 8HW

patchy numbness of the right leg but a number of new symptoms quickly developed including weakness affecting particularly proximal muscles; headaches; persistent visual disturbance with zig-zag lines and green and red lights in both visual fields; oral ulceration; polyuria and palpitations with dyspnoea on walking 20 yards. In addition to the rash and polyarthritis, examination now revealed grade 4 weakness of proximal muscles, and two areas of 'cotton wool' spots in the right retina. Investigations showed a reduced WCC (4.1 x 1071) withalymphopeniaof 0.9 x 1071 and reduced platelets (94 x 1071) . Serum C4 was low at 0.18 g/1 (normal range 0.25-0.65 g/1) but C3 and CH50 were normal, as was Clq binding. There was thus no evidence for circulating immune complexes. ANA, dsDNA, ENA and RF were again negative. Plasma viscosity and CRP were normal. Urine volume was 4.4 I/day without glycosuria but there was moderate proteinuria (3.2 g/24 h) and microscopic haematuria. There were no urinary casts and creatinine clearance was normal. Renal biopsy subsequently revealed mild mesangiopathic glomerulonephritis (WHO stage IIA), not thought severe enough to require intervention. Creatine kinase was elevated at 452 U/l (normal range 24-195 U/l) and a muscle biopsy showed fibre atrophy under electron microscopy which was consistent with a myopathic process. AST was 73 U/l, but other liver function tests were normal. An electrocardiogram showed flattening of T waves in the lateral chest leads; occasional ventricular ectopics superimposed on sinus rhythm were recorded over 24 h. Chest X-ray was normal. Prednisolone 60 mg daily resulted in slowly improving muscle strength and less breathlessness. Electromyography suggested focal mononeuritis. Fundoscopy (Fig. 1) revealed multiple cotton wool spots in both fundi, consistent with SLE retinopathy. Electroencephalography showed excess slow wave activity but a cranial computerized tomography (CT) scan of the head was normal. Thirteen days later the patient suddenly deteriorated with confusion, diplopia in all directions and severe weakness such that he was unable to walk unaided. A short, generalized, tonic-clonic seizure occurred. Three daily pulses of methyl prednisolone 1 g and

Submitted 18 May; accepted 21 May 1992. Correspondence to P. Creamer. 0263-7103/92/090619 + 04 $08.00/0

© 1992 British Society for Rheumatology 619

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CASE REPORT WHILST on holiday in Tenerife in December 1990, a 25year-old man developed a rash on his hands and feet. It was persistent and itchy and spread over a few days to involve the trunk and left thigh. At the same time he began to develop flu-like symptoms. Two weeks later he noticed arthralgia affecting wrists, interphalangeal joints, shoulders and ankles and was prescribed naproxen by his General Practitioner who also referred him for dermatological review. The patient was heterosexual and married with a 2-year-old son. His father was Chinese, his mother European, but there was no relevant family history. He had a previous history of acne and urticaria. On examination a lichenoid rash in the distribution described was noted. There was minor capillary dilatation in some nail folds. A symmetrical synovitis of wrists and interphalangeal joints was detected but there was no lymphadenopathy or hepatosplenomegaly. Investigation at that time showed a haemoglobin of 13.9 g/dl and white cell count (WCC) of 4.4 x 1071. The lymphocyte count was low at 1.0 x 1071 (reference range 1.5-4.0 x 109). The platelet count was 121 x 1071 (150-400 x 109). Plasma viscosity was normal but C-reactive protein (CRP) was slightly elevated at 12 mg/1 (normal range

Seronegative systemic lupus erythematosus.

We have presented a patient with severe clinical SLE but negative serological tests. This diagnostic difficulty, together with a presentation limited ...
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