Guillain–Barré syndrome as presenting feature in a patient with lupus nephritis, with complete resolution after cyclophosphamide treatment

Various neurological features have been reported in association with systemic lupus erythematosus (SLE). However, Guillain–Barré syndrome (GBS) as a presenting feature of SLE appears to be rare [1–3]. We report a patient presenting with GBS, in whom lupus nephritis was subsequently diagnosed. The GBS failed to respond to intravenous immunoglobulin treatment, but both GBS and lupus nephritis responded very favourably to intravenous pulses of cyclophosphamide and prednisone. A 20‐year‐old woman was referred to our hospital because of a 10‐day history of progressive muscle weakness of arms and legs, paraesthesias in both hands and feet, and diplopia. Her medical history was unremarkable. Physical examination on admission revealed a blood pressure of 150/110 mmHg and cardiac enlargement. Neurological examination showed a left abducens paresis, absent deep tendon reflexes, and severe symmetrical proximal muscle weakness. Laboratory examination revealed an erythrocyte sedimentation rate of 84 mm/h; C‐reactive protein 71 mg/l; urea 12.3 mmol/l; creatinine 150 μmol/l; serum albumin 17 g/l; LDH 434 U/l; Coombs‐positive haemolytic anaemia; but normal thrombocyte and leukocyte counts. Virological and bacteriological examination showed only a recent para‐influenza infection, but no signs of a Campylobacter jejuni infection. Qualitative urine analysis revealed the presence of protein. Later, also an active urinary sediment was found (10–25 leukocytes, hyaline and coarse granular casts, 25–50 dysmorphic erythrocytes). The cerebrospinal fluid revealed a normal cell count, total protein, lactate, pyruvate and brain‐specific proteins (neuron‐specific enolase, myelin basic protein and S‐100 protein). A slight IgM synthesis (0.4 mg/l) was found, but no synthesis of IgG or IgA. Oligoclonal immunoglobulin bands were absent.