The patient was a 32-year-old woman with chief complaints of fever and abdominal pain, with no particular past or familial medical history. Regarding the history of the present illness, the patient visited the Dermatology Department of our hospital for cheek erythema in March 2002. Laboratory testing showed leucopenia (2,100/?l), lymphopenia (560/?l), hypocomplementemia, and positive antinuclear antibody (1:1,280), anti-DNA, and antiphospholipid antibody, and the patient was referred to our department. The patient met the SLE criteria, however, no active lesion was present and, thus, aspirin was administered at a low dose for the antiphospholipid antibody positivity, and the course was observed. On July 21, 2006, fever of 37.5?C and lower abdominal pain developed with no advance sign, and numbness and pain in both the hands and legs appeared the following day. The patient visited the hospital for aggravation of abdominal distension and pain. Ileus was diagnosed, and the patient was admitted emergently. On admission, she was in a markedly confused state. Body temperature was 37.8?C, but the heart rate was 60/min, showing relative bradycardia. Light and convergence reflexes were absent, and the tongue was dry. The abdomen was distended, tense and tympanitic. Sounds of intestinal peristalsis were absent over the abdomen. The skin was dry with anhidrosis. No deep tendon or abnormal reflexes were noted, and glove and stocking-type sensory abnormality was present. On laboratory testing on admission, white blood cells (WBC) were 3,700/?l; lymphocytes, 222/?l; hemoglobin, 10.8 g/dl; platelets, 19.6 x 104/?l; and erythrocyte sedimentation rate (ESR), 15 mm/1h. Biochemical testing and urinalysis were normal. On immunoserological testing, CRP was 1.1 mg/dl; C3,50 mg/dl (normal 86-160 mg/dl); C4, 4 mg/dl (17-45 mg/dl); and CH50, 13 U/ml (25-50 U/ml); indicating hypocomplementemia. Antinuclear antibody titer was 1:320 (homogenous, speckled); anti-cardiolipin antibody, 11 U/ml (normal value › 8 U/ml); lupus anticoagulant (LAC) (dilute Russell’s viper venom time), 1.83 (› 1.3); and LAC (phospholipid neutralization), 10.7 sec (› 6.3 sec); showing positivity. Anti-DNA antibody (RIA) was 2.9 IU/ml (normal value › 6 IU/ml); anti-dsDNA antibody, 10 IU/ml (› 10 IU/ml); anti-CL?2GPI, 2.2 U/ml (› 3.5 U/ml); anti-RNP, (-); anti-Sm, (-);anti-SSA, (-);anti-SSB, (-); and antiribosomal P, (-); all normal values. Paralytic ileus was diagnosed on admission (Fig. 1). At the same time, disappearance of the light reflex, dyshidrosis, anuresis, respiratory sinus arrhythmia, and Valsalva test positivity were present, showing broad autonomic neuropathy. On admission, the patient was in acute confusional state, the cerebrospinal fluid (CSF) was clear with a normal opening pressure and contained 89 mononuclear cells/mm3. Protein content of the CSF was 81 mg/dl (normal range 10-40mg/dl) and the IgG index was 0.63 (normal range 0.20-0.85). The IL-6 level was markedly high (1,850 pg/ml). Oligoclonal bands were absent and all CSF cultures were negative. Electroencephalography (EEG) showed diffuse slow waves. Brain and cervical/thoracic vertebral magnetic resonance imaging (MRI) detected no abnormality. Sensory nerve conduction velocity was impossible to identify, with sural nerve, radial nerve, and ulnar nerve polyneuritis diagnosed, and NPSLE, mainly accompanied by autonomic neuropathy, however, also various neuropathologies, such as CNS and peripheral neuropathies, was diagnosed. Pulse corticosteroid therapy (intravenous infusion of methylprednisolone, 1000 mg/day for 3 days) on the 3rd-5th days was performed, followed by prednisolone 60 mg/day, and the acute confusional state, paralytic ileus, and polyneuritis rapidly improved. The patient became capable of changing position, however, repeatedly lost consciousness in the standing position, revealing severe orthostatic hypotension. Voluntary micturition was hardly possible, and a large volume of urine was retained, making removal of the urethral catheter difficult. Hepatobiliary scintigraphy detected digestive tract dysfunction, which may have been a sign of autonomic neuropathy (Fig. 2 a-c). Plasma exchange using 30 units of fresh frozen plasma on the 19th, 27th, 31st, 34th day and a 2nd course of pulse corticosteroid therapy on the 27-29th days were performed, however, were ineffective. CSF reexamination on the 41st day showed IL-6 1.6 pg/ml, TP 46 mg/dl, and improvement; however, hypocomplementemia persisted. IVCY (750mg) was administered on the 55th day, thereafter, orthostatic hypotension and vesicorectal disorder resolved. Nausea persisted, even after the remission of paralytic ileus, and oral ingestion was difficult. The 2nd course of IVCY on the 83rd day improved the symptoms on hepatobiliary scintigraphy (Fig. 2 d-f), hypocomplementemia, which increased slowly, normalized, and all autonomic neuropathies improved. She was discharged with PSL 25mg on the 101st hospital day (Fig. 3).