Discussion
This was a case of SLE that developed with broad autonomic signs. Autonomic neuropathy is included in the neuropathology of SLE, and its presence or absence has been investigated in some studies,5-8 however, the number of reports is fewer than those on central nervous lesions and mental symptoms. ACR prescribes 5 objective tests to examine autonomic nervous function: postural change test (blood pressure response, heart rate response), respiratory rate variation, Valsalva test positivity, and sweating test positivity.13 This patient was positive for all items, and showed severe hypotension, inducing syncope in the standing position in the postural change test. The patient was admitted for paralytic ileus, however, at the same time, light and convergence reflexes were absent, and anuresis occurred several days later, indicating the acute onset of pan-dysautonomia, and the degree was severe. Two courses of pulse corticosteroid therapy resolved paralytic ileus, however, difficulty in oral ingestion due to nausea continued. Hepatobiliary scintigraphy identified reduced gallbladder contractility, which was improved by IVCY. For this patient, the effect of pulse corticosteroid therapy was insufficient, and IVCY was effective.
Complication of SLE by pan-dysautonomia is a rare pathology, and only 4 cases have been reported.14-17 All reported cases showed acute onset, and the initial symptom of SLE was autonomic neuropathy in 3 cases. In all 4 cases, the response of acute pan-dysautonomia to corticosteroid was very good. As well as acute pan-dysautonomia, only this patient showed CNS and peripheral neuropathy. This patient is exceptional as complicated acute pan-dysautonomia in SLE (Table 1). Our patient had been diagnosed with SLE based on skin eruption and immunological abnormality 3 years before the appearance of autonomic symptoms, and hypocomplementemia had persisted with no apparent organ disorder. On admission, severe autonomic neuropathy was noted, however, no characteristic clinical sign of SLE, such as skin eruption, arthritis, and nephritis was present, and anti-DNA antibody was also negative. However, acute confusional state, brain-wave abnormality, and an increase in the cerebrospinal fluid protein level were detected, based on which SLE was diagnosed. If the patient had not visited a medical institution, and the course before admission was not clear, the diagnosis of SLE would have been difficult.
Acute, broad, and severe autonomic neuropathy with no underlying disease is called AIAN.9-11 AIAN and Guillain-Barre syndrome have many common points: acute onset, the presence of preceding infection in many cases, a high frequency of albuminocytologic dissociation, and the presence of serological inflammatory findings and inflammatory cell infiltration on nerve biopsy in some cases,11,18 and steroid treatment, plasma exchange, and massive ?-globulin administration have been applied from the viewpoint that immunological abnormality is present in the background. Recently, the presence of anti-ganglionic nicotinic acetylcholine receptor (AChR) antibody in AIAN has been reported,19 and the disease concept, autoimmune autonomic neuropathy, has been proposed as a disease group that develops autonomic neuropathy via an autoimmune mechanism.20 Guillain-Barre syndrome involves motor and sensory neuropathy accompanied by autonomic neuropathy, however, anti-ganglioside antibody is present, being recognized as an autoimmune disease targeting peripheral nerves.21 The presence of patients with a high, specific antibody titer against mixed antigens of phospholipid and ganglioside or several gangliosides has been reported.22,23 Similarly, findings suggesting the involvement of autoantibodies, such as anti-NR2 glutamate receptor antibodies, in CNS lupus have been increasingly reported.24-27
Unfortunately, we were not able to measure the titer of these autoantibodies; however, persistent hypocomplementemia suggested the involvement of autoantibodies in the onset of autonomic neuropathy. The neuropathies observed in this patient may also have resulted from autoantibodies.