Background and aims Pan-dysautonomia hasn’t been well studied in patients with systemic lupus erythematous (SLE) and the optimal treatments are still to be determined. We report two cases of SLE with pan-dysautonomia and a review of the literature.
Methods Two cases with SLE complicated with pan-dysautonomia were carefully documented and described. Medline, EMBASE, Web of Science and PubMed were searched and 8 other cases were reviewed.
Results Case 1: A 23 year-old female SLE patient with central and peripheral nervous system (CNS and PNS) involvements for 5 years, presented pan-dysautonomia, including severe orthostatic hypotension, dysuria and gastro-intestinal psedo-obstruction. Acetylcholine-receptors (AChR) antibody was detected in her cerebrospinal fluid (CSF). After treating with prednisone, tacrolimus, midodrine and fludrocortisone, her symptoms gradually improved in 12 months. Case 2: A 44 year-old female presented with pan-dysautonomia, anti-nuclear and anti-Ro antibody positivity and hypocomplementemia. AChR antibody titer also elevated in her CSF. After similar treatment, and additional plasma exchange for 6 times, all symptoms improved in 3 months. Comblined with other 8 reported cased, 9 were female, 4 and 3 cases complicated with CNS and PNS involvement respectively, 3 cases had AChR antibody detected and all were postive, 4 cases received methylprednisolone pulse therapy. Cyclophosphamide (n=3), azathiopurine (n=3), and tacrolimus (our case 1) were also used. Plasma exchange was applied in our case 2.
Conclusions Pan-dysautonomia can be an initial symptom of SLE. AchR antibody may be useful in early recognising this rare disease. Plasma exchange along with prompt immunosuppressive therapy seem to lead to an early remission of the disease.
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