sle is a disorder of immune dysregulation leading to autoimmunity involving virtually any organ . being a multisystem disorder , there is a broad spectrum of clinical presentation , and it often presents a diagnostic dilemma ( 1 ) . it has peak prevalence in african american women of reproductive age in the united states ( 2 ) . sle also affects the central nervous system , the peripheral nervous system , and the autonomic nervous system and is associated with worse prognosis ( 3 , 4 ) . neuropsychiatric manifestations of lupus may precede the onset of lupus , occur at the time of diagnosis or later in the course of the disease ( 3 , 5 ) . american college of rheumatology has defined 19 primary neuropsychiatric syndromes in lupus ( 3 ) . peripheral nervous system involvement is seen in less than 10% of all nervous system manifestations ( 5 ) . it has an annual incidence of about two cases per 100,000 individuals , and is the most common cause of acute flaccid paralysis in the united state ( 6 ) . antecedent infectious triggers leading to gbs have been identified including campylobacter jejuni , cytomegalovirus , epstein barr virus , mycoplasma pneumoniae , and haemophilus influenzae 
 ( 6 , 7 ) . associations of gbs have also been described with hiv infection , immunizations , lymphoma , and bone marrow and organ transplantation . gbs as initial manifestation of lupus is exceedingly rare and has been reported in a few cases in the literature ( 812 ) . we report a 38-year - old female who presented with aidp as initial manifestation of sle . natural history of gbs is variable in terms of extent and severity of involvement ( 6 ) . therefore , it is of utmost importance to diagnose the condition in a timely manner to prevent potentially life - threatening complications . in our patient , diagnosis was made in a timely manner , and prompt treatment of gbs with ivig was initiated . furthermore , our patient was treated with a dose of cyclophosphamide to control sle along with pulse - dose steroids . eventually , she had complete neurological recovery approximately 8 months after the initial presentation . gbs in lupus is a complex and poorly understood phenomenon but likely involves immunological mechanisms . whether gbs unmasks an underlying autoimmune disorder or lupus flare triggers gbs is currently unknown . first , simultaneous occurrence of gbs and sle can be explained by a common etiological trigger , for example , epstein molecular mimicry where an immune response is triggered because of cross - reactivity is an implicated pathogenesis in gbs since many cases of gbs are preceded by infections ( 6 ) . it is possible that cross - reacting epitopes not only invoke an immune response against neurons causing gbs but also against other organs and at the same time contribute to sle - like presentation . it is noteworthy that our patient did not have any antecedent diarrhea , respiratory illness , or immunization ; hence , this mechanism is not a likely explanation in this patient . second , a widespread immunological response in sle may cause autoantibody formation against gangliosides which can potentially elicit demyelinating polyneuropathy such as gbs ( 1 ) . elevated proinflammatory cytokines such as interleukin-6 and interleukin-8 have been found in patients with sle with neurological symptoms ( 14 ) . it is plausible that cell - mediated immunity and complement activation play important roles as well . third , vascular phenomena in sle including vasculitis , micoangiopathy , and premature atherosclerosis leading to ischemic demyelination may trigger a gbs - like response ( 15 ) . it is interesting to note that our patient had lupus anticoagulant and phosphatidylserine igg antibodies which , theoretically , may confer a hypercoagulable state causing microinfarction and demyelination . last , host - specific factors such as genetics or ethnicity and environmental factors may be involved . ivig have shown efficacy against gbs and is the first line therapy along with plasmapheresis ( 6 , 16 ) . proposed mechanism involves antagonization of circulating pathological antibodies by anti - idiotypic antibodies . besides , modulation of cell - mediated immunity and complement pathways are also possible ( 17 ) . the present patient received ivig for treatment of gbs in the background of another autoimmune disorder . its mechanism of action involves alteration in the lysosomal ph interfering with antigen presentation by macrophages . inhibition of peptide - major histocompatibility complex ( mhc ) fails to stimulate t - helper lymphocytes , thereby causing downregulation of autoimmune response against autoantigens . although hydroxychloroquine is the first line agent in sle , it is not sufficiently immunosuppressive to control organ - threatening acute flares or severe neuropsychiatric sle . in this regard , low - dose cyclophosphamide has demonstrated efficacy in the treatment of non - thrombotic neuropsychiatric lupus ( 18 ) . cyclophosphamide is an alkylating agent that inhibits dna replication by adding alkyl radicals into dna strands forming dna crosslinks . it exerts its immunosuppressive effects in sle and other autoimmune disorders by suppressing both t- and b - lymphocytes . however , its use in the treatment of gbs in lupus has not been studied . it appears that low - dose cyclophosphamide combined with corticosteroids improved the overall outcome in patients with sle where gbs was the initial presentation ( 9 , 10 , 12 ) . the association of gbs with lupus is extremely rare and likely has an immunological basis . it seems to have implications for both treatment and prognosis , and identification is important as it may imply important therapeutic decisions . early diagnosis of gbs is extremely important to initiate therapy to limit morbidity and mortality . this case not only illustrates a rare and unusual presentation of a common disorder but also a complete neurological recovery after being treated with immunosuppressive therapy . therefore , low - dose cyclophosphamide with corticosteroids should be considered when encountered with a similar situation .
systemic lupus erythematous ( sle ) is frequently encountered in clinical practice ; a widespread immunological response can involve any organ system , sometimes leading to rare and diagnostically challenging presentations . 
 we describe a 38-year - old female who presented with symmetric numbness and tingling of the hands and feet , and cervical pain . 
 imaging studies were not diagnostic of any serious underlying pathology . 
 the patient developed ascending paresis involving lower extremities and cranial muscles ( dysphagia and facial weakness ) . 
 guillain  barr syndrome ( gbs ) was diagnosed on the basis of electromyography and lumbar puncture showing albuminocytologic dissociation . 
 intravenous immunoglobulins ( ivig ) were administered for 5 days . supported by anti - dsdna antibody , oral ulcers , proteinuria of 0.7 g in 24 h , and neurological manifestation , 
 she was diagnosed with lupus . 
 after completion of ivig , she received pulse - dose corticosteroids and one dose of low - dose cyclophosphamide . her neurological symptoms improved and she had complete neurological recovery several months after her initial presentation . 
 literature search provides evidence of co - occurrence of lupus and gbs occurring mostly later in the course of the disease . however , gbs as initial manifestation of sle is exceedingly rare and less understood . 
 the association of gbs with lupus is important to recognize for rapid initiation of appropriate therapy and for consideration of immunosuppressive therapy which may affect the outcome .