Case Presentation: 59-year-old female with Celiac disease and Hypertension presented with polyneuropathy and distal extremity weakness, 6 weeks following shingles vaccine. Her symptoms had slowly progressed with increased numbness, paresthesia, weakness and an inability to ambulate over the previous 6 weeks until admission. She was in her normal state of health prior to that. On the admission physical exam: the patient had sensory loss in both hands and feet. She also had bilateral foot and wrist drop. Blood work showed mildly elevated Antinuclear antibody of 1:80 ratio and a positive anti-smooth muscle antibody as well as elevated C-Reactive Protein of 2.8 mg/dl and Erythrocyte Sedimentation Rate of 106 mm/hr. Electromyography of upper and lower extremities showed sensory peripheral axonal polyneuropathy. Nerve and muscle biopsies were negative for evidence of vasculitis, ANCA was negative. Patient had reported history of dry eyes and dry mouth but had a negative SSA and SSB. This prompted a salivary gland biopsy to rule out Sjogren’s disease. The biopsy was noninformative, subsequent orbital MRI showed normal salivary gland. Infectious work-up during hospitalization was unremarkable. CSF studies were normal, and extensive malignancy workup was negative. During her admission, the patient was evaluated by Medicine, Rheumatology, Neurology and hematology/oncology services. Given her largely negative workup, and the history of Recent shingrix vaccine, the suspicion was high for ASIA syndrome. She was treated with pulse dose steroids of 1 g/day for 3 days then she was transitioned to weight-based steroids with gradual taper every 2 weeks. This regimen, along with physical therapy, slowly continued to improve her sensory and motor deficits.
Discussion: ASIA syndrome (Autoimmune/inflammatory Syndrome Induced by Adjuvants) also known as Shoenfeld’s syndrome, is a fairly new entity that encompasses several autoimmune conditions that are induced following the exposure to adjuvants included in vaccines to boast immunogenicity. In genetically susceptible individuals, adjuvant administration can lead to overt autoimmune disease induction as well as autoantibody production. Several major and minor criteria need to be satisfied to diagnose ASIA syndrome such as symptoms of neurologic deficits, myalgias and arthralgias, fatigue and cognitive disturbances, memory loss, and neurological disabilities and absence of other more likely diagnosis. In many cases culprit adjuvant goes unidentified hence a high clinical suspicion is required to make the diagnosis. In our case, the patient did have a clear adjuvant that was introduced to her body 6 weeks prior to her symptoms but this could have been easily missed without proper history.
Conclusions: Since clinical manifestations of ASIA syndrome overlap with various other autoimmune and neurological disorders, the diagnosis can be easily missed specially in the absence of a clearly identified trigger. Increased awareness facilitates early diagnosis and prevents exposure to adjuvants in those subjects with high risk for autoimmunity. This Autoimmune disease should be on top of the differential diagnosis in patients’ hospitalized with neurological deficits following adjuvants along with other neurologic, rheumatologic and paraneoplastic syndromes that need to be ruled out prior to making the final diagnosis. Hence, early suspicion and treatment can prevent prolonging and worsening of a disease with debilitating symptoms.