Case Report
We present a man with 2 years of leg weakness, starting with trouble
rising from bed and red papular rash. He was inpatient for 3 weeks with
improvement on prednisone. After discharge, he required a wheelchair and
had intermittent aches, fever and periorbital edema. His past medical
history included connective tissue disorder, eczema, costochondritis,
arthritis, and sleep apnea.
Examination showed periorbital edema, diminished breath sounds, and
papular rash on his extremities. Neurologically, the only finding was
5-/5 strength of bilateral hip flexors. EMG revealed a length dependent,
axonal neuropathy. Muscle biopsy showed CD3 positive cells and atrophic
angulated fibers, suggestive of neurogenic atrophy. Sural nerve biopsy
showed loss of myelinated fibers, regenerative clusters and thinly
myelinated axons. CT Chest showed bibasilar ground glass opacities.
Abnormal labs were ESR 51, CRP 35.4, CK 1874 (peak 5973), elevated
aldolase and Sjogren’s anti-SSA. A myositis panel revealed positive EJ
autoantibodies. The patient was diagnosed with EJ-antibody
antisynthetase syndrome. He was treated with Cyclosporine, improved
significantly and was lost to follow up.