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.