Abstract Details

Title An overlap syndrome: Myositis specific and Myositis associated antibodies in dermatomyositis

Topic Autoimmune Neurology

Presentation(s) Autoimmune Neurology Posters (7:00 AM-5:00 PM)

Poster/Presentation
Number 049

Objective To report a rare case of paraneoplastic dermatomyositis.

Background Dermatomyositis is a rare multi-system inflammatory disorder that presents with proximal muscle weakness and characteristic skin findings. Cause for dermatomyositis is thought to be multifactorial involving immunologic and environmental factors making each case unique and challenging to diagnose.

Design/Methods A 42-year-old woman initially presented for progressive weakness over a 10-month period of time. Weakness developed with left then right upper extremity weakness followed by difficulty climbing stairs. On examination, patient was awake, alert, cognitively intact with no cranial nerve deficits. Muscle strength testing notable for proximal muscle weakness in upper and lower extremities with strength intact distally. She had no sensory abnormalities in all modalities. Reflexes were hypoactive diffusely in upper extremities and normal in lower extremities. Plantar reflexes were flexor bilaterally.

Results Electrodiagnostic testing revealed evidence of irritable myopathy without evidence of large fiber polyneuropathy or neuromuscular junctional disease. Lab work showed a creatinine kinase (CK) 2126 U/L, aldolase 44.4 U/L, elevated transaminases, a high RF titer and positive cyclic citrullinated peptide level. Myositis antibody panel significant for elevated TIF-1y and MI2-α antibodies. Jo1 antibody and SRP antibodies both returned negative. Skeletal muscle biopsy of the left deltoid showed inflammatory myopathy with histologic features of dermatomyositis. CT of the thorax revealed a thymic cyst that was confirmed by thoracoscopic biopsy. Patient was treated with high dose steroids, symptoms resolved and CK level trended down.

Conclusions Dermatomyositis is linked to several autoantibodies with each one carrying specific clinical features and prognosis. We present a case of dermatomyositis associated with two antibodies that have varying features with TIF-1y carrying concern for underlying malignancy. Thymic cyst are also rarely associated with dermatomyositis. It is therefore critical to test for these antibodies and consider malignancy in all patients diagnosed with dermatomyositis.

Authors/Disclosures
Sarah Germaine, DO (Legacy Health) PRESENTER	Dr. Germaine has nothing to disclose.
Hamza Coban, MD (UConn Health Neurology)	Dr. Coban has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for EMD Serono.
Ian L. Dimaandal, MD	Dr. Dimaandal has nothing to disclose.
Annie S. Daniel, MBBS (Department of Neurology , Hospital for Special Care , New Britain , Ct)	Dr. Daniel has nothing to disclose.

��ɫ��

��ɫ��