The clinical materials of the patient with dermatomyositis complicated with Sjogren’s syndrome with coexistence of anti-Jo-1 and anti-SRP antibodies were collected，all kinds of information， including gender， age of onset， clinical manifestations， laboratory data and follow-up information were recorded， and the clinical data were retrospectively analyzed and the related literature were reviewed.

The female patient aged 52 years old was admitted to hospital because of “interstitial pneumonia” due to intermittent cough， expectoration， shortness of breath and general weakness for 2 months. The patient’s hands had mild “mechanic’s hands” like changes， and the limb muscle strength was grade 3. The laboratory examination showed that the creatine kinase level was increased significantly， and anti-Jo-1 antibody and anti-SRP antibody were positive； the imaging results showed the pulmonary interstitial changes；the muscle pathological results showed atrophy， degeneration and inflammatory cell infiltration， and the labial gland pathology showed xerostomia； the clinical diagnosis was DM complicated with SS. After treatment with glucocorticoid combined with mycophenolate mofetil， the condition was improved and the patient was discharged from the hospital. The patient was followed up for 5 months， the creatine kinase level was completely decreased to normal， and the clinical symptoms of DM were significantly improved.

Double myositis-specific autoantibodies positive DM complicated with SS has a low incidence， and its clinical manifestations are atypical and easily involve the lung； the clinical manifestations， pathological examination and muscle zymography test are the mainstays for the diagnosis of this disease， and in this patient glucocorticoids combined with immunosuppressive regimens are effective. The patients should be followed up regularly during treatment process.