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Monoclonal gammopathy (MG) is common in autoimmune diseases (AID), but its progression to hematological neoplasm (HN) and the predictors for the progression are unclear. Patients diagnosed with AID and MG in our hospital from January 2010 to June 2017 were reviewed and followed. Cox proportional hazard regression analysis was applied. Of 160 patients with AID and MG, the most common AID was primary Sjӧgren's syndrome (37, 23.1%). Thirty-nine (24.4%) patients developed HN during follow-up (median: 3.7 years, IQR: 0.3–5.5 years). The cumulative probability of HN progression was 21.8% at one year and 29.3% at six years after the finding of MG. High levels of monoclonal protein (> 14.35% of total serum protein) (HR 11.71, 95%CI: 5.37–25.54), significant weight loss (HR 6.24, 95%CI: 2.87–13.59), and reduction of other types of immunoglobulins (HR 3.02, 95%CI: 1.40–6.48) are independent risk indicators for HN whose presence warrants vigorous follow-up and monitoring. • Monoclonal gammopathy (MG) in autoimmune diseases (AID) denotes the risk of hematological neoplasms. • Around one-fifth of MG in AID patients develop hematological neoplasms in the first year of MG diagnosis. • M protein level and other immunoglobulins type reductions are independent predictors for HN progression. [ABSTRACT FROM AUTHOR]