Immunological disturbances may result in altered immunoglobulin concentrations and kappa/lainbda light chain (k/X) ratios. We measured the k/X ratios of total serum immunoglobulins and of polyclonal IgG, A, and M separately as well as concentrations of these immunoglobulins in fourteen patients with juve nile onset mixed connective tissue disease. When comparing the patient group with a reference group the mean serum IgG and IgA concentrations were respectively 2.98 G/L (p=0.0012) and 0.79 G/L (p=0.0114) higher in the group of patients with juvenile onset mixed connective tissue disease. The mean IgM concentration was 0,39 G/L (p = 0.0002) lower. The mean k/X ratios of total serum immunoglobulins, serum IgG, and serum IgA were respectively 0.20 (p=0.0226), 0.28 (p~0.0016) and 0.10 (p=0.0732), higher in the group of patients with mixed connective tissue disease as compared with the reference group. Mean serum IgM k/X ratio, however, was 0.21 (p = 0.0046) lower. The alterations of the serum immunoglobulin concentrations and of the k/X ratios reflect immunological disturbances in patients with juvenile onset mixed connec tive tissue disease. The increased concentration of serum IgG and raised IgG k/X ratio and decreased concentration of serum IgM with decreased IgM K/X ratio in dicate that the synthesis of K-bearing immunoglobulins mainly is affected.