Immunoglobulin abnormalities in patients with myotonic dystrophy

Abstract

In order to further investigate immunoglobulin (Ig) abnormalities in patients with myotonic dystrophy (MyD), we examined 65 patients with MyD for their serum concentration of IgG in relation to their immunological functions as well as other clinical and laboratory findings. Turnover of IgG was also examined. We found significant and selective reduction of serum IgG in patients with MyD. The serum concentration of IgG in patients with MyD had a significant negative correlation with duration of illness, which suggested that serum IgG levels in MyD decreased constantly throughout the disease course. Peripheral blood lymphocyte subpopulations, including functional T cell subsets, in vitro lymphocyte proliferation, and in vitro IgG production were normal. The plasma cell population in bone marrow was also normal. The IgG turnover study using 125I-labelled IgG as a tracer revealed an increased extravascular IgG pool, and an increased capillary permeability of intravascular IgG into the extravascular compartment, in patients with MyD. These observations suggested that the Ig abnormalities in patients with MyD were not due to a broader immunological derangements as previously postulated by other authors. The abnormal distribution of IgG is a possible factor leading to reduced serum concentration of IgG in patients with MyD.

Strong association between myotonic dystrophy type 2 and autoimmune diseases

“…Cases include autoimmune mediated hyperthyroidism, Addison’s disease, Hashimoto’s thyroiditis, thrombocytopenic idiopathic purpura and myasthenia gravis. Also, hypogammaglobulinaemia and increased frequency of autoantibody formation has been described in DM1 patients 14 15 16 17 18 19 20. To date, no cohort study has confirmed these sporadic observations and no mechanism of action has been elucidated conclusively.…”

Massive abscess with prolonged respiratory failure due to newly diagnosed myotonic dystrophy

“…One report described significant, selective reduction of serum concentrations of immunoglobulin (Ig)G in MD1 patients. [8] In this case, the normal serum IgG level of 1511 mg/dL might not have been associated with MD1.…”

Enhanced serum immunoglobulin G clearance in myotonic dystrophy-associated hypogammaglobulinemia: a case series and review of the literature

“…Other work has found a longer duration of DM1 is associated with lower serum IgG levels, both in cross-sectional analysis and in longitudinal study of individual patients with DM1 over time [7]. Suzumura et al found no differences in broad lymphocyte populations or mitogen-driven proliferation [7], nor any differences in in vitro production of IgG between patients with DM1 and healthy controls. Bone marrow cellularity and plasma cell counts were also comparable [7].…”

“…Suzumura et al found no differences in broad lymphocyte populations or mitogen-driven proliferation [7], nor any differences in in vitro production of IgG between patients with DM1 and healthy controls. Bone marrow cellularity and plasma cell counts were also comparable [7]. Injection of radiolabeled 125 -I-IgG led to more rapid clearance of the 125 -I-IgG from day 2–4 in six patients with DM1 compared with seven healthy controls.…”

“…Injection of radiolabeled 125 -I-IgG led to more rapid clearance of the 125 -I-IgG from day 2–4 in six patients with DM1 compared with seven healthy controls. There was no difference in the fractional catabolic rate or t 1/2 over the remaining 2 weeks of the study [4, 7].…”