Simultaneous peripubertal onset of multireactive autoimmune diseases with an unusual long-lasting remission of type 1 diabetes mellitus.
Although it is well known that patients with type 1 diabetes mellitus are susceptible to other autoimmune diseases, the simultaneous occurrence of clustered distinct autoimmune diseases is uncommon. We report a 16-year-old girl, previously diagnosed as having coeliac disease and IgA deficiency, who at 13 years of age developed a clustering of distinct autoimmune diseases, including type 1 diabetes mellitus, rheumatoid arthritis (RA) and euthyroid autoimmune thyroiditis, eventually resulting in a simultaneous long-term remission. The clinical picture was associated with a functional immunodeficiency characterized by a defect in proliferative responses to T cell predominant mitogens and a normal response to the B cell predominant mitogen. In addition, the T cell activation markers HLA-DR, IL-2 receptor and transferrin receptor) were not upregulated. The clinical course of this immunodeficiency paralleled the outcome of the autoimmune diseases. After the abrupt onset, spontaneous clinical remission of both diabetes mellitus and RA was observed. Insulin was first reduced in dose and then discontinued completely at 15 months, in the presence of normal C peptide secretion and normal metabolic control (HbA1c 5.8%). Anti-glutamate decarboxylase (GAD65) and anti-IA-2 antibodies remained persistently high. During the remission phase a normalization of the functional immune defect was observed. The gradual resolution of the multisystemic diseases as well as the normalization of immune function in our patient is unusual. This case may be of considerable value in furthering our knowledge of the immunological mechanisms implicated in these rare multireactive syndromes.
Valerio
G
G
Department of Paediatrics, Federico II University, Naples, Italy.
Franzese A
Iovino
A F
AF
Tanga
M
M
Alessio
M
M
Pignata
C
C
D000293:Adolescent; D001172:Arthritis, Rheumatoid / Q000150:complications* / Q000276:immunology; D001323:Autoantibodies / Q000097:blood; D002096:C-Peptide / Q000097:blood; D002446:Celiac Disease / Q000150:complications* / Q000276:immunology; D003922:Diabetes Mellitus, Type 1 / Q000150:complications* / Q000276:immunology; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D005951:Glucose Tolerance Test; D006801:Humans; D017098:IgA Deficiency / Q000150:complications* / Q000276:immunology; D008934:Mitogens / Q000502:physiology; D012075:Remission, Spontaneous; D013967:Thyroiditis, Autoimmune / Q000150:complications* / Q000276:immunology; D015854:Up-Regulation
D001323:Autoantibodies; D002096:C-Peptide; D008934:Mitogens