The association of TIDM and SLE is rather rare, but in the event of occurrence can complicate the course of the other. Genetic predisposition, autoimmunity and viral infections are the main etiopathological factors implicated in the pathogenesis of type 1 diabetes mellitus and an association between TIDM and Celiac disease (CD) has a high incidence. This is probably due the human leukocyte antigen (HLA) DR3- DQ2 and DR4-DQ8 that is common to both the diseases.
A 9 year-old girl, was diagnosed with TIDM with Hba1c of 9.3, she was started on Insulin degludec and glulisine and was maintaining normal sugars. Her IgA anti-tissue transglutaminase antibodies (AtTG) was positive 101 RU/ML, which was followed up with a duodenal biopsy showing villus atrophy and increased intra-epithelial cells, confirming the diagnosis of CD and was advised a gluten free diet. Her anti thyroid peroxidase (TPO) was > 573 IU/ml (positive) but her TSH and Free T4 were within normal limits and hence were regularly followed for her thyroid functions regularly.
After three years,she developed high-grade fever and macular rashes on her face,palms and sole.Her blood and urine cultures were sterile.Anti-nuclear antibodies (ANA) and anti double stranded DNA (ds DNA) sent for evaluation of fever,were strongly positive. The thyroid function tests, anti TPO and anti thyroglobulin were repeated and were strongly positive with low free T4 and high TSH, she was started on tab thyroxine 25ug once a day.
The child came back a month later with facial swelling, with a diagnosis of SLE, 24 hour urinary protein and urine protein creatinine ratio was sent. Her blood pressure was 100/60 mm of Hg. The results were very high (in table), following which a kidney biopsy was planned. The biopsy reported focal proliferative lupus nephritis class III (ISN/RPS classification 2004). She was given intravenous methyl prednisolone pulse for three days and then started on oral mycophenolate and oral corticosteroids.
Genetically predisposed patients are known to have associated autoimmune conditions manifesting together.Although reports say 30% of patients with SLE may develop two or more autoimmune disorders, type 1 diabetes mellitus is a very rare association.
There needs to be an awareness that 2 or more auto immune conditions can exist in the same patient or even develop progressively. This would be the first case to report the tetrad of TIDM, CD, autoimmune hypothyroidism and SLE in the same child.
19 - 21 Sep 2019
European Society for Paediatric Endocrinology