CASE SUMMARY: A 54-year-old Caucasian male was admitted to our tertiary Centre complaining of severe
weight loss, diarrhoea,
abdominal pain, salivary gland swelling, sicca symptoms and
diplopia. On examination, bilateral palpable masses in the projection of major salivary glands, severe protrusion of the left eyeball and
cachexia were noted. The patient was previously diagnosed with
autoimmune thyroiditis and endocrine ophthalmopathy. The magnetic resonance imaging (MRI) of the head revealed enlarged extraocular muscles indicating
orbital myositis. The biopsy from the salivary gland mass indicated
sialadenitis. Abdominal MRI showed signs of
autoimmune pancreatitis, and a serological test revealed the elevated serum
IgG4 concentration. The patient was then diagnosed with
IgG4-RD and successfully treated with
prednisolone. There was a significant clinical, serological and radiological improvement after one month of treatment and no signs of relapse within twenty months. However, it took almost 18 years and the efforts of eight different medical specialists to establish the correct diagnosis.
CONCLUSION: