Published online Aug 6, 2020. doi: 10.12998/wjcc.v8.i15.3267
Peer-review started: April 15, 2020
First decision: April 26, 2020
Revised: May 7, 2020
Accepted: July 15, 2020
Article in press: July 15, 2020
Published online: August 6, 2020
Immunoglobulin G4-related disease (IgG4-RD) is a multisystemic mass forming immune-mediated disease that affects almost every organ and is a diagnostic challenge for every clinician. There is a lack of adequate epidemiological data worldwide, and evidence-based treatment recommendations are not yet established. We report the first case of IgG4-RD from Lithuania and the Baltic Sea region presented with thyroiditis, orbital myositis, orbitopathy, uveitis, scleritis, sialadenitis, autoimmune pancreatitis and prostatitis.
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.
A comprehensive approach to the patient is essential to improving the recognition of rare immune system conditions, such as IgG4-RD.
Core tip: Immunoglobulin G4-related disease (IgG4-RD) is a rare multisystemic immune-mediated disease that affects almost every organ and mimics various malignant, infectious or other inflammatory conditions. Therefore, the correct diagnosis and proper treatment are often delayed. We present the first clinical case of multi-organ IgG4-RD from Lithuania and the Baltic Sea region that reveals the importance of a comprehensive approach to the patient in order to achieve effective recognition of the rare immune system malfunction conditions such as IgG4-RD.