Background IgG4-related disease (IgG4-RD) is an increasingly recognised immune-mediated fibro-inflammatory condition, most commonly affecting the pancreas.
Objectives The aim of our study was to determine the incidence, organ involvement and outcome of IgG4-RD in the Maltese population.
Methods The case notes of all patients who had their IgG4 level checked between 2008 and 2015 at Mater Dei Hospital were reviewed. Patients who had their IgG4 level checked inappropriately were excluded. Patients were then stratified according to the “Comprehensive diagnostic criteria for IgG4-related disease” .
Results 25 patients were included in this retrospective, observational study. 60% (n=15) of patients were males and mean age was 70 years (SD±64.8). The mean IgG4 level was 0.7159 g/L (SD±0.851) (normal range 0.052–1.250g/L).
Organs involved included: pancreas (n=15), biliary tree (n=3), salivary glands (n=1), lymph nodes (n=8), lungs (n=4), thyroid gland (n=2), bone (n=1), heart (n=1), sinuses (n=2). 36% (9 patients) had involvement of more than one organ.
Blood tests at time of diagnosis demonstrated a raised mean ESR (65.7mm1st Hr; SD±36.1) and a raised mean CRP (143 mg/L SD± 112). White cell count was mostly normal (mean 11.7x109/L SD±4.91).
92% of patients (n=23) had organ swelling on radiological testing. 32% (n=8) of patients had raised IgG4 levels. A histological diagnosis was obtained in 8% (n=2) of patients presenting with cervical lymphadenopathy and sinusitis respectively. According to the “Comprehensive diagnostic criteria for IgG4-RD”1, the diagnosis was definite in 4% (n=1), possible in 28% (n=7) and probable in 4% (n=1) of patients. 64% (n=16) only had organ swelling on imaging studies, satisfying 1 criterion.
One patient was diagnosed with malignancy during the course of disease. Patients with hepatobiliary and pancreatic involvement underwent ERCP and stenting. Patients who developed pancreatic pseudocyst as a complication of pancreatitis underwent drainage. One patient (4%) developed pancreatic insufficiency and 32% (n=8) experienced recurrent pancreatitis requiring hospital admission. Four patients (16%) passed away.
Conclusions In concordance with current literature, our study has shown that IgG4-RD in the Maltese population is a rare disorder affecting mainly elderly males. The commonest organs involved were the pancreas and lymph nodes. The inflammatory markers ESR and CRP were always raised, thus acting as possible biomarkers for monitoring of disease activity.
Umehara H, Okazaki K, Masaki Y, Kawano M, Yamamoto M, Saeki T et al. Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011. Mod Rheumatol. 2012 Feb;22(1):21–30.
Disclosure of Interest None declared