*Corresponding author: Makki H Fayadh, Advanced center for day care sur-
gery, Abu Dhabi, UAE, Tel: +971 6227700; E-mail: makkihf@yahoo.com
Citation: Fayadh MH, Awadh S (2019) High IgG4 in a Patient with, Diabetes
Mellitus, Ulcerative Colitis, Liver Cirrhosis with Papillary Thyroid Cancer. J Clin
Stud Med Case Rep 6: 072.
Received: August 07, 2019; Accepted: September 16, 2019; Published: Sep-
tember 23, 2019
Copyright: © 2019 Fayadh MH and Awadh S. This is an open-access article
distributed under the terms of the Creative Commons Attribution License, which
permits unrestricted use, distribution, and reproduction in any medium, provided
the original author and source are credited.
Background
IgG4-Related Disease (IgG4 RD) is a clinical entity including a
wide variety of diseases, formerly diagnosed as Mikulicz’s disease,
Autoimmune Pancreatitis (AIP), interstitial nephritis, prostatitis an-
dretroperitoneal fbrosis [1-4].
Characteristics common to all forms of IgG4-RD include elevat-
ed serum IgG4 concentration and tissue infltration by IgG4-positive
plasma cells, accompanied by tissue fbrosis and sclerosis.
As increased serum concentrations of IgG4 have been observed in
several diseases with aberrant immunological condition unrelated to
IgG4-RD, such as malignant tumors, autoimmune diseases especially
rheumatoid arthritis and allergic diseases, increased IgG4 concentra-
tion is not a specifc marker for IgG4-RD [2].
Since this complex multisystem disease represented a single
pathogenetic disorder manifesting in a variety of target organs, the
diagnosis of IgG4-RD is largely based on biopsy results showing en-
hanced infltration by IgG4-positive plasma cells, storiform fbrosis,
obliterative phlebitis and moderate eosinophilia, all of which are fre-
quently observed in the affected tissues of these patients [1-8].
A high number of IgG4-positive plasma cells in tissue is a hall-
mark of IgG4-RD, even when serum IgG4 concentrations are below
the cut-off level [4]. IgG4-RD tends to be both under diagnosed and
over diagnosed [1-8].
Each criterion contains organ-specifc clinical symptom and char-
acteristic radiological fndings of IgG4-RD, even with steroidal trial
in some.
We reportedour initial experience in the United Arab Emirate on
high IGG4 associated with pachymenigitis, infammatory bowel
diseases, celiac disease, thyroiditis, retroperitoneal fbrosis, auto im-
mune pancreatitis & sclerosing cholangitis [6].
Case Study
A Male 55 years old with BMI 25, with strong family history of
diabetes mellitus & myocardial infarctions in brothers and father. His
diabetes mellitus started 1997 treated with oral hypoglycemic with
poor control, insulinstarted 2005, and developed left sided ulcerative-
colitis 2015 treated with mesalazine 3.2 g, azathioprine 200mg and
courses of budesonide 9 mg.
Frequent relapses of colitis with positive ameba and another re-
lapse with positive clostridium diffcile infection treated with metro-
nidazole successfully (Figures 1&2).
January 2016 because of the colitis frequent relapses, adalimumab
started but 6 months later developed recurrent deep vein thrombosis
lower limbs and discovered to have factor V Leiden mutation, treated
with oral anti-coagulants.
Hematuria developed, ultrasound showed small bilateral kidney
stones with large prostate. 2018 because of darkening of the skin and
Fayadh MH and Awadh S, J Clin Stud Med Case Rep 2019, 6: 72
DOI: 10.24966/CSMC-8801/100072
HSOA Journal of
Clinical Studies and Medical Case Reports
Case Report
Makki H Fayadh* and Salim Awadh
Advanced Center for Day Care Surgery, Abu Dhabi, UAE
High IgG4 in a Patient with,
Diabetes Mellitus, Ulcerative
Colitis, Liver Cirrhosis with
Papillary Thyroid Cancer
Abstract
Objectives: As increased serum concentrations of IgG4 have been
observed in several diseases with aberrant immunological condition
such as malignant tumors, autoimmune diseases especially rheuma-
toid arthritis and allergic.
We describe a patient with high IgG4, liver cirrhosis, the ulcer-
ative colitis, diabetes mellitus, prostatitis, venous thrombosis and
thyroid cancer.The relationship between elevated IgG4 and these
diseases is discussed.
Case study: A 56 years old male with diabetes mellitus on insulin,
ulcerative colitis on azathioprine & adalimumab developed deep skin
pigmentation and liver enzymes elevation with portal hypertension
and liver cirrhosis followed by portal vein thrombosis with persistent-
ly elevated IgG4, azathioprine & adalimumab were stopped. Goli-
mumab started but after 3 infusions,papillary thyroid cancer devel-
oped, total thyroidectomy followed by radio iodine.
Ulcerativecolitis was treated with vedolizumab with good re-
sponse. Liver disease progressed with bleeding varices and portal
vein thrombosis with factor Leiden V mutation. IgG4 was high which
may high direct or indirect relation to liver cirrhosis, diabetes melli-
tus and thyroid cancer. The safety of immunosuppressive & biologic
agents in patients with high IgG4 need to be reviewed. Awareness
is needed about the relationship between high IgG4 and multiple
complex diseases is needed
Conclusion: A patient with elevated IgG4, liver cirrhosis, diabetes
mellitus, and ulcerative colitis developed skin pigmentation and pap-
illary thyroid cancer, the role of IgG4 is questioned.
Keywards: Diabetes mellitus; Diseases; IgG4; Liver cirrhosis; Thy-
roid cancer