IgG4 –related disease is a new evolving immune-mediated disease
that was recently discovered, with  a
wide range of clinical features that involves multiple systems with new
clinical presentations being discovered every day, diagnosis depends on
clinical and characteristic histopathology findings  upon biopsy that shows dense lymphoplasmacytic
infiltration of IgG 4 plasma cells associated with storiform fibrosis ,since it
can cause tumor like masses ,it could mimic malignancies as lymphoma.

    We report a 58 years old
Malaysian gentleman with a background history of hypertension, chronic kidney disease,
who was in his usual state of health till 2 years back when he started to
develop slowly progressive painless cervical lymphadenopathy ,parotid gland enlargement,
bilateral orbital swelling and fatigue, ,patient  denied any fever, night sweats or weight loss,
Seen by many specialties through these 2 years with multiple emergency department
visits with no  good response to
antibiotics trial therapy and no conclusive diagnosis was made.

Ultrasound neck confirmed the presence of multiple submaxillary, salivary
and parotid gland enlargement, fine needle aspiration of left maxillary lymph node
was inconclusive, follow up ultrasound showed increase in the size of the
affected lymph nodes.

 Infections as EBV,CMV ,HIV
and  tuberculosis, ruled out,Sjogrens
syndrome versus lymphoma  was suspected
at that stage but patient had negative ANA,ANTI RO/LA .

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after one year of extensive investigations patient was admitted with painless
obstructive jaundice,pruririts and decreased appetited had a cholestatic
pattern liver impairment, with negative hepatitis serology and negative
screening for autoimmune hepatitis,MRCP done that showed a  Pancreatic head mass lesion causing obstruction
of the pancreatic  and bile ducts, Enlarged
peripancreatic lymph nodes, Multiple parenchymal lesions of both kidneys, Circumferential
thickening and diffusion restriction of the wall of a limited part of the aorta
inferior to the renal arteries, impressive of focal aortitis The possibilities
of lymphoma  versus IgG4-related disease were
considered as a highly probable diagnosis. Subsequent
blood  studies were performed demonstrating
elevated IgG-4 (1,980  mg/dL), EUS- FNA of pancreatic head mass ruled out
pancreatic head malignancy ,so right groin excisional lymph node biopsy was
done `that showed Increased nodal IgG4 plasma
cells consistent with IgG4-related disease (more
than 30/hpf).

Flow cytometry was not suggestive of lymphoma.

Diagnosis of IgG4 –related disease was made after discussion in
a multidisciplinary team and patient was started on pulse steroid therapy for 3
days followed by oral prednisolone 1mg/kg.

Follow up visit revealed marked improvement in the patient’s symptoms,
normalization of liver function tests and decrease in IgG 4 level from 1,980 to
400 mg/dl,so steroids were tapered down by 5mg every week and azathioprine


It is important to note that IgG 4-related  disease is a mimicker of malignancy, This case
highlights the importance of keeping high index of suspicion for IgG 4-related as
delay in diagnosis as what happened with this patient could lead to irreversible
fibrosis and organ damage,clinicians should be more aware of this new disease
entity ,putting into consideration that it is
treatment responsive,so early diagnosis could lead to decrease morbidity and



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