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2013 ; 2013
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Immunoglobulin G4-related disease with scant tissue IgG4
#MMPMID23749829
Makiishi T
; Shirase T
; Hieda N
; Maeda S
BMJ Case Rep
2013[Jun]; 2013
(ä): ä PMID23749829
show ga
A 56-year-old man was admitted to our hospital for renal dysfunction and
symmetrical swelling of submandibular glands. Laboratory and imaging findings
were consistent with immunoglobulin G4-related disease (IgG4RD). Histological
findings of the submandibular gland and the kidney were also consistent with
IgG4RD. However, the patient did not have elevated serum or tissue IgG4 levels.
Oral prednisolone therapy, initially 50 mg/day and gradually tapered over 12
months, improved his laboratory abnormalities and the swelling of his affected
organs. These findings prompted our final diagnosis of IgG4RD. IgG4RD is a newly
recognised disease with an unknown aetiology. This case suggests that IgG4
antibodies do not play a primary role in the aetiology of IgG4RD. Furthermore,
clinicians should not exclude the diagnosis of IgG4RD in patients lacking
elevated IgG4 levels in their affected tissues, particularly if they have other
features of IgG4RD. Steroid therapy should be considered for such patients.