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Presenting IgG4-related disease: an emerging entity in Pakistan


 Background

IgG4 related disease (IgG4-RD) is a spectrum of immune mediated disorder involving various organs of the body. In this study the clinical spectrum of possible IgG4-RD was explored.
Methods

Subjects tested for serum IgG4 and all biopsies of subjects with suspected IgG4-RD received at the clinical laboratory of a tertiary care hospital from April 2015 to December 2019 were included. Medical charts of subjects registered were reviewed and telephonic interviews were conducted. Subjects were divided into two groups: group I had biochemical evidence of IgG4-RD while group II had histopathological evidence of IgG4-RD. “Comprehensive diagnostic criteria for IgG4-RD, 2011” was used for labeling patients as possible, probable and definitive IgG4-RD.
Results

A total of 177 study subjects were recruited in the current study. Group I included 10 children and 105 adults whereas group II had 5 children and 57 adults. Out of the total 177 subjects definitive, probable and possible IgG4-RD were seen in (n = 2, 1.1%), (n = 61, 34.4%) and (n = 114, 64.4) subjects respectively. The commonest organs involved in all the study subjects were pancreas (57.6%), submandibular gland (12.4%) and liver (6.2%).
Conclusion

The clinical feature of IgG4-RD include single or multiple organ involvement with pancreas being the most frequently affected organ in the current population. Amalgamation of clinical, biochemical and histopathological findings are essential for the IgG4-RD, although none is pathognomonic by itself.