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Introduction of an Integrated Pathology Image Management, Artificial Intelligence, and Reporting System

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IgG4-Related Disease: A Reminder for Practicing Pathologists.

Steven C Weindorf¹, John Karl Frederiksen¹

¹From the Department of Pathology, University of Michigan, Ann Arbor. Dr Frederiksen is now with the Department of Pathology, Jackson Memorial Hospital, Miami, Florida.

Archives of Pathology & Laboratory Medicine

|October 27, 2017

Summary

Immunoglobulin G4-related disease (IgG4-RD) is a systemic autoimmune disorder presenting as masses in nearly every organ. Pathologists are crucial for diagnosing IgG4-RD, preventing severe complications.

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Area of Science:

Pathology
Immunology
Rheumatology

Background:

Immunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory condition characterized by IgG4-positive plasma cell infiltrates.
Initially identified as autoimmune pancreatitis, IgG4-RD is now recognized across multiple organ systems.
Diverse and nonspecific clinical signs often delay diagnosis, leading to misdiagnosis as malignancy.

Purpose of the Study:

To increase pathologists' awareness of IgG4-related disease.
To review the epidemiology, clinical features, and histopathology of IgG4-RD.
To aid in the early diagnosis and management of IgG4-RD.

Main Methods:

Review of existing literature on IgG4-related disease.
Analysis of histopathological features across various organs.

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Synthesis of clinical manifestations and epidemiological data.

Main Results:

IgG4-RD presents with tumefactive masses and lymphoplasmacytic infiltrates.
Histopathology reveals abundant IgG4+ plasma cells.
Early recognition is vital for preventing irreversible comorbidities.

Conclusions:

Pathologists play a pivotal role in diagnosing IgG4-RD.
Familiarity with IgG4-RD histopathology is essential.
Timely diagnosis facilitates treatment and improves patient outcomes.