18F-FDG PET/CT Manifestations of IgG4-related Disease

Abstract

Immunoglobulin G4-related disease (IgG4-RD) was not recognised as a systemic condition until 2003, when extra pancreatic manifestations were identified in patients with autoimmune pancreatitis. Since then, IgG4-RD has been described to involve virtually every organ system. It is highly responsive to immunosuppressants but can have detrimental effects if left untreated. Early recognition of the disease is, therefore, critical. The diagnosis of IgG4-RD is frequently challenging owing to its non-specific clinical manifestations, indolent nature and broad differential diagnoses. Although histopathological examination remains the cornerstone of diagnosis, imaging plays an important role in establishing extent of disease and identifying areas suitable for biopsy. 18F-Fluorodeoxyglucose (18F-FDG) positron emission tomography/computed tomography (PET/CT) has been demonstrated to be useful in assessing organ involvement, guiding biopsy and monitoring disease response. The 18F-FDG PET/CT scan is highly sensitive and able to evaluate multiorgan involvement in a single examination, a key advantage over conventional imaging modalities. A potential pitfall is its low specificity. As such, detailed knowledge of the imaging findings in IgG4-related disease is required to avoid misdiagnosis. This pictorial review aims to depict the diverse spectrum of imaging findings of IgG4-RD and the key imaging features to distinguish it from other important differential diagnoses.