Isolated Mass-Forming IgG4-Related Cholangitis as an Initial Clinical Presentation of Systemic IgG4-Related Disease

Abstract

IgG4-related disease (IgG4-RD) may involve multiple organs. Although it usually presents as diffuse organ involvement, localized mass-forming lesions have been occasionally encountered in pancreas. However, the same pattern has been seldom reported in biliary tract. A 61-year-old male showed a hilar bile duct mass with multiple enlarged lymph nodes in imaging studies and he underwent trisectionectomy under impression of cholangiocarcinoma. Gross examination revealed a mass-like lesion around hilar bile duct. Histopathologically, dense lymphoplasmacytic infiltration and storiform fibrosis were identified without evidence of malignancy. Immunohistochemical stain demonstrated rich IgG4-positive plasma cell infiltration. Follow-up imaging studies disclosed multiple enlarged lymph nodes with involvement of pancreas and perisplenic soft tissue. The lesions have been significantly reduced after steroid treatment, which suggests multi-organ involvement of systemic IgG4-RD. Here, we report an unusual localized mass-forming IgG4-related cholangitis as an initial presentation of IgG4-RD, which was biliary manifestation of systemic IgG4-related autoimmune disease.

Recent advances in understanding and managing IgG4-related disease

“…Similarly, Kim et al . presented a case of a patient in whom there was concern for cholangiocarcinoma and who underwent a left trisectionectomy but was ultimately diagnosed with IgG4-related disease 20 . IgG4-related disease can also be initially mistaken for lymphoma, as both diseases can present with lymph node swelling as an initial symptom 21 , 22 .…”

An overview of the diagnosis and management of immunoglobulin G4–related disease

“…38 The rarity, diverse presentation and lack of a gold standard test often result in delayed diagnosis, usually after investigation for malignant growths has occurred. 39,40 Thus, an understanding of the clinical, radiologic, laboratory tests (in particular serum concentrations of IgG4) and a focused histopathology examination are required to ensure that IgG4-related disease has been considered in the differential diagnosis. 41,42 Organ-specific guidelines, such as the HISORt (histology, imaging, serology, other organ involvement, response to steroid therapy) criteria for IgG4-related sclerosing cholangitis, facilitate this multimodal diagnostic approach but may blind specialists to clinical manifestations outside their area of interest.…”

Pathologic interpretation of endoscopic ultrasound–guided fine needle aspiration cytology/biopsy for pancreatic lesions

“…However, if the patient history or radiologic findings are vague, it may be difficult to differentiate conventional chronic pancreatitis from autoimmune pancreatitis or cholangitis. Especially when presenting as a local mass-forming lesion, it is very difficult to suspect autoimmune disease if EUS-FNA cytology/biopsy provides only surface mucosa [16]. It should be emphasized that the pathologic diagnosis of autoimmune disease should be made after excluding malignancy in the differential diagnosis.…”