A case of IgG4-related hepatic inflammatory pseudotumor replaced by an abscess after steroid treatment

Abstract

BackgroundHepatic inflammatory pseudotumor (IPT) is a rare disease which often mimics a malignant tumor and is therefore often misdiagnosed and surgically resected. Recently, a concept of IgG4-related diseases (IgG4-RD) has been proposed that is becoming widely recognized and includes IgG4-related hepatic IPT. Corticosteroids are widely accepted as the standard treatment.Case presentationA 72-year-old Japanese man, who had been followed for ten years after surgery and chemotherapy for treatment of hilar and lower bile duct cancers, developed intermittent fever and abdominal pain and visited this hospital. Blood examinations revealed an inflammatory reaction, worsened glucose intolerance, and an increased level of serum IgG4 (137 mg/dL). Computed tomography (CT) revealed a 5 cm-sized mass in hepatic segment 7. Because of his cancer history, not only was a benign mass suspected, but there was also the possibility of a recurrent biliary malignancy. Liver biopsy was performed and the histology met the criteria for IgG4-related IPT. Corticosteroid therapy was initiated and his symptoms quickly resolved. However, two months later, a repeat CT demonstrated that the hepatic mass had been replaced by an abscess. The abscess was initially refractory, despite tapering corticosteroid treatment, controlling diabetes by intensive insulin therapy, administration of antibiotics, and percutaneous abscess drainage. Finally, after six months, the condition resolved.ConclusionThe diagnosis of hepatic IPT is sometimes difficult. To differentiate it from a malignant tumor, histological examination is necessary. Although corticosteroids are recognized as the standard therapy, unexpected and critical complications can develop in cases of IgG4-related hepatic IPT.

Steroid Therapy and Steroid Response in Autoimmune Pancreatitis

“…By contrast, some of our cases of IgG4-related diseases demonstrated adverse events after steroid treatment, namely, a case of IgG4-related mesenteric pseudotumor causing intestinal perforation due to the excessive steroid response [115] and a case of IgG4-related hepatic pseudotumor developing an abscess, probably due to the combined hyperglycemic and ischemic conditions [120]. Clinicians also need to stay aware of any adverse events that might possibly occur after steroid therapy.…”

“…with type 1 AIP hypophysitis [107] skull and vertebral lesions [108][109][110] dacryoadenitis [103] neurosensory hearing loss [4] orbital lesions [109] sialoadenitis [103] chronic thyroiditis [4] meningitis [111] hilar lymphadenopathy [88] inflammatory pseudotumors esophagitis [112] interstitial pneumonitis [104] breast [113] sclerosing mesenteritis [114,115] sclerosing cholangitis [21,35,54] lung [116,117] Rosai-Dorfman disease [118,119] retroperitoneal fibrosis [105] liver [35,36,104,120] gastroenteritis [121] tubulointerstitial nephritis [106] gastric ulcer [4] vasculitis [122] swelling of the papilla of Vater [123] neuropathy [124] with type 2 AIP hepatopathy [35] myopathy [125] inflammatory bowel diseases [2,4,12,14] (ulcerative colitis and Crohn's disease)…”

An Enigmatic Liver Mass in a Child

“…18 Many case reports have suggested variable success with corticosteroid use. Shibata et al 19 had reported a similar case of 72-year-old man with hepatic IPT developed abscess after starting corticosteroids therapy, abscess resolved in the next 6 months. In this patient, steroid had resulted in liver abscess with recurrent hospital admission for fever.…”

Intratumor Abscess in a Posttraumatic Hepatic Inflammatory Pseudotumor Spreading Out of the Liver

“…Furthermore, we speculated that the abscesses in the IPT had been infected by the bile duct. A case of hepatic IPT that developed an abscess in the tumor has been reported ( 6 ), and several biliary diseases are thought to be the main cause of liver abscess ( 7 ). Fortunately, the abscess in our patient improved following treatment with antibiotics and drainage.…”