Fumihito Toshima

IgG4-Related Disease: Dataset of 235 Consecutive Patients

AbstractImmunoglobulin G4-related disease (IgG4-RD) is a recently discovered systemic condition, in which various organ manifestations are linked by a similar histological appearance. Our knowledge of this condition is still fragmented, as most studies have examined only a few dozen patients or focused on a particular organ manifestation. This study was conducted to learn the demography and patient characteristics of IgG4-RD using a large cohort. A total of 235 consecutive patients with IgG4-RD, diagnosed in 8 general hospitals in the same medical district, were identified by searching the institutions’ radiology database. Inclusion criteria were histology-proven IgG4-RD according to the Pathology Consensus Statement and/or definitive type 1 autoimmune pancreatitis meeting the International Consensus Diagnostic Criteria. Clinical notes and images of selected patients were retrospectively reviewed. All patients were adults (M/F = 4/1). The median age was 67 years (range 35–86). Nine tenths were diagnosed in their 50s to 70s. Among 486 manifestations identified in total, the most common was pancreatitis diagnosed in 142 patients (60%), followed by sialadenitis (34%), tubulointerstitial nephritis (23%), dacryoadenitis (23%), and periaortitis (20%). The majority of patients (95%) had at least 1 of the 5 most common manifestations. Male and female patients differed in their organ manifestations (periaortitis more common in males and sialodacryoadenitis more common in females). Serum IgG4 (normal ⩽135 mg/dL) was elevated to >135 mg/dL in 208 patients (88%) and >270 mg/dL in 167 (71%). The IgG4 value was significantly higher in patients with multiorgan involvement than in those with a single manifestation (median 629 mg/dL vs 299 mg/dL, P < 0.01). Of 218 patients, for whom both IgG4 and IgG values were available, the IgG4/IgG ratio was raised to >10% in 194 (89%). Corticosteroids were effective, but the relapse rate was estimated to be 24% in the study period (median 37 months). During the follow-up, 15 malignant diseases were diagnosed in 13 patients (6%). This figure is similar to the incidence (12.9 cancers) expected from the Japanese nationwide study for cancer epidemiology (standardized incidence ratio 1.16). In conclusion, this reliable dataset could improve the characterization of IgG4-RD, particularly its unique demography and the frequency of each organ manifestation.

Adenosquamous carcinoma of pancreas: CT and MR imaging features in eight patients, with pathologic correlations and comparison with adenocarcinoma of pancreas

PurposeTo reveal the CT and MR imaging features of adenosquamous carcinoma of pancreas (ASqC) in eight patients.MethodsThis study was approved by our institutional review board. Eight patients with ASqC were included in this study. Radiologic and pathologic findings were reviewed in each lesion. Additionally, radiologic imaging findings were compared between ASqC and controlled adenocarcinoma groups.ResultsSignificant differences between ASqC and adenocarcinoma groups were noted in lesion shape, enhancement pattern on dynamic CT images, the presence or absence of necrosis, and tumor thrombus in the portal vein (PV) system. Compared with adenocarcinoma, ASqC tended to be round-lobulated shape (100% vs. 57.6%), have necrotic portions (100% vs. 39.4%), and have tumor thrombus in the PV system (37.5% vs. 6.1%). Extensive central necrosis was found in six (75%) of ASqC lesions. More lesions in ASqC group (62.5% vs. 12.1%) showed the highest absolute attenuation on pancreatic arterial phase (PAP) or portal venous phase (PVP) images, although the average attenuation values of all ASqC lesions on PAP, PVP, and delayed phase images were almost the same. Five (83.3%) of six resected lesions appeared as nodular type macroscopically. Microscopically, all lesions did not show infiltrating growth pattern, but showed an intermediate growth pattern, and were surrounded incompletely by fibrous tissue.ConclusionsASqC tended to be a round-lobulated lesion with extensive central necrosis. Additionally, tumor thrombus in the PV system was often present. These CT and MR imaging features could be a useful clue for diagnosing ASqC.

Variations in feeding arteries of hepatocellular carcinoma located in the left hepatic lobe

The left hepatic lobe is divided into three subsegments according to anatomical landmarks; however, there are several variations in the vascular territories of the left hepatic arterial branches. Hepatocellular carcinoma (HCC) located near the umbilical fissure or at the left side of the umbilical portion of the left portal vein has frequent crossover blood supply. HCC located in the caudal aspect of the lateral segment has a variety of feeding arteries, and is infrequently supplied by the caudate artery or the medial subsegmental artery (A4), and by the lateral left hepatic arteries. HCC located in the posterior aspect of segment 4 is frequently supplied by the caudate artery or a small A4 branch arising from the caudate artery. In addition, the left inferior phrenic, right and left internal mammary, right and left gastric, cystic, and omental arteries are well known extrahepatic collateral pathways supplying HCC in the left hepatic lobe, especially when the hepatic artery is attenuated by previous transcatheter arterial chemoembolization (TACE). Interventional radiologists should have sufficient knowledge of vascular territories in the left hepatic arterial branches and extrahepatic collaterals to perform effective TACE for HCC located in the left hepatic lobe.

Imaging and pathological features of gastric lesion of immunoglobulin G4-related disease: A case report and review of the recent literature.

Abstract We describe a 67-year-old man with immunoglobulin G4-related disease (IgG4-RD) presenting with optic neuropathy, dacryoadenitis, periaortitis, retroperitoneal fibrosis, and a gastric mass-like lesion. A mass-like lesion measuring 52 × 40 mm in the antrum of the stomach was found incidentally through whole-body screening for other organ involvement of IgG4-RD using contrast-enhanced computed tomography (CT). Histology of the stomach revealed that the lesion was also IgG4-related and was located in the submucosal layer extending to the subserosal region. This case suggests that the stomach can also be a site of involvement of IgG4-RD.

Qualitative and quantitative evaluation for morphological changes of the splenic artery in autoimmune pancreatitis: novel imaging findings for differentiation from pancreatic adenocarcinoma

PurposeTo determine whether morphological changes can occur in the splenic artery (SPA) of autoimmune pancreatitis (AIP) cases, and if present, to compare them with those in pancreatic adenocarcinoma (PAC) to clarify any arterial morphological differences between AIP and PAC.MethodsA total of 101 AIP cases were included in this study. The presence or absence of morphological change in the SPA was assessed, using arterial phase axial computed tomography images. Subsequently, imaging parameters (imaging pattern, capsule-like rim, other organ involvement, splenic vein [SPV] stenosis, and SPA calcification) were compared between cases with and without morphological changes. Additionally, comparison analyses (visual SPA assessment and % minimal lumen diameter [MLD] stenosis) among normal pancreas, PAC, and AIP groups were performed using early arterial phase (EAP) reconstructed images.ResultsIn 25 (24.8%) AIP cases, marginal irregularities of the SPA were present. The presence of the capsule-like rim and SPV stenosis were significantly associated with the arterial morphological changes. All cases with morphological changes had a capsule-like rim. Visual assessment using EAP reconstructed images revealed irregularities of the SPA in 9 of 38 AIP cases (23.7%); however, arterial narrowing was not detected in any cases. % MLD stenosis in AIP group was significantly lower than that in PAC group (p < 0.0001).ConclusionsAlthough approximately one-quarter of AIP cases potentially demonstrate marginal irregularity in the SPA when it is surrounded by an apparent capsule-like rim, arterial luminal narrowing rarely occurs in contrast to PAC. These arterial findings can help to distinguish AIP from PAC.