A 78-year-old woman who had undergone an axillobifemoral artery bypass with a prosthetic graft for Leriche syndrome presented 1 month later with cough and fever. A clinical examination revealed obvious redness in the right groin. Routine laboratory tests uncovered inflammation and methicillin-sensitive-Staphylococcus aureus was cultured from blood samples. Mitral valve vegetations were identified by echocardiography, and after a diagnosis of infective endocarditis, specific intravenous antibiotics were immediately administered. One month later, CT revealed a large pseudoaneurysm of the posterior left ventricular wall that had not been present at the time of admission. Transesophageal echocardiography and magnetic resonance imaging showed an aneurysmal cavity arising from the wall just below the posterior mitral valve leaflet. The patient agreed to undergo cardiac surgery due to the high likelihood that the pseudoaneurysm would rupture. The mitral annulus and leaflet were normal at surgery. We resected the posterior leaflet, closed the cavity using a Xenomedica patch, and reconstructed the leaflet. We did not remove the pseudoaneurysm using an extracardiac approach because the likelihood of damaging the coronary arteries and the coronary sinus was quite high. The postoperative course was uneventful. At follow-up 1 year later, the patient was afebrile and both CT and echocardiography showed that the cavity was completely filled by the thrombus. The imaging findings were useful in determining the surgical approach.

Background : This study was performed to evaluate surgical outcomes after cardiovascular surgery (including urgent surgery) in patients 85 or older. Methods : A retrospective analysis was performed on 39 patients (mean age, 86.3 years ; age range, 85-90 years) who underwent total arch replacement (n=4), ascending aorta replacement (n=4), descending aorta replacement (n=1), aortic valve replacement (AVR ; n=13), mitral valve replacement or valvuloplasty (n=3), coronary artery bypass grafting (CABG ; n=9), CABG+AVR (n=4), tumor resection (n=1) between June 2008 and December 2012 at Dokkyo Medical University Hospital. Results : Six hospital deaths occurred. One patient died due to bleeding from a ruptured descending thoracic aortic aneurysm, and another patient died due to gastrointestinal perforation from non-occlusive mesenteric ischemia (NOMI) after urgent AVR. The other deaths were related to various complications, including lung cancer, cholecystitis, myocardial infarction, and Takotsubo cardiomyopathy, during the postoperative period. Overall 30-day mortality was 2.6%, hospital mortality was 12.8%, duration of hospital stay after surgery was 41.3 days, duration of intensive care unit (ICU) stay was 3.8 days and ventilator time was 49.1 h. Twenty patients underwent elective surgery, and 19 patients underwent urgent surgery. The two groups had similar preoperative characteristics, except for the number of patients with aortic disease. No significant difference was evident in hospital mortality (26.3% vs. 5%, p=0.065) or 30-day mortality (0% vs. 5.3%, p=0.3) when comparing the two groups. However, the duration of hospital stay (58.9 days vs. 27.5 days, p=0.049), ICU stay (6.74 days vs. 1.05 days, p=0.002) and ventilator time (89.9 h vs. 8.2 h, p=0.006) was significantly longer in the urgent surgery group than in the elective surgery group. Fourteen patients (70%) in the elective surgery group and four patients (21.1%) in the urgent surgery group were able to be discharged from the hospital to their homes within 30 days after surgery. These data demonstrated that cardiovascular surgery in patients 85 years of age or older was associated with satisfactory outcomes, and outcomes associated with elective surgery were even better than those associated with urgent surgery. Conclusions : Therefore, advanced age does not represent a contraindication of conventional cardiovascular surgery. Rather, the decision for surgery should take the patient's preoperative condition, the severity of concurrent medical disease, the wishes of the patient, and the predicted functional outcomes into account.

BACKGROUND/AIMS: Ulcerative colitis (UC) is sometimes associated with autoimmune pancreatitis (AIP). Infiltration of immunoglobulin G4 (IgG4)-positive plasma cells is sometimes detected in the colonic mucosa of AIP or UC patients. This study aimed to clarify the relation between UC and IgG4. METHODS: Associations with UC were reviewed in 85 AIP patients. IgG4 immunostaining was performed on biopsy specimens from the colonic mucosa of 14 AIP and 32 UC patients. RESULTS: UC was confirmed in two cases (type 1 AIP, n=1; suspected type 2 AIP, n=1). Abundant infiltration of IgG4-positive plasma cells in the colonic mucosa was detected in the case of suspected type 2 AIP with UC and two cases of type 1 AIP without colitis. Abundant infiltration of IgG4-positive plasma cells was detected in 10 UC cases (IgG4-present, 31%). Although 72% of IgG4-absent UC patients showed mild disease activity, 70% of IgG4-present patients showed moderate to severe disease activity (p<0.05). CONCLUSIONS: UC is sometimes associated with AIP, but it seems that UC is not a manifestation of IgG4-related disease. Infiltration of IgG4-positive plasma cells is sometimes detectable in the colonic mucosa of UC patients and is associated with disease activity.
Adult ; Aged ; Colitis, Ulcerative/*blood/complications ; Colon/*chemistry ; Female ; Humans ; Immunoglobulin G/*analysis ; Intestinal Mucosa/*chemistry ; Male ; Middle Aged ; Pancreatitis/*blood/complications ; Retrospective Studies

BACKGROUND/AIMS: Diffuse or segmental irregular narrowing of the main pancreatic duct (MPD), as observed by endoscopic retrograde cholangiopancreatography (ERCP), is a characteristic feature of autoimmune pancreatitis (AIP). METHODS: ERCP findings were retrospectively examined in 40 patients with AIP in whom irregular narrowing of the MPD was detected near the orifice. The MPD opening sign was defined as the MPD within 1.5 cm from the orifice being maintained. The distal common bile duct (CBD) sign was defined as the distal CBD within 1.5 cm from the orifice being maintained. Endoscopic findings of a swollen major papilla and histological findings of specimens obtained from the major papilla were examined in 26 and 21 patients, respectively. RESULTS: The MPD opening sign was detected in 26 of the 40 patients (65%). The distal CBD sign was detected in 25 of the 32 patients (78%), which showed stenosis of the lower bile duct. The patients who showed the MPD opening sign frequently showed the distal CBD sign (p=0.018). Lymphoplasmacytic infiltration, but not dense fibrosis, was histologically detected in biopsy specimens obtained from the major papilla. CONCLUSIONS: On ERCP, the MPD and CBD adjacent to the major papilla are frequently maintained in patients with AIP involving the pancreatic head. These signs are useful for diagnosing AIP on ERCP.
Autoimmune Diseases/*diagnosis/pathology ; *Cholangiopancreatography, Endoscopic Retrograde ; Common Bile Duct/pathology ; Female ; Humans ; Male ; Middle Aged ; Pancreas/pathology ; Pancreatic Ducts/pathology ; Pancreatitis/*diagnosis/pathology ; Retrospective Studies

IgG4-related disease (IgG4-RD) is a potentially multiorgan disorder. In this study, clinical and serological features from 132 IgG4-RD patients were compared about organ correlations. Underlying pathologies comprised autoimmune pancreatitis (AIP) in 85 cases, IgG4-related sclerosing cholangitis (IgG4-SC) in 12, IgG4-related sialadenitis (IgG4-SIA) in 56, IgG4-related dacryoadenitis (IgG4-DAC) in 38, IgG4-related lymphadenopathy (IgG4-LYM) in 20, IgG4-related retroperitoneal fibrosis (IgG4-RF) in 19, IgG4-related kidney disease (IgG4-KD) in 6, IgG4-related pseudotumor (IgG4-PT) in 3. Sixty-five patients (49%) had multiple IgG4-RD (two affected organs in 36 patients, three in 19, four in 8, five in 1, and six in 1). Serum IgG4 levels were significantly higher with multiple lesions than with a single lesion (P<0.001). The proportion of association with other IgG4-RD was 42% in AIP, the lowest of all IgG4-RDs. Serum IgG4 level was lower in AIP than in other IgG4-RDs. Frequently associated IgG4-RDs were SIA (25%) and DAC (12%) for AIP; AIP (75%) for IgG4-SC; DAC (57%), AIP (38%) and LYM (27%) for IgG4-SIA; AIP (26%) and LYM (26%) for IgG4-DAC; SIA (75%), DAC (50%) and AIP (45%) for IgG4-LYM; SIA (58%), AIP (42%) and LYM (32%) for IgG4-RF; AIP (100%) and SIA (67%) for IgG4-KID; and DAC (67%) and SIA (67%) for IgG4-PT. Most associated IgG4-RD lesions were diagnosed simultaneously, but IgG4-SIA and IgG4-DAC were sometimes identified before other lesions. About half of IgG4-RD patients had multiple IgG4-RD lesions, and some associations were seen between specific organs.
Adult ; Aged ; Autoimmune Diseases/*epidemiology/*immunology ; Comorbidity ; Female ; Humans ; Immunoglobulin G/*immunology ; Japan/epidemiology ; Male ; Middle Aged ; Multiple Organ Failure/*epidemiology/*immunology ; Organ Specificity/immunology ; Prevalence ; Risk Factors ; Statistics as Topic

BACKGROUND/AIMS: The objective of this study was to compare the clinical characteristics of patients with autoimmune pancreatitis (AIP) with or without Mikulicz's disease (MD) and with MD alone. METHODS: We investigated the clinical findings in 15 AIP patients with MD (group A+M), 49 AIP only patients (group A), and 14 MD only patients (group M). RESULTS: The male-female ratio was significantly higher in group A+M (73%, p<0.05) and group A (78%, p<0.01) than group M (21%). Serum immunoglobulin G (IgG) levels were significantly higher in group A+M than in group A (p<0.01) and group M (p<0.05). Serum IgG4 levels were significantly higher in group A+M than in group A (p<0.01). Other organ involvement was observed in 73% (11/15) of patients in group A+M. The number of patients with diabetes mellitus was significantly higher in group A+M (66%, p<0.01) and group A (51%, p<0.05) than in group M (7%). All of the patients responded well to steroid therapy, but the relapse rate in group A+M (33%) was significantly higher than that in group A (3%, p<0.01). Salivary gland function was impaired in all groups compared with the control group, but the degree of dysfunction was less in group A compared with group A+M and group M. CONCLUSIONS: The relapse rate of AIP in MD patients was significantly higher than that of AIP in patients without MD.
Diabetes Mellitus ; Humans ; Immunoglobulin G ; Mikulicz' Disease ; Pancreatitis ; Recurrence ; Salivary Glands

BACKGROUND/AIMS: Few studies have differentiated immunoglobulin G (IgG) 4-related sclerosing cholangitis (IgG4-SC) from hilar cholangiocarcinoma (CC). Thus, we sought to investigate useful features for differentiating IgG4-SC from hilar CC. METHODS: We retrospectively compared clinical, serological, imaging, and histological features of six patients with IgG4-SC and 42 patients with hilar CC. RESULTS: In patients with hilar CC, obstructive jaundice was more frequent (p<0.01), serum total bilirubin levels were significantly higher (p<0.05), serum CA19-9 levels were significantly higher (p<0.01), and serum duke pancreatic monoclonal antigen type 2 levels were frequently elevated (p<0.05). However, in patients with IgG4-SC, the serum IgG (p<0.05) and IgG4 (p<0.01) levels were significantly higher and frequently elevated. The pancreas was enlarged in all IgG4-SC patients but only in 17% of hilar CC patients (p<0.01). Salivary and/or lacrimal gland swelling was detected in only 50% of IgG4-SC patients (p<0.01). Endoscopic retrograde cholangiography revealed that the hilar or hepatic duct was completely obstructed in 83% of hilar CC patients (p<0.01). Lower bile duct stenosis, apart from hilar bile duct stenosis, was more frequent in IgG4-SC patients (p<0.01). Bile duct wall thickening in areas without stenosis was more frequent in IgG4-SC patients (p<0.01). CONCLUSIONS: An integrated diagnostic approach based on clinical, serological, imaging, and histological findings is necessary to differentiate IgG4-SC from hilar CC.
Bile Ducts ; Bilirubin ; Cholangiocarcinoma ; Cholangiography ; Cholangiopancreatography, Endoscopic Retrograde ; Cholangitis, Sclerosing ; Constriction, Pathologic ; Hepatic Duct, Common ; Humans ; Immunoglobulin G ; Immunoglobulins ; Jaundice, Obstructive ; Lacrimal Apparatus ; Pancreas ; Retrospective Studies

BACKGROUND/AIMS: Autoimmune pancreatitis (AIP) is frequently associated with diabetes mellitus (DM). This study evaluated the effect of steroid therapy on the course of DM in AIP. METHODS: Glucose tolerance was examined in 69 patients with AIP. DM onset was classified as either a simultaneous onset with AIP or an exacerbation of pre-existing DM. Based on the changes in the HbA1c levels and insulin dose, the responses of DM to steroids were classified as improved, no change, or worsened. RESULTS: Thirty (46%) patients were diagnosed as having DM (simultaneous onset, n=17; pre-existing, n=13). Three months after starting the steroid treatment, the DM improved in 13 (54%) of 24 DM patients. The DM improved in 55%, had no change in 36%, and worsened in 9% of the 11 simultaneous onset DM patients, and it improved in 54%, had no change in 31%, and worsened in 15% of the 13 pre-existing DM patients. At approximately 3 years after starting the steroid treatment, the DM improved in 10 (63%) of 16 patients. The pancreatic exocrine function improved in parallel with the changes in the DM in seven patients. CONCLUSIONS: Because approximately 60% of DM associated with AIP is responsive to steroids in the short- and long-terms, marked DM associated with AIP appears to be an indication for steroid therapy.
Diabetes Mellitus ; Glucose ; Humans ; Insulin ; Pancreatitis ; Steroids