Comparison of CEA and CA19-9 as a predictive factor for recurrence after curative gastrectomy in gastric cancer.

BACKGROUND: Our aim of was to compare importance of the tumor markers (TMs) serum carcinoembryonic antigen (CEA) and carbohydrate antigen (CA) 19-9 in prediction of recurrence after curative gastrectomy for gastric cancer. METHODS: We reviewed retrospectively the clinical records of 149 patients who underwent curative gastrectomy for stage I-III gastric cancer and whose CEA and CA19-9 levels were determined once preoperatively and for more than 3 years postoperatively. We investigated whether the clinicopathological characteristics of patients including age, sex, pathological disease stage, operative approach, type of gastrectomy, and degree of lymph node dissection as well as preoperative positivity of CEA and CA19-9 were risk factors for recurrence in univariate and multivariate analyses. Rate of recurrence was compared between patients positive and negative for postoperative CEA or CA19-9. We also calculated sensitivity, specificity, positive and negative predictable values of postoperative positivity of CEA and CA19-9 for recurrence. The lead time was compared between CEA and CA19-9 that was defined as the time of the first detection of increases in tumor markers and confirmation of recurrence on imaging modalities. RESULTS: The number of patients positive for preoperative CEA was 25 (17%) and for CA19-9 was 11 (7%). Recurrence was confirmed in 29 (19%) patients. Stage III disease, preoperative positivity for CA19-9 but not CEA, and total gastrectomy were risk factors for recurrence in univariate analysis, but stage III disease was the only risk factor for recurrence in multivariate analysis. Forty and 15 patients were positive for postoperative CEA and CA19-9, respectively. The recurrence rate of 47% (7/15) in patients positive for postoperative CA19-9 was greater than that in negative patients (22/134 = 16%), but it did not differ between patients who were positive or negative for postoperative CEA. Specificity for CA19-9 was greater than that for CEA (P < 0.05). The lead time of CEA (3.9 ± 4.7 months) was not different from that of CA19-9 (6.1 ± 7.1 months). CONCLUSIONS: These results indicate that CA19-9 rather than CEA is likely to be more useful for the detection of recurrence after curative gastrectomy for gastric cancer.

Effects of Pyrolysis Temperature and Chemical Modification on the Adsorption of Cd and As(V) by Biochar Derived from Pteris vittata.

Phytoremediation can be applied successfully to solve the serious worldwide issue of arsenic (As) and cadmium (Cd) pollution. However, the treatment of biomass containing toxic elements after remediation is a challenge. In this study, we investigated the effective use of biomass resources by converting the As hyperaccumulator P. vittata into biochar to adsorb toxic elements. Plant biomass containing As was calcined at 600, 800, and 1200 °C, and its surface structure and adsorption performances for As(V) and Cd were evaluated. Pyrolysis at 1200 °C increased the specific surface area of the biochar, but it did not significantly affect its adsorption capacity for toxic elements. The calcined biochar had very high adsorption capacities of 90% and 95% for As(V) and Cd, respectively, adsorbing 6000 mmol/g-biochar for As(V) and 4000 mmol/g-biochar for Cd. The As(V) adsorption rate was improved by FeCl3 treatment. However, the adsorption capacity for Cd was not significantly affected by the NaOH treatment. In conclusion, it was found that after phytoremediation using P. vittata biomass, it can be effectively used as an environmental purification material by conversion to biochar. Furthermore, chemical modification with FeCl3 improves the biochar's adsorption performance.

The composition of the human fecal microbiota might be significantly associated with fecal SCFA levels under hyperbaric conditions.

The fecal microbiota and short-chain fatty acids (SCFAs) play important roles in the human body. This study examined how hyperbaric conditions affect the fecal microbiota and fecal SCFAs. Fecal samples were obtained from 12 divers at three points during deep-diving training (before the diving training, at 2.1 MPa, and after decompression). At 2.1 MPa, the changes in the frequency of Clostridium cluster IV and fecal iso-valerate levels were positively correlated, and the changes in the frequencies of Bacteroides and Clostridium subcluster XIVa were inversely correlated. After decompression, positive correlations were detected between the changes in the frequency of Bifidobacterium and fecal n-valerate levels and between the changes in the fecal levels of iso-butyrate and iso-valerate. On the other hand, inverse correlations were detected between the changes in the frequency of Clostridium cluster IX and fecal iso-butyrate levels, between the changes in the frequency of Clostridium cluster IX and fecal iso-valerate levels, and between the changes in the frequencies of Bacteroides and Clostridium cluster IV plus subcluster XIVa. During the study period, the changes in fecal iso-butyrate and iso-valerate levels were positively correlated, and inverse correlations were seen between the changes in the frequency of Clostridium cluster IV and fecal propionate levels and between the changes in the frequencies of Prevotella and Clostridium subcluster XIVa. These findings suggest that hyperbaric conditions affect the fecal microbiota and fecal SCFA levels and that intestinal conditions reversibly deteriorate under hyperbaric conditions.

Influence of age on postoperative complications especially pneumonia after gastrectomy for gastric cancer.

BACKGROUND: The aim of this study was to investigate the influence of patients' age on postoperative morbidities including pneumonia. METHODS: We reviewed the clinical records of 211 patients with stages I - III gastric cancer undergoing curative distal gastrectomy (DG) or total gastrectomy (TG). Patients were classified into an elderly (≧80 y.o.) or a control (< 80 y.o.) group. We compared patient characteristics (sex ratio, disease stage, degree of lymph node dissection, number of retrieved lymph nodes, and type of reconstruction) and early postoperative outcomes (operation time, intra-operative blood loss, and postoperative morbidity including pneumonia, and mortality) between the two groups separately in DG and TG. RESULTS: There were 134 and 77 patients who underwent DG and TG, respectively. The numbers of patients in the elderly and control groups were 25 and 109 in DG and 12 and 65 in TG. The percentage of female patients in the elderly group was greater than that in the control group in both DG and TG. The extent of lymph node dissection did not differ between two groups in TG; in contrast in DG, the rate of a D1 dissection was greater in the elderly group than in the control group. There were no differences between the two groups in distribution of disease stage, number of retrieved lymph nodes, operation time, and blood loss in DG and in TG. Overall postoperative morbidity did not differ between two groups after DG and after TG. The rate of infectious complications in the elderly group was not different from that in the control group after DG and after TG. The incidence of pneumonia was more frequent in the elderly group compared to the control group after DG (8% vs. 1%, P < 0.05) but not after TG (17% vs. 5%). When patients were compared between the elderly and the control groups regardless of type of gastrectomy, the incidence of pneumonia in the elderly group (4/37 (11%)) was greater than that in the control group (4/174 (2%), P < 0.05). CONCLUSIONS: These results suggest that pneumonia is increased in patients older than 80 years after DG.

Clinical subsets associated with different anti-aminoacyl transfer RNA synthetase antibodies and their association with coexisting anti-Ro52.

OBJECTIVE: To characterize clinical features of polymyositis/dermatomyositis (PM/DM) patients with different anti-aminoacyl transfer RNA synthetase (ARS) antibodies and their association with anti-Ro52. METHODS: Autoantibodies in sera from 97 Japanese patients (36 PM, 56 DM, and 5 clinically amyopathic DM), who satisfied Bohan and Peter or modified Sontheimer's criteria, were characterized by immunoprecipitation and enzyme-linked immunosorbent assay. Clinical information was from medical records. Features associated with different anti-ARS and anti-Ro52 antibodies were analyzed. RESULTS: The prevalence of anti-ARS was similar to other studies (Jo-1, 22%; EJ, 4%; OJ, 1%; PL-12, 1%), except for a high prevalence of anti-PL-7 (12%), which allowed us to characterize patients carrying this specificity. Serum creatine kinase >3000 IU/l was less common in anti-PL-7-positive patients (57%) than anti-Jo-1-positive patients (18%) (p = 0.0328) and was not found in anti-EJ-positive individuals. Interstitial lung disease was common in anti-ARS-positive patients (97%) (p < 0.0001 vs. 48% in anti-ARS-negative). Anti-Ro52 antibodies were frequently detected with anti-ARS (59%) (57% in anti-Jo-1, 67% in anti-PL-7) (vs. 21% in anti-ARS-negative, p < 0.0002). Anti-Ro52 was associated with overlap syndrome (26%) (vs. 7% in anti-Ro52-negative, p = 0.0119). CONCLUSIONS: Patients with different anti-ARS in combination with anti-Ro52 appear to be associated with distinctive clinical subsets.

Adenocarcinoma associated with perianal fistulas in Crohn's disease.

AIM: The purpose of the present study was to examine the possible relation between anorectal carcinoma and infliximab therapy for Crohn's disease. PATIENTS AND METHODS: This study reviewed the medical records of patients with perianal Crohn's disease, who have required surgical intervention at the Tohoku University Hospital since May 2002, when infliximab was approved as a remedy for Crohn's disease in Japan. RESULTS: Ninety-two patients underwent surgery due to perianal Crohn's disease between May 2002 and December 2011. Four out of 92 patients were diagnosed as having anorectal carcinoma associated with perianal fistula. All four patients had advanced carcinoma, and received infliximab before the diagnosis of cancer was made. Infliximab was administered due to an exacerbated anal lesion in three patients. CONCLUSION: Careful inspection and obtaining of a biopsy sample under anesthesia is recommended for patients with Crohn's disease who have long-standing anal fistulas, especially before infliximab administration due to a possible exacerbation of anal symptoms.

Clinical characteristics of gastric cancer in patients with familial adenomatous polyposis.

Familial adenomatous polyposis is an autosomal dominant hereditary disease leading to the development of numerous colorectal polyps with malignant potential. Extra-colonic neoplasms are observed often in patients with familial adenomatous polyposis, but clinical characteristics of gastric cancer associated with familial adenomatous polyposis are not well understood. We studied the clinical characteristics of five Japanese patients who developed gastric cancer after undergoing colectomy for familial adenomatous polyposis. Gastric cancer was found on gastroduodenal endoscopy performed during postoperative follow-up in all five patients. Mean ages at the time of colectomy and at the time of diagnosis of gastric cancer were 39.2 and 58 years, respectively. Importantly, gastric fundic gland polyps were undetectable in these five patients. The mean duration between colectomy and diagnosis of gastric cancer was more than 20 years in three of five patients (mean: 19 years and 1 month). Cancers were multiple in three of five patients. Two patients developed metachronous gastric cancer in the gastric remnant. All five patients presented with well to moderately differentiated adenocarcinoma; four of the five patients were stage IA. Characteristics of the gastric cancer with familial adenomatous polyposis include a long duration between occurrence of gastric cancer and colectomy, metachronous cancers, multicentric lesions, and a high rate of well to moderately differentiated adenocarcinoma. Long-term and periodic gastroduodenal surveillance endoscopy is recommended for patients with FAP who underwent colectomy.

Young-onset peri-anorectal leiomyomatosis: report of a case.

A 23-year-old female was referred with constipation that lasted for 2 years. Preoperative examinations revealed multiple submucosal tumors beside the anorectum, along with subcutaneous tumors in the left buttock. The pathological diagnosis was leiomyoma. Low anterior resection of the rectum with regional lymph node dissection, along with the resection of the subcutaneous tumors in the left buttock through the transdermal approach, was performed, since multiple tumor formation indicated a high malignant potential. The tumors were diagnosed as multiple leiomyomas with no malignancy. Disease categories such as intravenous leiomyomatosis, leiomyomatosis peritonealis disseminata, Alport syndrome, and Currarino syndrome have been reported to be associated with leiomyomatosis; however, the current case of "peri-anorectal leiomyomatosis" was not classified into any of these. The patient was monitored with careful checkups, and the postoperative course was satisfactory for over 5 years without any sign of recurrence or metastasis. Although the clinicopathological features of this case are quite rare and no therapeutic guidelines for such a disease have yet been established, radical resection should be considered, and the elucidation of the histogenesis of this disease will help establish future therapeutic guidelines.

Criteria for the glucagon provocative test in the diagnosis of gastrinoma.

INTRODUCTION: The glucagon provocative test is useful for the diagnosis of gastrinoma. The aim of this study was to determine the criteria for the glucagon provocative test. METHODS: This study reviewed 8 patients that underwent the glucagon provocative test preoperatively and in whom the diagnosis was confirmed as gastrinoma histologically. The glucagon provocative test was performed by administering glucagon (20 µg/kg) intravenously, followed by 20 µg/kg h for the next 30 min, and plasma gastrin levels were measured 3 and 1 min before and 3, 5, 7, 10, 15, 20, and 30 min after the administration of glucagon. This study evaluated the peak value of plasma gastrin and the time required to reach the peak. RESULTS: Two of the 8 patients had multiple endocrine neoplasm type 1. The basal plasma gastrin levels ranged from 524 to 10,300 pg/ml. The time required to reach the peak was 3-10 min for all patients. The increase in the peak from the basal value was 235-8,920 pg/ml, and the percentage of increase was 38-337 %. CONCLUSIONS: These results suggest that a diagnosis of gastrinoma should thus be made when plasma gastrin levels peak within 10 min after glucagon administration, with an increase of greater than 200 pg/ml and greater than 35 % of the basal value.

Results of modified uncut Roux-en-Y reconstruction after distal gastrectomy for gastric cancer.

BACKGROUND/AIMS: The frequent occurrence of bile gastritis and esophagitis associated with dehiscence of 'uncut' jejunal portion was of concern for "uncut" Roux-en-Y reconstruction after distal gastrectomy. Our aim was to study if our technique of a modified, uncut Roux-en-Y procedure would decrease this dehiscence. METHODOLOGY: Ten patients with gastric cancer underwent distal gastrectomy with a modified, uncut Roux-en-Y reconstruction. Transmural silk stitches were added around the staples at the "uncut" portion in attempt to prevent dehiscence of the staple line. Dehiscence of the jejunum at the enterically closed site was investigated endoscopically or fluoroscopically. RESULTS: Mean operative time and intraoperative blood loss were 246 minutes and 381 mL, respectively. Morbidity occurred in three patients. No dehiscence was observed in any of the patients examined. CONCLUSIONS: These results suggest the possibility that our technique of a modified, uncut Roux-en-Y reconstruction after distal gastrectomy decreases dehiscence of enterically closed portion.

An adhesin-like protein, Lam29, from Lactobacillus mucosae ME-340 binds to histone H3 and blood group antigens in human colonic mucus.

A cell-surface 29-kDa protein (Lam29, cysteine-binding protein of the ABC transporter) from Lactobacillus mucosae ME-340 showed an adhesin-like property for human ABO blood group antigens expressed on the gastrointestinal mucosa. In addition, here we report that Lam29 also bound to an 18-kDa protein on human colonic mucus. By ligand blot assay and N-terminal amino acid sequence of the protein, it was identified as human histone H3. By ligand blot and microplate binding assays with recombinant histone H3, binding between Lam29 and histone H3 was confirmed. The adhesion of ME-340 cells to histone H3 was significantly inhibited by 26% after the addition of 2.5 mg/mL Lam29 as compared to the absence of Lam29 (p<0.01). By GHCl extraction and transcription attenuation of ME-340 cells, binding reduction of ME340 cells against histone H3 was detected at 12% and 13% respectively, as compared to control cells by the BIACORE assay (p<0.01). These data indicate that Lam29 shows multiple binding activities to blood group antigens and histone H3 in human colonic mucus. This is the first report to indicate that lactobacilli expressing Lam29 adhere to histone H3 on gastrointestinal mucosa.

IL-23 directly enhances the proliferative and invasive activities of colorectal carcinoma.

Interleukin-23 (IL-23) plays an essential role in the mucosal immune system. It has been suggested that IL-23 is able to induce carcinogenesis as well as inflammation and a recent study revealed that IL-23R is expressed in colorectal carcinoma cells. However, neither the differences in the IL-23R expression among the patients nor the concrete functions of IL-23 in colorectal carcinoma cells have been revealed. The aim of the present study was to examine the characteristics of IL-23R expression in colorectal carcinoma and the direct effects of IL-23 on colorectal cancer cells. We examined the IL-23R expression in human colorectal cancer tissue samples by immunohistochemistry. Cell proliferation and invasion assays under IL-23 stimulation were performed using cultured cells derived from colorectal cancer. ELISA and real-time PCR were used to evaluate the transforming growth factor (TGF)-ß production due to IL-23 stimulation. All of the TNM stage IV patients were positive for IL-23R. IL-23R expression in the carcinoma tissue was also relatively high at the deepest point of invasion in certain cases. The proliferative and invasive activities and/or TGF-ß production of DLD-1 cells increased by IL-23 stimulation, whereas no change was observed in the activities of MIP101 and KM12c cells. IL-23 directly enhanced the malignancy of the colon carcinoma cells. An autocrine mechanism via TGF-ß production may underlie these effects. IL-23 is therefore a potential target for cancer immunotherapy. However, the homogeneity in IL-23R expression and the effects of IL-23 on colorectal carcinoma cells should be considered.

A shift from colon- to ileum-predominant bacteria in ileal-pouch feces following total proctocolectomy.

BACKGROUND: We previously investigated fecal flora of the pouch after total proctocolectomy using terminal restriction fragment polymorphism analysis. Although the results of the cluster analysis demonstrated clearly that bacterial populations, including an unidentified bacteria generating a 213-bp PCR fragment, moved toward a colon-like community in the pouch, it did not track changes in the individual species of fecal bacteria. AIMS: The aim of the present study was to estimate genome copy number of ten bacterial species, clusters, groups, or subgroups (including the bacteria generating 213-bp fragment in the previous study) in feces samples from pouches at various times following ileostomy closure. METHODS: A total of 117 stool samples were collected from patients with ulcerative colitis after surgery as well as healthy volunteers. We used real-time polymerase chain reaction of the 16S rRNA gene to estimate genome copy numbers for the nine bacterial populations and the bacteria generating 213-bp fragment after identification by DNA sequencing. RESULTS: We demonstrated a time-dependent increase in the number of anaerobic and colon-predominant bacteria (such as Clostridium coccoides, C. leptum, Bacteroides fragilis and Atopobium) present in proctocolectomy patients after stoma closure. In contrast, numbers of ileum-predominant bacterial species (such as Lactobacillus and Enterococcus faecalis) declined. CONCLUSIONS: Our data confirm previous findings that fecal flora in the pouch after total proctocolectomy changes significantly, and further demonstrate that the number and diversity of ileal bacteria decreases while a more colon-like community develops. The present data are essential for the future analysis of pathological conditions in the ileal pouch.

The long-term clinical course of pouchitis after total proctocolectomy and IPAA for ulcerative colitis.

BACKGROUND: Pouchitis is the most common long-term complication after restorative total proctocolectomy and IPAA for ulcerative colitis. OBJECTIVE: We examined the incidence, clinical aspects, and long-term outcome of patients with idiopathic pouchitis. DESIGN: This study was a retrospective review of medical records. PATIENTS: Included in the study were 284 patients with ulcerative colitis who underwent a total proctocolectomy and IPAA. MAIN OUTCOME MEASURES: We evaluated the cumulative risk and long-term outcome of pouchitis including the duration of disease, pattern of relapse, and responsiveness to antibiotic therapy. RESULTS: Sixty-four patients developed idiopathic pouchitis. The cumulative risk was 10.7% at 1 year, 17.2% at 2 years, 24.0% at 5 years, and 38.2% at 10 years. At their first pouchitis episode, 45 patients had acute pouchitis, 19 patients had chronic pouchitis, and all patients received antibiotic therapy with oral ciprofloxacin and/or metronidazole. The efficacy of the therapy was 96.6% initially. Forty-five patients had antibiotic-responsive pouchitis, 17 patients had antibiotic-dependent pouchitis, and 2 patients had antibiotic-refractory pouchitis at their first episode. Whereas 20 of 45 patients (44.4%) with initially acute pouchitis experienced 2 or more relapses, 16 of 19 patients (84.2%) with initially chronic pouchitis had 2 or more relapses. After taking into account the relapses, the number of patients with antibiotic-responsive pouchitis decreased from 45 to 40, the number with antibiotic-dependent pouchitis increased from 17 to 20, and the number with antibiotic-refractory pouchitis increased from 2 to 4. Among the 4 patients with antibiotic-refractory pouchitis, 3 patients had Clostridium difficile-associated pouchitis. LIMITATIONS: This study was retrospective. CONCLUSION: The patients with chronic pouchitis at the first episode tend to have a higher incidence of relapse. In some patients, the responsiveness to antibiotic therapy changes during follow-up. When patients with pouchitis do not respond to standard antibiotic therapy, then the occurrence of C difficile infection should be considered.

A unique variant of afferent limb syndrome after ileal pouch-anal anastomosis: a case series and review of the literature.

BACKGROUND: Afferent limb syndrome is a relatively rare cause of small bowel obstruction after restorative total proctocolectomy with ileal pouch-anal anastomosis for patients with ulcerative colitis or familial adenomatous polyposis. DISCUSSION: This report describes three patients who developed recurrent small bowel obstruction after ileal pouch-anal anastomosis. The bowel obstruction was caused by torsion of the ileum at the inlet of the ileal J-pouch, which was thought to be a variant of afferent limb syndrome. This variant of afferent limb syndrome is characterized by a flexible afferent limb of the pelvic pouch due to the lack adhesion of the ileum in the abdominal cavity. Preoperative diagnosis required multiple series of contrast small bowel enemas. Strictureplasty and ileopexy effectively resolved the recurrent bowel obstruction caused by this variant of afferent limb syndrome.

Differentiating rectal carcinoma by an immunohistological analysis of carcinomas of pelvic organs based on the NCBI Literature Survey and the Human Protein Atlas database.

The treatments and prognoses of pelvic organ carcinomas differ, depending on whether the primary tumor originated in the rectum, urinary bladder, prostate, ovary, or uterus; therefore, it is essential to diagnose pathologically the primary origin and stages of these tumors. To establish the panels of immunohistochemical markers for differential diagnosis, we reviewed 91 of the NCBI articles on these topics and found that the results correlated closely with those of the public protein database, the Human Protein Atlas. The results revealed the panels of immunohistochemical markers for the differential diagnosis of rectal adenocarcinoma, in which [+] designates positivity in rectal adenocarcinoma and [-] designates negativity in rectal adenocarcinoma: from bladder adenocarcinoma, CDX2[+], VIL1[+], KRT7[-], THBD[-] and UPK3A[-]; from prostate adenocarcinoma, CDX2[+], VIL1[+], CEACAM5[+], KLK3(PSA)[-], ACPP(PAP)[-] and SLC45A3(prostein)[-]; and from ovarian mucinous adenocarcinoma, CEACAM5[+], VIL1[+], CDX2[+], KRT7[-] and MUC5AC[-]. The panels of markers distinguishing ovarian serous adenocarcinoma, cervical carcinoma, and endometrial adenocarcinoma were also represented. Such a comprehensive review on the differential diagnosis of carcinomas of pelvic organs has not been reported before. Thus, much information has been accumulated in public databases to provide an invaluable resource for clinicians and researchers.

Effects of ileal interposition on glucose metabolism in obese rats with diabetes.

BACKGROUND: Ileal interposition (IT), in which the distal ileum is transposed isoperistaltically into the proximal jejunum, is considered as a procedure for metabolic or antidiabetes surgery. Our aim was to study the effects of IT on glycemic control, fat metabolism, and hormonal changes in obese rats with spontaneous diabetes. METHODS: Animals were divided into either an IT or a sham (SH) group. They underwent an oral glucose tolerance test (OGTT) before and 4 and 8 weeks after the operation. All animals were killed 10 weeks after operation for analyses of tissue weight (liver, pancreas, epididymal fat, brown fat), immunoblotting of uncoupling protein-1 (UCP1) protein in brown adipose tissue (BAT), and fasting plasma levels of glucose, insulin, glucagon-like peptide (GLP)-1, peptide YY (PYY), glucose-dependent insulinotropic polypeptide (GIP), and leptin. RESULTS: Body weight increased postoperatively in both groups compared with preoperative weight, but it did not differ between the 2 groups. Eight weeks postoperatively, integrated blood glucose levels during the OGTT were decreased in IT compared with SH (P < .05). Fasting plasma levels of insulin, GLP-1, and GIP did not differ between the 2 groups, but PYY levels were higher in the IT animals (P < .01). The weight of epididymal and BATs, homeostasis model assessment insulin resistance, and fasting plasma leptin levels were decreased in the IT group (P < .05). Expression of UCP1 was higher in IT than SH animals (P < .05). CONCLUSION: These results suggest that IT improves glucose and lipid metabolism by decreasing insulin resistance and epididymal fat, and increased expression of UCP1 in BAT might be among the mechanisms responsible.

Cutaneous polyarteritis nodosa associated with HLA-B39-positive undifferentiated spondyloarthritis in a Japanese patient.

We present the case of a 43-year-old man diagnosed with HLA-B39-positive spondyloarthritis who developed cutaneous lesions consistent with cutaneous polyarteritis nodosa (CPN). Previous studies indicated an elevated incidence of HLA-B39 in HLA-B27-negative Japanese patients with spondyloarthritis. This case suggested that CPN may also occur in association with forms of HLA-B39-positive spondyloarthritis. The rarity of this association is emphasized. Therapy with corticosteroid and methotrexate improved both the cutaneous lesions and the clinical symptoms of spondyloarthritis.

Longterm survival and associated risk factors in patients with adult-onset idiopathic inflammatory myopathies and amyopathic dermatomyositis: experience in a single institute in Japan.

OBJECTIVE: To analyze clinical characteristics, survival, causes of death, and risk factors associated with mortality in patients with adult-onset idiopathic inflammatory myopathies (IIM) in Japan. METHODS: We retrospectively investigated 197 patients diagnosed with adult-onset IIM at our hospital from 1984 to 2009 according to Bohan and Peter criteria for polymyositis (PM)/dermatomyositis (DM) and modified Sontheimer's criteria for clinically amyopathic DM (ADM). RESULTS: Survival in the whole group at 1, 5, and 10 years was 85%, 75%, and 67%, respectively. Mortality in cancer-associated myositis was the worst (25% at 5 yrs), followed by clinically ADM (61% at 5 yrs) and primary DM (77% at 5 yrs). Primary DM had significantly low survival compared to primary PM (91% at 5 yrs; p = 0.0427). Among the 53 patients who died were 6 patients with ADM (11%) and 20 patients with primary DM (38%). Interstitial lung disease (ILD) was the main cause of death in clinically ADM (71%) and primary DM (60%), most of which occurred within the first few months. Fewer patients died in primary PM (9%) and overlap myositis (13%). Independent risk factors for death were older age (HR 1.031; 95% CI 1.009-1.053) and skin ulcers (HR 3.018; 95% CI 1.340-6.796) in the whole group and ILD with mild serum creatine kinase levels (< 500 IU/l; HR 3.537; 95% CI 1.260-9.928) in primary DM. CONCLUSION: Survival of clinically ADM and primary DM was low, mainly due to fatal ILD, compared to primary PM. Establishing therapeutic strategy for ILD may improve the survival in our patient population.