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(2011). FDG-PET/CT of polymyalgia rheumatica. Modern Rheumatology: Vol. 21, No. 3, pp. 334-336.

Objective Proton pump inhibitors (PPIs) are frequently coadministered with calcineurin inhibitors (CNIs) such as tacrolimus (TAC) and cyclosporin A (CSA), to treat or prevent upper gastrointestinal complications in Japanese patients with connective tissue diseases (CTDs). The coadministration of PPIs increases the blood concentration of TAC due to drug interaction. We retrospectively investigated the influence of the coadministration of PPIs and CNIs, as well as the influence of the cytochrome P450 (CYP) 2C19 gene polymorphism status, on the blood concentrations of TAC and CSA in patients with CTDs. Methods Patients treated with TAC (n=35) or CSA (n=30) were enrolled and divided into three groups according to the PPI they received: lansoprazole (LPZ)-combined, rabeprazole (RPZ)-combined, and non-PPI-combined groups. We compared the blood concentrations of TAC or CSA and the incidences of adverse events among the three groups. CYP2C19 gene polymorphisms were also assessed to investigate its influence on the blood concentration of TAC or CSA. Results LPZ significantly increased the blood concentration of TAC 12 hours after TAC administration (p=0.030 and p=0.003, respectively) and CSA (p=0.047 and p=0.014, respectively) in comparison with RPZ and non-PPI-combined treatment. There were no significant differences in the mean CSA blood concentration two hours after administration in patients with or without PPI treatment, in the incidence of adverse events, or in the CYP2C19 gene polymorphism status among the three groups. Conclusion Combining agents that are mainly metabolized by CYP3A4 such as LPZ elevates the blood concentrations of TAC and CSA, which could leading to adverse events.

Patients with SSc have the presence of several autoantibodies, some of which are SSc-specific antibodies associated with the clinical features of SSc (9-13). The presence of anti-topoisomerase I (topo-I) antibody is associated with the development of skin fibrosis and progressive ILD in SSc. Anti-centromere antibody (ACA) is seen in patients with limited skin disease and is a minor risk factor for the development of ILD. However, it remains unclear whether SSc-specific antibodies are associated with pulmonary function and radiological findings of HRCT. We divided SSc patients into 5 clusters on the basis of SSc-specific autoantibodies present and retrospectively evaluated the clinical and radiological features of ILD among these 5 SSc patient clusters. Abstract Background: Patients with systemic sclerosis have the presence of several autoantibodies, some of which are systemic sclerosis-specific autoantibodies that are related to the clinical features of this disease. We retrospectively evaluated the clinical and radiological features of systemic sclerosis in patients with interstitial lung disease on the basis of these autoantibodies. Method: This study comprised 226 patients with systemic sclerosis. We divided patients with systemic sclerosis into 5 clusters based on the systemic sclerosis-specific autoantibodies present and compared the clinical and radiological features of interstitial lung disease among these 5 systemic sclerosis clusters. Results: Of 226 patients, interstitial lung disease was present in 73 (32.3%) patients. Clustering by Ward cluster analysis subsequently identified 5 major clusters of patients. Clusters of patients with anti-topoisomerase I antibody and without systemic sclerosis-specific autoantibodies had a higher incidence of interstitial lung disease compared to the other clusters (75.0% and 51.9%, respectively). Patients in these two clusters had higher percentages of cough and dyspnea, higher incidence of traction bronchiectasis and honeycomb-like cysts in computed tomography, and a decrease in percentage predicted of vital capacity. In contrast, patients with anti-centromere antibody had few clinical and laboratory findings and the lowest incidence of interstitial lung disease (16.8%). Patients with anti- ribonucleic antibody or nucleolar pattern in anti-nuclear antibody had moderate findings among the 5 clusters. Conclusion: The results showed that there were differences in clinical and radiological findings among patients with systemic sclerosis-specific autoantibodies.

A case of a nasal-type NK/T cell lymphoma of the orbit with distant metastases is reported. The orbital lymphoma was angiodestructive with prominent necrosis and frequent apoptosis. Lymphoma cells were positive for CD56, cytotoxic molecules (granzyme B and TIA-1), and EBV. Despite local radiotherapy, the patient had a highly aggressive clinical course. A postmortem examination revealed distant metastases to the heart and lungs exhibiting angiocentric growth. TCR and Ig genes of a cardiac lesion were in germ line configuration. This case demonstrated that nasal-type NK/T cell lymphomas with a poor prognosis can occur in the orbit.

Characterizing the binding mechanism of Bt (Bacillus thuringiensis) Cry toxin to the cadherin receptor is indispensable to understanding the specific insecticidal activity of this toxin. To this end, we constructed 30 loop mutants by randomly inserting four serial amino acids covering all four receptor binding loops (loops α8, 1, 2 and 3) and analysed their binding affinities for Bombyx mori cadherin receptors via Biacore. High binding affinities were confirmed for all 30 mutants containing loop sequences that differed from those of wild-type. Insecticidal activities were confirmed in at least one mutant from loops 1, 2 and 3, suggesting that there is no critical amino acid sequence for the binding of the four loops to BtR175. When two mutations at different loops were integrated into one molecule, no reduction in binding affinity was observed compared with wild-type sequences. Based on these results, we discussed the binding mechanism of Cry toxin to cadherin protein.

Matrix metalloproteinase 9 (MMP-9) is a risk factor for cardiovascular events. The serum MMP-9 levels were measured before and 2 weeks after treatment with infliximab (3 mg/kg) in 12 rheumatoid arthritis (RA) patients. The serum average MMP-9 level was 238.5 ng/ml before treatment with infliximab in RA patients (normal range: less than 43.8 ng/ml). Infliximab reduced the serum average MMP-9 level significantly (161.66 ng/ml, P = 0.0425). The serum MMP-9 level was high in the RA patients with active disease, and it was reduced by infliximab independently of the reduction in disease activity. Thus, infliximab may reduce the risk of cardiovascular events directly. Keywords:: rheumatoid arthritis, matrix metalloproteinase 9, acute coronary syndrome

Dermatomyositis (DM) and polymyositis (PM) are types of autoimmune inflammatory muscle disease that mainly damage proximal limb muscles, with DM involving characteristic skin findings such as Gottron's sign and heliotrope eruption (Bohan & Peter, 1975a, 1975b). Interstitial pneumonia (IP) is often associated with DM/PM and is one of the important prognostic factors. Above all, rapidly progressive IP (RPIP), which has the worst prognosis, is resistant to corticosteroid drugs and is strongly associated with clinical amyopathic DM (CADM), which is unlikely to show myositis (Kameda & Takeuchi, 2006). In response, combination therapies of corticosteroid drugs and immunosuppressive drugs have recently been administered early in the onset of IP, and outcomes have been improved. Here, we review the pathogenesis, the clinical and laboratory findings, and treatment of IP associated with DM/PM.

Microscopic polyangiitis (MPA) is a necrotizing vasculitis of the small vessels. Among the nerve lesions of MPA, the incidence of multiple mononeuritis is high, but cranial nerve palsy is rarely reported. A female patient with oculomotor nerve palsy associated with MPA. The 68-year-old patient was admitted to our hospital with a high fever, numbness and weakness of the extremities, and muscle weakness. Multiple mononeuritis and purpura were observed. The urine was positive for occult blood and protein and the creatinine level was 1.2 mg/dL, indicating renal impairment. The levels of C-reactive protein (15.5 mg/dL) and myeloperoxidase-antineutrophil cytoplasmic antibody titers (600 ELISA units) were elevated. MPA was diagnosed, and 45 mg/day prednisolone was initiated. On the fifth day after the initiation of treatment, the patient suddenly developed diplopia and blepharoptosis of the left eye. Anisocoria and decreased light reflex as well as limited supraduction, infraduction, and adduction were also observed in the eye. Left oculomotor nerve palsy was diagnosed. The palsy gradually improved with continued prednisolone treatment. We encountered a rare case of MPA complicated by oculomotor nerve palsy.

A 67-year-old woman was admitted to our hospital because of erythema in the eyelids and fingers of both hands. Slight muscle weakness in the proximal limbs, a heliotrope rash, Gottron's sign, and mechanic's hand were observed. Creatine kinase serum levels were slightly elevated (376U/l), and the patient tested negative for all autoantibodies except for the antinuclear antibody. Chest computed tomography showed interstitial pneumonia (IP). Based on these findings, the patient was given a diagnosis of dermatomyositis (DM) associated with IP. Combined prednisolone (1 mg/kg/day) and cyclosporin-A (Cy-A 4 mg/kg/day) therapy was initiated; the patient showed marked improvement, but the Cy-A caused liver damage. Therefore, Cy-A was tapered off, and tacrolimus was simultaneously started, initially at a dose of 1 mg/day, building up to a sustained dose of 4 mg/day. The patient's IP and liver condition improved. Tacrolimus is a useful drug for the treatment of DM-IP in cases where Cy-A causes intolerable adverse reactions.

We analyzed the alternation in serum protein by infliximab therapy using proteomics-based technique. More than 50 gel spots were seen to increase or decrease in correlation with clinical improvements of rheumatoid arthritis (RA). Spots of interest were identified by two dimensional electrophoresis and mass spectrometry. Infliximab therapy reduced the inflammatory proteins such as C-reactive protein, serum amyloid protein A, serum amyloid protein P, and alpha1-acid glycoprotein, while the therapy increased Apo A-I, retinol-binding protein, vitamin D-binding protein, and gelsolin. This suggested that infliximab therapy shifted the inflammatory status of serum protein profile of RA patients to normal and modified the extracellular actin-scavenging system as well as vitamin and lipid profile.

A 57-year-old man had been suffering from dermatomyositis and systemic sclerosis presenting interstitial pneumonia since Jan, 2004. On February 2, 2005, he was admitted to our hospital because of thrombocytopenia, hematuria and proteinuria continuing from January, 2005. Hemolytic anemia and thrombocytopenia progressed, and fragmented red cells were present in peripheral blood on the 11th hospital day. Thrombotic microangiopathy (TMA) was diagnosed and he was treated with plasma infusion and methylprednisolone pulse therapy. Bloody sputum and hypoxia were observed, and HRCT of the chest on the 12th hospital day revealed multiple infiltrates and consolidations throughout all lung fields. His hypoxia was exacerbated and he was placed on a ventilator on the 13th hospital day. Bronchoscopic examination showed bleeding from both bronchi and he was diagnosed with complicating diffuse alveolar hemorrhage. He died of progressive respiratory failure. TMA and diffuse alveolar hemorrhage are rare but important complications of dermatomyositis and systemic sclerosis and need special care.

A 33-year-old woman presented with fever, malar rash, lymphadenopathy and pancytopenia 3 weeks after parturition. Serum C3 level was decreased and antinuclear antibody, anti-dsDNA antibody and anti-SS-A antibody were positive. Serum ferritin level was elevated (3454 ng/ml), and bone marrow aspirate revealed hemophagocytosis. She was diagnosed having systemic lupus erythematosus (SLE) associated with hemophagocytic syndrome (HPS). Oral prednisolone (55 mg/day) was initiated. Clinical manifestations and pancytopenia were improved and serum ferritin level was decreased. However, elevated anti-dsDNA antibody titer and reduced C3 level continued for a month after steroid therapy. The additional therapy of tacrolimus (3 mg/day) improved clinical and laboratory findings. This is a rare SLE case associated with HPS as primary manifestation after parturition.

A 55-year-old woman developed bilateral leg edema in June 2006. Since the edema tended to worsen, she visited our hospital on November 11. Laboratory tests showed a serum albumin level of 2.5 g/dl with 3+ proteinuria, and suggested nephrotic syndrome, which led to her hospitalization on November 14. The findings of discoid erythema, an antinuclear antibody titer of 1: 640, anti-ds DNA antibody titer of 16.8 IU/ml, and ISN/RPS class III (A/C)+V lupus nephritis on kidney biopsy led to the diagnosis of systemic lupus erythematosus. Treatment with prednisolone at 1 mg/kg/day was initiated. Despite an increase in complement levels and decreases in anti-ds DNA antibody titers and immune complexes, proteinuria persisted; therefore, the patient was concomitantly given 125 mg/day of azathioprine, which was discontinued because of the poor improvement of proteinuria and development of myelosuppression, and replaced by 3 mg/day of tacrolimus (Tac), with a consequent marked improvement in proteinuria. We report a patient with refractory proteinuria due to class III/V lupus nephritis who achieved a Tac-induced complete remission.

Lipase from Rhizopus chinensis cells was purified and characterized. The molecular mass of purified lipase was 28.4 kDa and the optimal temperature and pH for its activity were 37 degrees C and 5.5, respectively. Purified lipase exhibited high hydrolytic activity against fatty acid methyl esters such as methyl caprylate, methyl laurate, and methyl palmitate. Freeze-dried lipase catalyzed the transesterification between olive oil and methyl laurate in n-hexane.

We retrospectively examined the effect of combination therapy with prednisolone and cyclosporin A (CSA) for dermatomyositis (DM) presenting with acute/subacute interstitial pneumonia (A/SIP), the daily CSA dose, and the time from diagnosis of A/SIP to initiation of CSA treatment.Subjects were 16 DM patients with A/SIP. Seven patients were treated initially with 1 mg/kg/day prednisolone. When IP was progressive, CSA was added (Group A). Nine patients were treated initially with 1 mg/kg/day prednisolone and 4 mg/kg/day CSA, and 2-h postdose blood concentration (C2) monitoring was used to maintain the serum CSA level at 1000 ng/ml (Group B).Four of 7 patients in Group A (57%) and 1 of 9 patients in Group B (11%) died of respiratory failure related to IP (p = 0.06). Combination therapy with prednisolone and CSA ator= 200 mg/day initiated within 15 days of diagnosis was effective for treatment of DM-A/SIP. The trough level (C0) and daily CSA dose were higher in Group B (201.3 ng/ml and 200.0 mg/day, respectively) than in Group A (140.0 ng/ml and 166.4 mg/day). CSA was continued in all patients without severe side effects. No patient died of infection.Combination therapy of corticosteroids and CSA should be initiated during the early stage of IP. The daily CSA dose should also be controlled with measurement of serum CSA concentration to achieve maximal immunosuppressive effect. C2 monitoring is a useful tool for this control.

A 67-year-old female noticed dyspnea on exertion associated with the development of erythema in the eyelids and the bilateral fingers, and was admitted to our hospital on July 21, 2004. Proximal muscle weakness in the limbs, heliotrope rash, and Gottron's sign were observed, but the CK level was normal (194 U/l). All autoantibodies except for rheumatoid factor were negative. Hypoxemia and interstitial pneumonia on chest CT images were observed. Based on these findings, a diagnosis of advanced interstitial pneumonia associated with dermatomyositis was made. Combination immunosuppressive therapy was initiated with corticosteroid pulse therapy and cyclosporin-A (Cy-A), resulting in marked improvement. The Cy-A trough concentration was markedly high (456.4 ng/ml). When cytomegalovirus infection developed, the dose of Cy-A was reduced. Although the blood trough concentration of Cy-A was maintained at an adequately high level, the patient died of recurrence of rapidly progressive interstitial pneumonia. Careful observation is required when the dose of Cy-A is reduced for a patient with interstitial pneumonia associated with dermatomyositis. Furthermore, it is suggested that the trough concentration level of Cy-A is not always a useful parameter.

Background Cottin proposed that patients with emphysema of the superior lung field and fibrosis of the inferior lung field on thoracic CT should be regarded as having combined pulmonary fibrosis and emphysema (CPFE). They reported poor prognosis and marked reduction in pulmonary diffusion on a respiratory function test (1). Since then, various reports of CPFE have been published. However, there have been few reports on CPFE in patients with connective tissue disease (CTD). Objectives To investigate the clinical characteristics of CPFE in patients with CTD who consulted our hospital and compare the prognoses. Methods Of patients with interstitial pneumonia (IP) in the presence of rheumatoid arthritis (RA), dermatomyositis (DM), ANCA-associated vasculitis (AAV), or systemic sclerosis (SSc) who were treated in our hospital between August 1988 and October 2014, we identified the patients diagnosed of CPFE on thoracic HRCT findings. We retrospectively examined their clinical characteristics and prognoses. Patients in whom a low attenuation area accounted for 10% or more of the superior lung field on HRCT were regarded as having emphysema (2). Results Overall, 512 patients had IP, and 116 had CPFE. RA-CPFE was detected in 61 (34.1%) of 179 patients with RA-IP. DM-CPFE was detected in 13 (11.6%) of 112 patients with DM-IP. AAV-CPFE was detected in 19 (20.7%) of 64 patients with AAV-IP. SSc-CPFE was detected in 23 (14.6%) of 157 patients with SSc-IP. CPFE was more frequent in RA and AAV patients. The mean age of the CPFE patients was 68.4±9.4 years. They consisted of 78 males and 38 females. There were 99 smokers (85.4%, Brinkman index: 904±514). Before the start of treatment, the LDH, KL-6, A-aDO2, %FVC, FEV1.0/FVC, RV/TLC, %DLco/VA, and eRVSP values were 245±101 U/mL, 841±783 U/mL, 37.1±30.0, 88.1±21.2%, 76.6±15.3%, 51.7±18.1%, 47.5±20.2%, and 32.3±11.9 mmHg, respectively. In the DM-CPFE and SSc-CPFE patients, the FEV1.0/FVC was higher than in the AAV-CPFE and RA-CPFE patients, and the %FVC and %DLco/VA were lower. Eighty-one patients had received PSL therapy, and 79 had received immunosuppressant. In 9, IVCY was combined. During the course, lung cancer was detected in 17 patients. Twenty-three patients died (exacerbation of IP: 12, lung cancer: 4, and others: 7). The mean survival was 12.5±1.1 years. The 3-, 5-, and 10-year survival rates after the start of treatment were 89.9, 75.3, and 66.5%, respectively. The prognosis of the DM-CPFE patients was poorer, and that of the SSc-CPFE patients was favorable. The prognoses of patients with A-aDO2 more than 35 and those with %DLco/VA less than 34 were significantly poor. Conclusions CPFE with CTD was frequent in patients with RA or AAV. Its incidence was higher in males and smokers. Respiratory function tests showed reduction in diffusing capacity. The prognosis of the SSc-CPFE patients was favorable, whereas that of the DM-CPFE patients was poor. Prognostic factors were A-aDO2 and %DLco/VA. References 1. Cottin V et al., Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity. Eur Respir J. 2005;26(4):586-93. 2. Mejia M et al., Idiopathic pulmonary fibrosis and emphysema: decreased survival associated with severe pulmonary arterial hypertension. Chest. 2009 Jul;136(1):10-5. Disclosure of Interest None declared

Background Dermatomyositis (DM) is frequently complicated with interstitial pneumonia (IP), causing increased morbidity and mortality. It is important to identify a useful serum marker associated with the progression, severity, and prognosis of DM-IP patients. Activated CD8+ T cells play an important role in the pathogenesis of DM-IP. LIGHT (the name of which is derived from ‘homologous to lymphotoxins, exhibits inducible expression, competes with herpes simplex virus glycoprotein D for herpes simplex virus entry mediator [HVEM], and expressed by T lymphocytes’) is a member of the TNF superfamily and activates CD4+ and CD8+ T cells, monocytes and macrophages, natural killer cells, immature dendritic cells, and platelets. LIGHT mainly binds to the HVEM receptor on T cells and transmits co-stimulatory signals. HVEM signals stimulated by LIGHT more strongly induce CD8+ T cells than CD4+ T cells. LIGHT has been reported as a biomarker of inflammatory diseases, such as rheumatid arthritis, ankylopoietic spondylarthritis, inflammatory bowel disease, and atopic dermatitis, but its association with the pathology of DM-IP has not been clarified. Objectives Serum CD8+ T-cell activator, LIGHT, and Th1/Th2/Th17 cytokines were measured in DM-IP patients and compared with clinical parameters to investigate their usefulness. Methods The correlations between the clinical findings and serum LIGHT and Th1/Th2/Th17 cytokine levels were investigated in 21 patients with DM-IP (14 with rapidly progressive IP [RPIP] and 7 with chronic IP [CIP], including 4 fatal cases of IP). Results The median serum LIGHT level was 119 (16-335.4) pg/mL, which was higher than that in healthy control subjects and DM patients without IP. The median serum IL-6 level was 14.7 (2.4-154.5) pg/mL (n=13). The other cytokines were detected in only a few patients. The median serum LIGHT level in DM-RPIP patients (156 [49.6-335.4] pg/mL) was significantly higher than that in DM-CIP patients (94.3 [16-164.2] pg/mL) (P =0.02). The serum IL-6 level did not correlate with either progression or outcome of DM-IP. ROC curve analysis determined a serum LIGHT level of ≥120 pg/mL to be the cut-off value for the rapid progression of DM-IP. Serum LIGHT levels correlated significantly with %DLco (R =0.55, P =0.04) and total ground-glass opacity scores (R =0.72, P =0.0002). The serum LIGHT level significantly decreased to 100.5 (12.4-259.3) pg/mL 4 weeks after treatment initiation (P =0.04). Conclusions The serum LIGHT level may be a promising marker of disease progression and severity in patients with DM-IP. Disclosure of Interest None declared

Cold agglutinin disease (CAD) is a rare cause of anaemia in patients with systemic lupus erythematosus (SLE). CAD is usually refractory to glucocorticosteroid, and other immunosuppressive and/or cytotoxic therapies. We report the case of a 55-years old woman with SLE and CAD that did not respond to high-dose methylprednisolone, cyclosporin A, and double filtration plasma pheresis. Because several recent case reports and studies have indicated promising results of rituximab treatment for CAD and for SLE, rituximab was given weekly at 375 mg/m2in two doses. The rituximab was well tolerated, and there were no adverse effects. The hemolysis and SLE improved markedly, and the patient remained disease free eight months later. This is the first report of successful rituximab treatment of CAD in a patient with SLE. We conclude that rituximab is worth trying in such patients if they fail to respond to conventional treatment.

We analyzed the changes in the serum protein profile by infliximab using two-dimensional gel electrophoresis and mass spectrometry. More than 50 gel spots were seen to increase or decrease in correlation with clinical improvements of RA. The spots corresponding to CRP, C3, and Apo J showed reduced staining intensity, while the spots corresponding to Apo A-I, RBP, and transthyretin were enhanced. The protein profile of RA patients treated with infliximab was mostly similar to that of normal healthy controls except for several protein spots. This suggested that infliximab normalized the serum protein profile of RA patients, leading to modification in the serum lipid profile and antioxidant status in RA.

A 46-year-old male demonstrated edematous fingers on both hands in November 2003, and interstitial pneumonia was noted on chest X-ray during a medical check-up in December 2003. Since muscular weakness and fever developed thereafter, and interstitial pneumonia was aggravated on chest X-ray and CT, the patient was admitted to our hospital on March 25, 2004. Heliotrope-like erythema, and Gottron's sign were noted. Laboratory findings showed the following ; LDH 876 U/l ; CK 224 U/l ; CRP 5.68 mg/dl ; and KL-6 3270 U/ml. Autoantibodies such as anti-Jo-1 antibody were all negative. Chest X-ray and CT showed ground-glass opacity in the bilateral lower dorsal regions of the lung, and reduced volume of the inferior lobe. He was diagnosed as having dermatomyositis (DM) associated with progressive interstitial pneumonia. Although a combination of steroid pulse therapy and Cyclosporin-A were administered, the pulmonary lesions became aggravated. Additional intravenous Cyclophosphamide (IVCY) was initiated on the 6th hospital day, and interstitial pneumonia was markedly improved. Cases of progressive interstitial pneumonia associated with DM that are negative for anti-Jo-1 antibody and show a low ratio of CK/LDH are resistant to various treatments. Our case suggested that combination therapy with steroid, Cyclosporin-A, and IVCY is useful for the treatment of progressive interstitial pneumonia with DM.

To the Editor: Pulmonary involvement of rheumatoid arthritis (RA) includes drug-induced pulmonary toxicity, interstitial pneumonia (IP) due to RA itself, airway lesions, pleural lesions, and pulmonary infections due to the use of immunosuppressants. It is sometimes difficult to differentiate between these conditions. Pulmonary toxicity has been reported to be often induced by methotrexate and leflunomide but rarely by azathioprine (AZA). We describe a patient with RA and IP, in whom AZA induced pulmonary toxicity. The patient was a 72-year-old man diagnosed with RA (Steinbrocker stage III, class 2), treated with 5 mg/day prednisolone (PSL) and with clinically low disease activity. He developed a nonproductive cough 2 years ago. Chest radiograph and high-resolution computed tomography (HRCT) of the chest at that time revealed ground-glass opacities (GGO) in the bilateral peripheral lung fields and honeycomb-like cysts in the dorsal side of both lower lung fields, leading to a diagnosis of IP. Pulmonary function tests showed a vital capacity (VC) of 99.3%, and DLCO 54.2%. After he developed exertional dyspnea (Hugh-Jones Grade II) in March 2006, he was referred to our hospital and was admitted on June 20, 2006. Fine crackles were audible in both lower lung fields. Blood tests showed a white blood cell … Address correspondence to Dr. Ishida; E-mail: t-takeuchi{at}poh.osaka-med.ac.jp

BACKGROUND: Acute/subacute interstitial pneumonia in dermatomyositis (DM-A/SIP) is a disease associated with a poor prognosis that resists treatment with glucocorticosteroids (GC) and progresses rapidly in a period of weeks to months to death. We retrospectively studied outcomes, prognostic factors, and their relations with survival rate in patients with DM-A/SIP treated with early cyclosporine A (CSA)/GC combination therapy and 2-hour postdose blood concentration monitoring. METHODS: This study comprised 32 DM-A/SIP patients who were simultaneously treated with CSA and prednisolone. Clinical and laboratory findings were compared between those who died due to DM-A/SIP and those surviving 24 weeks after beginning of therapy. Prognostic factors were extracted, and their relations with the survival rate were evaluated. RESULTS: Of the 32 DM-A/SIP patients, 25 survived, 5 died of DM-A/SIP, and 2 died of infections. In those who died due to DM-A/SIP, ferritin level and the alveolar-arterial oxygen gradient were significantly increased compared with the survivors (P

Aorto‐oesophageal fistula is a life‐threatening disorder that can be caused by reflux oesophagitis. When reflux oesophagitis complicates systemic sclerosis, diffuse shallow ulcers appear in many ca...

Objectives We investigated changes in the prognosis and prognostic factors with respect to remission induction therapy in patients with microscopic polyangitis (MPA). Methods Among patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in whom remission was induced in our hospital between 1995 and 2010, the subjects were 60 who were regarded as having MPA according to the classification algorithm established by Watte et al. (2007) (1). We compared the prognosis during the remission induction period between two groups: patients in whom remission was induced between 1995 and 2004, and those in whom remission was induced between 2005 and 2010. In addition, we analyzed prognostic factors in the early phase of treatment. Results The subjects consisted of 23 males and 37 females. The median age (IQR) was 71 years (range: 59 to 75 years). According to EUVAS’ severity classification of AAV(2), the lesion type was evaluated as localized in 4 patients, early systemic in 7, systemic in 26, and severe/refractory in 23 (2 with cerebral hemorrhage, 2 with hemorrhage of the digestive tract, 1 with pancreatitis, 10 with diffuse pulmonary hemorrhage, and 2 who were positive for anti-basement-membrane antibody). Using the Kaplan-Meier life table method, the 180-day survival rate was estimated to be 79%. However, after 2004, it improved to 90%. When analyzing prognostic factors using Cox’s proportional hazard model, there were significant differences in the BVAS score and rate of decrease in the ANCA level between survival and primary disease-related death groups. Conclusions With respect to remission induction therapy for MPA, the prognosis after was more favorable than before 2004. When examining prognostic factors, the results suggest that BVAS-based assessment and control of the rate of decrease in the ANCA level are useful. References Watts R, Lane S, Hanslik T, et al: Development and validation of a consensus methodology for the classification of the ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studies. Ann Rheum Dis 2007; 66:222-227. Mukhtyar C, Guillevin L, Cid MC, et al: EULAR recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis 2009; 68: 310-317. Disclosure of Interest None Declared

Background Chest CT scan of patients with MPO-ANCA positive interstitial pneumonia often identifies a UIP pattern. This condition shows UIP-dominant pathological features, accompanied by characteristics differing from idiopathic pulmonary fibrosis such as poor fibroblastic foci. If these pathological differences are taken into account, the prognosis may differ between MPO-ANCA-positive interstitial pneumonia and idiopathic pulmonary fibrosis patients. (1) Objectives The prognosis was investigated in patients with MPO-ANCA-positive interstitial pneumonia having received immunosuppressive therapy beginning soon after disease onset. Methods Of the patients with microscopic polyangiitis admitted to our hospital between 2001 and 2012, MPO-ANCA-positive patients complicated by interstitial pneumonia (MPA-ILD) were enrolled in this study, in accordance with the EMEA Classification (2007). The clinical data, prognosis, and other variables were investigated in these patients. Results There were 32 cases of MPA-ILD (14 males and 18 females), with a mean age of 71.8 years (range: 48-87). The data at the start of treatment were: MPO-ANCA 379±486 EU, KL-6 692±551 U/ml, Aa-DO2 33.6±58, %FVC 80.9±18%, %DLco/VA 61.5±19%, and RV/TLC 42.0±8.4%. Chest CT scan at the start of treatment revealed a UIP pattern in 25 cases and a non-UIP pattern in 7 cases. All patients received treatment with PSL, combined with immunosuppressors in 29 cases (CY in 11 cases) and apheresis in 6 cases. After treatment, the MPO-ANCA level was below the detectable limit in most cases. On analysis of the prognosis, 5 patients died (from exacerbation of interstitial pneumonia in 1 case, infection + alveolar bleeding in 1 case, pulmonary hypertension in 1 case, and sudden death in 2 cases), with the median survival period being 124.6 months and the 5-year- survival rate being 85.5%. Following treatment, the ground glass opacity on chest CT disappeared, and the cyst tended to grow slightly. Conclusions The prognosis of MPA-ILD patients was favorable, suggesting that is better than that of usual idiopathic pulmonary fibrosis patients. When immunosuppressive therapy at sufficiently high dose levels, started soon after disease onset, was continued, MPO-ANCA became negative and remained so thereafter. We suggest that the prognosis of MPA-ILD patients may be improved if immunosuppressive therapy is applied more effectively. References Tanaka T, Otani K, Egashira R, Fukuoka J, et al: Interstitial pneumonia associated with MPO-ANCA: clinicopathological features of nine patients. Respir Med. 2012; 106: 1765-70. Disclosure of Interest None Declared

The importance of epidermal keratinocyte-produced antimicrobial peptides in the control of skin infections has been described. However, these peptides are not produced sufficiently in tissues surrounding the burn area. In this study, linage- CD34+ cells that infiltrated into burn area tissues were shown to be inhibitory on the antimicrobial peptide production by human epidermal keratinocytes (NHEK). Peripheral blood mononuclear cells (PBMC) taken from 5 severely burned patients (3rd degree, more than 40% total body surface area burn) enrolled in these experiments. NHEK (1 x 105 cells/ml, lower chamber) were cultured with 1 x 105 cells/ml of healthy donor PBMC or patient PBMC in a dual-chamber transwell. Forty-eight hrs after cultivation, cells in the lower chamber were assayed for human β-defensin-1 (HBD-1) and cathelicidin (CAP-18) mRNAs by RT-PCR. Without any stimulation, mRNAs for HBD-1 and CAP-18 were consistently expressed by NHEK. mRNA expression for HBD-1 and CAP-18 by NHEK was not influenced when peptide producer cells were transwell-cultured with normal donor PBMC. However, the expression of HBD-1 and CAP-18 mRNAs by NHEK was greatly suppressed when they were transwell-cultured with patient PBMC. Suppressor cells in patient PBMC for the peptide production by NHEK were determined to be linage- CD34+ cells. Linage- CD34+ cells isolated from healthy donor PBMC did not have any suppressor cell activities. These results indicate that linage- CD34+ cells isolated from severely burned patient PBMC are suppressor cells on the antimicrobial peptide production by epidermal keratinocytes. This study was supported by SHC NA #8610.

Investigations were made on the dissolution rate of rayon pulp fibers, original and degraded, and the amount of amorphous regions in undissolved fibers in emulsion xanthation. The dissolution process of fibers was also observed microscopically.The results are as follows:a) The lower the degree of polymerization is, the higher the rate of dissolution becomes, and the range of optimum alkali concentration for dissolution becomes wide.b) The amount of amorphous regions in undissolved fibers increases during xanthation, and is maximum when alkali concentration is about 10%.c) Although xanthation reaction occurs only at the contact points of CS2 droplets with fibers, occasional contacts are sufficient for the progress of xanthation. For this reason viscose-formation by simultaneous reaction in emulsion form takes place.