[COMPONENT ANALYSIS OF THREE ANISAKIASIS ALLERGY CASES IN OUR DEPARTMENT].

Reactivity to an anisakis allergen component was examined in three patients with a history of an anisakiasis anaphylaxis. Case 1, a 38-year-old man, allergic symptoms appeared 0.5 hours after ingestion, and the component Ani s 1 and 3 were positive. Case 2, a 44-year-old woman, allergic symptoms appeared 4 hours after ingestion, and components Ani s 3 and 12 were positive. Case 3, a 36-year-old woman, developed allergic symptoms 7 hours after ingestion of fish and shellfish, and tested positive for Ani s 1, 4, and 12. Case 3 reacted strongly to both heated and unheated Anisakis extract, while cases 1 and 2 reacted weakly to heated Anisakis extract. The most common allergen was Ani s 12, followed by Ani s 1, when analyzed in conjunction with existing reports on 10 cases. Anisakis IgE was class 3 or higher in all cases. Analysis of 13 cases showed 2 cases sensitized to Ani s 4 and moderate or higher anaphylaxis, while Ani s 4-sensitized patients were reported to be more likely to develop severe disease. It is possible that the patients sensitized to Ani s 4 need to be careful about the severity of their allergic symptoms.

The role of pain catastrophizing in pain perception among patients with rheumatoid arthritis without clinical signs of inflammation.

OBJECTIVE: Pain in rheumatoid arthritis (RA) is considered to be associated with non-inflammatory factors, including physical disabilities, psychiatric disorders, and pain catastrophizing (PC). PC is reportedly a key driver in the development of pain in patients with RA without clinical signs of inflammation; however, previous studies enroled patients with RA who were potentially in an inflammatory state. Hence, our aim was to investigate the role of PC as the possible link between pain, physical disabilities, and psychiatric disorders in patients with RA without clinical signs of inflammation. MATERIALS AND METHODS: In this cross-sectional study, 81 patients with RA without clinical signs of inflammation were included; all patients had serum C-reactive protein levels <0.5 mg/dL, without any inflammatory joints. We examined the demographic and clinical data and administered the pain visual analogue scale (VAS), pain catastrophizing scale (PCS), Health Assessment Questionnaire Disability Index (HAQ-DI), and patient version of the Brief Scale for Psychiatric Problems in Orthopaedic Patients (pBS-POP). A series of multivariate-adjusted multiple regression analyses were performed to examine the associations between PC and pain intensity, physical disabilities, and psychiatric disorders. RESULTS: We found associations between all the above-mentioned variables in separate models with HAQ-DI, pBS-POP, and PCS scores. However, in the model associated with pain VAS, the PCS score (ß = 0.34, p = 0.0073) emerged as the only variable showing a statistically significant association. CONCLUSIONS: PC is associated with pain in patients with RA without clinical signs of inflammation, and this association may be mediated through pathways involving physical disabilities and psychiatric disorders.

Quantitative bone SPECT/CT for evaluating treatment response in patient with sternoclavicular arthritis.

We report here a case of sternoclavicular arthritis due to SAPHO syndrome in a 60-year-old female in which quantitative values determined using bone SPECT/CT were useful to evaluate response. After celecoxib and alendronate sodium hydrate therapy, the chief complaints were well relieved and post-treatment Tc-99m HMDP bone SPECT/CT examination showed decreased uptake. The maximum standardized uptake value (SUV), peak SUV, mean SUV, metabolic bone volume, and total bone uptake of the untreated lesion were 18, 16, 10, 17 mL, and 180, respectively, which were decreased to 8, 7, 5, 15 mL, and 75, respectively, after the treatment. In comparison with pre-treatment situation, those parameters were decreased by -56%, -56%, -50%, -12%, and -58%, respectively, following celecoxib and alendronate sodium hydrate therapy, likely reflecting treatment response. Quantitative bone SPECT/CT may be useful to evaluate joint inflammatory activity and treatment response in a patient with osteoartritis.

Clinical value of the threshold derived from bone single-photon emission computed tomography/computed tomography in differentiating sternoclavicular arthritis.

OBJECTIVE: With the recent improvements in the quantitative accuracy of single-photon emission computed tomography (SPECT)/ computed tomography (CT), the value of using standardized uptake value (SUV) in bone SPECT/CT for quantitative assessment has been reported.We established a threshold for inflamed and normal areas of the sternoclavicular joint and examined the clinical value of bone SPECT/CT. SUBJECTS AND METHODS: The threshold between the inflamed and normal areas of the sternoclavicular joint was initially calculated. The diagnostic performance of the calculated threshold was subsequently compared with the visual assessment of the whole-body image. The clinical value of the threshold was examined in cases of ambiguous visual assessment and a sub-analysis with pustuloticarthro-osteitis (PAO) patients was done. RESULTS: The threshold between the inflamed and the normal area in the 93 sternoclavicular joints of 51 patients was 4.46. The area under the ROC curve (AUC), accuracy, sensitivity, and specificity of SUVmax for differentiating sternoclavicular arthritis were 0.92, 0.86, 0.88, and 0.85, respectively. Similarly, the AUC of visual assessment were 0.87, and the difference was not significant (P=0.11). In 25 patients with PAO, the AUC, accuracy, sensitivity, and specificity of SUVmax were 0.94, 0.90, 0.96, and 0.84, respectively with a significant higher AUC of visual assessment (0.82, P=0.032). Furthermore, for cases where there was ambiguous uptake upon visual assessment, the accuracy, sensitivity, and specificity of SUVmax were 0.84, 1.00, and 0.71, respectively, which was useful to judge regarding the initiation of treatment. CONCLUSION: Quantitative assessment using SUVmax and the threshold found using bone SPECT/CT for the presence of sternoclavicular arthritis is clinically useful and can be a useful tool for the initiation of treatment, especially in PAO patients.

Quantitative 67Ga-citrate SPECT/CT for evaluating disease activity in patients with interstitial lung disease.

OBJECTIVE: To determine whether results of a standardized uptake value (SUV)-based semi-quantitative analytic method for gallium-67 (67Ga)-citrate single photon emission tomography/computed tomography (SPECT/CT) reflects disease activity in patients with interstitial lung disease. SUBJECTS AND METHODS: Gallium-67-citrate SPECT/CT was used to evaluate disease activity in 24 patients with interstitial pneumoniaon clinical grounds at a single institution from June 2018 to August 2020. SUV in a given volume of interest over the bilateral pulmonary parenchyma was calculated using a dosimetry software package. Correlations of maximum SUV (SUVmax) and mean SUV (SUVmean) with clinical factors, including KL-6, lactate dehydrogenase (LDH), and C-reactive protein (CRP), were evaluated in all 24, as well as in 15 patients with spirometry results using Pearson's rank correlation test. RESULTS: The mean bilateral pulmonary SUVmax value showed a moderately significant correlation with KL-6 (Pearson's correlation coefficient r=0.51, P=0.012) and LDH (r=0.51, P=0.010), a weak non-significant correlation with DLCO% (r=-0.26, P=0.34), and no correlation with CRP (r=-0.01, P=0.94), FVC% (r=0.11, P=0.71), or FEV1.0% (r=0.14, P=0.62). Eleven patients with high KL-6 (≥1000U/mL) were defined as having disease activity. Maximum SUV sensitivity, specificity, and accuracy for predicting interstitial lung disease activity were 72.7%, 76.9%, and 75.0%, respectively, with a best cut-off value of 3.78. CONCLUSION: Semi-quantitative values obtained with 67Ga-citrate SPECT/CT showed a moderate correlation with KL-6 and moderate diagnostic performance for predicting disease activity of interstitial lung disease. It is rather unlikely that quantitative 67Ga-citrate SPECT/CT will have a role into the algorithm of interstitial lung disease.

Recurrent myocardial infarction associated with vasculo-Behçet's disease: a case report.

A 39-year-old Japanese man presented with chest oppression in February 2017. Electrocardiogram showed ST-elevation myocardial infarction (MI), and cardiac catheterisation revealed thrombotic occlusion of the right coronary artery (RCA), which was treated with thrombectomy, and he received warfarin. Three days after discharge, he complained of chest oppression again, and re-cardiac catheterisation showed thrombi occlusion of the circumflex artery (LCX) and 90% stenosis with thrombosis in the proximal site of the anterior descending artery (LAD) and RCA. Drug eluting stent was implanted in the LAD and RCA; aspirin and prasugrel hydrochloride were added to warfarin. Before discharge, coronary computed tomography angiography (CTA) found new thrombi in the RCA, LAD, and LCX, and he was referred to our hospital on suspicion of Behçet's disease (BD). Past medical history was notable for recurrent aphthous stomatitis, a pudendal ulcer, and Crohn's disease, for which he had been taking infliximab (5 mg/kg) every 8 weeks until December 2016. Notably, his C-reactive protein (CRP) level increased before and after each MI, suggesting that the thrombi were caused by inflammation. Consequently, we concluded that his abnormalities were manifestations of vasculo-BD. After 3 days of hospitalisation, treatment with prednisolone and colchicine was started. His CRP and D-dimer levels decreased, and coronary CTA after 8 days showed disappearance of the thrombi. We tapered the prednisolone dose, and cardiovascular events have not been observed for 7 months after the treatment initiation. In summary, we report a rare case of MI associated with vasculo-BD and review the relevant literature.

A retrospective analysis of 7 cases of familial mediterranean fever.

BACKGROUND: Familial mediterranean fever (FMF) is a single inherited autoinflammatory disease characterized by periodic fever with relatively short duration of 1 to 3 days and sterile serositis. Although the prevalence rate is highest in the Mediterranean coastal area, a large number of cases have been reported recently by genetic analysis by identification of MEFV (Mediterranean fever) which is responsible gene in Japan too. In outpatient department of rheumatology, diagnosis and treatment of FMF is performed in cases where fever and abdominal pain attack are repeated for a short period of time. PATIENTS AND METHODS: We examined cases in which symptoms considered periodic seizures were repeated, excluding autoimmune diseases, infectious diseases, and malignant tumors. In both cases, genetic analysis is performed as auxiliary diagnosis. RESULTS: Seven cases satisfied the Tel-Hashomer criteria criteria and MEFV gene mutation was detected. Everyone was a female, and half had seizure symptoms at menstruation. Even though there is a difference in the amount of colchicine to be used, either one is effective. CONCLUSION: In cases of periodic symptoms or cases called periodic fever, exclusion diagnosis is carried out, there is a need to suspect FMF, determine the effect of colchicine, and perform genetic analysis.

[A case of anti-PL-7 antibody positive polymyositis with thrombotic microangiopathy].

  A 65-year-old woman with a 17-year history of polymyositis and 8-year history of rheumatoid arthritis who was treated with a low dose of prednisolone and tacrolimus (Tac) was admitted to our hospital because of general malaise and hypertension. Blood tests showed thrombocytopenia, hemolytic anemia with fragmented erythrocytes, and hypercreatinemia. Based on these clinical features, she was diagnosed with thrombotic micro-angiopathy (TMA). Thrombocytopenia and hemolytic anemia with fragmented erythrocytes improved with the discontinuation of Tac and plasma exchange; however, hypertension and renal dysfunction persisted. TMA due to calcineurin inhibitor (CNI) nephropathy was suspected based on the histopathological findings of renal biopsy. However, the condition was atypical of a CNI nephropathy because the trough level of Tac was lower than that reported previously and renal dysfunction persisted after drug discontinuation. She had mild sclerodactylia and Raynaud's symptoms, although the diagnostic criteria for systemic sclerosis (SSc) were not satisfied. Moreover, the patient tested positive for anti PL-7 antibody. The relationship between anti PL-7 antibody and pathogenesis of SSc has been reported. In this case, it was suspected that CNI nephropathy worsened because of the potential basic factors of SSc. These findings indicate that TMA may occur in patients testing positive for anti PL-7 antibody who are treated with Tac.

[A case of axial spondyloarthritis acute onset as opportunity tonsil foci infection].

  A 49-year-old female with a chief complaints of arthralgia, and a medical history is Hashimoto's disease presented to us. She had been previously treated for Sjögren's syndrome at our hospital. She had anterior chest and polyarticular pain. On admission, her blood test results were as follows: white blood cells, 12700/µl; C reactive protein, 24.8 mg/dl; erythrocyte sedimentation rate 122 mm/h, Anti-streptolysin O, 1179 IU/ml;an, ASK, 10240. She had tenderness in both her hand and finger joints, recurrent episodes of tonsillitis and pustular eruption. Her imaging studies were remarkable for inflammation of the sacroiliac joint and bone erosion of the hand joint, among other findings. We considered a diagnosis of either axial spondyloarthritis or synovitis acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome due to an opportunistic tonsillar infection. The differential diagnosis between axial spondyloarthritis or SAPHO syndrome is difficult to make. We discuss this case in the context of previous literature.

A case of familial Mediterranean fever who complained of periodic fever and abdominal pain diagnosed by MEFV gene analysis.

Familial Mediterranean fever (FMF) is a hereditary autoinflammatory disease caused by Mediterranean FeVergene (MEFV) mutations on Chromosome 16, and characterized by periodic fever of and serositis. FMF is the result of gain-of-function mutations in pyrin that lead to interleukin-1ß activation. FMF can be classified as "typical" and "atypical" types based on clinical finding and genetic screening. Although MEFV genotyping has enabled FMF to be confirmed in some cases, the diagnosis remains predominantly clinical since genotyping has shown that the disease is characterized by variable manifestations in Japanese. In 1976, the first report performed on the case of Japanese FMF with periodic fever of and serositis. Since 2002, genetic analyses are performed on Japanese FMF patients by K. Shiozaki et al. and N. Tomiyama et al. In our case, she was a 25-year-old Japanese woman with at periodic fever and abdominal pain. MEFV gene analysis demonstrated a heterozygous mutation of variant M694I, leading to a diagnosis of FMF. After the increase dose (up to 3 mg/day) of colchicine, periodic fever and abdominal pain disappeared. It is the important candidate of FMF for differential diagnosis with unexplained periodic fever and serositis, such as our case.

Reliability and clinical utility of Enzyme-linked immunosorbent assay for detection of anti-aminoacyl-tRNA synthetase antibody.

Anti-aminoacyl-tRNA synthetase (ARS) antibody is one of the myositis-specific autoantibodies to make a diagnosis of polymyositis (PM) and dermatomyositis (DM). Recently a new enzyme-linked immunosorbent assay (ELISA) kit of concurrently detected anti-ARS antibodies (anti-Jo-1, anti-PL-7, anti-PL-12, anti-EJ and anti-KS) have become to measure in the clinical setting. To evaluate the reliability of this ELISA kit, we measured anti-ARS antibodies in 75 PM and DM patients using by this ELISA assay and compared them with the results by RNA immunoprecipitation assay. Between the measurements of anti-PL-7, anti-PL-12, anti-EJ and anti-KS autoantibodies by ELISA assay and RNA-IP assay, the concordance rate of reproducibility is 95.1% and the positive agreement rate is 90.9% and negative agreement rate is 96.0% and kappa statistic is 0.841. Between the measurements of existing anti-Jo-1 antibody ELISA kit and anti-ARS antibody ELISA kit, the concordance rate of reproducibility is 96.9%, the positive agreement rate is 100%, negative agreement rate is 96.1% and kappa statistic is 0.909. The lung involvement in patients with PM and DM patients are positive of anti-ARS antibodies and anti-melanoma differentiation associated gene5 (MDA5) antibody at a rate around 70%. Then most life-threatening ILD with anti-MDA5 positive clinically amyopathic dermatomyositis patients could be highly guessed when anti-ARS antibodies are negative.

A case of anti-aminoacyl tRNA synthetase antibody syndrome complicated by hemophagocytic syndrome.

A 48-year-old woman had suffered from a fever and general fatigue, and visited the other hospital for fever elevation in November 2013, at which time interstitial lung disease was revealed. In January 2014, she experienced an eruption in the hand and developed peripheral blood flow damage. Under a diagnosis of adult Still's disease, the patient was administered 0.5 mg of betamethasone as well as cyclosporin at 75 mg/day. In November 2014, general fatigue, fever, and headache were noted, while MRI revealed an enlarged hypophysis and laboratory findings were positive for the anti-pituitary cell antibody, thus a diagnosis of autoimmune hypophysitis was made. Although disease activity was low, she requested hospitalization and was admitted by the Division of Endocrinology and Metabolism at our hospital in May 2015, though only observed. Fever developed again, along with interstitial lung disease, Raynaud's phenomenon, and pain in the crural area again, and we considered the possibility of another disease. After stopping administration of betamethasone and cyclosporin, we made a diagnosis of anti-aminoacyl tRNA synthetase antibody syndrome, and administered methylprednisolone at 500 mg for 3 days as well as prednisolone at 35 mg/day following steroid pulse therapy. Although her condition soon improved, fever, muscle pain, and pancytopenia returned after 3 days. Bone marrow findings revealed the existence of hemophagocytosis, for which we again gave methylprednisolone at 500 mg for 3 days and cyclosporin at 125 mg/day. Thereafter, the patient recovered and was discharged from the hospital.

Quantitative evaluation of myocardial function by a volume-normalized map generated from relative blood flow.

Our study aimed to quantitatively evaluate blood flow in the left ventricle (LV) of apical hypertrophic cardiomyopathy (APH) by combining wall thickness obtained from cardiac magnetic resonance imaging (MRI) and myocardial perfusion from single-photon emission computed tomography (SPECT). In this study, we considered paired MRI and myocardial perfusion SPECT from ten patients with APH and ten normals. Myocardial walls were detected using a level set method, and blood flow per unit myocardial volume was calculated using 3D surface-based registration between the MRI and SPECT images. We defined relative blood flow based on the maximum in the whole myocardial region. Accuracies of wall detection and registration were around 2.50 mm and 2.95 mm, respectively. We finally created a bull's-eye map to evaluate wall thickness, blood flow (cardiac perfusion) and blood flow per unit myocardial volume. In patients with APH, their wall thicknesses were over 10 mm. Decreased blood flow per unit myocardial volume was detected in the cardiac apex by calculation using wall thickness from MRI and blood flow from SPECT. The relative unit blood flow of the APH group was 1/7 times that of the normals in the apex. This normalization by myocardial volume distinguishes cases of APH whose SPECT images resemble the distributions of normal cases.