Sonneruiz1115

AL amyloidosis (AL) is a systemic disorder due to extracellular tissue deposition of amyloid fibrils, composed of immunoglobulin light chains. Since the description of AL involving temporal arteries in 1986, this disorder has been known as one of the differential diagnoses of giant cell arteritis (GCA). We encountered a case of an elderly female presenting with headache and tender and enlarged temporal arteries, that was pathologically diagnosed with temporal artery involvement of AL due to Bence-Jones-type MM. To our knowledge, this was the first case of AL with temporal artery involvement in Japan, that presented with GCA-like features. Literature review of AL cases with temporal artery involvement showed close similarity between these disorders, but suggested that vasculature involvement (extremity claudication, kidney or heart), macroglossia, carpal tunnel syndrome and normal or low ( less then 0.5 mg/dL) CRP levels may predict AL rather than GCA. Physicians should keep in mind that AL involving temporal arteries can be a pitfall in the diagnosis of GCA, as seen in our and previous cases.Rat-bite fever (RBF) is characterized by fever, rash and arthritis, mainly caused by Streptobacillus moniliformis. We present a case of inflammatory erosive polyarthritis with sepsis caused by Streptobacillus notomytis, a novel species isolated from S. moniliformis. A 67-year-old man presented with fever, purpura and peripheral arthritis. After blood cultures were performed, loxoprofen administration was initiated. Arthritis partially improved, but left first metatarsophalangeal (MTP) arthritis persisted. A gram-negative rod was detected in the blood culture, and meropenem administration was started that improved arthritis. Ultrasonography and computed tomography revealed bone erosion in the left first MTP joint, leading to the diagnosis of septic arthritis. 16S rRNA gene sequence analysis revealed that this strain was S. notomytis. Further questioning revealed the patient may have had contact with rats. It is essential to consider Streptobacillus infection in the differential diagnosis of erosive polyarthritis.A 30-year-old female patient had been administered 5-mg/day prednisolone for systemic lupus erythematosus. She developed hypertension, dyslipidemia, moon face, central obesity, hypertrichosis, and impaired glucose tolerance. Although iatrogenic Cushing syndrome was initially suspected, we made a diagnosis of Cushing syndrome caused by a right adrenal adenoma, on the basis of the endocrine function test result and imaging findings. After surgery, the Cushingoid signs disappeared. Autoimmune diseases are often treated with corticosteroids; therefore, a differential diagnosis of primary Cushing syndrome should be made adequately.Eosinophilic myositis is characterised by peripheral blood eosinophilia and eosinophilic infiltration in muscles, and is comprised three subtypes focal eosinophilic myositis (FEM), eosinophilic polymyositis, and eosinophilic perimyositis. Muscle involvement of FEM is usually limited to the lower legs, and pulmonary complications have not been reported. selleck compound We report a rare case of FEM in the left adductor pollicis complicated by lung lesions. A 37-year-old woman developed swelling of the first web space in the left hand. Magnetic resonance imaging (MRI) of the left hand demonstrated increased signal on fat-suppressed T2-weighted imaging in the left adductor pollicis. A muscle biopsy specimen demonstrated perimysial and endomysial infiltration of mononuclear cells and eosinophils, and necrosis and regeneration of muscle fibres. Chest computed tomography (CT) revealed upper-lobe-dominant ground-glass opacities (GGO). Both focal myositis and pulmonary lesions improved without treatment. This case suggests that FEM could involve adductor pollicis and have pulmonary lesions. In this case, myositis and GGO resolved spontaneously. Some FEM cases treated with glucocorticoids were reported in the past. Further studies are required to determine whether patients with FEM require therapeutic intervention.Antiphospholipid antibody syndrome (APS) is defined by the presence of clinical symptoms caused by antiphospholipid antibodies. When APS occurs during pregnancy, it is conventionally treated with low-dose aspirin or heparin. In cases refractory to conventional treatment, intravenous immunoglobulin (IvIg) is sometimes added. We present the case of an APS patient with severe thrombocytopenia who experienced a successful pregnancy after treatment that included intravenous rituximab and IvIg. As far as we know, this is the first report demonstrating a positive pregnancy outcome in this context. Physicians may consider prescribing not only IvIg but also rituximab during the first trimester of pregnancy in APS patients with severe obstetrical complications and thrombocytopenia refractory to conventional treatment.Intravenous immunoglobulin (IVIG) is a standard therapy for Kawasaki disease (KD), because it prevents formation of coronary artery aneurysm, a major complication of KD. Herein, we report a 3-year-old boy with KD complicated by haemolytic anaemia (HA) which developed following two courses of IVIG. Although both direct and indirect antiglobulin tests and anti-M antibodies were positive in his blood obtained after the onset of HA, indirect antiglobulin tests and anti-M antibodies were negative either in the blood sample before the treatment or the same lot of IVIG products as those used for the therapy, suggesting autoimmune mechanism. This is, to our knowledge, the first report of autoimmune HA caused by anti-M autoantibodies after IVIG therapy in KD.Despite considerable research effort, there is a significant need for safe agents that stimulate bone formation. Treatment of large or complex bone defects remains a challenge. Implantation of small molecule-induced human bone marrow-derived mesenchymal stromal cells (hBMSCs) on an appropriate tricalcium phosphate (TCP) scaffold offers a robust system for noninvasive therapy for spinal fusion. To show the efficacy of this approach, we identified a small molecule curcuminoid that when combined with TCP ceramic in the presence of hBMSCs selectively induced growth of bone cells after 8- or 25-day incubations, alkaline phosphatase was elevated. Treatment of hBMSCs with curcuminoid 1 and TCP ceramic increased osteogenic target gene expression (i.e., Runx2, BMP2, Osteopontin, and Osteocalcin) over time. In the presence of curcuminoid 1 and TCP ceramic, osteogenesis of hBMSCs, including proliferation, differentiation, and mineralization, was observed. No evidence of chondrogenic or adipogenic potential using this protocol was observed.