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Objective The early diagnosis of rheumatoid arthritis (RA) improves disease outcomes. Using bilateral magnetic resonance imaging (MRI), we investigated whether or not tenosynovitis at the level of the metacarpophalangeal (MCP) and wrist joints, as well as non-symmetrical versus symmetrical involvement, predicts RA development in undifferentiated arthritis (UA) patients. Methods We collected the clinical and serological findings as well as bilateral gadolinium-enhanced 1.5-T MRI data of UA patients after 1 year. A multivariate logistic regression analysis was used to determine the association of tenosynovitis in UA with RA development. Ninety-one UA patients from the Nagasaki Early Arthritis Clinic who did not meet the 2010 European League Against Rheumatism/American College of Rheumatology classification criteria for RA were selected. Tenosynovitis at the MCP and wrist joints was scored according to the RA MRI scoring system. Results Of these 91 UA patients, 29 (31.9%) progressed to RA, with a median disease duration of 3 months, despite only 10.9% being positive for anti-cyclic citrullinated peptide antibody (ACPA). A univariate analysis showed higher MCP tenosynovitis scores, MCP flexor tenosynovitis, and symmetrical MCP tenosynovitis in the RA development group than in the non-development group (p<0.05). A multivariate analysis showed that symmetrical MCP tenosynovitis was independently associated with RA development after adjusting for age, gender, swollen joint count, C-reactive protein level, and ACPA positivity (odds ratio: 4.96). The presence of symmetrical MCP tenosynovitis had low sensitivity (35%) but high specificity (87%) for RA development. Conclusion MRI-detected tenosynovitis, especially symmetrical findings at the MCP joint, is predictive of RA development in a UA population with low ACPA positivity.

We encountered a 78-year-old Japanese man with IgG4-related sialoadenitis complicated with marked eosinophilia. We diagnosed him with IgG4-RD (related disease) with a submandibular gland tumor, serum IgG4 elevation, IgG4-positive plasma cell infiltration, and storiform fibrosis. During follow-up after total incision of the submandibular gland, the peripheral eosinophil count was markedly elevated to 29,480/μL. The differential diagnosis of severe eosinophilia without IgG4-RD was excluded. The patient exhibited a prompt response to corticosteroid therapy. His peripheral blood eosinophil count was the highest ever reported among similar cases. We also review previous cases of IgG4-RD with severe eosinophilia.

A 54-year-old woman developed drop head syndrome (DHS), Raynaud's phenomenon and creatine kinase (CK) elevation. She did not meet the international classification criteria of dermatomyositis/polymyositis, as we observed no muscle weakness, grasping pain or electromyography abnormality in her limbs, and anti-aminoacyl tRNA synthetase (ARS) antibody was negative. Cervical magnetic resonance imaging and a muscle biopsy of the trapezius muscle revealed myositis findings as the only clinical observations in muscle. These findings, along with her anti-U1-ribonucleoprotein (RNP) antibody positivity and leukopenia, resulted in a diagnosis of mixed connective tissue disease (MCTD). Prednisolone treatment significantly improved her myositis. To our knowledge, this is the first report of DHS as the only muscle complication of MCTD.

A 71-year-old woman being treated with methotrexate (MTX) and tacrolimus (TAC) for rheumatoid arthritis (RA) was admitted to our hospital and underwent surgery for gastric perforation and peritonitis. An endoscopic examination six days post-surgery showed an extensive ulcer in the stomach, and a biopsy revealed diffused large B-cell lymphoma. We diagnosed her with immunodeficiency-associated lymphoproliferative disorder (LPD) and discontinued the MTX and TAC. She underwent gastrectomy due to stenosis approximately two months after the first operation, but the histopathological findings of lymphoma had disappeared. LPD should be considered as a potential cause of gastric perforation during RA treatment.

A 53-year-old man with recurrent episodes of large joint pain and a low-grade fever at irregular intervals for 16 years developed right knee and ankle arthralgia, watery diarrhea, and abdominal pain. Following an ileum and colon biopsy, he was diagnosed with gastrointestinal amyloidosis. We suspected familial Mediterranean fever (FMF) based on his history and administered colchicine; his symptoms subsequently improved. Thus, he was diagnosed with atypical FMF. After tocilizumab administration, the amyloid deposits disappeared. This case suggests that physicians should consider FMF even in cases with atypical symptoms in order to prevent the progression of amyloidosis and that amyloid deposits can be eliminated by interleukin (IL)-6 inhibition.

A 70-year-old woman presented with a fever and pain in both lower extremities and the right shoulder and right upper arm continuously for approximately 3 months. 18 F-fluorodeoxyglucose-positron emission tomography/computed tomography ( 18 F-FDG/PET-CT) revealed the accumulation of FDG in the right shoulder, lumbar spinous processes, both ischial tuberosities, and both hips and greater trochanters, indicating polymyalgia rheumatica (PMR). In addition, upper gastrointestinal endoscopy revealed esophageal carcinoma. After endoscopic submucosal dissection was performed, her musculoskeletal symptoms spontaneously improved, and the 18 F-FDG/PET-CT findings decreased. In light of these findings, she was diagnosed with paraneoplastic syndrome. When atypical features of PMR, such as asymmetric symptoms occur, we should search for malignancies.

A 44-year-old Japanese man with a 14-year history of limited cutaneous systemic sclerosis (SSc) was admitted with a fever, hypertension, anemia, thrombocytopenia, and renal dysfunction. On admission, hypertension, hyperreninemia, acute renal dysfunction, hemolytic anemia, and thrombocytopenia led to the diagnosis of scleroderma renal crisis (SRC) complicated with thrombotic microangiopathy (TMA). The patient had also been infected with influenza B virus almost six days before admission. Following treatment with plasma exchange, an angiotensin-converting enzyme inhibitor, and an anti-virus agent, his general condition improved. He had no risk factors for SRC. In SSc patients, an influenza virus infection might trigger SRC complicated with TMA.

A 56-year-old Japanese woman with muscle weakness, increased creatine kinase and aldolase levels, and characteristic cutaneous lesions was diagnosed with anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5 antibody)-positive dermatomyositis. She also had interstitial lung disease (ILD). After corticosteroid and tacrolimus combination therapy was started, bicytopenia and elevated serum ferritin and transaminase emerged. Because the bone marrow tissues were hypoplastic with hemophagocytes, she was diagnosed with concomitant autoimmune-associated hemophagocytic syndrome (HPS). Intravenous cyclophosphamide pulse therapy and plasmapheresis were performed. The laboratory findings indicated improved abnormalities, and the ILD did not progress. Anti-MDA5 antibody-positive dermatomyositis can be complicated by HPS.

A 70-year-old Japanese woman presented to our hospital with gait disturbance and cognitive dysfunction. Since she had arthritis, lymphopenia, hypocomplementemia, and anti-nuclear and anti-double-stranded DNA antibodies, she was diagnosed with systemic lupus erythematosus (SLE). T2-weighted magnetic resonance imaging revealed bilateral hyperintensities in the putamen. Based on her cognitive impairment, muscle rigidity, and high levels of interleukin-6 in the cerebrospinal fluid, we believed she had developed a complication of a neuropsychiatric disease and administered corticosteroids and intravenous cyclophosphamide therapy. Her cognitive function fully recovered, and her gait disturbance improved. Attending to cognitive impairment in elderly SLE patients is necessary.

A 72-year-old Japanese woman diagnosed with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis was admitted to our hospital with hearing loss, temporal pain, and sudden blindness. We finally diagnosed recurrent granulomatosis with polyangiitis and initiated methyl-prednisolone pulse therapy (1,000 mg) followed by prednisolone (30 mg/day) and rituximab (RTX). After the third RTX administration, she developed bloody stools along with acute thrombocytopenia and low complement levels. We diagnosed rituximab-induced acute thrombocytopenia (RIAT), and her platelet counts spontaneously recovered. This case suggests that after RTX therapy RIAT may sometimes cause severe thrombocytopenia, and that monitoring the complements may be useful for making an early diagnosis of RIAT.

A 67-year-old Japanese man was diagnosed with granulomatosis with polyangiitis based on the presence of right maxillary sinusitis, proteinase 3 antineutrophil cytoplasmic antibody positivity, and right scleritis. A conjunctival biopsy specimen showed neutrophil-predominant infiltration around the vessels without granuloma. Because there was a risk of blindness, pulsed methylprednisolone and intravenous cyclophosphamide pulse therapy (IVCY) were started. However, it was ineffective, and peripheral ulcerative keratitis newly emerged. We promptly switched the treatment from IVCY to rituximab, and ophthalmologists performed amniotic membrane transplantation, which avoided blindness. The close and effective working relationship between physicians and ophthalmologists improved our patient's ocular prognosis.

A 65-year-old man was diagnosed with granulomatosis with polyangiitis (GPA) based on the detection of high myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA), vasculitis and granulomas in a lung biopsy specimen and crescentic glomerulonephritis in a kidney biopsy specimen. Soon after the initiation of intravenous methylprednisolone pulse therapy (mPSL pulse) and intravenous cyclophosphamide pulse therapy (IVCY), the patient experienced cough and hemoptysis. Based on emerging anemia and bilateral diffuse lung consolidation on computed tomography, we judged that diffuse alveolar hemorrhage (DAH) was complicated by GPA. The patient's DAH improved following additional mPSL pulse and IVCY. Physicians should be aware of the possible occurrence of DAH, even when a patient's symptoms improve after mPSL pulse and IVCY.

A 78-year-old woman diagnosed with cyclic neutropenia 5 years previously had been treated with recombinant granulocyte-colony stimulating factor (G-CSF). She developed fever, tenderness and distension of temporal arteries after the treatment with G-CSF. Magnetic resonance imaging and ultrasonography revealed wall thickening of the temporal arteries. She was therefore diagnosed with giant cell arteritis (GCA). Small vessel vasculitis has been reported as a complication of G-CSF. However, the development of large vessel vasculitis after G-CSF treatment is quite rare. To our knowledge, the present case is the first report of GCA suspected to be associated with coexisting cyclic neutropenia and G-CSF treatment.

A 55-year-old man was diagnosed with remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome. Contrast-enhanced computed tomography for cancer screening showed a mass with low-density centers with an enhanced rim in the left iliopsoas muscle. We suspected an iliopsoas abscess and performed computed-tomography-guided puncture of the mass. Both Gram staining and the culture of the fluid were negative. We diagnosed the patient with RS3PE syndrome with iliopsoas bursitis and administered low-dose corticosteroids without antibiotics. The symptoms, including left hip pain, quickly disappeared following treatment. Clinicians should be aware that iliopsoas bursitis may resemble an iliopsoas abscess. As a result, it is important to make an accurate differential diagnosis.

A 69-year-old man was diagnosed with granulomatosis with polyangiitis (GPA) based on the presence of skin granuloma, refractory otitis media, renal insufficiency and myeloperoxidase-antineutrophil cytoplasmic antibody positivity and slight lung opacity. He was treated with high-dose corticosteroid therapy. Despite the initial improvement of his renal function and a decrease in his C-reactive protein level, he suffered from an alveolar hemorrhage one week after the start of corticosteroid therapy. An anti-dsDNA antibody test was positive and the patient had hypocomplementemia. Elements of both GPA and systemic lupus erythematosus were thought to have affected his clinical course.

A 73-year-old man with a history of myasthenia gravis (MG) was diagnosed with rheumatoid arthritis (RA) based on a history of polyarthritis and positivity for anti-citrullinated protein antibodies (ACPA). He presented with a high level of serum vascular endothelial growth factor (VEGF) and RS3PE syndrome-like pitting edema in the extremities, which improved following treatment with low-dose prednisolone. This is an interesting case of ACPA-positive RA associated with RS3PE syndrome-like pitting edema and a high VEGF level.

We herein report the case of a 21-year-old woman with refractory adult-onset Still's disease who developed central venous catheter-related methicillin-resistant Staphylococcus aureus sepsis during aggressive immunosuppressive therapy. She subsequently experienced septic pulmonary embolism (SPE) and sacroiliitis during treatment with intravenous vancomycin and was successfully treated with long-term oral linezolid therapy. This case suggests that the occurrence of methicillin-resistant Staphylococcus aureus infection in immunosuppressive patients can trigger severe clinical manifestations such as SPE and septic sacroiliitis and that linezolid is suitable for treating such conditions.

We herein describe a 71-year-old woman with adult-onset Still's disease (AOSD) who developed fever, myalgia, and pancytopenia. The bone marrow aspiration and muscle biopsy revealed hemophagocytic syndrome (HPS) and inflammatory myopathy with abundant macrophages (IMAM). Immunostained specimens were positive for expression of retinoic acid-inducible gene-I (RIG-I), which recognizes viral RNA in infiltrated mononuclear cells as well as muscle tissues. These findings suggest that RIG-I may be involved in induction of HPS and IMAM in AOSD.

Type B insulin resistance is characterized by the appearance of autoantibodies to the insulin receptor. We present a 59-year-old Japanese man with type B insulin resistance complicated with systemic lupus erythematosus (SLE). A high titer of anti-insulin receptor autoantibodies was revealed when SLE was defined as active disease. Intravenous boluses of cyclophosphamide (IVCY) with oral prednisolone and cyclosporin A induced remission of SLE, and a subsequent disappearance of anti-insulin receptor autoantibodies, followed by a recovery of glucose intolerance. This is a rare and important case report showing a clear correlation between anti-insulin receptor autoantibodies of type B insulin resistance and SLE disease activity.

Objective: Mizoribine (MZR) is an immunosuppressant that inhibits nucleic acid metabolism and is a relatively safe disease-modifying anti-rheumatic drug (DMARD). We evaluated the efficacy and safety of one single dose per day for patients with rheumatoid arthritis (RA)., Patients and Methods: In this study 32 patients with RA received MZR therapy. We evaluated the average dose of MZR and prednisolone, response to treatment and peak plasma level of MZR., Results: The average dose of MZR was 146.1±31.2 (range: 50-200) mg/day. The average dose of prednisolone was 4.63±3.59 (range: 0-14) mg/day. The average plasma level of MZR, measured after 3 hours, was 2.20±0.49 µg/mL in the responder group and 1.59±0.82 µg/mL in the non-responder group (p=0.020). The treatment with MZR for 24 weeks was completed by 71.9% of patients and the proportion of patients who achieved a good and moderate response rate according to the European League Against Rheumatism (EULAR) criteria was 56.3% at 24 weeks. The plasma level of MZR which was greater than or equal to 2.12 µg/mL was significantly correlated with the clinical response (p<0.01). Only one of thirty-two cases discontinued the treatment, because of skin eruption., Conclusion: This study included patients that could not be treated with other DMARDs and/or biologic agents because of age, interstitial pneumonia and other complications. We show that MZR may be a useful and relatively safe therapy for patients in this group.

The most common renal manifestation of Sjögren's syndrome is tubulointerstitial nephritis, and glomerular disease is rare (3). A 62-year-old woman with primary Sjögren's syndrome developed nephrotic syndrome. Kidney biopsy was consistent with membraneous glomerulonephritis. Steroid pulse therapy was not effective. Three months later she was diagnosed with non-Hodgkin's lymphoma of the tongue, and she was given CHOP therapy and radiation. Both the lymphoma and membraneous glomerulonephritis were resolved.

We have experienced a Takayasu arteritis (TA) patient, successfully treated with infliximab, who did not respond well to conventional therapy with glucocosteroid and methotrexate. Takayasu arteritis had developed in a 24-year-old woman (March 2003) who had been treated with glucocorticoid including methylprednisolone pulse therapy and methotrexate; however, she relapsed during the tapering of the dosage of oral prednisolone. Nineteen months after the first administration of glucocorticoid, 3 mg/kg of infliximab was introduced to the patient. The therapeutic efficacy of infliximab was markedly demonstrated; the patient's C-reactive protein (CRP) value returned to almost normal range with subsequent tapering of the dosage of oral prednisolone in the absence of further relapse. This is the first case presentation of TA in Japan successfully treated with infliximab.

We have studied the serology of 6 patients with palindromic rheumatism. None of the patients fulfilled the classification criteria for rheumatoid arthritis at the entry; however, 4 out of the 6 patients were seropositive for IgM rheumatoid factor (IgM-RF) at entry. Sequential serological study was performed in 4 patients; IgM-RF changed from seronegative to seropositive in one patient, and the titer increased in another patient. Anti-cyclic citrullinated peptide antibody (anti-CCP Ab) at the entry was detected in only one of the 6 patients; that patient later developed RA. Although follow-up is necessary, the present study may suggest the importance of serological examination, especially anti-CCP Ab, in patients with palindromic rheumatism.

We report a 60-year-old woman with neurosarcoidosis. She was referred to our hospital for examination of the cause of pain in left Th4-6 dermatome. Chest X-ray and computed tomography (CT) revealed bilateral hilar and mediastinal lymphadenopathy, and her serum angiotensin converting enzyme (ACE) level was elevated. Histological finding of mediastinal lymph nodes consisted with sarcoidosis. Therefore, her pain was thought to be spinal root pain caused by neurosarcoidosis. With the administration of prednisolone, her symptom and bilateral hilar lymphadenopathy disappeared, and serum ACE level became normal. It is important to pay attention to neurosarcoidosis when patients show unknown spinal root symptom, although it is rare.

A 39-year-old man with no significant medical history was admitted to our hospital with severe abdominal pain and melena. Computed tomographic (CT) scans demonstrated superior mesenteric venous thrombosis. Although thrombolysis and anticoagulant therapy was started immediately, symptoms of strangulation ileus developed. Laparotomy was therefore performed and revealed necrotic stenosis of the ileum. The patient, his father and sisters showed low protein C levels. Direct sequencing analysis of their protein C gene revealed a heterozygous mutation at codon 169 corresponding to the cleavage site of the activation peptide, which was referred to as protein C Tochigi.

Limy bile is a rare condition characterized by excessive precipitation of calcium carbonate in the gallbladder. Cases of complicated hyperparathyroidism are extremely rare. There is only one reported case, where serum and urine calcium levels were high. On the other hand, the presence of limy bile in the common bile duct is also very rare, and has been reported in only 20 cases. We report a patient with obstructive jaundice in whom the initial abdominal radiography showed limy bile in the gallbladder and common bile duct, and laboratory values showed a high serum calcium level and highly sensitive PTH (HS-PTH) level.

Objective: There is an increasing number of accidents by erroneous ingestion of button batteries in recent years; the batteries arouse the interest of infants because of their attractive shape and luster. The batteries remaining in the gastrointestinal tract and discharging electric current over a long period of time may induce ulceration or perforation, thus must be carefully considered the selection of appropriate treatment., Methods: We remove erroneously ingested button batteries with two tubes with ferrite magnets nearly the same size as the button batteries themselves., Patients: Four cases of erroneous ingestion of button batteries., Results: We easily removed button batteries from the stomach within 5 minutes in all cases with two magnet-attached tubes., Conclusion: We present this battery removal device together with a literature review, because it seems convenient and useful.

We report a 65-year-old woman with thyrotropin (TSH) secreting pituitary adenoma, who was diagnosed based on the lack of inhibition of serum TSH despite an increased serum free thyroxine (T4), a low response of serum TSH to thyrotropin releasing hormone, and a pituitary tumor as revealed by magnetic resonance imaging. The pituitary adenoma was, however, inoperable due to chronic respiratory failure. The treatment with octreotide in a dose of 100 microg b.i.d. resulted in inhibition of serum TSH and free T4 to euthyroid levels and considerable shrinkage of the pituitary tumor. These effects were continued over 8 months after the start of octreotide therapy without any adverse effects. These findings add further evidence that octreotide is useful for treating inoperable TSH secreting pituitary adenoma.

A 42-year-old woman with biopsy-proven chronic hepatitis B, who had been treated with human leukocyte-derived interferon-alpha (huLe-IFN alpha) therapy for two months was found to have liver tumors on routine abdominal ultrasonography examination. She underwent laparotomy, and partial hepatectomy was performed under the clinical diagnosis of hepatocellular carcinoma. The lesions were diagnosed histologically as pseudolymphoma based on the massive infiltration of small mature lymphocytes and the presence of hyperplastic lymph follicles with germinal centers. Immunohistochemistry revealed polyclonal origin of the involved lymphocytes. The possible association between IFN alpha treatment and chronic hepatitis B with the development of pseudolymphoma is discussed.

Alpha-interferon was used for anti-cancer or anti-viral therapy in two patients with preexisting autoimmune thyroid disease and in seven patients with chronic viral hepatitis who had no history of thyroid dysfunction. Primary hypothyroidism developed in the two patients who had a history of autoimmune thyroid disease, while no changes in thyroid function were observed in the other seven patients. Modulation of the immune system by alpha-interferon may have been responsible for the development of hypothyroidism in these two patients. Therefore, autoantibodies to the thyroid and the thyroid function should be assessed in patients undergoing alpha-interferon therapy.