A case of rheumatoid arthritis complicated with mucous membrane pemphigoid.

Rheumatoid arthritis (RA) is a disease of unknown aetiology that causes irreversible joint destruction and has been known to present with not only various extra-articular symptoms, but also various autoimmune disorders. Mucous membrane pemphigoid (MMP) is a chronic autoimmune disease characterised by inflamed and eroded mucosa. The prognosis of MMP can be poor, so early diagnosis and prompt initiation of therapy are necessary for optimal management.

Anti-glomerular basement membrane antibody disease complicated by posterior reversible encephalopathy syndrome.

Anti-glomerular basement membrane (anti-GBM) antibody disease is a rare type of small-vessel vasculitis. Posterior reversible encephalopathy syndrome (PRES) is a syndrome of heterogeneous aetiologies grouped together based on similar neuroimaging findings. We report a rare case of a patient who received treatment for anti-GBM antibody disease who developed PRES.

Reactivation of cytomegalovirus predicts poor prognosis in patients on intensive immunosuppressive treatment for collagen-vascular diseases.

Reactivation of cytomegalovirus (CMV) occurs during intensive immunosuppressive therapies. However, the influence of CMV reactivation on prognosis in patients with immunosuppressive therapies for collagen-vascular diseases (CVD) is not fully understood. To determine whether CMV reactivation affects the prognosis of patients with CVD and to identify risk factors of CMV reactivation, we reviewed, retrospectively, the medical records of 109 CVD patients who were treated with glucocorticoid (prednisolone ≥20 mg/day) and were tested for CMV antigen (CMV-Ag).

Systemic sclerosis complicated by arrhythmogenic right ventricular dysplasia that was misinterpreted as pulmonary arterial hypertension.

A 58-year old Japanese woman who had been diagnosed with and managed for systemic sclerosis (SSc) with pulmonary arterial hypertension died suddenly. However, the autopsy revealed marked right ventricular dilatation, and the myocardium had been replaced by fatty tissue. These findings were consistent with arrhythmogenic right ventricular dysplasia (ARVD).

Rituximab was effective on refractory thrombotic thrombocytopenic purpura but induced a flare of hemophagocytic syndrome in a patient with systemic lupus erythematosus.

We report the case of a patient with systemic lupus erythematosus (SLE) who first revealed hemophagocytic syndrome (HPS), which was treated successfully with glucocorticoid and intravenous cyclophosphamide. The patient then demonstrated refractory thrombotic thrombocytopenic purpura (TTP) with normal a disintegrin and metalloprotease with thrombospondin motifs (ADAMTS)-13 activity that responded well to rituximab. After rituximab treatment, the patient showed a flare of HPS that was controlled by additional intravenous cyclophosphamide treatment.

Collision of advanced gastric adenocarcinoma and gastrointestinal stromal tumour: a case report.

We present a case of an 83-year-old female patient with a collision tumour of an advanced Borrmann type 4 gastric cancer and a large gastric gastrointestinal stromal tumour (GIST). According to the deformity of the gastric wall caused by the GIST, type 4 cancer was difficult to identify by oesophagogastroduodenoscopy (OGD). The patient died of progressive gastric cancer related disease.

Clinical evaluation of patients with inflammatory connective tissue diseases complicated by cytomegalovirus antigenemia.

We evaluated the occurrence of cytomegalovirus (CMV) infection and the background characteristics in twenty-three hospitalized patients with inflammatory connective tissue diseases including systemic lupus erythematosus, polymyositis/dermatomyositis, rheumatoid vasculitis, microscopic polyangitis, and Takayasu's arteritis. Cytomegalovirus antigenemia was demonstrated in 10 of 23 evaluable patients. Five of ten patients with CMV antigenemia developed symptomatic CMV disease (all cases of fever, two cases of liver involvement, two cases of interstitial pneumonia, and one case of unknown organ involvement), whereas the remaining five patients were asymptomatic.

Peripheral T cell lymphoma-associated hemophagocytic syndrome in a patient with human adjuvant disease.

Hemophagocytic syndrome (HPS) is a rare complication of lymphoma. We report a 70-year-old woman with human adjuvant disease who developed lymphoma-associated HPS (LAHPS) and elevated serum soluble interleukin (IL)-2 receptor. Despite intensive therapy, she died of pneumonia.

A novel mechanism for the regulation of IFN-gamma inducible protein-10 expression in rheumatoid arthritis.

Chemokines play an essential role in the progression of rheumatoid arthritis (RA). In the present study we examined the expression and regulatory mechanisms of IFN-gamma inducible protein (IP)-10 in RA synovitis. RA synovial fluid contained greater amounts of IP-10 than did synovial fluid from patients with osteoarthritis.

Interaction of monocytes with vascular endothelial cells synergistically induces interferon gamma-inducible protein 10 expression through activation of specific cell surface molecules and cytokines.

To further understand the regulatory mechanisms involved in the process of angiogenesis, the present study was designed to determine the expression and regulation of interferon gamma-inducible protein 10 (IP-10) in peripheral blood monocytes and human umbilical vein endothelial cells (HUVECs). We found that the interaction of monocytes with HUVECs resulted in synergistic increases in IP-10 expression and secretion, which consequently inhibited endothelial tube formation in vitro. Induction of IP-10 was mediated via specific cell surface molecules, as indicated by the finding that IP-10 secretion was significantly inhibited by anti-CD40 ligand antibody, and to a lesser extent by anti-CD40 antibody.

[A case with systemic lupus erythematosus presenting with reversible edematous lesion in cerebellum].

We report the case of a 24-year-old woman with systemic lupus erythematosus (SLE). The patient presented with cervical erythema and multiple arthralgia in December, 1996. Based on the high level of antinuclear antibody and the positivity for anti-double-stranded-DNA antibody, we diagnosed the patient as having SLE.

[A case of rheumatoid arthritis complicated with a pneumonitis during concomitant treatment with methotrexate and bucillamine].

A 70-year-old female was diagnosed as having rheumatoid arthritis (RA) in 1971, which was then treated with steroid and nonsteroidal anti-inflammatory drugs. In 1999, after total replacement of her knee joint, 4 mg of methotrexate (MTX) per week was administered. Two months after the MTX administration, 200 mg of bucillamine per day was administered.