Laryngeal edema in drug reaction with eosinophilia and systemic symptoms syndrome due to sulfasalazine.

We present a case of a 47-year-old man with drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome. The patient had been diagnosed with rheumatoid arthritis and sulfasalazine was prescribed 4 weeks before admission. Initial symptoms with fever and rash worsened even after a discontinuation of the medication, and concomitant symptoms developed including typical manifestations of facial rash and edema sparing the periorbital area, as well as atypical laryngeal edema.

Clinical experience in 115 patients with arthritis and/or enthesitis who met the classification criteria for psoriatic arthritis (CASPAR) within the last two years-Possible association with malignant disorders.

Among about 400 patients with active arthritis and/or enthesitis who were referred to our department within the last two years, 140 of them were strongly suspected as having psoriatic arthritis by a comprehensive diagnostic procedure and after consulting specialists from dermatology, orthopedics, and radiodiagnostics at our institution and other institutions. Among them, 115 patients strictly met the classification criteria for psoriatic arthritis (CASPAR). Among the 115 patients, 19 patients (9 males and 10 females) had current psoriasis and 96 patients (22 males and 74 females) did not have current psoriasis.

Vertebrobasilar infarction related to giant cell (temporal) arteritis: case report.

An 84-year-old male with a 3-month history of headache and elevated C-reactive protein levels was admitted for biopsy of the superficial temporal artery, which led to the diagnosis of giant cell arteritis (GCA). Two days after prednisolone therapy was initiated, the patient began to experience transient vertigo attacks. Two days later, dysarthria, left-sided hemiparesis, right abducens palsy, and horizontal nystagmus developed.

Serum β2-microglobulin level is a useful indicator of disease activity and hemophagocytic syndrome complication in systemic lupus erythematosus and adult-onset Still's disease.

This study demonstrates whether serum β2-microglobulin (β2-MG) level can be an indicator of the status of systemic lupus erythematosus (SLE) and adult-onset Still's disease (AOSD), and development of hemophagocytic syndrome (HPS) complication. Serum β2-MG level was compared between the active and inactive statuses of SLE and AOSD in hospitalized patients. Active status was defined as a state for which a therapy was introduced.

Serum KL-6 level as an indicator of active or inactive interstitial pneumonitis associated with connective tissue diseases.

Objective: To elucidate the cut off levels of serum KL-6 indicating patients with interstitial pneumonitis (IP) and patients with active IP associated with connective tissue diseases (CTDs).

Methods: CTD patients whose serum KL-6 level was measured were included. IP was diagnosed on the basis of medical records including XP/CT findings, and active IP was assumed in case that intervention for IP was newly added.

[Massive pulmonary thromboembolism found at autopsy in a patient with a 14-year history of polymyositis-associated nonspecific interstitial pneumonia].

A 64-year-old woman with a 14-year history of refractory nonspecific interstitial pneumonia (NSIP) associated with polymyositis was admitted urgently to our hospital due to acute exacerbation of dyspnea. The first episode of dyspnea had occurred 14 years earlier and the diagnosis of cellular and fibrotic NSIP had been made by radiographic examinations and open lung biopsy. Her NSIP initially responded well to high-dose prednisolone therapy (40mg/day), but flared when the dose of prednisolone was tapered.

Acute exacerbation of preexisting interstitial lung disease after administration of etanercept for rheumatoid arthritis.

A 70-year-old woman with a 6-year history of seropositive rheumatoid arthritis (RA) and asymptomatic interstitial lung disease (ILD) began taking etanercept for ongoing arthritis despite treatment with methotrexate (MTX) and bucillamine. MTX was discontinued before introduction of etanercept. She developed lung injury 8 weeks after starting etanercept.

[A case of systemic sclerosis and Sjogren's syndrome with cardiac tamponade due to steroid-responsive pericarditis].

A 75-year-old woman was admitted to our hospital because of abnormal lung shadow and necrosis of the left feet. She had a history of Raynaud's phenomenon from her twenties. On admission, she was diagnosed as having diffuse systemic sclerosis (SSc) and Sjögren's syndrome (SjS) because of scleroderma, interstitial pneumonia (IP), positive result of anti-Scl-70 and SS-A antibody, sicca, decreased tear excretion, and dysfunction of salivary glands.

Reactive hemophagocytic syndrome in a case of systemic lupus erythematosus that was diagnosed by detection of hemophagocytosing macrophages in peripheral blood smears.

A 38-year-old woman with pancytopenia and liver dysfunction was diagnosed with active systemic lupus erythematosus (SLE). On days 9 and 10 of admission, peripheral blood smears showed macrophages phagocytosing platelets, and reactive hemophagocytic syndrome (HPS) was diagnosed. Hemophagocytic syndrome was successfully treated with high-dose prednisolone therapy and one course of methylprednisolone pulse therapy.

Sarcoidosis with acute recurrent polyarthritis and hypercalcemia.

A 45-year-old woman had bleary eyes and recurrent episodes of fever and arthritis in the knees and ankles. The patient had anterior uveitis, negative findings of the tuberculin test, and an increased serum lysozyme level, but bilateral hilar lymphadenopathy (BHL) was absent. During the course of her disease, the serum calcium and angiotensin-converting enzyme levels gradually increased to above the normal level, and the patient was clinically diagnosed as having sarcoidosis.

Trimethoprim-sulfamethoxazole-induced hypersensitivity syndrome associated with reactivation of human herpesvirus-6.

A 27-year-old man who had a history of bronchial asthma, eosinophilic enteritis, and eosinophilic pneumonia presented with fever, skin eruptions, cervical lymphadenopathy, hepatosplenomegaly, atypical lymphocytosis, and eosinophilia two weeks after receiving trimethoprim (TMP)-sulfamethoxazole (SMX) treatment. After the withdrawal of TMP-SMX and the administration of high-dose steroid, these systemic symptoms gradually resolved. During the disease course, the patient showed a transient increase in anti-human herpesvirus (HHV)-6 antibody titers and HHV-6 DNA in the peripheral blood, indicating the reactivation of a latent HHV-6 infection.

[Case of congestive heart failure associated with hypereosinophilia developed during antibiotics treatment].

A 92-year-old man with a history of bronchial asthma and allergic rhinitis received antibiotics for sepsis by methicillin-resistant Staphylococcus aureus and multidrug-resistant Enterococcus gallinarum. During the antibiotics treatment, skin eruptions, liver dysfunction, and hypereosinophilia developed, followed by dyspnea, congestive heart failure, electrocardiographic abnormalities, and diffuse mild myocardial hypokinesis. After the discontinuation of the antibiotics and the administration of steroid, skin eruptions, liver dysfunction, and hypereosinophilia improved parallel with the improvement of the congestive heart failure.

A case of necrotizing fasciitis caused by coagulase-negative staphylococcus: utility of magnetic resonance imaging for the preoperative diagnosis of necrotizing fasciitis.

A 70-year-old woman presented with fever and pain in the right lower extremity. Fat-suppressed gadolinium-enhanced T1-weighted magnetic resonance imaging (MRI) showed contrast-enhanced fascia, fluid accumulation, and hypointense signals in the muscles. Surgical interventions including incisions and insertion of drainage tubes were performed on the basis of the MRI findings.

Takotsubo (ampulla-shaped) cardiomyopathy associated with microscopic polyangiitis.

Recently, a cardiac disorder characterized by ballooning and hypokinesis at the apex has been described as takotsubo (ampulla-shaped) cardiomyopathy. We encountered a patient with a rare case of takotsubo cardiomyopathy associated with microscopic polyangiitis. A 70-year-old woman suddenly presented with ventricular dysfunction during the active phase of microscopic polyangiitis.

A case of acquired hemophilia caused by factor VIII inhibitor with rheumatoid arthritis, successfully treated with immunosuppressive treatment and recombinant activated factor VII.

Abstract We report the case of a patient who presented with acquired hemophilia associated with rheumatoid arthritis. The patient's factor VIII activity was less than 1% and factor VIII inhibitor was detected. Based on the blood analysis, the patient was diagnosed as having the factor VIII inhibitor.