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. Nineteen (16.5%) of the 115 patients had developed malignant tumor before the onset of arthritis, and 4 (3.5%) developed malignant tumor after the onset of arthritis. Twenty-two of the 23 patients who developed malignancy were female and 10 patients developed breast cancer. Differential diagnoses in these 23 patients may include paraneoplastic syndrome. We consider that it is important to take into account the possibility of paraneoplastic syndrome in patients with arthritis and/or enthesitis who apparently meet the CASPAR criteria, and detailed screening and monitoring of malignant disease may be beneficial to the patients.

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. Magnetic resonance (MR) imaging disclosed fresh infarctions in the vertebrobasilar territory. Since the patient became drowsy because of brainstem compression and hydrocephalus due to cerebellar swelling, emergency suboccipital decompression surgery and ventricular drainage were performed. Subsequently, the patient's consciousness levels improved. MR angiography revealed right vertebral artery (VA) occlusion and left VA stenosis due to arteritis. Ischemic stroke is a serious though relatively rare complication of GCA. Similar cases have been reported, in which ischemic stroke developed despite or possibly due to steroid therapy. To our knowledge, this is the first description of vertebrobasilar infarction associated with GCA in the Japanese population. The merits and potential demerits of steroid therapy are briefly discussed.

Serum ß2-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 ß2-microglobulin (ß2-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 ß2-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 ß2-MG level was also compared between patients with and without HPS complication. HPS was diagnosed on the basis of clinical and pathological findings. Laboratory markers of HPS including peripheral blood cell counts and levels of serum lactate dehydrogenase (LDH), serum ferritin, plasma fibrin/fibrinogen degradation product (FDP), and plasma D-dimer were examined to determine their correlations with serum ß2-MG level. Sixteen SLE and seven AOSD patients (all females, aged 39.0 ± 16.4) were included. The serum ß2-MG level was high in the active status of underlying diseases and decreased significantly after the therapy (3.5 ± 1.4 vs. 2.1 ± 0.8 mg/L, p < 0.001). Among patients with active status, the ß2-MG level was higher in patients with HPS (two with SLE and three with AOSD) than in patients without HPS (4.9 ± 1.8 vs. 3.3 ± 1.4 mg/L, p < 0.05). Serum ß2-MG level significantly correlated with the levels of serum LDH (r(s) = 0.42, p < 0.05), plasma FDP (r(s) = 0.58, p < 0.05), and plasma D-dimer (r(s) = 0.77, p < 0.01). Serum ß2-MG level would be a useful indicator of disease activity and development of HPS complication in patients with SLE and AOSD.

[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. Three years after the diagnosis of NSIP, a diagnosis of polymyositis was made based on her elevated serum level of creatinine phosphokinase and muscle biopsy findings. Her polymyositis was controlled well by prednisolone therapy, but her NSIP gradually worsened, despite treatment with several courses of methylprednisolone pulse therapy and immunosuppressive agents. She was brought to our hospital by ambulance due to acute progression of dyspnea and was admitted. Chest X-ray film revealed diffuse interstitial shadows and marked enlargement of the right atrium and bilateral pulmonary arteries. Transthoracic echocardiogram revealed severe dilatation of the right atrium and right ventricle with an estimated pulmonary artery systolic pressure of 84mmHg. She died of progressive respiratory failure on day 4 of hospitalization. Autopsy revealed massive thrombi that completely obstructed the bilateral pulmonary trunks. Histological examination revealed that these thrombi were organized, and her interstitial lung disease (ILD) was specified as fibrotic NSIP with microscopic honeycomb. Massive pulmonary thromboembolism is extremely rare in patients with polymyositis-associated NSIP, but it should be considered as a possible cause of acute exacerbation of respiratory failure. This case provides valuable information on the clinical and pathological course of polymyositis-associated NSIP.

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. Etanercept was discontinued and oral prednisolone 40 mg/day was begun, and her clinical findings gradually improved. Lung injury, although rare, is a recently noticed, potentially fatal adverse effect of all 3 licensed biological anti-tumor necrosis factor (TNF) agents. We recommend caution in the use of anti-TNF agents in elderly RA patients with preexisting ILD.

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. Detection of hemophagocytosing macrophages in peripheral blood smears would be a useful and noninvasive method of diagnosing SLE-associated HPS.

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. The histopathological examinations of surgically obtained biopsy specimens demonstrated suppurative fasciitis, widespread myonecrosis, and thromboses of the vessels, all of which were compatible with a diagnosis of necrotizing fasciitis. The bacterial cultures were positive for a coagulase-negative staphylococcus. Following the surgical interventions, the patient was successfully treated by aggressive antimicrobial therapy. MRI can thus be useful for differentiating necrotizing fasciitis from nonnecrotizing soft tissue infection and for planning the treatment of necrotizing fasciitis.

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. The findings on echocardiograms and electrocardiograms were consistent with those of takotsubo cardiomyopathy. The ventricular dysfunction completely resolved after treatment with 40 mg/day of prednisolone and methylprednisolone pulse therapy. This unique type of cardiomyopathy can be a complication of microscopic polyangiitis.

[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. Vancomycin hydrochloride and teicoplanin were suspected as the causative drugs on the basis of the treatment course. Although congestive heart failure is rare in the case of drug-induced hypereosinophilia, it is one of life-threatening complications. We describe herein a case of congestive heart failure associated with hypereosinophilia developed during antibiotics treatment, successfully treated with steroid after the discontinuation of the causative drug.