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. Rheumatologists should be aware that sulfasalazine is derived from sulfonamide and can possibly induce DRESS syndrome, one of the life-threatening drug eruptions.

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.

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. The cut off levels were determined by receiver operating characteristic (ROC) curve analysis. RESULTS: Among 240 (174 females) patients, 67 (42) had IP and 15 (9) had active IP. The ages of patients with and without IP, and with active IP and with inactive IP were 70.3±9.5 and 62.8±15.3, and 72.8±8.1 and 69.6±9.8, respectively. IP was significantly more prevalent in males and the elderly. The KL-6 levels were 990±90 and 301±12 U/mL in patients with and without IP, and 1,905±236 and 726±54 U/mL in those with active IP and with inactive IP, respectively. ROC curve analysis showed a cut off level of 509 U/mL for indicating IP, and that of 1,051-1,060 U/mL for indicating active IP. CONCLUSION: A serum KL-6 level of higher than 500 U/mL is a marker of the presence of IP, and a level of higher than 1,000 U/mL is a marker of the presence of active IP associated with CTDs.

[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.

[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. Seventy days after amputation of her left leg, she presented with edema, hypoxemia, chest discomfort, and fever. Blood test revealed inflammation and cardiac echography revealed pericardial effusion with a collapse sign of right atrium, thereby leading to the diagnosis of cardiac tamponade. After starting the daily dose of 20 mg of prednisolone, the pericardial effusion and cardiac tamponade sign disappeared. Pericarditis is seen in half of patients with SSc and rarely with SjS, and is usually asymptomatic. Pericarditis due to SSc has been reported unresponsive to steroid therapy, but several cases of steroid responsive pericarditis due to SSc or SjS have been reported. Clinically, they shared inflammatory responses and the presence of IP in the cases of SSc, which will be important when considering the pathogenesis and treatment of pericarditis due to SSc or SjS.

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.

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. The clinical features of arthritis were typical of those of Löfgrens syndrome although BHL and erythema nodosum were absent. The patient was successfully treated with 15 mg/day of prednisolone.

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. This is the first case of TMP-SMX-induced hypersensitivity syndrome associated with the reactivation of a latent viral infection.

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.