Fulminant elderly adult-onset Still disease effectively treated with tocilizumab and methotrexate: A case report.

RATIONALE: Adult-onset Still disease (AOSD) is a rare inflammatory disease of unknown etiology. AOSD is common in young or middle-aged adults; however, in recent years, there have been increasing reports of elderly AOSD. Differentiating AOSD from diseases such as infections and malignancies is difficult. Moreover, rare fulminant AOSD cases with resistance to corticosteroids and immunosuppressive drugs have been reported. PATIENT CONCERNS: An 80-year-old woman presented with flaccid fever, generalized arthralgia, and erythema of the anterior chest for 2 weeks. On day 5 of hospitalization, the patient developed pleural effusion with hypoxemia and her vital signs indicated rapid progression to shock. During the clinical course, the levels of inflammatory markers, including maximum level of ferritin and white blood cells (WBCs) were elevated (252,796 ng/mL and 86,500/µV, respectively) with disseminated intravascular coagulation syndrome (DIC) and macrophage activation syndrome (MAS). DIAGNOSIS: The patient was diagnosed with elderly AOSD as per the Yamaguchi criteria for AOSD. The state of disease was extremely severe with rapid progression and was, thus, categorized as a fulminant form of elderly AOSD. INTERVENTIONS: The patient was treated with prednisolone (PSL) pulse therapy (1000 mg/d) twice and plasma exchange in the intensive care unit for the primary disease and shock. Although she recovered from shock, she developed DIC and MAS. Methotrexate (MTX; 10 mg/d) improved the DIC and MAS. However, severe pleuritis recurred and the patient developed pericarditis; her primary disease was poorly controlled. Finally, tocilizumab (TCZ) was introduced using interleukin-18 (IL-18) as a surrogate marker. The IL-18 level was measured repeatedly following admission, with the peak level (170,000 pg/mL) recorded on the 75th day of hospitalization, immediately prior to introducing TCZ. OUTCOMES: The combined use of MTX, TCZ, and PSL was effective in suppressing elderly AOSD, which was unsuccessfully controlled with MTX and PSL. Frequent monitoring of IL-18 levels proved useful for differentiating elderly AOSD from other diseases. LESSONS: A fulminant form of elderly AOSD was treated with a combination of MTX, TCZ, and PSL. Repeated monitoring of IL-18 levels can be useful for decision-making in treating elderly AOSD.

Acute pancreatitis coincided with multiple arteriolar aneurysms in a patient with polyarteritis nodosa.

A 78-year-old man presented to our hospital with a history of 10 kg weight loss within 6 months previously and general fatigue and fever for 2 and 1 months, respectively. On hospitalisation, the patient was diagnosed with polyarteritis nodosa after multiple microaneurysms were observed in the liver, kidney, pancreas, and mesenteries. He achieved remission with the administration of 1000 mg methylprednisolone for 3 days, followed by prednisolone (55 mg/day). Steroids were successfully tapered with no re-elevation in inflammation. Two months after the administration of steroids, the patient complained of acute abdominal pain and developed severe acute pancreatitis. During treatment for pancreatitis, the patient died due to septic shock and disseminated intravascular coagulation. An autopsy revealed necrotising vasculitis in the intrapancreatic arteries and ischaemia of the downstream arterioles resulting in acute pancreatitis.

18F-fluorodeoxyglucose-positron emission tomography/computed tomography for the diagnosis of polymyalgia-like illnesses: a retrospective study.

BACKGROUND: Various inflammatory conditions may present with musculoskeletal symptoms similar to those of polymyalgia rheumatica (PMR). We investigated findings on 18F-fluorodexoxyglucose (FDG)-positron emission tomography/computed tomography (PET/CT) images that may differentiate PMR from polymyalgia-like illnesses. METHODS: We analyzed data from 25 patients with new-onset polymyalgia-like illnesses who fulfilled Bird's diagnostic criteria for PMR and had undergone FDG-PET/CT scan. To assess the uptake by major joints and synovial bursae, particularly at PMR-specific sites (shoulder, sternoclavicular, and hip joints, interspinous bursae, ischial tuberosities, and greater trochanters), we used visual scoring system to score FDG uptake: 0, no uptake (same as bone); 1, slight uptake; 2, moderate uptake (same as the liver); 3, greater uptake than the liver; and 4, uptake as strong as in the cerebellum. RESULTS: The final diagnoses were PMR in 17 patients and non-PMR in eight patients (three malignancies, two infections, one cholesterol crystal embolism, one ANCA-associated vasculitis, and one undefined diagnosis). Although the serum MMP-3 levels were significantly higher in patients with PMR, C-reactive protein and erythrocyte sedimentation rate mean values did not differ between the two groups. In PMR-specific sites, FDG accumulations were observed in all cases of PMR, with a high PET-positive score of 2.00 (range, 0-3), but it was low in non-PMR cases, with a PET-positive score of 1.00 (range, 0-3). CONCLUSIONS: The FDG accumulation patterns in polymyalgia-like illness differ from those in PMR, despite the similar clinical presentations of both conditions. An FDG-PET/CT scan is useful for differentiating PMR from other polymyalgia-like illnesses.

Two new species of Bairdiidae (Ostracoda: Crustacea) from the western Pacific coast of Japan.

Herein, two new bairdiid ostracod species in the western Pacific Coast of Japan, namely Neonesidea kamiyai sp. nov. and Bairdoppilata japonica sp. nov. are described. They are the fourth and first species described in their respective genus in Japan within the Recent Bairdiidae. The description of the two new species is based on scanning electron microscopic investigation of carapaces and on the analysis of anatomy and chaetotaxy of appendages. Scanning electron microscopy of the carapace and soft-part anatomy of appendages of the two new species provided the complete species description. An asymmetric brush-shaped organ, with the right part considerably larger than the left part, is found in both species; this characteristic is suggested to be synapomorphic for some taxa in the family Bairdiidae.

Refractory Dermatomyositis Complicated with Myelodysplastic Syndrome.

We herein describe a case of refractory dermatomyositis (DM) complicated with myelodysplastic syndrome (MDS). Despite intensive immunosuppressive therapies, the activity of myositis, skin ulcers, and interstitial pneumonia did not improve. The patient ultimately died following the progression of interstitial pneumonia. There are few reports of DM accompanying MDS to date, and any association in the pathogenesis between the two is still unclear. However, underlying MDS may have the potential to influence the therapeutic response of DM.

A case of multicentric Castleman's disease having lung lesion successfully treated with humanized anti-interleukin-6 receptor antibody, tocilizumab.

This report presents the case of a patient demonstrating multicentric Castleman's disease (MCD) with a lung lesion that was successfully treated with an anti-interleukin-6 receptor antibody, tocilizumab in combination with corticosteroid and tacrolimus. A 43-yr-old female with abnormal shadows on a chest X-ray was referred to the hospital for further examination. She was diagnosed as having MCD based on the characteristic pathology of inguinal lymph node, lung lesions, laboratory data, and undifferentiated arthritis. Corticosteroid and rituximab therapy did not fully ameliorate the symptoms; thus, the therapeutic regimen was changed to include tocilizumab, oral corticosteroid and tacrolimus. This regimen resulted in clinical remission and the dose of tocilizumab and corticosteroid could be tapered. Tocilizumab in combination with corticosteroid and tacrolimus may therefore be a beneficial treatment regimen for lung lesions associated with MCD.

The relationships between titers of anti-Ro or anti-La as measured by ELISA and salivary production rate with age correction.

The objective of this work was to clarify the clinical significance of titers of anti-Ro and anti-La, the relationships between titers of either anti-Ro or anti-La, and salivary production rate (SPR). These autoantibodies were titrated using enzyme-linked immunosorbent assay. The Saxon test was performed to measure SPR. Fifty-one females who had anti-Ro but not anticentromere antibodies or anti-U1RNP were enrolled. SPR decreased significantly with age. In order to exclude the effect of aging on SPR, we calculated the "SPR with age correction." According to the results of a multiple regression analysis, only the anti-La titer was significantly associated with SPR with age correction. The distribution pattern of the anti-La titers consisted of two subgroups (with a titer index cutoff of 100.0): a negative anti-La titer (anti-La<25.0) and low anti-La titer (25.0or=100.0). The concentration of serum IgG and the frequency of Sjögren's syndrome in the high anti-La titer group were significantly higher than those in the negative anti-La and low anti-La titer group. Several new aspects of the clinical significance of titrating anti-Ro and anti-La in comparison with SPR have been revealed.

Negative correlation of anti-U1RNP antibody titers and the amount of salivary secretion with age correction.

OBJECTIVE: The aim of this study was to reveal whether or not the presence of anti-U1RNP antibodies is associated with a low amount of salivary secretion (ASS). SUBJECTS AND METHODS: Twenty females (mean age 49+/-12 years) who had anti-U1RNP but not ACA, anti-Ro, or anti-La antibodies (anti-U1RNP-positive group), and 65 control females (mean age 50+/-12 years) were included in this study. The saxon test was performed to measure the ASS. RESULTS: After a correction for age by ANCOVA, ASS in the anti-U1RNP-positive group was significantly lower than ASS in the control group (p <0.001). In the control group, ASS was not significantly decreased with advanced age (r=-0.140, p=0.211). In the anti-U1RNP-positive group, ASS was decreased with age, without a significant difference (r=-0.379, p=0.100). In the next analysis, we introduced 'ASS with age correction', assuming that all subjects in the anti-U1RNP-positive group were 49 years of age. A negative correlation between the titers of anti-U1RNP antibodies and the ASS with the age correction in the anti-U1RNP-positive group was noted (r=-0.520, p=0.019). The log of the antinuclear antibodies titers, or titers of rheumatoid factor was significantly correlated with the titers of anti-U1RNP antibodies, respectively (r=0.466, p=0.038 and r=0.595, p=0.006; respectively). The pathological findings of minor salivary gland biopsy in 2 subjects were compatible with Sjögren's syndrome; one subject showed moderate lymphocytic infiltration. CONCLUSION: The presence of anti-U1RNP antibodies is associated with reduced ASS.

Primary biliary cirrhosis in female subjects with sicca-associated antibodies.

The aim of this study is to clarify the time course of primary biliary cirrhosis (PBC) in subjects possessing anticentromere antibodies (ACA), anti-Ro, and/or anti-La antibodies, and who used alkaline phosphatase (ALP) as a serological marker for PBC. Female subjects (n = 165), who had at least one of ACA, anti-Ro, and/or anti-La, were enrolled in this study. Groups A (ACA alone, n = 44), B (anti-Ro alone, n = 54), E (anti-Ro and anti-La, n = 52), and DFG (ACA with anti-Ro and/or anti-La, n = 14) were analyzed. Healthy females (n = 65) were used as a control. The frequencies of the PBC in groups A (13.6%) and DFG (14.3%) were higher than those in groups B (1.9%) and E (0.0%). The ALP levels increased with age in groups A and DFG and slightly increased with age in groups B and C, and the control group. After correcting for age by analysis of covariance, a comparison of ALP levels among the groups not having anti-M(2) was as follows: group A falling dots group DFG > group B falling dots group E falling dots the control group. The subjects with ACA might thus have PBC more frequently than either those with anti-Ro and/or anti-La, or the control subjects.

Intermittent intravenous cyclophosphamide pulse therapy for the treatment of active interstitial lung disease associated with collagen vascular diseases.

The availability of intravenous cyclophosphamide (CYC) pulse therapy for collagen vascular diseases (CVD)-associated interstitial lung disease (ILD) has been indicated. However, the standard protocol concerning the dosage and the interval of CYC infusion has not yet been established. The aim of this study is to elucidate the efficacy and the safety of our "divided administration" protocol of CYC for the treatment of CVD-ILD. The treatment protocol consists of two steps: step 1, CYC 400-500 mg at 10-day intervals for at least 30 days, and step 2, CYC 500 mg at 14-day intervals for at least 4 weeks. The ILD activities were monitored by respiratory symptoms, serum levels of KL-6 (a serological marker of IP), chest computed tomography (CT), and pulmonary function tests. Seventeen patients [nonspecific interstitial pneumonia (NSIP), 12 patients; usual interstitial pneumonia (UIP), 4; lymphocytic interstitial pneumonia (LIP), 1] accomplished the study protocol. The sessions of CYC infusion ranged from 5 to 20 (mean, 8.3). In all patients, respiratory symptoms were improved and the serum levels of KL-6 were decreased (from 1572 +/- 904 to 978 +/- 392 U/ml; P < 0.01). Chest CT findings were improved in 4 patients (23.5%): they were all classified as NSIP; not deteriorated, 13 patients (76.5%). An improvement in the vital capacity percentage (%VC) was recognized in 10 patients (78.6%) and in diffusing capacity of carbon monoxide (%DLco) in 8 patients (61.5%). Nevertheless, mean %VC and mean %DLco did not change significantly. No major adverse event(s) occurred. The efficacy and safety of our "divided administration" protocol of CYC for CVD-ILD was demonstrated.