Background Aortic dissection is an age-related and lethal vascular disease. Aging, which is associated with degeneration, is the major risk factor of aortic dissection. Telomeres are specialized DNA structures located at the end of eukaryotic chromosomes, the telomere length could be considered as an index of vascular aging. The purpose of present study was undertaken to investigate the relationship between the leukocyte telomere length and aortic dissection.
Methods and Results Seventy-two patients with aortic dissection and seventy-two sex- and age-matched subjects without vascular diseases were collected. Leukocyte telomere length ratio (T/S ratio) was measured using a quantitative PCR method and analyzed. A significantly shorter leukocyte telomere length in the patients with aortic dissection was found compared to the controls, [median 1.02 (interquartile range {IQR}:0.83-1.37) vs median 1.63 [IQR: 1.18-2.51), p<0.001]. The telomere length in the control group showed a trend of inverse correlation with age (r=-0.226, p=0.056), however, there was no significant correlation in aortic dissection (r=0.062, p=0.607). The short leukocyte telomere length was associated with aortic dissection, even after adjustment for other risk factor (OR=0.214, 95% CI: 0.085-0.537).
Conclusion Leukocyte telomere length could be an independent predictor of aortic dissection. Measurement of the leukocyte telomere length may be valuable for patients with a high risk of aortic dissection.

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Objective We examined the hypothesis that renal congestion is responsible for the decline in renal function in patients with heart failure (HF) via tubulointerstitial inflammation.
Methods First, in a longitudinal study, we retrospectively examined the relationship between cardiac functions and the decline of renal function during a period of 6.4±3.2 years in 20 patients who had a left ventricular ejection fraction of <40% and an estimated glomerular filtration ratio (eGFR) of <60 mL/min/1.73 m². Second, we compared the renal histology in autopsy cases of HF-induced renal dysfunction (HF-RD), cases of nephrosclerosis (NSC) and cases of neither RD nor HF (controls) in a cross-sectional study. Third, we retrospectively examined renal function in HF patients with predominantly right, but not left, ventricular dysfunction.
Results eGFR decreased at 9.4±4.6%/year in the cohort of the longitudinal study. The rate of eGFR decline was correlated with blood pressure and with diameter of the inferior vena cava (IVCd) (r=0.5) measured at the initial work-up. Multivariate analysis indicated that the IVCd is an independent determinant of decline of eGFR in HF. In the cross-sectional study, fibrosis, Rac1 expression, protein nitrosylation, and number of CD68-positive cells were increased in the tubulointerstitium in both cases of HF-RD and NSC. Peritubular capillaries in HF-RD were dilated by 35% without any change in density compared with those in the controls. In right sided HF, the reduction of IVCd after treatment was associated with improvement of eGFR.
Conclusion Venous congestion may contribute to HF-induced deterioration of renal function by augmenting oxidative stress-mediated inflammation in the tubulointerstitium.

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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.

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Acute suppurative thyroiditis is a rare disorder that is mostly found in the left lobe of the thyroid gland of children due to congenital patency of the pyriform sinus fistula. Here, we report a 61-year-old man with acute right-sided suppurative thyroiditis without pyriform sinus fistula. He also showed infectious hip arthritis, spondylitis and Roth's spots. He presented with heart failure and was diagnosed with infectious endocarditis by sequential transesophageal echocardiography. A replacement with a prosthetic valve was performed and cured him. It is important to recognize that infectious endocarditis can be a focus of acute suppurative thyroiditis.

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We treated a woman with membranous nephropathy in whom serious nephrotic syndrome (NS) continued even after the introduction of hemodialysis (HD). No response was seen with conservative treatment, including administration of steroids and albumin transfusion and body fluid management with HD. Hypoalbuminemia continued, and management of her general condition was problematic because of the hypotension, edema, pleural and peritoneal effusions. We performed percutaneous renal artery embolization (RAE), voluntary urine output disappeared for a short time, and good clinical course was subsequently seen with sustainable serum albumin levels of around 3 g/dL.

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Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis mediated by intravascular deposition of immune complexes. We report a 55-year-old man with PAN revealed by CNS vasculitis with subarachnoid hemorrhage and brainstem vasogenic oedema, associated with essential mixed cryoglobulinemia. In spite of aggressive treatment (steroids and cyclophosphamide), the patient became tetraplegic. A pathophysiological relationship between cryoglobulin and PAN can be suspected. Mixed cryoglobulinemia may trigger vasculitis with PAN features, possibly explaining the lack of treatment response.

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A 44-year-old Japanese woman was diagnosed with type 1 hereditary angioedema (HAE) at the age of 30. In March 2007, she began suffering from severe abdominal pain due to intestinal edema. After treatment with C1-INH concentrate, her symptoms disappeared. However, during the subsequent three years, the frequency of the attacks increased continuously, and C1-INH concentrate was necessary for treatment of every attack. The increase in the number of attacks might have been due to the frequent injection of C1-INH concentrate or the deterioration of her disease course. In a genetic investigation, the patient was found to have a novel mutation in the C1-INH gene.

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A 24-year-old man was referred to our hospital due to bilateral hilar lymphadenopathy on chest radiography. He had been under medication for aortitis syndrome and Crohn's disease for 12 years. Surgical biopsy from the anterior segment of the left upper lobe and mediastinal lymph nodes was performed under video-assisted thoracoscopy. Histopathological examination revealed epithelioid cell granulomas without caseous necrosis, compatible with sarcoidosis. Full sequence analysis of the CARD15 gene, which is reportedly related to the formation of granulomatous lesions in Crohn's disease and sarcoidosis revealed no mutation of CARD15 gene. This is the first report of concurrent sarcoidosis, Crohn's disease and aortitis syndrome in an individual.

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A 22-year-old healthy woman visited our clinic for seizure and consciousness loss. A thorough history taking and physical examination was negative except for persistent high blood pressure. Serial workup for suspicious secondary hypertension revealed secondary hyperaldosteronism. Further image study showed diminished unilateral kidney size, but the computed tomographic angiogram was unremarkable. Radionuclide renography disclosed a specific pattern of compromised unilateral renal function after captopril challenge, suggesting a high probability of renal artery stenosis. Renal artery angiography finally confirmed the diagnosis, and angioplasty with stenting successfully reversed the refractory hypertension despite the shrinking kidney size. Although isotopic study is gradually fading away among our diagnostic tools, the value it demonstrates in this case warrants attention.

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A 47-year-old woman with a recent clinical diagnosis of acute cholecystitis developed acute adrenal failure. Initial computed tomograms of the abdomen showed bilateral adrenal swelling which evolved into bilateral adrenal hemorrhage on repeat examination a few days later. Laboratory investigations revealed a previously undiagnosed primary antiphospholipid syndrome and homozygosity for the MTHFR C677T mutation with hyperhomocysteinemia. This case highlights the protean clinical manifestations of adrenal vascular accidents and the need for a thorough search for underlying prothrombotic states in this setting.

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