Clinical value of a high-fat and low-carbohydrate diet before FDG-PET/CT for evaluation of patients with suspected cardiac sarcoidosis.

BACKGROUND: We hypothesized that a high-fat and low-carbohydrate (HFLC) diet before FDG-PET/CT could identify patients with active cardiac sarcoidosis (CS). METHODS: Fifty-eight sarcoidosis patients with a suspicion of CS consumed a HFLC diet before FDG-PET/CT. Clinical, electrical, and other imaging investigations were compared to PET results. RESULTS: Using Japanese Ministry of Health and Welfare (JMHW) criteria as a gold standard, 21% (12/58) of patients had a CS. Sensitivity and specificity of PET (visual analysis) were 83% (10/12) and 78% (36/46), respectively, with a very good interobserver agreement (k = 0.86). 70% (7/10) of the patients with a positive PET and negative JMHW criteria exhibited abnormalities suggestive of CS either on MR (n = 3) or SPECT (n = 4). Comparison with the presence of delayed enhancement on magnetic resonance imaging helped to classify patients with active (PET positive) or non-active CS (PET negative). In addition, when MR and PET were both negative, none of the patients met the JMHW criteria. PET response under treatment was concordant with clinical evolution in 11/13 patients. CONCLUSIONS: FDG-PET/CT after HFLC diet is a sensitive tool for the diagnosis of active CS. Combined use of PET and MR is promising for the detection and characterization of CS lesions.

Acute exacerbation of idiopathic pulmonary fibrosis: outcome and prognostic factors.

BACKGROUND: Acute exacerbation is a substantial cause of death in patients with idiopathic pulmonary fibrosis with poorly described prognostic factors. OBJECTIVES: To review the features associated with acute exacerbation of idiopathic pulmonary fibrosis and assess its prognostic factors. METHODS: Thirty-seven occurrences of acute exacerbation of idiopathic pulmonary fibrosis were retrospectively reviewed in the medical records of 27 patients. Clinical presentation, radiographic studies, pulmonary function tests, laboratory data, treatment, and outcome were analyzed. RESULTS: Acute exacerbation of idiopathic pulmonary fibrosis occurred more frequently between December and May (75.7%) than between June and November (24.3%) (p = 0.01). In-hospital mortality was 27% and median survival was 4.2 months (range 0.2-36.6). Significant differences between nonsurvivors and survivors included the time elapsed between their admission and the initiation of treatment for acute exacerbation (6 vs. 3.1 days, p = 0.04), lactate dehydrogenase levels at admission (801 vs. 544.6 IU/l, p = 0.002), impairment of the prior forced vital capacity (51.2 vs. 65%, p = 0.01) and diffusing capacity for carbon monoxide (21.7 vs. 34%, p = 0.01). Furthermore, the evolution of gas exchange in the first 10 days after the initiation of treatment was associated with in-hospital and long-term mortality. CONCLUSIONS: Acute exacerbations of idiopathic pulmonary fibrosis are more frequent during winter and spring. The time between admission and initiation of treatment is a new reported prognostic factor that should be investigated further. This finding highlights the need for a fast diagnostic approach that should probably be standardized. Early gas exchange modifications reflect the response to treatment and predict the prognosis.

Progression of idiopathic pulmonary fibrosis: lessons from asymmetrical disease.

BACKGROUND: In idiopathic pulmonary fibrosis (IPF) the distribution and spatial-temporal progression of fibrotic changes may be influenced by general or locoregional conditions. From this perspective, patients with asymmetrical disease (AIPF) may be unique. METHODS: This retrospective study included 32 patients (26 men, mean ± SD age 69 ± 7 years) with AIPF, as defined by an asymmetry ratio (most affected--least affected fibrosis score)/(most affected + least affected fibrosis score) >0.2. The global fibrosis score was the average of the right and left scores. Patients with AIPF were compared with 64 matched controls with symmetrical IPF. RESULTS: Patients with AIPF did not differ from controls in global fibrosis score and forced vital capacity, but carbon monoxide transfer factor was less decreased (52 ± 19% vs 43 ± 13%, p=0.009). The rate of gastro-oesophageal reflux and acute exacerbations was significantly higher in patients with AIPF (62.5% vs 31.3%, p=0.006 and 46.9% vs 17.2%, p=0.004, respectively). In patients with AIPF the right side was more likely to be involved (62.5%); the median asymmetry ratio was 0.5 (range 0.24-1). Although the global fibrosis score worsened significantly in all 23 patients with AIPF with serial high-resolution CT scans (p<0.0001), pulmonary fibrosis remained asymmetrical in all except three. During follow-up, 15 patients with AIPF experienced 18 acute exacerbations. The first episode was virtually unilateral, occurring in the most affected lung in 10 patients (66.7%). Survival was similar between patients with AIPF and controls. CONCLUSION: AIPF may be related to locoregional factors including gastro-oesophageal reflux which may be responsible for both disease expansion and the occurrence of acute exacerbations.

Cardiac sarcoidosis.

Cardiac involvement is undeniably one of the most challenging issues in sarcoidosis. Although autopsy studies reveal heart lesions in 20 to 30% of sarcoid patients, fewer than 5% suffer from clinical disease. Cardiac sarcoidosis (CS) has a predilection for the myocardium, but the pericardium and endocardium may also be affected. CS manifestations are various and most frequently include the following: (1) aberrations of atrioventricular or intraventricular conduction, either silent or symptomatic; (2) ventricular arrhythmias; (3) subacute congestive heart failure; and (4) sudden death. CS must be detected in all sarcoid patients by means of detailed medical history, physical examination, and resting electrocardiogram (ECG) at first evaluation and during follow-up. In patients with suspected CS, further investigations are aimed at evaluating diagnosis and cardiac consequences. Unfortunately, no gold standard exists that would allow CS diagnosis with a level of confidence. Endomyocardial biopsy is an invasive procedure that lacks sensitivity. Patients need, at a minimum, specialized cardiologic advice, echocardiography, and 24-hour ambulatory ECG. Other diagnostic tools include thallium, technetium, and gallium scintigraphy, and more recently, 18F-fluorodeoxyglucose positron emission tomography and cardiac magnetic resonance (CMR). The respective role of these new imaging tools in the diagnostic approach remains to be defined. CMR has the advantage of not exposing patients to radiation, but it is not feasible in those with cardiac devices. In Western countries, heart involvement accounts for 13 to 25% of sarcoidosis-related deaths, and it is the leading cause of mortality in Japan. The main prognostic indicators are New York Heart Association functional class, left ventricular enlargement, and sustained ventricular tachycardia. Treatment is based on systemic corticosteroids with an initial dose between 30 mg/day and 1 mg/kg/day (which is usually maintained for at least 24 months), specific cardiologic agents, and the placement of a pacemaker or implantable cardiac defibrillator in case of an atrioventricular block or severe intractable ventricular arrhythmias. Cardiac transplantation is exceptionally required.

Uncommon Diseases of The Popliteal Artery: A Pictorial Review.

Uncommon diseases of the popliteal artery include cystic adventitial disease, popliteal artery entrapment syndrome (PAES) and popliteal artery aneurysm (PAA). Because all of these conditions may present with pain or intermittent claudication, imaging is crucial for differentiating them and directing management. Delayed diagnosis can lead to major complications, including acute limb ischemia. Our aim is to provide an illustrative overview of these conditions in order to make radiologists aware of them and avoid misdiagnosis for timely appropriate management. Teaching Points • Cystic adventitial disease diagnosis is based on evidence of cysts within artery walls. • A variety of anatomic variations may result in PAES. • PAES may be bilateral. • PAA is most commonly encountered in men. • Acute complications of PAA include acute thrombosis and distal embolization.

Bone fragility in sarcoidosis and relationships with calcium metabolism disorders: a cross sectional study on 142 patients.

INTRODUCTION: The prevention of fragility fractures in patients with sarcoidosis is a serious concern and the potential risk of hypercalcemia limits vitamin D and calcium supplementation. The objective of this study was to evaluate the risk factors for low bone mineral density (BMD) and fractures in sarcoidosis. In particular, we aimed to determine the link among bone fragility and calcium and vitamin D metabolism in this population. METHODS: We performed a cross-sectional analysis on 142 consecutive patients with histologically proven sarcoidosis. BMD and prevalence of vertebral fractures on X-rays were assessed and the association with potential risk factors was studied by regression analysis. RESULTS: Fragility fractures occurred in 23.5% of patients, despite a normal mean BMD in the study population. In a multivariate analysis, low dietary calcium, fracture, age, gender and menopause were associated with increased risk of low BMD. Low dietary calcium, high current corticosteroid dose and low creatinine clearance were associated with increased risk of fracture. Serum 25(OH)D between 10 and 20 ng/ml was significantly associated with higher BMD. Conversely, values greater than 20 ng/ml were associated with increased risk of fracture. Serum 25(OH)D level was inversely correlated with disease activity. Of note, vitamin D supplements increased serum 25(OH)D in a dose-dependent manner but had no effect on serum calcium level. CONCLUSIONS: Sarcoidosis patients have a high risk of fracture despite not having a lowered BMD suggesting that other independent factors are involved. Current corticosteroid dose, low dietary calcium and serum 25(OH)D levels are associated with bone fragility. In sarcoidosis, calcium and vitamin D supplementation might be warranted, but desirable 25(OH)D serum levels might be lower than those advised for the general population.

Contribution of multimodality imaging for positive and aetiological diagnosis of multiple brown tumours.

This report concerns a rare case of multiple brown tumours discovered in a setting of primary hyperparathyroidism in a 64-year-old patient presenting with weight loss and leg pain. Biological, radiological and pathological findings led to the diagnosis of brown tumours. The contribution of anatomic and nuclear imaging techniques to the diagnosis of brown tumours and their aetiological assessment as well as pre-surgery localisation of parathyroid adenomas in the context of primary hyperparathyroidism is discussed.