Clinical significance of soluble interleukin-2 receptor measurement in patients with idiopathic retroperitoneal fibrosis.

BACKGROUND: Idiopathic retroperitoneal fibrosis (iRPF) is a rare chronic fibro-inflammatory disorder of unknown etiology. Activated T-helper cells, which shed soluble interleukin-2 receptor (sIL-2R) into the circulation, may play a pathogenetic role. Hence, measuring sIL-2R may be of value in monitoring disease activity and treatment response in iRPF patients. METHODS: We performed a prospective inception cohort study of 82 patients with untreated (re)active iRPF stratified by elevated (> 623 U/mL) or normal sIL-2R level at baseline and compared disease characteristics among these groups. Baseline and changes in sIL-2R levels following treatment with tamoxifen (TMX) or prednisone (PDN) were analyzed and related to treatment response. RESULTS: Median sIL-2R level was 668 U/mL (IQR 502.8-827.5); 48 patients (59%) had elevated baseline sIL-2R levels. Patients with elevated sIL-2R presented with higher CRP (P = 0.049) and serum creatinine (sCr) levels (P < 0.001) and more often had hydroureteronephrosis (P = 0.01). There was an age and sex adjusted linear association between baseline sIL-2R and both CRP (P = 0.02) and sCr (P < 0.001). Baseline and serial levels of sIL-2R were predictive and concordant, respectively, with clinical response in patients treated with PDN. ROC curve analyses of sIL-2R on a continuous scale and PDN treatment success showed an AUC of 0.84. A serum sIL-2R cut-off value for PDN treatment success of ≤ 703 U/mL was found with a sensitivity of 100% and specificity of 72%. CONCLUSION: Serial measurement of sIL-2R may be of value in monitoring disease activity and PDN treatment response in iRPF patients.

Abdominal aortic diameter and cardiovascular status in patients with idiopathic retroperitoneal fibrosis.

Although much debated, an exaggerated inflammatory response to advanced atherosclerosis has been implicated in the pathogenesis of idiopathic retroperitoneal fibrosis (RPF). Clinical presentation, infrarenal abdominal aortic diameter and RPF mass thickness were retrospectively analyzed in 166 patients with idiopathic RPF seen at our referral center between April 1998 and December 2019. Patients were stratified to their infrarenal abdominal aortic diameter at presentation (i.e., non-ectatic [< 25 mm]; ectatic [25-29 mm]; and aneurysmal [≥ 30 mm]) to compare characteristics across groups with an undilated or dilated aorta. Ectatic or aneurysmal aortic dilatation was present in 34% of patients. Most clinical characteristics did not differ across abdominal aortic diameter stratified groups, but RPF mass thickness was greater in patients presenting with aortic aneurysmal dilatation compared to that in patients with an undilated aorta (49.0 mm [IQR 34.0-62.0] vs 32.5 mm [IQR 25.3-47.8]; P < 0.001). A positive linear association was found between aortic diameter on a continuous scale and RPF mass thickness (ß 0.32 [95% CI 0.34-0.96]; P < 0.001). This association remained significant after adjusting for age, sex and acute-phase reactant levels (ß 0.28 [95% CI 0.15-0.95]; P < 0.01). Treatment success across aortic diameter stratified groups did not differ (P = 0.98). Treatment induced RPF mass regression was not associated with an increase in aortic expansion rate (P = 0.44). Aortic dilatation was prevalent among patients. Infrarenal abdominal aortic diameter was independently associated with RPF mass thickness. Findings support the concept that at least in a subset of patients, RPF may be secondary to advanced atherosclerosis.

Effects of 12-week treatment with dapagliflozin and gliclazide on faecal microbiome: Results of a double-blind randomized trial in patients with type 2 diabetes.

AIMS/HYPOTHESIS: Patients with type 2 diabetes (T2D) are usually treated with (combinations of) glucose-lowering medication. The effects of these drugs can be influenced by intestinal microbiota and vice versa, as these drugs can also influence microbiome composition. However, as there is currently little clinical insight into this bug-drug interaction, our study aimed to evaluate the effects of 12-week treatment with the SGLT2 inhibitor dapagliflozin and sulphonylurea gliclazide on gut microbiome composition in T2D patients treated with metformin. METHODS: A total of 44 patients were randomized to either dapagliflozin or gliclazide treatment for 12 weeks. At baseline and after 12 weeks, faecal samples and 24-h urine were collected. During study visits, anthropometric data were measured and blood samples drawn after an overnight fast. Microbiome composition was determined by 16S rRNA gene sequencing. Plasma glucose, insulin, HbA1c and urinary glucose excretion were measured using conventional methods. RESULTS: While dapagliflozin and gliclazide similarly improved glycaemic control, dapagliflozin reduced and gliclazide increased fasting insulin. Dapagliflozin also greatly increased urinary glucose excretion whereas gliclazide did not, while body mass index, fat mass percentage and waist circumference were reduced by dapagliflozin, but increased by gliclazide. However, neither treatment significantly affected either gut microbiome alpha diversity or composition and, after treatment, no associations were found between microbiome composition and other clinical parameters. CONCLUSION: Even though gliclazide and dapagliflozin have different metabolic actions in patients with T2D, neither treatment altered the faecal microbiome, thereby suggesting that the observed metabolic changes are not mediated by their effects on the microbiota.

Geriatric Assessment and the Relation with Mortality and Hospitalizations in Older Patients Starting Dialysis.

BACKGROUND AND OBJECTIVES: A geriatric assessment (GA) is a structural method for identifying frail patients. The relation of GA findings and risk of death in end-stage kidney disease (ESKD) is not known. The objective of the GA in OLder patients starting Dialysis Study was to assess the association of GA at dialysis initiation with early mortality and hospitalization. DESIGN, SETTING, PARTICIPANTS, AND MEASUREMENTS: Patients ≥65 years old were included just prior to dialysis initiation. All participants underwent a GA, including assessment of (instrumental) activities of daily living (ADL), mobility, cognition, mood, nutrition, and comorbidity. In addition, a frailty screening (Fried Frailty Index, [FFI]) was applied. Outcome measures were 6- and 12-month mortality, and 6-month hospitalization. Associations with mortality were assessed with cox-regression adjusting for age, sex, comorbidity burden, smoking, residual kidney function and dialysis modality. Associations with hospitalization were assessed with logistic regression, adjusting for relevant confounders. RESULTS: In all, 192 patients were included, mean age 75 ± 7 years, of whom 48% had ≥3 geriatric impairments and were considered frail. The FFI screening resulted in 46% frail patients. Mortality rate was 8 and 15% at 6- and 12-months after enrolment, and transplantation rate was 2 and 4% respectively. Twelve-month mortality risk was higher in patients with ≥3 impairments (hazard ratio [HR] 2.97 [95% CI 1.19-7.45]) compared to less impaired patients. FFI frail patients had a higher risk of 12-month mortality (HR 7.22 [95% CI 2.47-21.13]) and hospitalization (OR 1.93 [95% CI 1.00-3.72]) compared to fit patients. Malnutrition was associated with 12-month mortality, while impaired ADL and depressive symptoms were associated with 12-month mortality and hospitalization. CONCLUSIONS: Frailty as assessed by a GA is related to mortality in elderly patients with ESKD. Individual components of the GA are related to both mortality and hospitalization. As the GA allows for distinguishing between frail and fit patients initiating dialysis, it is potentially of added value in the decision-making process concerning dialysis initiation.

Elevated serum IgG4 levels in diagnosis and treatment response in patients with idiopathic retroperitoneal fibrosis.

Idiopathic retroperitoneal fibrosis (iRPF) may be a manifestation of IgG4-related disease. Measuring serum IgG4 (sIgG4) may be of value in monitoring iRPF, but this has scarcely been evaluated. It is unknown if tamoxifen (TMX) affects sIgG4 levels. We performed a prospective inception cohort study of 59 patients with untreated (re)active iRPF stratified by elevated (>1.4 g/L) or normal sIgG4 level. Changes in sIgG4 levels following TMX initiation and, if treatment failed, during subsequent corticosteroid (CS) treatment were analyzed. The median sIgG4 level was 1.1 g/L (interquartile range (IQR) 0.4-2.2); 24 patients (40%) had elevated sIgG4 level. Patients with elevated sIgG4 tended to present with higher ESR (46 vs. 34 mm/h; P = 0.08) and more frequent locoregional lymphadenopathy adjacent to the mass (41.7 vs. 20.0%; P = 0.08). sIgG4 also correlated with ESR (ρ = 0.26; P = 0.05) and serum creatinine (SC) (ρ = 0.26; P = 0.04). Following TMX initiation, sIgG4 level decreased, particularly when achieving treatment success (P < 0.01). Odds ratio for TMX treatment success in patients with elevated sIgG4 level was 0.77 (95% CI 0.53-1.14; P = 0.19). After adjusting for age, sex, and SC, the odds ratio was 0.78 (95% CI 0.51-1.18; P = 0.24). ROC curve analyses of sIgG4 on a continuous scale and treatment success showed an AUC of 0.62. Treatment success and concurrent sIgG4 decrease (P < 0.01) were achieved in 78% of patients who converted to CS therapy. Patients with elevated sIgG4 level may be more inflammatory than patients with normal sIgG4 level, but this needs further study. TMX affects sIgG4 levels, but to a lesser extent than CSs. sIgG4 cannot be used as an outcome prediction tool, irrespective of which cutoff value was chosen.

Mesenteric panniculitis: prevalence, clinicoradiological presentation and 5-year follow-up.

OBJECTIVE: To determine prevalence, clinicoradiological characteristics and outcome of patients with mesenteric panniculitis (MP) in a large hospital-based population. METHODS: Consecutive abdominal CT examinations of 3820 patients were evaluated for MP. Clinical characteristics, therapy and outcome of patients with MP were evaluated during a 5-year follow-up period. A matched pair analysis was performed to further investigate the relation between MP and malignancy. RESULTS: 94 (2.5%) patients with MP were identified (mean age, 66.6 ± 11.2 years, 70.2% male). MP coexisted with malignancy (especially prostatic carcinoma) in 48.9% of patients, and this was slightly but significantly higher than in age- and sex-matched control patients (n = 188, 46.3%). In 48 patients, MP was presumed to be idiopathic. The most frequent presenting symptom was pain (54.3%). Laboratory findings revealed increased acute-phase reactants in half of the patients with MP. CT findings included increased density of mesenterial fat (mean, -56.8 ± 10.8 HU), fat ring sign, tumoural pseudocapsule and small soft-tissue nodules. Patients with MP (14.6%) developed significantly more malignancies during a 5-year follow-up than did the control group (6.9%). One patient was treated with prednisone without satisfactory response. CONCLUSION: The prevalence of MP in this study was 2.5%. In most patients, radiologic features included increased mesenteric fat density, fat ring sign and small soft-tissue nodules. MP was associated with a significant higher prevalence of coexisting malignancies and a higher prevalence of future cancer development. ADVANCES IN KNOWLEDGE: A more accurate prevalence of MP on CT is demonstrated. An underlying malignancy may play a role.

Long-term safety and efficacy of a tamoxifen-based treatment strategy for idiopathic retroperitoneal fibrosis.

BACKGROUND: Tamoxifen may be a viable treatment option for idiopathic retroperitoneal fibrosis (iRPF) but data are limited and its long-term safety and efficacy is unclear. We describe the long-term course and outcomes in a large group of patients with iRPF treated with tamoxifen monotherapy. METHODS: This is a single-center prospective, observational study of 55 patients with iRPF treated with tamoxifen for 2years from April 1998 through April 2011. Measurements included clinical improvement, laboratory parameters and follow-up computed tomographic (CT) scanning. Treatment success was the composite endpoint of clinical improvement, mass regression and definite resolution of ureteral obstruction. RESULTS: Forty-seven (85%) patients reported substantial resolution of symptoms after median treatment duration of 3.0weeks (IQR 1.4-4.8weeks). Repeated CT scanning showed mass regression in 39 (71%) patients at 4months and 47 (85%) patients at 8months of follow-up, respectively. Nineteen (34.5%) patients did not meet the composite endpoint of treatment success, 56% of whom responded satisfactorily to second-line immunosuppressive treatment. Recurrence-free survival in patients with treatment success after post-treatment follow-up of 21months (IQR 9.0-35.0months) was 68%. Tamoxifen was well tolerated. Pulmonary embolism occurred in 2 patients receiving tamoxifen and in one patient receiving second-line treatment. CONCLUSION: Tamoxifen is a safe and viable therapeutic option in the treatment of iRPF.

Listeria peritonitis in patients on peritoneal dialysis: two cases and a review of the literature.

Two cases are reported of patients on continuous ambulatory peritoneal dialysis who presented with peritonitis caused by Listeria monocytogenes. They were successfully treated with intraperitoneal and intravenous administration of amoxicillin. In patients on peritoneal dialysis, Listeria monocytogenes is a very rare cause of peritonitis, with only 11 cases reported to date, and mainly occurring in immunocompromised patients. In contrast to the majority of the reported cases, neither of our patients had received immunosuppressive drugs. To our knowledge, these are the first two cases of Listeria peritonitis reported in the Netherlands.

Life-threatening hypokalaemic paralysis associated with distal renal tubular acidosis.

A 56-year-old woman developed acute respiratory failure requiring mechanical ventilation due to acute hypokalaemic paralysis. There was no gastrointestinal potassium loss nor was she taking diuretics. Additional analyses revealed a normal anion gap metabolic acidosis with a positive urine anion gap. An acid-load test revealed a renal urine acidification defect, leading to the diagnosis of distal renal tubular acidosis. Normalisation of serum potassium level was established with oral bicarbonate supplementation and temporary potassium supplementation.

Autoantibodies against oxidized low-density lipoprotein and lipid profile in patients with chronic periaortitis: case-control study.

Chronic periaortitis is thought to result from an autoallergic reaction to oxidized low-density lipoprotein (OxLDL). No data exist on lipid profile and atherosclerotic biomarkers. We investigated circulating levels of OxLDL and of anti-OxLDL (aOxLDL) antibodies in patients with chronic periaortitis using the cross-sectional case-control study on 20 patients with chronic periaortitis. Patients were compared to 20 age- and sex-matched controls. aOxLDL antibodies were measured by ELISA and expressed as mean optical density values at 450 nm from duplicate measurements (OD(450)). aOxLDL antibody titers (median [interquartile range]) did not differ significantly between patients and controls (aOxLDL-IgM: 0.70 [0.24-1.08] vs. 0.54 [0.25-0.73] OD(450); aOxLDL-IgG: 0.59 [0.38-0.75] vs. 0.41[0.33-0.63]OD(450)). Female patients had higher aOxLDL-IgM levels than male patients (1.02 [0.46-1.38] vs. 0.29 [0.22-0.84] OD(450); P = 0.05). aOxLDL-IgM titers were lower in patients with cardiovascular disease (CVD) than in patients without CVD (0.22 [0.16-0.37] vs. 0.92 [0.70-1.30] OD(450); P = 0.003) and correlated positively with HDL-cholesterol (r = 0.47, 95% CI 0.02-0.69; P = 0.03) and inversely with diastolic blood pressure (r = -0.46, 95% CI -0.75 to -0.01; P = 0.03) and OxLDL/apoB ratio (r = -0.41, 95% CI -0.73 to 0.04; P = 0.06). No differences or associations were found between aOxLDL-IgG titers and other variables between or within patients and/or controls. In patients OxLDL levels correlated with smoking pack-years (r = 0.58, 95% CI 0.17-0.81; P = 0.007). Data suggest a differing innate immune response to OxLDL in patients with chronic periaortitis compared to controls. Whether this response is causally related to chronic periaortitis development remains to be clarified.

Prevalence of iatrogenic admissions to the Departments of Medicine/Cardiology/ Pulmonology in a 1,250 bed general hospital.

A recent meta-analysis in this journal showed incidences between 3.4 and 33.9%. Studies performed by pharmacists and epidemiologists produced lower incidences than internists' studies. We reassessed the prevalence of iatrogenic admissions in a study of internists. Iatrogenic disease was defined as adverse drug reactions according to the World Health Organization Definition and complications induced by non-drug medical interventions. Subsequent admissions at the Departments of Medicine/Cardiology/Pulmonology in a 1,250 bed general hospital in the Netherlands from May 2007 to August 2007 were studied. 2,000 consecutive admissions were studied: 576 (29%, 26-32%) were classified as possibly iatrogenic; out of these 380 (19%, 17-22%) as definitely iatrogenic, out of whom 229 (12%, 10-14%) had already been classified as iatrogenic by the admitting physicians. Patients with cardiac disease, hypertension, gastrointestinal conditions, anticoagulant treatment and use of NSAIDs were, particularly, at risk of iatrogenic admission with percentages of 22 (16-24), 13 (11-18), 12 (9-15), and 7 (5-11) %. An independent predictor of iatrogenic admissions was age with an odds ratio of 1.27 per 10 years (p = 0.0001). 1. At least 19% of admissions to the Departments of Internal Medicine/Cardiology/Pulmonology, and, maybe, even percentages up to 29% were due to adverse drug effects. 2. A large difference between the numbers of iatrogenic admission according to the physicians in charge of admission and the investigators, 229 versus 380 patients, was observed. 3. Most often iatrogenic admissions were observed with cardiac disease, hypertension, gastrointestinal conditions, anticoagulant treatment, and use of NSAIDs.

Pseudocyst formation in retroperitoneal fibrosis relapse.

We describe a 45-year-old male patient with recurrent retroperitoneal fibrosis (RPF), in whom a pseudocyst in the peri-aortic fibrotic mantle was diagnosed. Without any intervention other than oral treatment with tamoxifen, the pseudocyst showed significant regression. Although rare, pseudocyst formation may sometimes appear in RPF and may mimic other benign and malignant conditions. This is the first paper to describe pseudocyst formation in an RPF relapse.

(18)F-fluorodeoxyglucose position emission tomography (FDG-PET) for monitoring disease activity and treatment response in idiopathic retroperitoneal fibrosis.

OBJECTIVE: To evaluate the value of (18)F-fluorodeoxyglucose positron emission tomography (FDG-PET) in monitoring disease activity and predicting treatment response in idiopathic retroperitoneal fibrosis (iRPF). PATIENTS AND METHODS: This prospective study was approved by the institutional review board. Informed consent was obtained from all patients. Twenty-six patients with iRPF receiving tamoxifen monotherapy underwent repeated FDG-PET (baseline and, if positive, at 3 months) and computed tomographic (CT) scanning (baseline, 4 and 8 months). Maximal RPF mass thickness in 3 different view directions was measured on each CT scan; FDG-uptake was semi-quantified using a visual 4-point scale. Initial and follow-up PET scan results were correlated with clinical, laboratory and CT scan follow-up data. Treatment outcome was the aggregate measure of clinical, laboratory and CT-documented response to tamoxifen. RESULTS: FDG-PET was positive in 20 patients. Patients with positive PET scan result had higher C-reactive protein level (P=0.02) and larger mass size (P=0.01) compared with patients with negative PET scan result. Visual PET score correlated with C-reactive protein level (P=0.002) and CT-documented mass thickness (P=0.04). Visual PET score decreased following treatment (P<0.01). This decrease correlated with decrease in ESR (P<0.001) but not with CT-documented mass regression. Positive predicting value (PPV) of initial positive PET scan result was 0.63; PPV of negative follow-up PET scan result in patients with initial positive PET scan result was 0.66. CONCLUSION: FDG-PET is valuable in detecting (recurrent) disease activity. Short-term follow-up with FDG-PET cannot be routinely recommended for the therapeutic evaluation of RPF disease in tamoxifen-treated patients.

Meta-analysis of recent studies on patients admitted to hospital due to adverse drug effects.

The use of drugs has expanded during the previous decade. However, earlier studies on patients admitted for adverse drugs effects (ADEs) have been heterogeneous. The objectives of this study were to assess the number of recent admissions to hospital due to ADEs and to assess the degree of heterogeneity in recent studies. Prospective studies published in the past decade were therefore pooled and compared with the pooled results from earlier studies. The pooled overall percentage in recent studies (n = 20) was 5.4% (5.0 - 5.8) and this did not significantly differ from that in the earlier studies (n = 21, pooled percentage 4.7%, 3.1 - 6.2). The studies were clinically very heterogeneous with percentages of ADEs between 3.4 and 33.2%. The nature of the patient group could be held largely responsible for the clinical heterogeneity observed.

Idiopathic retroperitoneal fibrosis: prospective evaluation of incidence and clinicoradiologic presentation.

Retroperitoneal fibrosis (RPF) is a rare disorder of unknown etiology. Its incidence is unknown, and the insidious and nonspecific nature of symptoms may contribute to considerable diagnostic delay. We conducted the current study to assess the incidence and clinicoradiologic characteristics of idiopathic RPF. For this, we evaluated prospectively 53 consecutive patients with a diagnosis of idiopathic RPF at our tertiary care referral center from April 1998 through January 2008.Calculated annual incidence of RPF was 1.3/100,000 inhabitants. Mean age was 64 +/- 11.1 (SD) yr; male-female ratio was 3.3:1.0. Median duration of symptoms was 6.0 mo (IQR 3.0-12.0). Abdominal, flank, and/or back pain and discomfort were the major symptoms, with visual analogue scale scores of 49 +/- 27.2 mm and 43 +/- 29.4 mm for pain and discomfort, respectively. Female patients had higher erythrocyte sedimentation rate (ESR), higher white blood cell count, and lower hemoglobin content than male patients at presentation. Computed tomography-documented maximal mass thickness amounted to 35 +/- 16.6 mm; craniocaudal length amounted to 137 +/- 48.8 mm. RPF mass extension up to or above the level of the renal vessels was noted in 3 patients (6%). Six patients (11%) presented with atypical RPF localization and/or bulky mass. Localized lymphadenopathy adjacent to the RPF mass was observed frequently (25%). Patients with hydronephrosis (56%) presented earlier than patients without hydronephrosis, with higher creatinine and greater mass thickness but similar pain severity. Patients were typically at high cardiovascular risk with increased-often aneurysmal-infrarenal aortic diameter (25.0 mm, IQR 22.0-30.0). RPF mass distribution was similar in patients with or without aneurysmal dilation. Occupational asbestos exposure (20%) and asbestos-related pleural changes (17%) were frequent among males. Previous or concurrent chronic inflammatory disease and/or autoimmune disease was noted in 8 patients (15%). Multivariate analysis revealed an independent association of ESR values with severity of pain and discomfort. Smoking was independently associated with infrarenal aortic diameter.In summary, annual RPF incidence is higher than previously assumed. Age at diagnosis and male-female ratio seem to have changed over time. RPF typically affects patients at high cardiovascular risk, including increased aortic diameter. Clinical presentation is influenced by sex, severity of inflammation and presence of hydronephrosis. Prolonged asbestos exposure and asbestos-related pleural changes were frequent among males. Localized lymphadenopathy adjacent to the RPF mass occurs frequently and should not confuse RPF diagnosis.

Persistent chronic peri-aortitis ('inflammatory aneurysm') after abdominal aortic aneurysm repair: systematic review of the literature.

Data on the outcome of aneurysmal chronic peri-aortitis ('inflammatory aneurysm') after open surgical aneurysm repair are few and contradictory. To what extent this inflammatory process is reversed after endovascular aneurysm repair (EVAR) is even more unclear. The objective of this review was to study the outcome of peri-aortic fibrosis (PAF) and ureteral obstruction in patients with aneurysmal chronic peri-aortitis treated with open surgery or EVAR. Medical literature was searched for pertinent articles on the outcome of PAF and ureteral obstruction after open surgery or EVAR from 1970 through October 2007. Studies were included if specific follow-up data were available on outcomes of interest. A total of 19 studies were included comprising 478 patients (open surgical, n = 426; EVAR, n = 52). Age, sex and percentage of patients with ureteral obstruction (31% vs 37%) did not differ between groups. Regression of PAF occurred more frequently after open surgery compared to EVAR (86% vs 60%; p < 0.0001). Complete regression of PAF was more frequent after open surgery compared to EVAR (52% vs 14%; p < 0.0001). After excluding patients in whom concurrent ureterolysis was performed, the frequency of persistent ureteral obstruction remained lower in patients treated surgically compared to patients treated with EVAR, albeit not statistically significant (32% vs 56%; p = 0.09). In conclusion, although open surgery is superior to EVAR in achieving regression of chronic peri-aortitis, the frequency of persistent PAF and/or ureteral obstruction is not negligible. Additional medical and/or urological treatment should be considered in selected cases of aneurysmal chronic peri-aortitis.

[Hyponatraemia as a clinical manifestation of hypopituitarism caused by neurosarcoidosis]. / Hyponatriëmie als klinische manifestatie van hypopituïtarisme door neurosarcoïdose.

Severe hyponatraemia was observed in a 35-year-old man with progressive malaise; this was caused by hypopituitarism and secondary hypocortisolism as a result ofneurosarcoidosis. Sarcoidosis is a multisystem granulomatous disorder of unknown aetiology which can develop in any of the body's organs or tissues. The central nervous system is affected in only 5-15% of patients with sarcoidosis. Neurosarcoidosis is a rare disorder with clinical heterogeneity. Extensive diagnostic procedures, including MRI of the cerebrum and histological investigation, and structural outpatient follow-up are mandatory in patients in whom neurosarcoidosis is suspected. Treatment consists mainly of high-dose corticosteroids, which usually have to be taken long-term. Clinical course and prognosis are variable, and depend on the accompanying symptoms.

Clinical value of gallium-67 SPECT scintigraphy in the diagnostic and therapeutic evaluation of retroperitoneal fibrosis: a prospective study.

BACKGROUND: Gallium-67 (67Ga) scintigraphy may be useful in evaluating patients with retroperitoneal fibrosis (RPF), but a systematic assessment of its value is lacking. OBJECTIVE: Prospective evaluation of the value of 67Ga scintigraphy in assessing active RPF disease and in predicting treatment response. METHODS: Thirty-four patients with nonmalignant RPF treated with tamoxifen underwent 67Ga scintigraphy at baseline and--if baseline gallium scan was positive--at 3 months follow-up. Gallium scans were visually scored according to pathologic 67Ga-uptake compared to normal bone marrow 67Ga-uptake. Results were correlated with other (follow-up) measurements. Value of (follow-up) 67Ga scintigraphy in predicting treatment response was also assessed. RESULTS: Gallium scans were positive in 24 patients (71%). Mass thickness was greater in patients with positive gallium scan compared with patients with negative gallium scan (P = 0.006). Visual gallium score correlated with mass thickness (P = 0.006). Visual gallium score decreased significantly following tamoxifen treatment (P < 0.0001). Decrease in visual gallium score correlated with decreases in C-reactive protein and erythrocyte sedimentation rate (P = 0.019) and with decrease in mass thickness (P < 0.01). Positive predicting value (PPV) of positive baseline gallium scan was 0.71; PPV of negative follow-up gallium scan in patients with initial positive scan was 0.89. 67Ga scintigraphy detected extra-abdominal involvement in one patient and recurrent active disease in two symptomatic patients with normal acute-phase reactants and stable residual mass. CONCLUSION: 67Ga scintigraphy is useful in assessing (recurrent) activity of RPF disease and in evaluating treatment response in patients with initial positive gallium scan.