Capi-score: a quantitative algorithm for identifying disease patterns in nailfold videocapillaroscopy.

OBJECTIVES: The European Alliance of Associations for Rheumatology (EULAR) supports the use of nailfold videocapillaroscopy (NVC) to identify disease patterns (DPs) associated with systemic sclerosis (SSc) and Raynaud's phenomenon (RP). Recently, EULAR proposed an easy-to-manage procedure, a so-called Fast Track algorithm, to differentiate SSc from non-SSc patterns in NVC specimens. However, subjectivity among capillaroscopists remains a limitation. Our aim was to perform a software-based analysis of NVC peculiarities in a cohort of samples from SSc and RP patients and, subsequently, build a Fast Track-inspired algorithm to identify DPs without the constraint of interobserver variability. METHODS: NVCs were examined by 9 capillaroscopists. Those NVCs whose DPs were consensually agreed (≥2 out of 3 interobservers) were subsequently analysed with an in-house developed software. Each variable's results were grouped according to the consensually agreed DPs in order to identify useful hallmarks to categorise them. RESULTS: Eight-hundred and fifty-one NVCs (21 957 images) whose DPs had been consensually agreed were software-analysed. Appropriate cut-offs set in capillary density and percentage of abnormal and giant capillaries, tortuosities and hemorrhages allowed DP categorization and the development of the CAPI-Score algorithm. This consisted of 4 rules: Rule 1, SSc vs non-SSc, accuracy 0.88; Rules 2 and 3, SSc-early vs SSc-active vs SSc-late, accuracy 0.82; Rule 4, non-SSc normal vs non-SSc non-specific, accuracy 0.73. Accuracy improved when the analysis was limited to NVCs whose DPs had achieved full consensus among interobservers. CONCLUSIONS: The CAPI-Score algorithm may become a useful tool to assign DPs by overcoming the limitations of subjectivity.

Fetal cardiac function in late-onset intrauterine growth restriction vs small-for-gestational age, as defined by estimated fetal weight, cerebroplacental ratio and uterine artery Doppler.

OBJECTIVE: Among late-onset small fetuses, a combination of estimated fetal weight (EFW), cerebroplacental ratio (CPR) and mean uterine artery (UtA) pulsatility index (PI) can predict a subgroup of fetuses with poor perinatal outcome; however, the association of these criteria with fetal cardiac structure and function is unknown. Our aim was to determine the presence and severity of signs indicating cardiac dysfunction in small fetuses, classified as intrauterine growth-restricted (IUGR) or small-for-gestational age (SGA), according to EFW, CPR and UtA-PI. METHODS: A cohort of 209 late-onset small fetuses that were delivered > 34 weeks of gestation was divided in two categories: SGA (n = 59) if EFW was between the 3(rd) and 9(th) centiles with normal CPR and UtA-PI; and IUGR (n = 150) if EFW was < 3(rd) centile, or < 10(th) centile with a CPR < 5(th) centile and/or UtA-PI > 95(th) centile. The small population was compared with 150 appropriately grown fetuses (controls). Fetal cardiac morphometry and function were assessed by echocardiography using two-dimensional M-mode, conventional and tissue Doppler. RESULTS: Compared with controls, both IUGR and SGA fetuses showed larger and more globular hearts (mean left sphericity index ± SD: controls, 1.8 ± 0.3; SGA, 1.5 ± 0.2; and IUGR, 1.6 ± 0.3; P < 0.01) and showed signs of systolic and diastolic dysfunction, including decreased tricuspid annular plane systolic excursion (mean ± SD: controls, 8.2 ± 1.1; SGA, 7.4 ± 1.2; and IUGR, 6.9 ± 1.1; P < 0.001) and increased left myocardial performance index (mean ± SD: controls, 0.45 ± 0.14; SGA, 0.51 ± 0.08; and IUGR, 0.57 ± 0.1; P < 0.001). CONCLUSIONS: Despite a perinatal outcome comparable to that of normal fetuses, the population of so-defined SGA fetuses showed signs of prenatal cardiac dysfunction. This supports the concept that at least a proportion of them are not 'constitutionally small' and that further research is needed.

Progression of subclinical cardiovascular disease in patients with HIV.

OBJECTIVE: Human immunodeficiency virus (HIV) infected patients are at increased risk of cardiovascular disease (CVD). Multidetector computed tomography (MDCT) stratifies cardiovascular risk in asymptomatic patients with subclinical atherosclerosis. The aim of this study was to determine the ability of MCTD and clinical and laboratory parameters to assess subclinical CVD progression in HIV patients. METHODS: Prospective longitudinal cohort study of patients with at least 10 years of HIV infection and 5 years of antiretroviral therapy history, low cardiovascular risk and monitored for 6 years (2015-2021). All patients underwent clinical assessment, blood analysis, carotid ultrasound, and gated MDCT in 2015 and 2021. RESULTS: Sixty-three patients (63.5% male) with a mean age of 49.9 years (standard deviation [SD], 10.5) were included in 2015; 63 of them were followed until 2021. Comparing the results from 2015 with those from 2021, Systematic Coronary Risk Estimation-2 (SCORE2) was 2.9% (SD, 2.1) vs. 4.4% (SD,3.1); Multi-Ethnic Study of Atherosclerosis score (MESA risk) was 3.4 (SD 5.8) vs. 6.0 (SD 8.6); coronary artery calcification CAC) score >100 was 11.1% vs. 25.4% (P < 0.05); and 11% vs. 27% had carotid plaques (P = 0.03). CONCLUSIONS: After six years of follow-up, an increase in SCORE2, carotid plaques, CAC scoring and MESA risk was observed. MDCT findings, along with other clinical and laboratory parameters, could play an important role as a marker of CVD progression in the evaluation of patients with HIV and low cardiovascular risk.

High prevalence of upper urinary tract involvement detected by 111indium-oxine leukocyte scintigraphy in patients with candiduria.

The purpose of this investigation was to assess the prevalence of upper urinary tract involvement in patients with candiduria by means of (111)indium-oxine-labeled leukocyte scintigraphy. An observational cohort study of patients with confirmed candiduria was conducted in an acute-care teaching hospital in Spain from March 2006 through February 2009. An (111)In-labeled leukocyte scan was performed in order to assess the upper urinary tract involvement. A series of non-matched patients without candiduria nor bacteriuria undergoing scintigraphy to exclude infections in other sites than the urinary tract was also studied. Demographics, baseline illness, and clinical data were recorded. Candiduria was detected in 428 patients, and scintigraphy was performed in 35 of these patients. Twenty-nine patients without candiduria nor bacteriuria were also studied. Positive renal scintigraphy was documented in 24 (68%) patients with confirmed candiduria and in 3 (10%) patients without candiduria (p < 0.005). Renal uptake was not associated with a higher mortality nor with re-admissions. Subclinical pyelonephritis could be more frequent in patients with candiduria than it has been previously considered.

Evaluation of endothelial function and subclinical atherosclerosis in patients with HIV infection.

The aim of this study was to analyse the association between human immunodeficiency virus (HIV) related clinical and analytical parameters and the presence of subclinical atherosclerosis as well as endothelial dysfunction. This was a prospective cohort study of HIV-positive patients who underwent intima media thickness (IMT) determination and coronary artery calcium scoring to determine subclinical atherosclerosis. To detect endothelial dysfunction, the breath holding index, flow-mediated dilation and the concentration of endothelial progenitor cells (EPCs) were measured. Patients with an IMT ≥ 0.9 mm had an average of 559.3 ± 283.34 CD4/µl, and those with an IMT < 0.9 mm had an average of 715.4 ± 389.92 CD4/µl (p = 0.04). Patients with a low calcium score had a significantly higher average CD4 cell value and lower zenith viral load (VL) than those with a higher score (707.7 ± 377.5 CD4/µl vs 477.23 ± 235.7 CD4/µl (p = 0.01) and 7 × 104 ± 5 × 104 copies/ml vs 23.4 × 104 ± 19 × 104 copies/ml (p = 0.02)). The number of early EPCs in patients with a CD4 nadir < 350/µl was lower than that in those with a CD4 nadir ≥ 350 (p = 0.03). In HIV-positive patients, low CD4 cell levels and high VL were associated with risk of developing subclinical atherosclerosis. HIV patients with CD4 cell nadir < 350/µl may have fewer early EPCs.

Graded contrast echocardiography in pulmonary arteriovenous malformations.

To compare the results of transthoracic contrast echocardiography (TTCE) adding a grading scale with the results of thoracic computed tomography (CT) in order to optimise the use of both techniques. 95 patients with hereditary haemorrhagic telangiectasia (HHT) were examined with TTCE and thoracic CT to detect pulmonary arteriovenous malformations (PAVMs). According to previous studies, TTCE was divided into a four grade scale depending on the degree of opacification of the left ventricle after the administration of a contrast agent. Of the 95 patients (50.5% female; mean age 46 yrs), none with normal or grade 1 TTCE had detectable PAVMs on thoracic CT. Shunts of grades 2, 3 and 4 were associated with PAVMs according to thoracic CT in 25, 80, and 100% of the cases. There was a statistically significant association between the TTCE grade and the detection of a PAVM by thoracic CT. There were also statistically significant associations between TTCE grade and the cardiac cycle when the contrast was first visible in the left atrium, and size of the feeding artery. Graded TTCE and timing of left atrium opacification may be useful techniques in selecting HHT patients for PAVM screening with thoracic CT scans.

Lupus nephritis with preserved kidney function associated with poorer cardiovascular risk control: A call for more awareness.

BACKGROUND: Despite the improvement in the prognosis of lupus nephritis (LN), the cardiovascular morbimortality remains high. The early recognition and remission of flares, while trying to avoid the metabolic adverse effects of medication, must be mandatory. AIM: The aim of our study was to assess the cardiovascular (CV) risk profile in a cohort of lupus patients with preserved kidney function after a nephritis episode, compared to patients without a nephritis flare. METHODS: 130 patients diagnosed of SLE (32 with previous nephritis flare and 98 without) were studied in order to evaluate the CV risk profile, despite the preserved kidney function. RESULTS: The most prevalent risk factors were sedentary lifestyle (57.6%), overweight/obesity (38.3%) and dyslipidemia (36%), followed by smoking (32%) and hypertension (16%). Though more than a half (53.1%) was taking CV medication, a high percentage did not reach a therapeutic target value, especially regarding obesity (11.5%) and cholesterol levels (LDL-C of 16%). The prevalence of dyslipidemia (53.1% vs 30.6%), smoking (46.6% vs 27.5%), left ventricular hypertrophy (LVH) (21.4% vs 6.4%) and lower HDL-C (48.6mg/dL vs 55.4mg/dL) were significantly different in the group with previous nephritis flare. Moreover, young patients with lupus nephritis, received more pulses of corticosteroids and cyclophosphamide, had higher prevalence of hypertension, LVH, higher proteinuria, hospital admissions and waist circumference, constituting the subgroup of patients with greater aggregation of CV risk factors. CONCLUSIONS: Patients with previous nephritis flare showed a poor control of CV risk factors despite the preserved renal function, these patients would require a closer therapeutic management.

Ventilation/Perfusion SPECT lung scintigraphy and computed tomography pulmonary angiography in patients with clinical suspicion of pulmonary embolism.

UNLABELLED: The aim was to compare ventilation/perfusion SPECT lung scintigraphy (V/Q-SPECT) and computed tomography pulmonary angiography (CTPA) in patients with suspicion of pulmonary embolism (PE). MATERIAL AND METHODS: This prospectively designed study included 53 patients with intermediate or high clinical probability of PE. A V/Q-SPECT and CTPA was performed on all patients. The V/Q-SPECT was interpreted according to the European Association of Nuclear Medicine and Molecular Imaging (EANMMI) guidelines. CTPA was reported as positive, negative, or indeterminate. RESULTS: CTPA was positive in 22 cases, negative in 28, and indeterminate in 3. V/Q-SPECT was positive in 27 cases, negative in 24, and non-diagnostic in 2. In the 22 with positive CTPA, V/Q-SPECT was positive in 18, negative in 3, and non-diagnostic in 1. In the 28 with negative CTPA, V/Q-SPECT was positive in 8, negative in 19, and non-diagnostic in 1. In the 3 with indeterminate CTPA, V/Q-SPECT was positive in 1 and negative in 2. In the 2 non-diagnostic cases V/Q-SPECT, CTPA was positive in 1 and negative in one. In the 10 high clinical probabilities, CTPA and V/Q-SPECT were positive in 7, negative in 2, and in 1, CTPA was positive and V/Q-SPECT negative. In the 38 intermediate probability group, CTPA and V/Q-SPECT were positive in 11, negative in 17, with CTPA negative and V/Q-SPECT positive in 8, and in 2 CTPA was positive and V/Q-SPECT negative. The results show that V/Q-SPECT detected PE in 5 patients more than CTPA. CONCLUSION: Our results show a 77% concordance of both techniques. Overall V/Q-SPECT detected PE in 18% more patients than CTPA in the intermediate group. Both techniques have a complementary role when a diagnosis cannot be made with one of them.

Talc granulomatosis. A differential diagnosis of interstitial lung disease in HIV patients.

Consumers of adulterated drugs may present with talc-induced interstitial lung disease. A transbronchial biopsy specimen showing granulomas with intracellular talc crystals is necessary to confirm this diagnosis. In patients infected with HIV, such a condition can be indistinguishable at first glance from opportunistic infections or other pulmonary diseases. A case is presented of a seropositive patient whose chest radiographs showed a diffuse interstitial pattern.

Factitious disorders mimicking systemic lupus erythematosus.

Factitious disorders are one of the most difficult challenges to the sagacity of the physician. Self-inflicted injuries and diseases have been recognized since Biblical times. In the Middle Ages, hysterics were known to place leeches in their mouths to simulate hemoptysis and to abrade their skin to reproduce skin conditions. Münchausen syndrome, originally described in 1951, is the term applied to persons who seek medical care by feigning illness in the absence of any organic medical or surgical disease. Since the first report of the syndrome, many case reports have documented the performance of unneeded operations and the administration of dangerous drugs to these patients. Rheumatologic manifestations in this syndrome are rare, and include septic arthritis, osteomyelitis, destructive arthropathy, reflex sympathetic dystrophy and systemic lupus erythematosus (SLE). We reviewed the English literature for the past 29 years and found only 8 patients with Münchausen syndrome who simulated SLE. It is interesting to consider the damage mechanisms (some of the patients satisfied 4 or more of the criteria for the classification of SLE) and how a complex disease with a broad spectrum of manifestations such as lupus can be simulated, the only limits being the patient's knowledge and imagination.

An unusual form of pneumatosis intestinalis associated with appendicitis.

A rare case of pneumatosis intestinalis is described in a patient with appendicitis. Gas in the colonic wall, epiploic appendices and mesentery caused a new appearance not previously reported on plain radiography or CT. The relevance of an associated redundant right colon is also discussed.

Prosthetic mesh used for inguinal and ventral hernia repair: normal appearance and complications in ultrasound and CT.

The use of prosthetic mesh has now become accepted practice in the treatment of patients with both inguinal and ventral hernias. This pictorial review illustrates the various radiological appearances of these meshes and also demonstrates the post-operative complications associated with their implantation.

[Bronchial granular cell myoblastoma associated with epidermoid carcinoma]. / Mioblastoma bronquial de células granulares asociado a carcinoma epidermoide.

A 43-year-old male simultaneously presented a bronchial granular cell myoblastoma affecting the bronchi of the lower right lobe, and a bronchogenic epidermoid carcinoma with mediastinal spread. In both cases diagnosis was established through bronchial and mediastinal biopsies, with posterior immunohistochemical evaluation to confirm the nature of the neoplastic lesions. The clinicopathological characteristics of this rare observation not previously reported in our country are commented.

Collateral biliary circulation in portal hypertension syndrome.

OBJECTIVE: to describe collateral circulation in the gallbladder wall in relation with portal hypertension syndrome, and to determine whether this syndrome is frequently associated portal vein thrombosis. METHODS: images were retrospectively reviewed for 9 patients with previously diagnosed portal hypertension syndrome, in whom ultrasound results suggested the presence of varices of the cystic vein. RESULTS: four patients showed signs suggesting portal vein thrombosis. The gallbladder wall showed diffuse hypoechoic thickening in all patients, 7 of whom had intramural dilation of tubular, tortuous appearance. Ultrasound findings, however, were not very specific, and differential diagnosis with a large number of other entities is required to rule out other possible causes of focal and diffuse thickening of the gallbladder wall. The use of Doppler sonographic techniques made it possible to determine the cause of the varices, and to confirm suspicions of portal thrombosis. This method was found to be just as sensitive as ultrasound imaging, and much more specific. Angiograms obtained in 3 patients for different reasons confirmed the ultrasonographic findings in all cases. CONCLUSIONS: this study confirms the association between thromboses and varices, and analyzes the physiopathological hypotheses invoked to explain this association. We emphasize the need for correct diagnosis, given the frequency of surgical iatrogenic bleeding or misdiagnosis resulting from confusion with other possible causes of gallbladder wall thickening. Doppler ultrasound is considered the ideal diagnostic method as it is harmless, sensitive and specific.

[Cranial hyperostosis as a metastasic adenocarcinoma presentation form]. / Hiperostosis craneal como forma de presentación de un adenocarcinoma metastásico.

Hyperostosis is a volume-unit osseous increase of very diverse etiology. We present the case of a 68-year woman with a cranial hyperostosis debuting with frontal protrusion, headache and neurologic symptoms. Image proves demonstrated a hyperostosis in the calotte and meningeal enhancement, without intracerebral lesions nor malignant cells in the cerebrospinal fluid. Analytic data were unspecific. Cranial biopsy showed huge neoplastic infiltration in bone and meninges. Primary site remained unknown after a CAT and a mammography.

[Munchausen's syndrome: a diagnostic challenge]. / Síndrome de Munchausen: reto al diagnóstico.

The Munchausen's syndrome is a variant of chronic factitious illness with predominantly physical signs and symptoms under the conscious control of the patient without any obvious gain. The disorder has also been called hospital addiction, professional patient syndrome and in the field of dermatology, dermatitis artefacta. Munchausen's syndrome may mimic many different conditions, especially acute abdominal pain, hemorrhagic disorders, rheumatologic manifestations, factitious fever, and injury of skin. We describe a 28-year-old woman, who was admitted at a local teaching hospital and studied during three years with complaints of a bleeding ulcer on the left knee, severe enough to require blood transfusions. Many procedures and tests were performed without pathologic findings. Afterward, she was seen at our institution; during the study, the suspicion had arisen that the patient had manipulated her ulcer, after the diagnosis of factitious disease was communicated, she admitted. Munchausen's syndrome may have a broad spectrum of manifestations; early recognition can avoid unnecessary treatments and invasive diagnostic studies, with their inherent risk of complications.