Pulmonary and abdominal sarcoidosis, the great imitators on imaging?

Sarcoidosis is an insidious disorder that virtually affects every body organ. Lungs are the site most often affected (in up to 90% of patients) followed by intra thoracic more often than peripheral lymph nodes and other sites can be involved in different percentages. The evaluation of pulmonary sarcoidosis is best performed with high-resolution computed tomography (HRCT), as traditional chest X-ray has a low resolution and can be negative or give non-significant results. Disorders such as interstitial lung diseases (ILDs), tuberculosis, lung cancer and lymphangitis carcinomatosis can manifest with similar radiological findings that can deceive clinics and radiologists. The need of a clear distinction between these conditions is important not only for diagnostic purposes but also because treatment differs significantly in different conditions. However, conventional Ultrasound (US) can be negative if small lesions are present and false negative images can result if US is not followed by a contrast-imaging technique. Contrast enhanced computed tomography (CECT) and magnetic resonance imaging (CEMRI) are preferred to detect single or multiple masses, which appear hypodense and hypointense after contrast agent administration, respectively. We think that a correct algorithm should include a thorough clinical and radiological evaluation, a definite biopsy of affected tissues revealing classical non-caseating granulomas and a certain exclusion of conditions that can give similar clinical/histophatological patterns before considering the diagnosis of sarcoidosis. Only in these cases, a diagnosis of sarcoidosis can be sufficiently achieved before starting an appropriate treatment.

1T magnetic resonance imaging in the diagnosis of giant cell arteritis: comparison with ultrasonography and physical examination of temporal arteries.

OBJECTIVE: To assess the usefulness of 1T magnetic resonance imaging (MRI) of temporal arteries and to compare 1T MRI with duplex ultrasonography (US) and physical examination of temporal arteries for the diagnosis of giant cell arteritis (GCA) in patients with suspected GCA. METHOD: The superficial temporal arteries of 20 consecutive patients with a suspected diagnosis of GCA were examined using a 1T MRI scanner. Fat-saturated multislice T1-weighted spin-echo images were acquired perpendicularly to the orientation of the vessel. In all cases, MRI results were compared to US and temporal artery examination findings. Temporal artery biopsies were performed in all patients. RESULTS: Mural contrast enhancement of the temporal arteries on MRI had a sensitivity of only 33.3% and a specificity of 87.5% for the diagnosis of biopsy-proven GCA. Compared with the diagnosis of GCA by the American College of Rheumatology criteria, MRI had a sensitivity and specificity of 27.2% and 88.9%, respectively. Temporal artery abnormalities on physical examination and the presence of a hypoechoic halo on US had a higher sensitivity (66.7% and 77.7%, respectively) and a higher specificity (100% for both) compared to MRI findings. CONCLUSION: 1T MRI is not useful for the diagnosis of GCA because of its low sensitivity. US and physical examination of temporal arteries had a better diagnostic accuracy. However, our data does not exclude a diagnostic role for higher-resolution MRI.

The incidence of venous thromboembolism following gynecologic laparoscopy: a multicenter, prospective cohort study.

BACKGROUND: Information on the incidence of venous thromboembolism (VTE) following laparoscopic procedures is inadequate and there is currently no solid evidence to guide the use of thromboprophylaxis in this setting. Gynecologic laparoscopy is a common procedure, and is frequently performed in low-risk patients. To our knowledge, there are no clinical studies specifically designed to assess the incidence of VTE in this setting. METHODS: In a prospective cohort study, consecutive patients undergoing gynecologic laparoscopy underwent compression ultrasonography (CUS) and clinical assessment to evaluate the incidence of clinically relevant VTE. CUS was performed 7 +/- 1 and 14 +/- 1 days postoperatively. A subsequent telephone contact was scheduled at 30 and 90 days. No patient received pharmacologic or mechanical prophylaxis. Patients with malignancy or previous VTE were excluded from the study. RESULTS: We enrolled 266 consecutive patients; mean age was 36.3 years, range: 18-72. The most common indications for laparoscopy were ovarian cysts in 25.6% of patients, endometriosis in 21.0% of patients, unexplained adnexal masses in 12.4% of patients, and infertility in 7.5% of patients. The mean duration of the procedure was 60.5 min (range: 10-300 min). In particular, in 55.6% of patients the duration exceeded 45 min. There were neither episodes of CUS detected DVT (0/247; 0%, 95% CI 0-1.51%) or clinically relevant VTE after follow-up (0/256; 0%, 95% CI 0-1.48%). No patient died of fatal pulmonary embolism (0/266; 0%, 95% CI 0-1.42%). CONCLUSIONS: Gynecologic laparoscopy in non-cancer patients is a low-risk procedure for postoperative VTE.

Clinical experience with retrievable vena cava filters: results of a prospective observational multicenter study.

BACKGROUND: Retrievable inferior vena cava (IVC) filters offer the attractive possibility to be definitive or to be removed when they become unnecessary. OBJECTIVE: The purpose of this study was to evaluate the efficacy and the likelihood to remove the retrievable IVC filter ALN. METHODS: A total of 30 patients (13 males and 17 females, mean age 57 +/- 15 years) underwent placement of ALN filters. Indications for implantation were acute venous thromboembolism (VTE) with a contraindication to anticoagulation in 26 cases (86%), primary prophylaxis after major trauma in two cases (7%) or before surgery in two patients with very high thromboembolic risk (7%). RESULTS: The filter was successfully placed in all patients. After a median follow-up of 18.2 months, there were three cases (10%) of trapped emboli within the filter, one case (3%) of asymptomatic migration of the filter toward the heart and two patients (7%) had deep vein thrombosis (DVT) recurrences. ALN retrieval was attempted through transjugular approach in 18 patients (60%) and the maneuver was successful in 14 of them (78%); when the decision of removal was taken more than 3 months after the implantation, the retrieval was possible only in four of eight patients (50%). The median implantation period was 123 days (range: 30-345). CONCLUSIONS: The present study shows the efficacy of ALN filter; it also demonstrates the feasibility and safety of retrieval after a medium-term period of placement. Removal after 3 months after implantation can be unsuccessful and maximum implantation time requires further studies.

Incidence of cancer after a first episode of idiopathic venous thromboembolism treated with 3 months or 1 year of oral anticoagulation.

BACKGROUND: A prolonged treatment with oral anticoagulants has been claimed to reduce the incidence of newly diagnosed cancer in the long-term follow-up of patients with venous thromboembolism. OBJECTIVES: In a multicenter prospective study we assessed the incidence of newly diagnosed clinically overt cancer in patients with a first episode of idiopathic venous thromboembolism (VTE) treated with oral anticoagulants for 3 months or 1 year. PATIENTS AND METHODS: Consecutive patients with an idiopathic venous thromboembolism who had completed 3 months of oral anticoagulant therapy without having a recurrence, bleeding or newly diagnosed cancer were randomized to discontinue oral anticoagulant therapy or to continue it for nine additional months. Idiopathic venous thromboembolism was defined as thrombosis occurring in the absence of known cancer, known thrombophilia, or temporary risk factors for venous thromboembolism. All patients were followed up for at least 1 year after randomization. RESULTS: A total of 429 patients, 265 patients with DVT and 164 with PE, were followed up for an average of 43.7 months after randomization. A newly diagnosed cancer occurred in 32 patients (7.5%), 13 (6.2%) of the 210 patients treated for 3 months and 19 (8.7%) of the 219 patients treated for 1 year (RR = 0.71, 95% confidence interval 0.36-1.41). CONCLUSIONS: The incidence of newly diagnosed clinically overt cancer is not reduced in patients with idiopathic venous thromboembolism treated with 1-year anticoagulant treatment compared with patients treated for 3 months.

[Left atrial thrombosis in patients with antiphospholipid antibody syndrome and mesenchymal abnormal septum]. / Trombosi atriale destra in paziente con sindrome da anticorpi antifosfolipidi e setto anomalo mesenchimale.

Antiphospholipid antibodies are a heterogeneous family of immunoglobulins that includes lupus anticoagulant and anticardiolipin antibodies. They are strongly associated with a clinical syndrome characterized by venous and arterial thrombosis and spontaneous fetal losses. This syndrome may be primary or else secondary to autoimmune or neoplastic diseases. The cardiovascular system is frequently involved with mitral or aortic insufficiency, juvenile myocardial infarction, and primitive pulmonary hypertension. However, the occurrence of intracardiac thrombi is rare. We describe a case of an intracardiac right atrial thrombus in a 19-year-old asymptomatic woman who was admitted in December 1998 to the Thrombosis Center owing to the finding, during routine work-up, of a prolonged activated partial thromboplastin time (71 s) and thrombocytopenia (71 x 1000/mm3), a positive antinuclear antibody test (1/320), positivity for lupus anticoagulant, and increased IgG (92 GPL-U/ml) and IgM (27 MPL-U/ml) anticardiolipin antibodies. Six months later, the patient presented with headache, edema and cyanosis of the face and jugular swelling. Transthoracic and transesophageal echocardiography revealed a right atrial mass which was clearly distinguishable from the tricuspid valve and extended to the superior vena cava. The patient was successfully submitted to surgical excision of the thrombus. Histology revealed that the mass was adherent to an abnormal septum consisting of mesenchymal tissue. Although the American Rheumatology Association criteria for the diagnosis of systemic lupus erythematosus were not fulfilled, the positivity of antinuclear antibody test is in favor of a lupus-like syndrome. The decision to opt for surgical excision of the thrombus was determined by the unclear nature of the atrial mass. It may be necessary that such patients be submitted to anticoagulant therapy for the rest of their lives or temporarily (6-12 months). This underscores the importance of the anatomical abnormality as a promoting factor. Transthoracic echocardiography (as well as transesophageal echocardiography in selected cases) must be considered as an essential component of the initial diagnostic work-up in patients presenting with antiphospholipid antibodies.

Thrombosis associated with the prothrombin G-->A20210 mutation in Behçet's disease.

We describe 2 cases of Behcet's disease (BD) and thrombosis who were heterozygous for the prothrombin G-->A20210 mutation. In one case, progressive uncontrolled thromboses led to death. Case-control studies are needed to support the hypothesis of the role of the prothrombin A20210 allele as a risk factor for venous thrombosis in some patients with BD.

[The antiphospholipid syndrome during chronic lymphatic leukemia. An association with anti-factor VIII antibodies]. / Sindrome da anticorpi antifosfolipidi in corso di leucemia linfatica cronica. Associazione con autoanticorpi anti-fattore VIII.

Activated partial thromboplastin time may be prolonged as the result of either of two different autoimmune complications of chronic lymphocytic leukemia: the development of antiphospholipid antibodies, such as lupus anticoagulant or anticardiolipin antibodies, or anti-factor VIII inhibitors, such as acquired hemophilia A. In the rare simultaneous occurrence of both inhibitors, differential diagnosis of a prolonged activated partial thromboplastin time poses a number of problems during laboratory work-up, due to mutual interference of the commonly performed tests. Only careful clinical follow-up can disclose the significance of the laboratory findings. We report the case of concurrent antiphospholipid antibodies (lupus anticoagulant positivity, anticardiolipin antibodies; IgM 3880 MPL/mL and IgG 265 GPL/mL) and anti-factor VIII antibodies (46.8 Bethesda Units) in a patient with chronic B-cell lymphocytic leukemia who had prolonged activated partial thromboplastin time (78.8 s). The relationship between lymphoproliferative and antiphospholipid syndrome, laboratory work-up in the case of the association of antiphospholipid and anti-factor VIII antibodies, and related problems that occur during clinical management of the patient are also discussed.

[Celiac disease (familial) associated with sarcoidosis. Clinical case and review of the literature]. / Associazione di malattia celiaca (familiare) e sarcoidosi. Caso clinico e revisione delle letteratura.

The authors report a case of a 42 year-old patient, female, already suffering in 1990 from pulmonary sarcoidosis at 0 radiological stage, together with uveitis and relapsing erythema nodosum, with dyspepsia and weight loss which benefited from corticosteroidal therapy, repeatedly applied at the relapses of Erythema Nodosum. This therapy induced clinical recovery and marked weight gain. After 3 years (in 1993), the appearance of chronic diarrhoea, weight loss, oedemas of the lower limbs and altered laboratory findings which suggested malabsorbtive syndrome, made us verify with clinical-instrumental examinations (serum AGA IgA and IgG, Xilose test, perendoscopic jejunal biopsy) the diagnosis of fully clinically expressed adult coeliac disease. The screening of close relatives, showed that the patient's brother, HLA like (HLA A1-B8), was suffering from a less expressive coeliac disease. After a wide review of the literature, authors emphasize particular aspects of both diseases, reporting clinical manifestations, possible morbid linkage and prognostic factors. They underline epidemiological, pathogenetic and genetic/immunological similarity. bound to support a possible non-causal linkage of the diseases, even within the family. The authors think this linkage to be underestimated, because it is not often searched for on identified.

[Ovarian hyperstimulation syndrome with bilateral hydrothorax and ascites]. / Sindrome da iperstimolazione ovarica con idrotorace bilaterale ed ascite.

Ovarian hyperstimulation syndrome (OHSS) is an unusual complication of ovarian stimulation with exogenous gonadotrophins. We describe a case of severe OHSS with bilateral hydrothorax and ascites. We discuss the different pathogenetic hypothesis and the differential diagnostic possibility.