P. Garau

Joint and tendon involvement in systemic lupus erythematosus: an ultrasound study of hands and wrists in 108 patients

OBJECTIVE To estimate the prevalence of, and identify factors associated with, hand and wrist US alterations in a large cohort of SLE patients. METHODS One hundred and eight consecutive SLE patients were recruited and classified according to arthropathy type and the musculoskeletal item of the British Isles Lupus Assessment Group (BILAG) 2004 score. US examinations were performed on hand and wrist flexor tendons, wrist extensor tendons, second and third MCP and wrist joints bilaterally using a multi-planar scanning technique. RESULTS US examination showed joint involvement in 42/108 (38.8%) subjects, tendon involvement in 44/108 (40.7%) and both in 22/108 (20.3%). Patients with rhupus syndrome (n = 8) carried a higher incidence of inflammatory changes (87%) and erosions (87%) compared with the six with Jaccouds arthropathy (50% and 17%, respectively) and the 94 with non-deforming X-ray non-erosive arthropathy (37% and 21%, respectively). Power Doppler signal was prevalent in patients scoring A (n = 4) or B (n = 9) on the musculoskeletal item of the BILAG 2004, and was significantly more frequent at the joint (92%) and tendon (54%) level than in the 26 patients scoring C (19%, P = 0.0007 and 15%, P = 0.016, respectively) and in the 69 scoring D (3%, P < 0.0001 and 3%, P < 0.0001). US changes in patients who scored C or D were more expressed at the tendon level (50% and 29%, respectively) than at the joint level (35% and 9%, respectively). CONCLUSION The picture of musculoskeletal US in SLE depends on arthropathy subtype and disease activity. US examination could be a valid and reliable tool to monitor musculoskeletal features and therapeutic outcomes in SLE patients.

Abnormalities of magnetic resonance imaging of the central nervous system in patients with systemic lupus erythematosus correlate with disease severity

SummaryForty randomly selected patients with systemic lupus erythematosus (SLE) were studied by clinical and serologic parameters and magnetic resonance imaging (MRI). Abnormal MRI was found in 15/40 patients (37,5%): all 15 cases showed multiple widespread small-sized areas of increased signal in T2 in the white matter; in one of these patients MRI also displayed a large area with a reduced signal in T1 and an increased signal in T2 involving both the white and the gray matter. Among the 15 patients with abnormal MRI, only 7 had neuropsychiatric symptoms. The presence of MRI changes was highest in patients with organic type symptoms and was associated to the highest disease severity scores. A long-term follow up of asymptomatic patients would be useful to establish whether the application of MRI is appropriate for the assessment of CNS involvement in SLE.

Absence of epicardial coronary stenosis in patients with systemic sclerosis with severe impairment of coronary flow reserve

Systemic sclerosis (SSc) is known to be characterised by a diffuse microvascular pathological process leading to cutaneous and visceral changes and to related clinical manifestations. Both necropsy studies1,2 and in vivo investigations3–5 have shown that in a number of patients with SSc there is evidence of a coronary microvascular disease, while coronary artery disease does not exceed that seen in a control group. In particular, myocardial perfusion defects on thallium-201 scintigraphy usually occur in the absence of angiographic evidence of coronary stenosis.3 Recently, we used a new and non-invasive method of contrast enhanced, transthoracic, second harmonic echo Doppler in patients with SSc to evaluate the coronary flow reserve (CFR), a functional variable measuring the ability of the coronary microvasculature to adapt its lumen to a vasodilating stimulus.6 We detected a significant reduction of the CFR …

Bleomycin-induced scleroderma: Report of a case with a chronic course rather than the typical acute/subacute self-limiting form

Abstract: We report a case of bleomycin-induced scleroderma in a 35-year-old woman treated with chemotherapy for Hodgkin’s disease. Approximately 6 months after the first chemotherapy cycle, the patient developed skin sclerosis in both arms. The lesion showed no signs of spontaneous clinical amelioration and treatment with steroids was unsuccessful. A partial remission of the skin sclerosis was instead obtained by the administration of d-penicillamine. A family history revealed other cases of autoimmune diseases and HLA typing showed the presence of antigens associated with scleroderma. The association between bleomycin therapy and scleroderma is discussed.

Tracheo-bronchial Mucociliary Clearance in Patients with Primary and Secondary Sjögren's Syndrome

We determined the tracheo-bronchial mucociliary clearance (MCC) in order to evaluate a possible impairment of this function in patients affected by Sjögrens syndrome (SS) with or without overt clinical symptoms of xerotrachea. The MCC was expressed as flow rate (mm/min) and studied in 22 non-smoking SS patients (10 pSS and 12 sSS) and in 8 control subjects by specifically adapted ventilation lung scintigraphy (VLS). The MCC in the control group was 5.9 +/- 1.1 mm/min. No values were produced for MCC in 16 SS patients (8 pSS and 8 sSS) in the time interval considered and were reduced in the remaining 6 SS patients (3.3 +/- 1.2 mm/min). In all nine cases with clinical evidence of xerotrachea no values for MCC were obtained. A significant correlation was found between the MCC values and the rate of stimulated salivary excretion determined by dynamic scialoscintigraphy in the same patients (p < 0.001). These preliminary data show that the majority of SS patients studied presented with MCC impairment, always found when clinical symptoms of xerotrachea were present.

Rituximab treatment for 'rhupus syndrome': clinical and power-Doppler ultrasonographic monitoring of response. A longitudinal pilot study.

Objective To evaluate the safety and efficacy of rituximab in patients suffering from rhupus unresponsive to therapy with non-biological disease-modifying anti-rheumatic drugs (DMARDs). Methods Six patients fulfilling criteria for both rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE) and with a DAS28 score >5.1 were enrolled to receive two fortnightly 1000 mg rituximab doses at baseline and after 28 weeks. All patients underwent clinical, laboratory, and power- Doppler (PD) ultrasonographic (US) assessment at baseline and after 14, 28 and 56 weeks. Results A sustained improvement in DAS28, SLEDAI, HAQ, laboratory markers and ultrasound indices together with a significant reduction in the daily dose of prednisone were observed throughout follow-up. Conclusion Rituximab may be a safe and effective therapeutic option in refractory rhupus patients.

Safety of Abatacept in Rheumatoid Arthritis With Serologic Evidence of Past or Present Hepatitis B Virus Infection

Rheumatoid arthritis (RA) with concomitant hepatitis B virus (HBV) infection represents a therapeutic challenge due to the risk of HBV reactivation under immunosuppressive treatment. To date there are few data coming from anecdotal case reports that concern HBV reactivation following treatment with abatacept. This observational retrospective study was aimed to assess the safety profile of abatacept in this particular clinical setting.

Prognostic impact of coronary microcirculation abnormalities in systemic sclerosis: a prospective study to evaluate the role of non-invasive tests

IntroductionMicrocirculation dysfunction is a typical feature of systemic sclerosis (SSc) and represents the earliest abnormality of primary myocardial involvement. We assessed coronary microcirculation status by combining two functional tests in SSc patients and estimating its impact on disease outcome.MethodsForty-one SSc patients, asymptomatic for coronary artery disease, were tested for coronary flow velocity reserve (CFR) by transthoracic-echo-Doppler with adenosine infusion (A-TTE) and for left ventricular wall motion abnormalities (WMA) by dobutamine stress echocardiography (DSE). Myocardial multi-detector computed tomography (MDCT) enabled the presence of epicardial stenosis, which could interfere with the accuracy of the tests, to be excluded. Patient survival rate was assessed over a 6.7- ± 3.5-year follow-up.ResultsNineteen out of 41 (46%) SSc patients had a reduced CFR (≤2.5) and in 16/41 (39%) a WMA was observed during DSE. Furthermore, 13/41 (32%) patients showed pathological CFR and WMA. An inverse correlation between wall motion score index (WMSI) during DSE and CFR value (r = -0.57, P <0.0001) was observed; in addition, CFR was significantly reduced (2.21 ± 0.38) in patients with WMA as compared to those without (2.94 ± 0.60) (P <0.0001). In 12 patients with abnormal DSE, MDCT was used to exclude macrovasculopathy. During a 6.7- ± 3.5-year follow-up seven patients with abnormal coronary functional tests died of disease-related causes, compared to only one patient with normal tests.ConclusionsA-TTE and DSE tests are useful tools to detect non-invasively pre-clinical microcirculation abnormalities in SSc patients; moreover, abnormal CFR and WMA might be related to a worse disease outcome suggesting a prognostic value of these tests, similar to other myocardial diseases.

Evaluation of cardiac functional abnormalities in systemic sclerosis by dobutamine stress echocardiography: a myocardial echostress scleroderma pattern.

In this study, we investigate the possibility of detecting, by dobutamine stress echocardiography (DSE), the presence of early or subclinical myocardial functional changes in patients with systemic sclerosis (SSc) without symptoms of ischaemic cardiac involvement.

Primary antiphospholipid syndrome with mesenteric venous thrombosis presenting with intestinal infarction: a case description

Sir—Antiphospholipid syndrome (APS) is an autoimmune hypercoagulability syndrome in which a wide variety of thromboembolic manifestations may occur. Thrombotic events can involve any arterial or venous vessel, although deep vein thrombosis in the lower limbs is the most frequent manifestation.1 Portal vein thrombosis is infrequent and thrombosis of the portal branches is rare, with only 12 cases reported in literature. We report a 53-year-old woman, who was admitted to the general surgery department complaining of nausea, vomiting and pain in the epigastrium, right hypochondrium and at the base of the homolateral hemithorax. On questioning, past medical history was unremarkable apart from hypertension that was being treated with atenolol 100 mg/day and enalapril 20 mg plus hydrochlorotiazide 12.5 mg/day. On physical examination, her abdomen was tender in the epigastrium and right hypochondrium. Hematochemical tests revealed leukocytosis (WBC 11.500/mm3), while hepatic, renal and coagulation parameters were within normal ranges. Abdominal X-ray was normal, and abdominal colourDoppler sonography and gastroduodenoscopy showed no abnormalities. Seven days after admission, the patient complained of a worsening of abdominal pain. Multiple and small air-fluid levels, particularly in the mid-low right quadrant were revealed by a further abdominal X-ray and portal thrombosis of the spleno-mesenteric axis was shown by colour Doppler sonography. Emergency surgery revealed 30 cm of cyanotic terminal ileum loop at about 60 cm from the ileocaecal valve and a mesenteric venous thrombosis in the corresponding section of the mesentery. Therefore, intestinal resection (about 50 cm), and terminoterminal, entero-enteric anastomosis were performed. Laboratory investigations at the time of the surgery showed erythrocyte sedimentation rate (ESR) 58 mm, international normalized ratio (INR) 1.02 ( 1.2), activated partial thromboplastin time (aPTT) 21 s (18–29 s) and fibrinogen 476 mg/dL (150–450 mg/dL). Rheumatoid factor, antinuclear antibodies, antiextractable nuclear antigens, anti-ds-DNA and anti-neutrophil cytoplasmatic antibodies (ANCA) were all negative. No congenital deficiencies of antithrombin III, protein C and protein S were present. Lupus anticoagulant (LA) was negative. Immunoglobulin M (IgM) anticardiolipin antibodies (aCL) were negative (3.4I U for a normal 5 IU) with positive IgG aCL of 46 IU ( 15). The histology revealed intense oedema of the submucosa and subserous tunica with diffuse haemorrhagic extravasation, diffuse leucocytoclastic vasculitis with scattered signs of recent thrombosis, diffuse dilation of lymphatic vessels and exudation foci. Primary APS (PAPS) was suspected and low molecular weight heparin was started. Two months after the first admission an abdomen echo colour-Doppler sonography showed signs of thrombosis of the mesenteric vein with signs of cavernomatosis, while calibre and flow of the right branch appeared normal. The upper mesenteric vein appeared pervious and normal for calibre and flow, with signs of venous ectasia in the epigastrium from porto-systemic shunts. Positivity for IgG aCL was confirmed with titres present at 40 IU. The diagnosis of PAPS was confirmed and the patient was switched to acenocumarol with a target INR of 2.0–3.0. After three years follow-up the patient was in reasonably good health. aCL were negative for two years and became positive (aCL IgG 40 IU) in the last period, however, in the absence of new symptoms and/or signs of APS. Portal vein thrombosis is rare.2 It occurs in association with several conditions that can act as inducing or precipitating factors. It can present as an acute, subacute or chronic manifestation with symptoms of portal hypertension or nonspecific symptoms (i.e., vomiting, abdominal pain and fever). Venae portae thrombosis as a clinical manifestation of APS has been scarcely reported in the literature3–13 and its exact frequency is not known despite a number of reports of APS involving hepatic circulation (i.e., Budd-Chiari syndrome).14 In our case, the thrombosis of the spleno-mesenteric axis presented as an intestinal infarct with a subacute onset in a patient who was otherwise healthy. Previous case reports3,4,12 show a presentation with signs and symptoms of portal hypertension or hepatic infarct. Four patients were affected by an underlying autoimmune disease: three by systemic lupus erythematosus and one by rheumatoid arthritis.