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1.  M mode and Doppler echocardiographic assessment of left ventricular diastolic function in primary antiphospholipid syndrome. 
British Heart Journal  1995;74(5):531-535.
BACKGROUND--High titres of serum antiphospholipid antibodies are a possible pathogenic factor for cardiac lesions in patients with systemic lupus erythematosus. OBJECTIVE--To test the hypothesis of a causal link between high titres of antiphospholipid antibodies in the serum and myocardial involvement in patients without systemic lupus erythematosus. PATIENTS AND DESIGN--18 patients with primary antiphospholipid syndrome (recurrent fetal loss, arterial and/or venous thrombosis, high titres of antiphospholipid antibodies, and no criteria for systemic lupus erythematosus) were prospectively studied by cross sectional, M mode, and pulsed Doppler echocardiography, and compared with 18 healthy controls. The pulsed Doppler indices of left ventricular diastolic function included isovolumic relaxation time and four mitral outflow indices: peak velocity of early flow, peak velocity of late flow, early to late peak flow velocity ratio, and rate of deceleration of early flow. Four computerised M mode indices were also measured: peak rate of left ventricular enlargement in diastole, peak rate of posterior wall thinning, peak velocity of lengthening of the posterior wall, and velocity of circumferential chamber lengthening. RESULTS--Compared with controls, patients with primary antiphospholipid syndrome had higher values for isovolumic relaxation time and peak velocity of late mitral outflow and lower values for early to late mitral peak outflow velocity ratio, rate of deceleration of early mitral outflow, peak rate of left ventricular enlargement in diastole, peak rate of posterior wall thinning, peak velocity of lengthening of the posterior wall and velocity of circumferential chamber lengthening. CONCLUSION--This abnormal pattern reflects an impairment of myocardial relaxation and filling dynamics of the left ventricle in patients with primary antiphospholipid syndrome who were free of any clinically detectable heart disease. These data suggest that high serum titres of antiphospholipid antibodies may be associated with subclinical myocardial damage.
PMCID: PMC484075  PMID: 8562240
3.  Abdominal manifestations in childhood‐onset systemic lupus erythematosus 
Annals of the Rheumatic Diseases  2006;66(2):174-178.
Background
Childhood‐onset lupus erythematosus is a rare disorder of unknown origin.
Objectives
To describe the frequency of gastrointestinal manifestations at presentation of systemic lupus erythematosus SLE and at follow‐up, and discuss the specific causes of these manifestations.
Methods
Medical records of 201 patients with childhood‐onset SLE followed up in French paediatric nephrological, haematological and rheumatological centres were reviewed and abstracted for gastrointestinal manifestations.
Results
Gastrointestinal involvement was recorded in 39 (19%) children. The median (range) age at the time of initial gastrointestinal manifestations was 11.3 (4.5–16) years. Gastrointestinal symptoms were present at or occurred within 1 month after diagnosis in 32% patients. Abdominal pain was the most frequent symptom, present in 34 (87%) patients. It was mostly related to lupus involvement, especially ascites (n = 14) and pancreatitis (n = 12), more rarely to treatment‐induced events (n = 1) or infection (n = 1) and never to events unrelated to SLE. Three children with surgical abdomen underwent a laparotomy before SLE was diagnosed, with a final diagnosis of lupus peritonitis and lupus acalculous cholecystitis. C reactive protein values were <40 mg/l in all but two patients who had surgical abdomen. Abdominal ultrasonography and computed tomography scans were abnormal in 58% and 83% of the evaluated patients, respectively. Corticosteroids, associated with intravenous cyclophospamide in eight patients, led to complete remission of gastrointestinal involvement in 30 of 31 treated patients.
Conclusion
Gastrointestinal involvement is common in children with SLE, and is mainly due to primary lupus involvement. Corticoidsteroid treatment should be promptly considered in children with lupus presenting with abdominal pain after infectious disease; side effects of treatment and intestinal perforation have been excluded.
doi:10.1136/ard.2005.050070
PMCID: PMC1798515  PMID: 16818463
4.  Reactive haemophagocytic syndrome in adult‐onset Still's disease: a report of six patients and a review of the literature 
Annals of the Rheumatic Diseases  2006;65(12):1596-1601.
Objective
To examine the prevalence and characteristics of patients with reactive haemophagocytic syndrome (RHS) complicating adult‐onset Still's disease (AOSD).
Methods
Of 50 patients with AOSD fulfilling Yamaguchi and Fautrel criteria followed in our department, clinical and laboratory data, course and treatment of six patients with histologically proven RHS and without any obvious cause other than AOSD were retrospectively recorded.
Results
RHS led to AOSD in two cases, whereas it appeared after a mean duration of 3.5 years from onset of AOSD in the other cases. The main symptoms were fever (n = 6), polyarthralgias or myalgias (n = 4), lymphadenopathy or splenomegaly (n = 3), pharyngitis (n = 3), rash (n = 3), pleuritis (n = 3), hepatomegaly (n = 1), normal or low leucocyte count (n = 4), anaemia (n = 6), lymphocytopenia (n = 6), thrombocytopenia (n = 4), hyperbasophilic lymphocytes (n = 2), abnormal liver function tests (n = 6) and increased serum triglyceride level (n = 6). Serum ferritin concentration was constantly increased (>10 000 µg/l in five cases, with <5–35% in glycosylated form). Two patients presented with coagulopathy. Treatment comprised corticosteroids (n = 4) and intravenous immunoglobulins (n = 3), whereas prednisone was unchanged in one case. One death due to pneumonia occurred 15 days after RHS. With a follow‐up ranging from 2 to 7.5 years, the other patients were in remission with prednisone plus etanercept (n = 1), prednisone plus methotrexate (n = 1), low‐dose prednisone (n = 2) or without treatment (n = 1).
Conclusion
RHS is not uncommon in AOSD. It should be evoked in a patient with AOSD in the absence of hyperleucocytosis, thrombocytopenia, lymphopenia and coagulopathy, or in the presence of high serum ferritin and triglyceride levels.
doi:10.1136/ard.2005.046904
PMCID: PMC1798476  PMID: 16540551
5.  Anti‐cyclic citrullinated peptide antibodies in hepatitis C virus associated rheumatological manifestations and Sjögren's syndrome 
Annals of the Rheumatic Diseases  2006;65(3):394-397.
Objective
To investigate the diagnostic reliability of anti‐CCP antibodies (anti‐CCP Ab) in distinguishing hepatitis C virus (HCV) associated rheumatological manifestations and Sjögren's syndrome from rheumatoid arthritis.
Methods
147 HCV infected patients (HCV RNA positive) were compared with 64 patients with definite rheumatoid arthritis in a retrospective study. Anti‐CCP Ab were detected using the Immunoscan ELISA kit (second generation) and rheumatoid factor (RF) by the FIDIS™ Rheuma kit.
Results
Among the 147 HCV infected patients (77 women; mean (SD) age 58 (16) years), 77 (52%) had a mixed cryoglobulin (MC), 38 (26%) an MC associated systemic vasculitis, 35 (24%) arthralgia/arthritis, and seven (5%) definite Sjögren's syndrome. HCV infected patients with arthralgia were more often RF positive than those without arthralgia (54% v 27%; p = 0.003), but less often than patients with rheumatoid arthritis (54% v 81%; p = 0.009). Anti‐CCP Ab were detected in only two HCV infected patients with arthralgia (5.7%), in none without arthralgia or with Sjögren's syndrome, and in 78% of patients with rheumatoid arthritis. With a specificity of 93.5% and a positive predictive value of 96% for rheumatoid arthritis, anti‐CCP Ab were the most specific biological marker.
Conclusions
Anti‐CCP antibodies are very rarely found in HCV infected patients with rheumatological manifestations or Sjögren's syndrome. They are reliable serological markers to distinguish these from patients with rheumatoid arthritis.
doi:10.1136/ard.2005.038042
PMCID: PMC1798048  PMID: 16474032
hepatitis C virus; arthritis; Sjögren's syndrome; anti‐CCP; rheumatoid factor
9.  MRI in 31 patients with Behçet's disease and neurological involvement: prospective study with clinical correlation. 
Thirty one patients with Behçet's disease and neurological manifestations were prospectively studied with MRI. Cerebral venous thrombosis was diagnosed in 10 patients. MRI performed during the acute illness in eight patients showed an abnormally high signal on the T2 weighted sequences in the occluded sinus. MRI showed minor flow abnormalities suggestive of partial recanalisation of the sinus in two cases at a later clinical stage. MRI can be an alternative, non-invasive, investigation to intravenous cerebral angiography. In 13 patients with central nervous system involvement, MRI performed during the acute illness showed multiple hyperintense lesions on T2 weighted sequences. They were usually less than 5 mm, scattered and confluent, mainly in the white matter, distributed in the hemispheric white matter in nine cases, brainstem in eight, basal ganglia and thalamus in five, and cortex in two. MRI abnormalities were usually associated with appropriate clinical deficits, but were larger and more disseminated than expected.
Images
PMCID: PMC1015062  PMID: 8331356
10.  Factors associated with Pneumocystis carinii pneumonia in Wegener's granulomatosis. 
Annals of the Rheumatic Diseases  1995;54(12):991-994.
OBJECTIVE--To determine the factors associated with the occurrence of Pneumocystis carinii pneumonia (PCP) in Wegener's granulomatosis (WG). METHODS--We retrospectively compared a group of 12 patients with WG and PCP (PCP group), with 32 WG patients without PCP followed over the same period in the same centres (control group). RESULTS--The mean delay of onset of PCP after the start of the immunosuppressive therapy was 127 (SD 128) days. Before treatment, the clinical and biological features of the two groups were similar, except for the mean lymphocyte count which was lower in the PCP group than in the control group (1060/mm3 v 1426/mm3; p = 0.04). During treatment, both groups were lymphopenic. There was a significant difference between the lowest absolute lymphocyte count in each group (244/mm3 in the PCP group v 738/mm3 in the control group; p = 0.001). During the first three months of treatment, the lymphocyte count was less than 600/mm3 at least once in 10 of the 12 patients in the PCP group and in 11 of the 32 patients in the control group (p < 0.01). The mean cumulative dose of cyclophosphamide was greater in the PCP group than in the control group at the end of both the second (1.55 mg/kg/day v 0.99 mg/kg/day; p = 0.05) and the third (1.67 mg/kg/day v 0.97 mg/kg/day; p = 0.03) months. However, in multivariate analysis, the only two factors independently and significantly associated with the occurrence of PCP were the pretreatment lymphocyte count (p = 0.018) and the lymphocyte count three months after the start of the immunosuppressive treatment (p = 0.014). CONCLUSIONS--The severity of lymphocytopenia before and during immunosuppressive treatment is the factor best associated with PCP in WG.
PMCID: PMC1010066  PMID: 8546533
12.  The binding of some human antiendothelial cell antibodies induces endothelial cell apoptosis. 
Journal of Clinical Investigation  1998;101(10):2029-2035.
The pathogenic role of antiendothelial cell antibodies (AECA) remains unclear. They are frequently associated with antibodies to anionic phospholipids (PL), such as phosphatidylserine (PS), which is difficult to reconcile with the distribution of PL molecular species within the plasma membrane. Since it is already known that PS is transferred to the outer face of the membrane as a preclude to apoptosis, the possibility exists that apoptosis is initiated by AECA. AECA-positive/anti-PL antibody-negative sera from eight patients with systemic sclerosis (SS) and 21 control patients were evaluated. Endothelial cells (EC) were incubated with AECA and the exposure of PS was established through the binding of annexin V. Hypoploid cell enumeration, DNA fragmentation, and optical and ultrastructural analyses of EC were used to confirm apoptosis. Incubation of EC with AECA derived from six of eight patients with SS led to the expression of PS on the surface of the cells. This phenomenon was significantly more frequent in SS (P < 0.04) than in control diseases. The redistribution of plasma membrane PS preceded other events associated with apoptosis: hypoploidy, DNA fragmentation, and morphology characteristic for apoptosis. Apoptosis-inducing AECA did not recognize the Fas receptor. We conclude that AECA may be pathogenic by inducing apoptosis.
PMCID: PMC508790  PMID: 9593758
17.  Behçet's disease. 
BMJ : British Medical Journal  1992;304(6836):1199-1200.
PMCID: PMC1881797  PMID: 1515786

Results 1-17 (17)