Successful treatment with immunosuppression, anticoagulation and vasodilator therapy of pulmonary hypertension in SLE associated with secondary antiphospholipid syndrome

Severe pulmonary hypertension (PHT) occurring in patients with systemic lupus erythematosus (SLE) is uncommon. Different modalities have been tried in the treatment for this condition but have not been effective because of progressive increase of pulmonary resistance over time. Our patient with SLE and PHT with antiphospholipid syndrome (APS), a condition which has previously never been described, responded rapidly to combination treatment with immunosuppression, anticoagulation and vasodilator therapy.     

Diagnosis and therapy of antiphospholipid antibody syndrome

PURPOSE: To report alterations of electrophysiologic tests, including the multifocal electroretinogram, in a case of acute zonal occult outer retinopathy. METHOD: We recorded photopic, scotopic, and single-flash electroretinograms and a multifocal electroretinogram in a 47-year-old woman with acute zonal occult outer retinopathy in the right eye. RESULTS: Her visual acuity was 20/20 in the right eye throughout the follow-up period. The electroretinograms indicated retinal impairment of the right eye, predominantly in the cones. The multifocal electroretinogram showed reduced responses corresponding to the visual field defect of the static perimetry. CONCLUSIONS: In acute zonal occult outer retinopathy, impairment of the retinal area results in a visual field defect. The multifocal electroretinogram can be useful in determining the location of the defect.     

The syndrome of thrombosis, thrombocytopenia, and recurrent spontaneous abortions associated with antiphospholipid antibodies: Hughes syndrome

aPL-associated thrombosis (Hughes syndrome) is widely recognized as a major cause of organ damage in autoimmune diseases. Beginning with the first symposium in 1984, international aPL symposia have facilitated research on aPL antibodies, and the clinical standardization of aPL tests. It is hoped that the present symposium will continue this tradition, because much remains to be learnt about the origin and pathogenicity of aPL antibodies. In addition, new insights are needed for more effective therapies to be developed.     

Anti-platelet antibodies in patients with systemic lupus erythematosus and the primary antiphospholipid antibody syndrome: their relationship with the observed thrombocytopenia

The role of antiphospholipid antibodies in the pathogenesis of the thrombocytopenia observed during primary antiphospholipid antibody syndrome (APAS) and systemic lupus erythematosus (SLE) remains controversial. We have used the MAIPA test to examine the frequency and specificity of anti-platelet antibodies directed against the major platelet membrane glycoproteins (GP IIb-IIIa, GP Ib-IX, GP Ia-IIa and GP IV) in patients where SLE and APAS were associated or not with thrombocytopenia. Results were compared with a series of 26 ITP patients, 46% of whom were shown to possess anti-platelet antibodies directed against one or more of the platelet surface glycoproteins. When APAS was associated with thrombocytopenia, 7/10 patients possessed antibodies against GP IIb-IIIa and/or GP Ib-IX. For SLE patients with thrombocytopenia, 6/10 patients were shown to have antiplatelet antibodies against GP IIb-IIIa, GP Ib-IX or GP IV. In contrast, for APAS (n=11) and SLE patients (n=11) without thrombocytopenia, only one patient had an antibody directed against GP IIb-IIIa and one patient had an antibody to GP IV. Our results suggest that antibodies directed against major platelet membrane glycoproteins may play a role in the thrombocytopenia that is seen during SLE and APAS.     

Nailfold capillary microscopy in patients with antiphospholipid syndrome

In this paper we tried to define the capillaroscopic pattern of anti phospholipid syndrome able to differentiate between the primary (PAPS) and the systemic lupus erythematosus-associated form (SLE-APS) and to be a predictive marker of thrombotic manifestations. Eight PAPS and five SLE-APS patients were studied. In each patient the evaluation was based on anti cardiolipin antibody levels, nailfold capillaroscopy, retinal fluorangiography and transcranial doppler sonography. Statistical analysis has been performed using chi 2 analysis. Morphological alterations of capillary loops, venular visibility and sludging of blood were often observed in both groups. While we found in higher prevalence a variability of capillary loop length in PAPS patients, the SLE-APL group significantly differed for the presence of microhaemorrhages (p < 0.001). When we evaluated the clinical history, a marked microcirculatory damage was related with the occurrence of thrombotic manifestations in the PAPS patients. Anti cardiolipin antibody levels, retinal fluorangiography and transcranial doppler sonography did not correlate with clinical history in either group. In conclusion, nailfold capillaroscopy can be usefully employed in the differentiation between primary and SLE-associated anti phospholipid syndrome, and it can help to identify the patients at higher risk of thrombotic disease.     

The characteristics of kidney involvement in a female patient with systemic lupus erythematosus and the antiphospholipid syndrome

A case of rapidly progressive nephritis is reported in a female patient having systemic lupus erythematosus (SLE) with antiphospholipid syndrome. Clinical presentation of progressive lupus nephritis with intensifying renal insufficiency, arterial hypertension, hematuria, nephrotic syndrome was associated with unusual morphological manifestations of mesangiocapillary glomerulonephritis with advanced vasculitis. The authors attribute a malignant nephritis course atypical for patients with antiphospholipid syndrome to development of renal vasculitis. The discussion covers lupus genesis of vascular involvement, a probable triggering role of antibodies to phospholipids in impairment of endothelial cells.     

Effective splenectomy in a patient with HIV-associated thrombocytopenia

A 31-year-old man presented with a 3-month history of petechial hemorrhages. Physical examination revealed no splenomegaly. The patient's platelet count was 1.0 x 10(9)/l and bone marrow aspiration showed an elevated number of megakaryocytes. A diagnosis of HIV-associated thrombocytopenia was made on the basis of HIV seropositive results. The CD4 cell count was 400 x 10(6)/l. No opportunistic infections indicating AIDS were detected. Initially the patient was treated with predonisolone, but showed only a transient response. He also failed to respond to zidovudine, lamivudine, or indinavir. Following splenectomy, however, his platelet count rose above 80 x 10(9)/l (normal level: 150-350 x 10(9)/l).     

Carnitine deficiency and carnitine therapy in a patient with Rett syndrome

The aim of the present study was to establish an alternative methodology for testing the antibacterial effects of different amalgams. The vitality of mutans streptococci grown in vitro on various amalgam surfaces was monitored with a vital fluorescence staining technique using fluorescein diacetate and ethidium bromide. The in vivo effect of amalgam-non-gamma 2 fillings on the vitality of dental plaque was assessed with the same method and compared with samples originating from enamel. The median in vitro vitality of mutans streptococci was estimated as 70% on glass, 50% on Amalcapnon-gamma 2 and Sybraloy, 20% on Amalcap F and 10% on Neo-Silbrin. In vivo plaque vitality on enamel varied from 60 to 70%. In contrast, plaque sampled from non-gamma 2-amalgam surfaces revealed significant reductions in vitality with a minimum value of 25% of one day old supragingival plaque. The vital fluorescence technique was shown as an easy and quick method to assess the bactericidal effect against biofilm bacteria of dental materials in vitro as well as in vivo.     

An elderly patient with Takayasu's arteritis associated with antiphospholipid antibodies

We report a patient with Takayasu's arteritis associated with antiphospholipid antibodies. An 84-year-old woman gradually developed gait and visual disturbances, dementia, myocardial infarction, and gangrene in all four limbs during a period of 8 years. Persistent inflammatory signs also continued for at least 8 years. Positive reaction for lupus anticoagulant by the diluted Russel viper venous time and positive reactions for cardiolipin antibodies were confirmed. However, she did not develop SLE. MR angiography revealed multiple and extensive occlusive changes in large vessels such as the aorta and its major branches. We believe antiphospholipid antibodies may have been related to severe occlusive vasculopathy in this patient.     

Obstetrical complications of antiphospholipid syndrome

Spontaneous miscarriages and fetal deaths take part of the definition of the antiphospholipid syndrome. Preeclampsia, fetal distress, preterm delivery, intrauterine growth retardation, mother's or newborn's vascular thrombosis are also included in the spectrum of clinical events linked to the antiphospholipid antibodies in pregnant women. The pathogenesis is not fully understood, but involves interactions between antibodies and phospholipidic molecules that are complexed to plasmatic proteins of the coagulation, or present on endothelial cell and platelet surfaces. The poor spontaneous prognosis of pregnancy in the context of the antiphospholipid syndrome is dramatically improved by preventive treatment. Therapeutic options are not codified and are various combinations of low-dose aspirin, heparin, steroids, intravenous immunoglobulins, and fish-oil derivatives. Excellent obstetrical care is mandatory in every case.     

Fibrinolytic treatment in primary antiphospholipid syndrome

Glycine (800 mg/kg ip) abolished the antimmobility effect of 1-aminocyclopropanecarboxylic acid (ACPC) given both ip (400 mg/kg) and ihp (30 micrograms) in the forced swimming test in rats, but did not affect the anticonflict activity of ACPC (200 mg/kg ip or 10 micrograms ihp) in the conflict drinking test.     

Progressive encephalopathy in antiphospholipid antibody syndrome

The most frequent neurological complications of antiphospholipid antibodies syndrome (APS) are cerebral infarctions. In cases with recurrent strokes this syndrome may be associated with multi infarct dementia. We report the case of a 40-year old man presenting as "primary" APS with multiple cerebral infarctions. This patient had insidiously and progressively developed a cognitive impairment predominant in memory. The progressive encephalopathy associated with APS is very different from multi infarct dementia and could be caused either by a vascular mechanism (vascular occlusion with fibrin thrombi or disseminated vasculitis) or by an immunological mechanism.     

Nodular regenerative hyperplasia associated with primary antiphospholipid syndrome

Nodular regenerative hyperplasia of the liver is characterized by diffuse nodularity of the hepatic parenchyma without fibrotic septa. It may be related to venous or arterial obstruction in the portal tract. We report a case of primary antiphospholipid syndrome associated with nodular regenerative hyperplasia in a 45-year old woman. The patient had an ischemic stroke, associated with an acute arterial ischemia of the left leg. She had high titers of serum anticardiolipin antibodies. Nodular regenerative hyperplasia of the liver was histologically confirmed and was associated with anicteric cholestasis. This case provides additional evidence that a thrombotic mechanism may play a role in the pathogenesis of nodular regenerative hyperplasia of the liver.