The diagnosis and treatment of a secondary antiphospholipid syndrome in systemic lupus erythematosus

AIM: The study of incidence of clinico-laboratory signs of antiphospholipid syndrome (APLS) and the results of its treatment in SLE. MATERIALS AND METHODS: Out of 120 patients with verified SLE, 13 (10.8%) had the signs of APLS. They underwent additional tests (platelet and lupus blood anticoagulant assays, assessment of immunity). RESULTS: APLS treatment consisted of suppression of antiphospholipid antibodies production by glucocorticosteroids and cytostatics, removal of antibodies by means of plasmapheresis, HBO therapy of thromboses and vascular defects. APLS in SLE frequently manifested with arterial thromboses, cerebrovasculitis, livedo, thrombocytopenia, lupus anticoagulant. CONCLUSION: Combined treatment of APLS in SLE improves the disease prognosis and prolongs life span.     

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.     

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.     

Psychiatric manifestations of systemic lupus erythematosus

Low-intensive laser radiation used by topical, intravenous, and transendoscopic routes was employed in the complex antituberculosis therapy of 120 patients with pulmonary tuberculosis concurrent with chronic nonspecific respiratory diseases (n = 48), diabetes mellitus (n = 20 patients), gastrointestinal diseases (n = 45). The results of treatment were compared with the parameters of 62 patients with the similar clinical and X-ray characteristics of a tuberculosis process and concomitant diseases. It was ascertained that laser therapy might enhance the efficiency of treatment of patients with concurrent abnormality statistically significantly, by reducing the time of bacterial isolation, closure of decay cavities in the lung tissue, hospital treatment and by accelerating the onset of stable remission of concomitant diseases.     

Pulmonary manifestations of systemic lupus erythematosus

Systemic lupus erythematosus (SLE) is an autoimmune disease that primarily affects young women. The respiratory system is more commonly involved in SLE than in any other collagen vascular disease. SLE may affect virtually all components of the respiratory system, including the upper airway, lung parenchyma, pulmonary vasculature, pleura, and respiratory muscles. Respiratory system involvement ranges from symptomatic to fulminant and life threatening. This article reviews the pulmonary manifestations of SLE, including drug-induced SLE.     

Antibodies to beta2-glycoprotein I: a potential marker for clinical features of antiphospholipid antibody syndrome in patients with systemic lupus erythematosus

OBJECTIVE: To clarify risk factors for the development of clinical features of antiphospholipid syndrome (APS) in patients with anticardiolipin antibodies (aCL) in systemic lupus erythematosus (SLE). METHODS: We studied 65 SLE patients, all with positive IgG and/or IgM aCL. Patients were divided into 2 groups; I: 29 SLE patients with features of APS (SLE/APS) and II: 36 aCL positive SLE patients without any feature of APS (SLE/aCL). Serum samples were collected from our serum bank. Anti-beta2-glycoprotein I (anti-beta2-GPI) were tested by ELISA using irradiated plates in the absence of cardiolipin. Anti-dsDNA antibodies were tested by standard Farr assay. RESULTS: There were no major differences between SLE clinical manifestations in both groups. However, the frequency of IgG anti-beta2-GPI was markedly increased in SLE/APS (18/29, 62%) than in SLE/aCL (4/36, 11%) (chi-squared 18.6, p=0.0001). The levels of anti-dsDNA antibodies in the same samples were slightly lower in SLE/APS. CONCLUSION: Our data suggest that increased levels of IgG anti-beta2-GPI may be a specific feature of SLE/APS patients rather than reflecting a polyclonal B cell activation.     

Study of the respiratory manifestations of systemic lupus erythematosus

Following a short discussion of the anatomopathological and clinical features of respiratory signs during systemic l.e., some clinical cases in which involvement of the respiratory system (pleural and/or parenchymal) was observed alone or was clearly predominant are reported. The cases also presented some unusual clinical characteristics. Clinical investigations must be extended to the systemic mesenchyma in the presence of pleuritis or pulmonary fibrosis of unclear aetiology, even where there are no extra-respiratory signs of S.L.E.     

Transient monocular blindness and antiphospholipid antibodies in systemic lupus erythematosus

Cation binding to the monovalent cation selective channel, gramicidin A, is shown to induce changes in the dipolar and chemical shift observables from uniformly aligned samples. While these changes could be the result of structural or dynamic changes, they are shown to be primarily induced by through-bond polarizability effects when cations are solvated by the carbonyl oxygens of the peptide backbone. Upon cation binding partial charges are changed throughout the peptide plane, inducing large changes in the 13C1 chemical shifts, smaller changes in the 15N chemical shifts, and even smaller effects for the 15N-13C1 and 15N-2H dipolar interactions. These conclusions are substantiated by characterizing the 15N chemical shift tensors in the presence and absence of cations in fast-frozen lipid bilayer preparations of gramicidin A.     

Shrinking spinal cord following transverse myelopathy in a patient with systemic lupus erythematosus and the phospholipid antibody syndrome

A 38-year-old woman with systemic lupus erythematosus and the phospholipid antibody syndrome was admitted because of rapidly evolving symptoms consistent with a transverse myelopathy at the TH9/10 level. Magnetic resonance imaging (MRI) showed slight diffuse swelling and increased signal intensity of the spinal cord. She was treated with high dose methylprednisolone plus azathioprine and aspirin. Four months later she had achieved almost complete remission with minimal residual sphincter disturbances. Despite the clinical recovery, repeated MRI at 4 months and 4 years showed diffuse and irreversible atrophy of the entire spinal cord.     

Antibodies to beta2-glycoprotein I in systemic lupus erythematosus: new laboratory marker of antiphospholipid syndrome

Antibodies to beta 2-glycoprotein in the serum of patients with antiphospholipid syndrome (APS) were found by many investigators, but their results appeared contraversional. We studied clinical significance of antibodies to beta 2-glycoprotein I (anti-beta 2-GPI) in patients with SLE. 69 patients with verified SLE were examined for lupus anticoagulant (LA), antibodies to cardiolipin (aCL) and anti-beta 2-GPI. 44(65%), 46(67%), 49(71%), 19(28%), 16(23%) patients were positive for LA, IgG-aCL, IgM-aCL, IgG-anti-beta 2-GPI and IgM-anti-beta 2-GPI, respectively. Hyperproduction of IgG-anti-beta 2-GPI correlated with APS development as a whole, its separate clinical symptoms (venous and arterial thromboembolism, obstetric pathology and thrombocytopenia) and some comcomitant clinical signs (trophic crural ulcer, hemolytic anemia, valvular heart disorders). Moreover, an increase in concentration of IgM-anti-beta 2-GPI was associated with habitual abortion. Both isotypes of anti-beta 2-GPI occurred more frequently in the sera positive by LA and aCL. It is interesting that we discovered IgG-anti-beta 2-GPI more often in early than late postthrombolytic period. Thus, anti-2b2-GPI is a new serological marker of APS. Its detection is clinically important for upgrading diagnosis of APS.     

Neuropsychiatric systemic lupus erythematosus and the syndrome of inappropriate secretion of antidiuretic hormone: a case report with very late onset systemic lupus erythematosus

The syndrome of inappropriate secretion of antidiuretic hormone (SIADH) associated with neuropsychiatric lupus (NP-SLE) is rare. We report a case of SIADH associated with the new onset of SLE in an 88-yr-old female. The unique features of this case include the extreme age of onset of SLE presenting with neuropsychiatric manifestations and positive antiribosomal P antibody titres. Both the NP manifestations of SLE and SIADH were highly correlated with the SLE disease activity. This case illustrates a novel presentation of NP-SLE with SIADH which may develop due to antibody-mediated hypothalamic dysfunction.     

Fatal hyperammonemia in a patient with systemic lupus erythematosus

We treated a 31-year-old woman with systemic lupus erythematosus, renal failure with nephrotic syndrome, and a long-standing seizure disorder, who developed severe hyperammonemia with a fatal outcome. Blood chemistry examination did not indicate liver disease, and amino acid concentrations did not suggest a defect in the urea cycle. Discontinuation of anticonvulsant treatment with valproic acid (VPA) failed to bring about improvement. We speculated that hyperammonemia in this case was induced by VPA, and the existence of other underlying factors, including the administration of aspirin and cimetidine, hypoalbuminemia, and renal failure might elevate the concentration of the serum free fraction of VPA.     

Hyperprolactinemia in males with systemic lupus erythematosus

Alanine dehydrogenase [EC 1. 4. 1. 1] was purified to homogeneity from a crude extract of Enterobacter aerogenes ICR 0220. The enzyme had a molecular mass of about 245 kDa and consisted of six identical subunits. The enzyme showed maximal activity at about pH 10.9 for the deamination of L-alanine and at about pH 8.7 for the amination of pyruvate. The enzyme required NAD+ as a coenzyme. Analogs of NAD+, deamino-NAD+ and nicotinamide guanine dinucleotide served as coenzymes. Initial-velocity and product inhibition studies suggested that the deamination of L-alanine proceeded through a sequential ordered binary-ternary mechanism. NAD+ bound first to the enzyme, followed by L-alanine, and the products were released in the order of ammonia, pyruvate, and NADH. The Km were 0.47 mM for L-alanine, 0.16 mM for NAD+, 0.22 mM for pyruvate, 0.067 mM for NADH, and 66.7 mM for ammonia. The Km for L-alanine was the smallest in the alanine dehydrogenases studied so far. The enzyme gene was cloned into Escherichia coli JM109 cells and the nucleotides were sequenced. The deduced amino acid sequence was very similar to that of the alanine dehydrogenase from Bacillus subtilis. However, the Enterobacter enzyme has no cysteine residue. In this respect, the Enterobacter enzyme is different from other alanine dehydrogenases.