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Biomedical subjects

R A Asherson

Publications and source records attributed to R A Asherson.

At least 73 records · Page 4Linked to original sources
PubMed1993

Avascular necrosis of bone and antiphospholipid antibodies in systemic lupus erythematosus.

Of a total of 800 patients with systemic lupus erythematosus, 37 were found to have developed avascular necrosis of bone (4.6%). Routine radiological screening of all joints was not performed and these 37 represented symptomatic patients only. Twenty-seven of these were positive for antiphospholipid antibodies (aPL) (73%). Both aPL positive and aPL negative patients had received large doses of oral steroids. The frequency of vasculitis and livedo reticularis, as well as arterial thrombosis was higher in the aPL positive group, but this did not reach statistical significance, perhaps because of the small numbers of patients involved. Although the difference in the frequency of thrombocytopenia was more marked in the aPL positive group (p < 0.08), once again, this was not statistically significant at the 5% level. Since the prevalence of aPL positivity in the general lupus population is about 30-40%, the presence of the aPL may be associated with an increased tendency to develop avascular necrosis. This is supported by recent reports of avascular necrosis occurring in patients with primary antiphospholipid syndrome in the absence of previous steroid administration.

Adolescent↗
PubMed1992

Primary idiopathic pulmonary hypertension complicated by pulmonary arterial thrombosis. Association with antiphospholipid antibodies.

OBJECTIVE: We report an unusual case of primary pulmonary hypertension and review the pertinent literature. METHODS: A 25-year-old Caucasian woman presented with progressive dyspnea and was found to have pulmonary hypertension. Antiphospholipid antibodies were present. The patient had a prolonged hospital course, was unresponsive to therapy, and died suddenly. RESULTS: Postmortem examination revealed a large thrombus affecting the right main pulmonary artery, with plexogenic arteriopathy bilaterally. CONCLUSION: This appears to be the first reported case of primary pulmonary hypertension complicated by thrombosis of a main pulmonary artery in association with antiphospholipid antibodies.

Adult↗
PubMed1992

Bilateral adrenal infarction, hypoadrenalism and splinter haemorrhages in the 'primary' antiphospholipid syndrome.

We describe a patient with a 3-year history of recurrent deep vein thromboses (DVT) of the lower limbs, who developed adrenal insufficiency following withdrawal of warfarin therapy. Multiple splinter haemorrhages of the nail beds were evident, simultaneous with the development of adrenal infarction in the absence of infective endocarditis. CT scans of the adrenal glands were consistent with bilateral adrenal infarctions. The patient had persistently high titres of IgG anticardiolipin antibodies (aCL) over the previous 4 years in the absence of antinuclear antibodies (ANA), antibodies to double stranded deoxyribonucleic acid (dsDNA) or extractable nuclear antigens (ENA). Thrombocytopenia and an intermittently positive Coombs' test had been noted. Previous episodes of DVT were associated with inadequate warfarin control and a period of warfarin resistance. He conforms to a diagnosis of a 'primary' antiphospholipid syndrome.

Adrenal Gland Diseases↗
PubMed1992

Urticarial vasculitis, IgA deficiency and C1 esterase inhibitor deficiency in the presence of an IgG monoclonal gammopathy--a case report.

A female patient, currently aged 40, has attended our Lupus Clinic for 8 years with a clinical picture of urticarial vasculitis, joint pains, severe myalgias and abdominal pain which has occurred in bouts at irregular intervals. She has been found to have consistently reduced C4 levels, C1-esterase inhibitor deficiency and a persistent monoclonal band in the gamma region on electrophoresis. Her serum IgA level has been continuously low. Her symptoms have failed to respond to antihistamines and a variety of other measures but have been partially alleviated by immunosuppressive therapy ('pulsed' cyclophosphamide). We believe that this is the first patient described showing this combination of clinical and laboratory features.

Adult↗
PubMed1992

Skin nodules and macules resembling vasculitis in the antiphospholipid syndrome--a report of two cases.

Two patients who demonstrated skin lesions in the presence of antiphospholipid antibodies are documented. The first had a long history of recurrent painful nodules. She had also suffered two deep vein thromboses. The second developed a rash on the lower limbs resembling vasculitis which did not respond to prednisolone, but to low dose salicylate therapy. Histology in both patients revealed microthrombosis of cutaneous vessels.

Adult↗
PubMed1992

Systemic lupus erythematosus complicated by tricuspid stenosis and regurgitation: successful treatment by valve transplantation.

Clinical tricuspid stenosis has not previously been reported in patients with systemic lupus erythematosus (SLE). A 25 year old woman with active SLE presented with signs of severe right ventricular failure. Cardiac catheterisation confirmed the diagnosis of tricuspid stenosis and regurgitation together with mitral regurgitation. This patient underwent successful tricuspid and mitral valve replacement.

Adult↗
PubMed1992

Antiphospholipid antibodies and HLA associations in primary Sjögren's syndrome.

Blood samples from 65 patients with primary Sjögren's syndrome were evaluated for the presence of antiphospholipid antibodies. Increased levels of antiphospholipid antibodies were found in 13 of 65 (20%) of patients. These antiphospholipid antibodies were predominantly of the IgA isotype, in contrast with the IgG isotype antiphospholipid antibodies found in patients with systemic lupus erythematosus (SLE). The presence of IgA antiphospholipid antibodies in the patients with primary Sjögren's syndrome was not significantly associated with arterial or vascular thrombosis, nor peripheral or central nervous system vasculitis. There was no association with laboratory determined features such as lupus anticoagulant or false positive results of the Venereal Disease Research Laboratory (VDRL) test. Oligonucleotide specific DNA amplification and hybridisation with allele specific probes was used to examine the HLA-D antigens occurring in this group of patients with primary Sjögren's syndrome. Of 13 patients with antiphospholipid antibodies, seven had the genotype HLA-DR2/DR3. However, compared with the whole group of 65 patients with Sjögren's syndrome, no increased occurrence of haplotype DR2 or DR3 was noted. These results suggest that gene interaction between DR2 and DR3 may play a part in the production of antiphospholipid antibodies in patients with Sjögren's syndrome. In contrast with patients with SLE, the IgA antiphospholipid antibodies in patients with Sjögren's syndrome are not risk factors for thrombosis or vasculitis. The presence of IgA antiphospholipid antibodies in patients with Sjögren's syndrome probably reflects its production at mucosal sites of inflammation and the absence of vasculopathy may be due to the inability of IgA antibodies to activate complement.

Female↗
PubMed1992

Distribution of two common idiotypes of anticardiolipin antibodies in sera of patients with primary antiphospholipid syndrome, systemic lupus erythematosus and monoclonal gammopathies.

The frequency of two common idiotypes of anticardiolipin antibodies (aCL) was determined in sera from three groups of subjects, patients with systemic lupus erythematosus (SLE), with primary antiphospholipid syndrome (PAPS) and with monoclonal gammopathies (MG), as compared to normal population. The idiotype 1.10, which was derived from a patient with active SLE and antiphospholipid syndrome, was found more frequently among patients with PAPS (10.5%, 10.5% and 22.2% in MG, SLE and PAPS, respectively) than the idiotype H3, which was derived from a human hybridoma monoclonal aCL generated from a healthy subject immunized with tetanus and diphtheria. The latter idiotype was detected in 8.7%, 6.5% and 11.7% of patients with MG, SLE and PAPS, respectively. Incidental findings in this study include a high prevalence of aCL among patients with MG (23%) and a high prevalence of anti-dsDNA antibodies, detected only by a sensitive enzyme-linked immunosorbent assay, among patients with PAPS. Our results indicate that idiotypic diversity exists among aCL derived from different sources. Some of these cross-reactive idiotypes may be more pathogenic than others.

Adult↗
PubMed1992

Multiorgan thrombotic disorders in systemic lupus erythematosus: a common link?

Multiorgan thrombotic disorders have been described in a variety of conditions including systemic lupus erythematosus. Among these are the 'catastrophic' antiphospholipid syndrome, disseminated intravascular coagulation and the thrombotic thrombocytopenic purpura-haemolytic uraemic syndrome. In this review we briefly analyse some specific clinical and haematological characteristics of these conditions and attempt to uncover common links between them.

Antiphospholipid Syndrome↗
PubMed1992

Hypertrophic cardiomyopathy in systemic lupus erythematosus and "lupus-like" disease. Chance association? A report of 2 cases.

Two patients, both women, one with "lupus-like" disease, age 51 years, the other a 45-year-old with systemic lupus erythematosus (SLE), developed symptoms and echocardiographic signs of hypertrophic cardiomyopathy. One patient had a family history of sudden maternal death. Neither patient had a history of sustained hypertension and there were no significant valvular lesions detectable to account for the septal and ventricular hypertrophy. The association of SLE or any related condition with hypertrophic cardiomyopathy has not been recorded. In one patient the question of a hereditary cardiomyopathy remains a possibility. The diagnosis of the condition was based on clinical and echocardiographic grounds alone. No endomyocardial biopsies were performed.

Cardiomyopathy, Hypertrophic↗
PubMed1992

HLA-DP genotyping in patients with systemic lupus erythematosus: correlations with autoantibody subsets.

In order to ascertain whether the HLA-DP locus plays a role in the genetic predisposition for systemic lupus erythematosus (SLE), 42 patients with SLE were typed for 17 different DPBeta haplotypes by locus specific amplification followed by allele specific oligonucleotide hybridization. Sera from the same patients were assayed for the presence of autoantibodies to Sm, RNP, Ro, La and of anticardiolipin antibodies (aCL). DPB1*0301 and DPB1*1401 were increased in patients compared with 107 healthy controls, mainly in those anti-Sm/RNP positive and aCL positive. Remarkably, DPB1*1401 and DPB1*0301 have a nearly identical nucleotide sequence, suggesting that an epitope shared by their membrane products is of major importance for the DP related susceptibility to SLE.

Adult↗