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Biomedical subjects

H Spiera

Publications and source records attributed to H Spiera.

At least 73 records · Page 4Linked to original sources

Myocardial infarction in four young patients with SLE.

Four patients with chronic systemic lupus erythematosus (SLE) in whom myocardial infarction occurred at an unusually early age are described. The evidence suggests that the coronary occlusion was due to atherosclerosis. There was no evidence that active arteritis played any role. The only risk factor for atherosclerotic disease was hypertension. All patients had had both central nervous system and renal disease and had been taking corticosteroids for a minimum of 9 years. It is suggested that hypertension aggravated by chronic corticosteroid administration may be an important risk factor for atherosclerosis in patients with SLE.

Adolescent↗

Cutaneous vasculitis complicating coeliac disease.

A 38 year old female, with chronic uncontrolled coeliac disease, presented with the rare complication of cutaneous leucocytoclastic vasculitis. Detailed study failed to identify any cause for the vasculitis, other than the underlying coeliac disease. Haematuria and proteinuria with mesangial nephritis were also demonstrated on renal biopsy with electron microscopic study. It is speculated that exogenous or endogenous antigens permeated the abnormal small bowel mucosa leading to formation of circulating immune complexes. Subsequent tissue deposition of these complexes then resulted in vasculitis and nephritis. The skin lesions cleared completely after treatment with a strict gluten free diet.

Adult↗

Binding of synthetic double-stranded DNA by serum from patients with systemic lupus erythematosus: correlation with renal histology.

Detection of antibody to double-stranded DNA by direct binding assays has proved useful in clinical management of patients with systemic lupus erythematosus (SLE). Recent confusion regarding specificity of these antibodies for SLE appears to be due, at least in part, to contamination of natural DNA preparations with nondouble-stranded DNA antigens. Measurement of binding of a synthetic, self-complementary DNA copolymer (dAT) rather than of natural DNA (KB) has been shown to obviate some of these difficulties, apparently because of freedom of dAT from nondouble-stranded DNA antigens. Among the advantages found in this way was a higher degree of specificity of antibodies to double-stranded DNA for clinically-judged active lupus nephritis than had been suspected. Since activity of nephritis is difficult to assess clinically, histologic data were sought to confirm these observations. Thirty-two kidney specimens were examined by light and/or electron microscopy. The degree of histologic activity and the amount and location of glomerular electron-dense deposits were semiquantitated blindly. The binding of both dAT and KB DNA was measured by the ammonium sulfate method. Correlation with the amount of electron-defense deposits was highly significant for dAT binding and somewhat less so for KB DNA binding as determined by both parametric and nonparametric statistical methods. Significant correlation with histologic activity was found for dAT but not KB DNA binding. These results are consistent with previous data and suggest that dAT binding may provide a useful, noninvasive means of clinically assessing both nephritis activity and the intensity of glomerular immune-complex deposition as reflected by the amount of electron-dense deposits. If it can be confirmed that the latter provides long-term prognostic information, then dAT binding (and perhaps its reponse to therapy) may also prove of value in this regard.

Adenosine Monophosphate↗

Interstitial pneumonitis complicating rheumatoid arthritis. Sustained remission with azathioprine therapy.

A patient with classic rheumatoid arthritis developed biopsy-proven diffuse interstitial pulmonary fibrosis and ventilatory insufficiency which appeared to be irreversible. The administration of azathioprine coincided with significant immediate improvement in pulmonary function and clinical status. During five years of continuous azathioprine therapy, progressive improvement in lung function has been accompanied by marked deterioration of the rheumatoid joint disease, suggesting that the pulmonary and joint lesions of rheumatoid disease may not be mediated by the same pathways.

Arthritis, Rheumatoid↗

Detection of anti-DNA antibody using synthetic antigens. Characterization and clinical significance of binding of poly (deoxyadenylate-deoxythymidylate) by serum.

Virtually all preparations of DNA used to detect antibody to native DNA (nDNA) by binding assays have been found to be subtly contaminated by single stranded DNA. Because recent DNA binding data have directly challenged the unique role previously attributed to these antibodies in systemic lupus erythematosus (SLE), resolution of the consequent ambiguity is of theoretical and practical importance. It is proposed that a synthetic nDNA molecule (dAT) might circumvent this difficulty by being antigenically equivalent to nDNA while, on theoretical grounds, lacking significant contamination with single stranded DNA or other cellular antigens. These expectations were generally confirmed by biochemical and immunological analyses. In clinical studies, sera from 124 pateints with SLE and from controls were examined for their ability to bind dAT. In contrast to results with KB binding, patients with non-SLE rheumatologic disorders were indistinguishable from normals by dAT binding. dAT binding was elevated in 85% of sera from SLE patients with clinically-judged active nephritis but in only 9% of those with inactive renal disease. Active non-renal disease, including cerebritis, was not associated with increased dAT binding. Individual non-lupus sera which bound increased amounts of KB DNA, failed to bind dAT. It is suggested that such binding resulted from contaminating non-nDNA antigens. When elevated, dAT binding, like KB binding, varied with disease activity and might thus be useful as a parameter thereof. In several patients elevated dAT binding led to the finding, on biopsy, of clinically silent, active, diffuse proliferative nephritis. It is concluded that use of synthetic nDNA antigens such as dAT may offer theoretical and practical advantages over naturally-derived preparations in detecting anti-nDNA, both clinically and for investigational purposes. Also, caution is urged in interpreting DNA binding data derived from incompletely characterized systems, particularly with regard to the occurrence of anti-nDNA antibodies in serum.

Adult↗

Multifocal fibrosis involving the thyroid, face, and orbits.

Our patient had fibrous thyroiditis with unusual facial and retroorbital fibrosis. Systemic fibrosis, which is more typical of the multifocal fibrosclerosing syndromes, was not evident. Treatment with steroid alone, surgery, and radiotherapy failed to maintain a remission. The combination of steroid and cyclophosphamide therapy appears to have been successful.

Bone Diseases↗