Biomedical subjects
Publications and source records attributed to R G Hull.
An 8 year old girl with seronegative pauciarticular onset juvenile chronic arthritis who developed progressive joint destruction and aortic incompetence is presented.
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A woman presented with multiple cerebral thromboses and skin lesions characteristic of Degos' disease. Her serum contained high titres of anticardiolipin antibodies and showed lupus anticoagulant activity, both known to be strong markers of a thrombotic tendency in systemic lupus erythematosus. This finding may have therapeutic implications for this usually fatal disease.
Primary Sjögren's syndrome (SS) can be associated and may present with neurological complications. We describe a 32-year-old woman with a debilitating sensory neuropathy, including trigeminal nerve involvement which was the initial manifestation of this disease.
The ability of nuclear magnetic resonance (NMR) tomographic imaging to show a change in proton spin-lattice relaxation time (T1 value) in the joints of patients with rheumatoid arthritis before and after treatment with intra-articular steroid has been assessed. Six patients with seropositive rheumatoid arthritis and clinical evidence of a popliteal cyst were examined by both NMR tomography and arthrography. In all cases the presence of active rheumatoid arthritis was shown by an increase in the T1 values of the synovium of the joints. After arthrography a consistent but small rise in T1 value was noted which fell to below the initial reading after treatment by intra-articular injection of an anti-inflammatory agent. In all cases the presence of the popliteal cyst was clearly shown by both NMR tomography and arthrography. The findings indicate that NMR tomography provides a sensitive method for the demonstration of inflammatory joint disease, popliteal cysts, and possibly for monitoring response to therapy.
Anticardiolipin antibodies have recently been described in association with arterial and venous thrombosis, and with neurological symptoms, in connective tissue diseases. In a study of 70 patients with Behçet's syndrome 13 patients had these antibodies. Of these 13 patients eight had a history of either retinal vascular pathology, cerebral infarction, or thrombophlebitis. The association of retinal vascular disease and the presence of anticardiolipin antibodies was statistically significant.
A 63 year old woman with a 16 year history of ankylosing spondylitis and peripheral joint involvement later presented with a large vessel arteritis affecting the branches of the arch of the aorta.
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Auranofin, an orally active gold preparation, was compared with sodium aurothiomalate in a double-blind trial in patients with rheumatoid arthritis fulfilling the ARA criteria, who had been stabilized on sodium aurothiomalate for at least six months. Twenty-four patients have so far been entered in the trial, of whom fourteen have been randomly allocated to receive auranofin and ten to receive sodium aurothiomalate. After initial stabilization, patients receive either auranofin 6 mg daily and placebo injection, or sodium aurothiomalate 50 mg monthly and placebo tablets. Five patients have completed one year on auranofin. The remaining nine patients were withdrawn because of loss of efficacy (two), side-effects, (five), loss of efficacy and side-effects (one) and default (one). Four patients have completed one year's treatment with sodium aurothiomalate. Of the remaining six patients, two were withdrawn because of side-effects, three because of poor disease control and one because of side-effects and poor disease control. Diarrhoea occurred in eight patients receiving auranofin. Rashes occurred in both groups but otherwise there were no serious side-effects. The efficacy of both drugs appeared similar, there being no significant differences in morning stiffness, fatiguability, visual analogue pain score, grip strength and articular index. There were also no significant differences in laboratory parameters of efficacy. Auranofin appears to control disease activity in rheumatoid arthritis but diarrhoea is a frequent side-effect.
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A case of Waldenström's macroglobulinaemia is presented where marrow examination was normal and production of the abnormal protein was centered on the lungs. Symptomatic and biochemical improvement was achieved with chlorambucil and prednisone. Lung manifestations in the disease are briefly reviewed.
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Two patients with lupus erythematosus and renal vein thrombosis are described. Both patients had the "lupus anti-coagulant" in their serum. It is postulated that in these patients the clotting tendency could be favoured by a cross reaction of the "lupus anti-coagulant" with phospholipids in the endothelial cell membrane, resulting in inhibition of prostacyclin release.