Renal vein thrombosis in systemic lupus erythematosus: association with the "lupus anticoagulant".

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.

A double-blind study comparing sodium aurothiomalate and auranofin in patients with rheumatoid arthritis previously stabilized on sodium aurothiomalate.

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 The remaining nine patients were withdrawn because of loss of efficacy (two), side-effects, (five), loss of efficacy and side-effects (one) and default (one).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 Of the remaining six patients, two were withdrawn because of side-effects, three because of poor disease control and one because of side-effects and poor disease control.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.

Outcome in juvenile arthritis.

Juvenile chronic arthritis has a number of subtypes with only seropositive juvenile rheumatoid arthritis and systemic juvenile chronic arthritis having equivalents in adult life. In 75% of patients the inflammatory disease has subsided by adulthood, leaving some with degenerative and mechanical problems. Systemic, polyarticular and pauciarticular subgroups, based on mode of presentation, have been related to prognosis. Seropositive polyarticular disease behaves as an aggressive form of adult rheumatoid arthritis. Standard methods of assessment are inappropriate in children. Active joint score is most useful. Radiographs are difficult to interpret because of growth and lack of early erosive disease. Growth and social outcome is important. Death occurs in 7% of cases and is due to infection and cardiac involvement during active systemic disease, and due to secondary amyloidosis later. Slow-acting drugs and surgical procedures may alter outcome. The aetiology of these diseases remains unknown and there is a need for diagnostic tests, particularly to identify those children who will do badly.

Pulmonary paraprotein production in Waldenström's macroglobulinaemia.

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.

Ankylosing spondylitis and an aortic arch syndrome.

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.

Pneumococcal septic arthritis in rheumatoid arthritis.

Septic arthritis is associated with a definite morbidity which may be related to a delay in diagnosis and hence treatment. The cases of three patients with rheumatoid arthritis and chronic chest disease where the joint sepsis was not the predominant feature are presented. The responsible organism was Streptococcus pneumoniae which had spread after recent chest infections. Minimal joint symptoms or general malaise in association with an unexplained rise in erythrocyte sedimentation rate in these circumstances warrant a search for joint sepsis.

Aortic incompetence in pauciarticular juvenile chronic arthritis.

An 8 year old girl with seronegative pauciarticular onset juvenile chronic arthritis who developed progressive joint destruction and aortic incompetence is presented.

Lesch-Nyhan syndrome presenting with renal insufficiency in infancy and transient neonatal hypothyroidism.

A 20-day-old male infant presented with acute renal failure. Three weeks later he developed acutely swollen, hot, red joints and tophi in his hands and feet. The serum uric acid was 2.2 mmol/l (normal 0.13-0.23 mmol/l) and the urinary oxypurine/creatinine ratio was 2.26 mmol (normal < 1.5 mmol). Complete deficiency of hypoxanthine guanine phosphoribosyl transferase (HGPRT) in intact erythrocytes confirmed Lesch-Nyhan syndrome. Neurological development was delayed and self-mutilation was observed at 22 months. Acute renal failure secondary to crystal nephropathy and tophaceous gout are unusual presenting features of this rare condition. This child also had transient neonatal hypothyroidism, which is not a recognized manifestation of the syndrome.

D-penicillamine and polymyositis: the significance of the anti-Jo-1 antibody.

A 57-yr-old lady developed polymyositis whilst taking D-penicillamine for RA. D-Penicillamine-induced polymyositis occurs in RA with a greater frequency than idiopathic polymyositis. Anti-acetyl choline receptor antibodies and ANA were positive, consistent with drug-induced disease. Anti-Jo-1 antibodies are considered specific for idiopathic myositis, and their presence was unexpected. Following withdrawal of the drug, the disappearance of the anti-Jo-1 and other antibodies coincident with clinical improvement, suggested that D-penicillamine was responsible for inducing antibody production.

Sjögren's syndrome presenting as a severe sensory neuropathy including involvement of the trigeminal nerve.

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.

Nuclear magnetic resonance (NMR) tomographic imaging for popliteal cysts in rheumatoid arthritis.

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.

Circulating human leucocyte elastase in rheumatoid arthritis.

Elastase-inhibitor complex (EIC) levels were determined in EDTA-plasma samples of 40 patients with connective tissue disease by a double antibody immuno-assay technique. In active rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE), EIC levels were significantly higher than in the normal controls (P less than 10(-8) and fell on remission. The mean EIC level in active RA was significantly greater than in inactive disease (P = 0.0001) but there was no statistically significant difference between the EIC levels in the acute and inactive disease states in SLE (P = 0.49). In active RA, there was a positive correlation between EIC levels and white blood cell count (WBC) but not with erythrocyte sedimentation rate (ESR). In SLE there was no significant correlation between EIC levels and ESR or WBC. EIC measurement may be useful in the objective assessment of activity in RA.

Anticardiolipin antibodies: occurrence in Behçet's syndrome.

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.

Degos' disease: association with anticardiolipin antibodies and the lupus anticoagulant.

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.