Lymphocyte studies in a patient with neurological complications of Behçet's syndrome.

We rapport here a case of Behcet's disease presenting with neurological complications who made a remarkable recovery to high dose steroids and azathioprine. Prior to initiation of steroid therapy the percentage of T lymphocytes (OKT3) was low, that of helper T lymphocytes (OKT4) was normal and suppressor T lymphocytes (OKT8) was slightly increased. The OKT4: OKT8 ratio was slightly reduced and the percentage of B lymphocytes was high. With steroid therapy there was an increase in the percentage of OKT3 and fall in the percentages of OKT4 and OKT8 and the OKT4: OKT8 ratio was maintained fairly normal. The percentage of B lymphocytes was reduced. On addition of azathioprine the percentage of OKT3 and OKT8 were substantially increased and the percentage of OKT4 was slightly increased. The net result was a significant reduction in the OKT4: OKT8 ratio. The percentage of B lymphocytes remained normal. On reviewing the absolute counts there was a paradoxical rise in OKT4 and OKT8 a week after high dose steroid therapy due to a substantial rise in the total number of lymphocytes. On reducing the dose of steroids the absolute count of OKT4 and OKT8 decreased in parallel with their percentages. A disparity was noted in the percentage of OKT3 and the sum of the percentages of OKT4 and OKT8, prior to commencement of therapy which was significantly reduced after treatment. It is likely that this disparity is due to the presence of doubly labelled cells (cortical thymocytes) released into circulation prematurely.

Prevention of vascular damage in scleroderma and autoimmune Raynaud's phenomenon: a multicenter, randomized, double-blind, placebo-controlled trial of the angiotensin-converting enzyme inhibitor quinapril.

OBJECTIVE: To evaluate the efficacy and tolerability of prolonged administration of quinapril, a long-acting angiotensin-converting enzyme inhibitor, in the management of the peripheral vascular manifestations of limited cutaneous systemic sclerosis (lcSSc) and in the prevention of the progression of visceral organ involvement in the disease. METHODS: This was a multicenter, randomized, double-blind, placebo-controlled study evaluating quinapril 80 mg/day, or the maximum tolerated dosage, in 210 patients with lcSSc or with Raynaud's phenomenon (RP) and the presence of SSc-specific antinuclear antibodies. Treatment was for 2-3 years. The primary outcome measure was the number of new ischemic ulcers appearing on the hands; secondary measures were the frequency and severity of RP attacks, skin score, treatments for ischemia, health status (measured by the Short Form 36 instrument), measures of kidney and lung function, and echocardiographic estimates of pulmonary artery pressure. An intent-to-treat analysis was used. RESULTS: Quinapril did not affect the occurrence of digital ulcers or the frequency or severity of RP episodes. It did not alter the treatments that were prescribed for either infected ulcers or severe RP symptoms. There was no apparent effect on the estimated tricuspid gradient. Health status was not affected by quinapril, and one-half of the patients who believed they had benefited from the trial treatment were in the placebo arm. Quinapril was not tolerated by one-fifth of the patients, with dry cough being the most frequent side effect. CONCLUSION: Administration of quinapril for up to 3 years had no demonstrable effects on the occurrence of upper limb digital ulcers or on other vascular manifestations of lcSSc in this patient population.

The assessment of disease activity in rheumatoid arthritis using a multivariate analysis.

A simple method of assessing 'index of disease activity' (IDA) in rheumatoid arthritis (RA) using a multivariate analysis (MVA) comprising morning stiffness (MS), pain scale (PS), grip strength (GS), articular index (AI), haemoglobin (Hb) and erythrocyte sedimentation rate (ESR) is described. The IDA of 99 patients with RA was assessed using MVA. The method could be used reliably and readily for random or longitudinal assessment, in drug trials and for comparing disease activity with other objective indices.

High definition computed tomography in rheumatoid arthritis associated pulmonary disease.

Eighteen patients with rheumatoid arthritis (American Rheumatism Association definition) were selected consecutively from a rheumatology clinic. All patients were examined with plain chest radiographs, thin slice computed tomography, and pulmonary function tests. Four patients with normal chest radiographs, and normal pulmonary function tests were found to have normal CT scans. In ten patients, abnormalities consistent with rheumatoid associated lung disease were demonstrated, including changes of interstitial fibrosis in seven cases. This fibrosis had a predominantly peripheral pattern on CT scan. Computed tomography was found to be more sensitive than plain radiographs in detecting abnormalities; however, all the patients in this series with CT lung changes had abnormalities on pulmonary function testing.

Bronchial obstruction due to respiratory mucosal sloughing in toxic epidermal necrolysis.

A 17 year old woman is described who nearly asphyxiated because of sloughing of the bronchial mucosa as a result of toxic epidermal necrolysis. Bronchoscopic aspiration of bronchial slough proved life saving.

Serum C-reactive protein in polymyalgia rheumatica. A prospective serial study.

A prospective serial study of 13 well-documented, previously untreated cases of polymyalgia rheumatica was undertaken in order to assess the behavior of the nonspecific indices of disease activity, erythrocyte sedimentation rate and serum C-reactive protein (CRP) concentration, during induction of disease remission by prednisolone therapy. The clinical manifestations of all patients responded rapidly and completely to steroids, and the serum CRP value, which was raised in all patients at presentation, fell to normal at a rate which precisely reflected the clinical improvement. The erythrocyte sedimentation rate also fell, but did so much more slowly than the CRP concentration and, in half the patients, was still not normal after 14 days. These results indicate that assay of serum CRP provides a precise means of objectively assessing the course of polymyalgia rheumatica during initial therapy with steroids, and suggest that routine measurements of CRP may make a useful contribution to the management of the disease.

Correlation of clinical parameters of disease activity in rheumatoid arthritis with serum concentration of C-reactive protein and erythrocyte sedimentation rate.

Serum C-reactive protein (CRP) levels were measured in 99 patients with definite or classical rheumatoid arthritis (RA). At the same time as the serum was obtained, the activity of the RA was assessed by assigning scores to a comprehensive set of subjective, semi-objective and objective clinical features. The results confirmed that the serum CRP concentration closely reflects activity of RA and is of value in its objective assessment. Erythrocyte sedimentation rate (ESR) followed the same pattern as CRP levels but all of the subjective and semi-objective criteria correlated more significantly with CRP levels than with ESR.

Anti-keratin antibodies in rheumatoid arthritis.

A naturally occurring antibody that reacts with the keratinised tissue of animal oesophagus was found in the serum of 75 out of 129 patients (58%) with classical or definite rheumatoid arthritis (RA) but not in sera from 105 healthy people. Detection of the antibody, which is unrelated to rheumatoid factor, is more specific for RA than the reaction in the sheep-cell agglutination test but less sensitive.

Anti-keratin antibodies in rheumatoid arthritis: frequency and correlation with other features of the disease.

Anti-keratin antibodies (AKA) were detected in 68 out of 98 patients (69%) with classical or definite rheumatoid arthritis (RA). The intensity of the AKA reaction correlated significantly with articular index (AI), grip strength (GS), erythrocyte sedimentation rate (ESR), serum C-reactive protein (CRP) concentration, serum amyloid A (SAA) protein concentration, the level of antibodies against single stranded DNA (ssDNA) and the IgM rheumatoid factor (RF) titre. A significantly higher number of patients with nodules and Sjögren's syndrome were AKA positive compared with patients without extra-articular features (EAFs) and the AKA titre was significantly greater in the former group. The mechanisms underlying appearance of AKA are not known but may relate to an as yet unidentified structural alteration of keratin in this disease or may just reflect the rheumatoid autoimmune diathesis.

Serum amyloid-A protein concentration in inflammatory diseases and its relationship to the incidence of reactive systemic amyloidosis.

Serum amyloid-A protein (SAA) is the putative precursor of amyloid-A (AA) protein which forms the fibrils in reactive systemic or secondary amyloidosis. By means of a novel immunoradiometric assay, the concentration of SAA was found to be greatly elevated in patients with rheumatoid arthritis and juvenile chronic arthritis and correlated with activity of their primary disease. However, in patients with systemic lupus erythematosus SAA levels were only modestly raised, even in those with severe active disease, unless significant intercurrent microbial infection was also present. In Crohn's disease SAA levels showed a pattern similar to that seen in rheumatoid arthritis, whereas in ulcerative colitis it resembled that of systemic lupus erythematosus. The level of SAA response in these different disorders corresponds with the incidence of reactive systemic amyloidosis in them. These observations support the view that major increases in SAA levels are a necessary condition for the deposition of this form of amyloid and suggest that prospective monitoring of the SAA concentration in predisposing diseases may help to identify those individuals who are most at risk for amyloidosis.

Correlation in rheumatoid arthritis of concentrations of plasma C3d, serum rheumatoid factor, immune complexes and C-reactive protein with each other and with clinical features of disease activity.

The concentrations of C3d in the plasma and of C-reactive protein (CRP), immune complexes and rheumatoid factor in the serum were measured in 99 patients with rheumatoid arthritis. Most patients had raised levels, the values of which correlated with disease activity assessed according to a newly described index based on multivariate analysis of subjective, semi-objective and objective features of the disease. There were also significant correlations between the values for plasma C3d and circulating immune complexes, immune complexes and rheumatoid factor, serum CRP and immune complexes, and serum CRP and plasma C3d. Measurement of plasma C3d provides a useful means of detecting in vivo complement activation, which may be involved in the pathogenesis of rheumatoid arthritis, but neither C3d levels nor any of the other variables correlated as closely with disease activity as did the serum CRP concentration.