LEFT VENTRICULAR FUNCTION IN SCLERODERMA

Scleroderma affects the left heart directly and indirectly viathe effects of systemic hypertension. Using transthoracic echocardiography,we evaluated 35 patients with scleroderma and compared themwith matched control subjects. Compared with controls, therewere no differences between left ventricular dimensions, wallthickness, calculated mass or fractional shortening. However,the left atrium was enlarged (P = 0.006) and the mitral decelerationtime was prolonged (P = 0.0005) in patients with scleroderma;suggesting abnormal diastolic function. After adjusting forpotential confounders, duration of Raynaud's was found to bean independent predictor of deceleration time (P = 0.04), E/Apeak velocity ratio (P = 0.04). A peak velocity (P = 0.004)and A velocity time integral (P = 0.0001), all measures of diastolicfunction. This group of individuals with scleroderma have evidenceof abnormal diastolic function of the left ventricle despitenormal left ventricular size and systolic function, and in theabsence of hypertrophy. This finding is independent of the useof vasoactive medications and history of systemic hypertension,and thus may be due to primary myocardial involvement by scleroderma.The tendency to abnormal diastolic function of the left ventriclecorrelated with the duration of Raynaud's phenomenon. KEY WORDS: Scleroderma, Echocardiography, Left ventricle, Diastolic function     

NAILFOLD CAPILLAROSCOPY: A BLINDED STUDY OF ITS DISCRIMINATORY VALUE IN SCLERODERMA, SYSTEMIC LUPUS ERYTHEMATOSUS, AND RHEUMATOID ARTHRITIS

The appearances of the nailfold capillaries can be used to distinguish between various connective tissue diseases. In a study of 30 patients (10 with scleroderma, nine with systemic lupus erythematosus, and 11 with rheumatoid arthritis), photographs were taken of the eight nailfolds of each patient (thumbs excluded) and then coded. Each of the photographs was later analysed by a rheumatology registrar and an attempt was made to predict the patient's diagnosis using only the appearance of the nailfold. The diagnostic specificity and sensitivity were 89% and 80%, respectively.
The results indicate that nailfold capillaroscopy, performed by a relatively inexperienced observer, can accurately distinguish between patients with scleroderma and those with systemic lupus erythematosus or rheumatoid arthritis. (Aust NZ J Med 1986; 16: 457–460.)     

DILTIAZEM INDUCES REMISSION OF CALCINOSIS IN SCLERODERMA

There is no widely accepted treatment for the calcinosis whichoccurs in scleroderma and dermatomyositis. We report a caseof a 62-yr-old woman with active scleroderma complicated bytuberose calcinosis. The calcinosis, which had previously beenunchanged for several years, regressed over a 2-yr period duringwhich diltiazem was used to treat hypertension. This effectcould not be explained by altered disease activity or renalfunction but, we suggest, may be due to inhibition of calciuminflux into cells. This treatment merits further evaluation. KEY WORDS: Systemic sclerosis, Calcinosis, Treatment, Diltiazem     

PHTHALYLSULPHATHIAZOLE IN RHEUMATOID ARTHRITIS

Sixteen patients with active rheumatoid arthritis were treatedwith phthalylsulphathiazole (4 g/day) over a period of 24 weeks.Although there was some statistically significant improvementin plasma viscosity, IgM, pain score, morning stiffness andsummated change score, this was either intermittent or not maintained.Five patients withdrew from the trial before completion, four(25%) with non-serious adverse reactions and one patient fromlack of efficacy; only one patient elected to remain on thedrug beyond the 24-week period. Low free and total sulphathiazoleserum concentrations were found, confirming that most of thedrug remained within the gut. This investigation suggests, certainlyat the dose used, that phthalylsulphathiazole does not havethe properties of a second-line agent. Higher doses of the drugwill not be ethically feasible. KEY WORDS: Sulphonamides, Second-line agent, Rheumatoid arthritis     

METHYLSULPHASALAZINE IN RHEUMATOID ARTHRITIS

Methylsulphasalazine, which differs from sulphasalazine by theaddition of one methyl group, may provide the benefits of theparent drug with fewer side-effects in rheumatoid arthritis(RA). We describe the outcome of its use in RA. Of 21 patientsentered into the study, 10 successfully completed 6 months oftherapy; five developed adverse effects, four with drew forreasons unrelated to drug treatment and two stopped becauseof inefficacy. No serious adverse effects were reported. A statisticallysignificant improvement in most clinical assessments was observedfrom weeks 8–12 onwards. Significant improvement in plasmaviscosity was observed and there was a trend towards improvementin serum CRP, his tidine and IgM concentrations. There was agood correlation between mean serial changes in clinical andbiochemical assessments indicating that the drug may exhibitthe properties of a second-line agent. Median steady-state serumconcen trations of methylsulphasalazine and methylsulphapyridinewere 26.6 µg/ml and 2.85 µg/ml respectively. KEY WORDS: Therapy, Sulphasalazine, Second-line agent     

THYMOPOIETIN IN RHEUMATOID ARTHRITIS

In a controlled study involving 36 patients, thymopoietin wasshown to be more effective than levamisole and as effectiveas penicillamine in improving the clinical status of patientswith rheumatoid arthritis. There were small reductions in erythrocytesedimentation rate and IgG which did not achieve statisticalsignificance. Rheumatoid factor titre did not change. Althoughits mechanism of action is almost certainly related to its immunomodulatoryproperties it does not seem to be the same as that of levamisole.     

DEFECTS OF THE RETINAL PIGMENT EPITHELIUM IN SCLERODERMA

We completed ocular examination, including retinal fluoroangiography,in 19 unselected patients with diffuse systemic sclerosis (scleroderma)and compared the findings with those made in 50 consecutivepatients with systemic lupus erythematosus, 18 with primarySjögren's syndrome, 20 with mixed connective tissue disease,and 20 healthy women. Five of 19 scleroderma patients had atrophyof the retinal pigment epithelium (26.3%) while none of thecontrols and only four of the 88 (4.5%) patients with otherconnective tissue diseases (P<0.01) had this anomaly. Atrophyof the pigmented epithelium of the retina may occur in sclerodermaas a result of damage of the choroidal plexus. KEY WORDS: Scleroderma, Retinal involvement, Retinal pigment epithelium atrophy     

DAPSONE IN RHEUMATOID ARTHRITIS

After a preliminary period of observation, 71 patients withconsistently active rheumatoid arthritis (RA) were given dapsone50 mg daily for one week, then 100 mg daily. There was a significantimprovement in subjective clinical state and there were significantfalls in serum C-reactive protein (CRP) and the ESR; the Rose-Waalertitre (RW) did not fall. The results were compared with those in 78 patients who hadgold. Subjective clinical improvement was slower with gold,but from six weeks the pattern of changes in clinical score,CRP and ESR was similar for both drugs. The RW fell during goldtreatment. The results suggest dapsone is effective in RA; over-all itappears to be slightly less effective than gold but much lesstoxic.     

BRONCHOALVEOLAR LAVAGE IN RHEUMATOID ARTHRITIS

Bronchoalveolar lavage (BAL) was performed on 70 RA patients,28 without extra-articular manifestations, nine with pulmonaryinvolvement, 13 with sicca-syndrome, 20 with other extra-articularmanifestations such as renal involvement, cutaneous vasculitisand rheumatoid nodules. Fifteen patients without rheumatic orpulmonary disease served as the control group. Compared withthe control group RA patients showed a statistically significantincrease of lymphocytes, especially of activated (DR+)T(CD3+)-helper(CD4+) cells, resulting in a significantly diminished percentageof alveolar macro-phages, B(CD21+)-lymphocytes, T-suppressor(CD8+) cells and an increased CD4/CD8 ratio. This cell distributionpattern was more pronounced in RA patients with lung involvementwith significant differences to the other RA patients with regardto lymphocytes, DR positive cells and CD4 positive/DR positivecells. It is concluded that these results indicate an alteredbalance of immunocompetent cells not only in the joints butalso in the lung. The changes are more distinct if local manifestationscan be diagnosed clinically. KEY WORDS: Bronchoalveolar lavage, Rheumatoid arthritis, Pulmonary manifestation, Lymphocyte subsets     

ANTICARDIOLIPIN ANTIBODIES IN RHEUMATOID ARTHRITIS

Anticardiolipin antibody (ACA) was present in the sera of 49%of 90 consecutive patients with rheumatoid arthritis (RA). TheACA was absent in 30 control patients with osteoarthritis. C-reactiveprotein levels equal to or exceeding 7 mg/dl were found in 10patients all of whom were ACA positive. ACA was present in a larger proportion of rheumatoid factor(RF) positive than of RF negative patients. Male sex and extra-articularmanifestations of RA were both more common in ACA positive thanACA negative patients. In the ACA positive group the lupus anticoagulant and VDRL testswere negative. However, a small number of patients had evidenceof vascular events. KEY WORDS: Anticardiolipin antibody, C-reactive protein, Rheumatoid arthritis, Osteoarthritis     

RAYNAUD'S PHENOMENON IN RHEUMATOID ARTHRITIS

The aim of this study was to evaluate the prevalence of Raynaud'sphenomenon in patients with rheumatoid arthritis (RA), and torelate this symptom to clinical, radiological and serologicalcharacteristics of the patients. All relevant information wasretrospectively obtained from the standardized clinical recordsof 322 RA in-patients first admitted to the Rheumatology Unitof Brest University Medical School. Raynaud's phenomenon wasfound in 54 (17.2%) of 322 RA patients. There was no significantcorrelation between demographic, clinical or radiological characteristics.However, the subgroup of RA patients with Raynaud's phenomenonhad a slightly higher prevalence of vasculitis than the subgroupwithout Raynaud's phenomenon. CRP level and C4 level were foundto be lower in the former than in the latter group, whereasESR and various serological findings (rheumatoid factor, antinuclearantibodies) were comparable in the two groups. We conclude thatthe prevalence of Raynaud's phenomenon is high in French RAin-patients, and that some clinical and biological abnormalities(vasculitis, low CRP level and low C4 level) suggest an associationbetween Raynaud's phenomenon and vasculitis in a few cases,whereas this association might be fortuitous in the remainder. KEY WORDS: Raynaud's phenomenon, Rheumatoid arthritis