Systemic sclerosis is not associated with clinical or ambulatory blood pressure.

BACKGROUND: Raynaud's phenomenon is often the first symptom and occurs eventually in more than 95% of patients with systemic sclerosis (SSc). Angiographic studies disclose narrowing and obstruction of the digital arteries, which on autopsy histologic study show prominent subintimal connective tissue proliferation without inflammation, as well as adventitial fibrosis. It is also known that primary cardiac problems include pericarditis, left ventricular or biventricular failure, serious supraventricular or ventricular arrhythmias emerge in patients with SSc. It is not known if these patients present hypertension or hypotension and which parameter of the ambulatory blood pressure may influence such a disease course. METHODS: A total of 85 subjects underwent clinical blood pressure (BP) readings, 24-hour ambulatory BP monitoring, left ventricular assessment by echocardiography and measurement of intima media thickness (IMT) of the right-left internal carotid arteries (RICA and LICA) and right-left common carotid arteries (RCCA and LCCA). The population consisted of 40 subjects with SSc according to the criteria of the American College of Rheumatology (SCL-group) who were not receiving any antihypertensive treatment and 45 healthy volunteers (control group). The two groups did not differ in age. RESULTS: Clinical systolic and diastolic blood pressure, clinical heart rate, mean 24 h systolic blood pressure, SD systolic blood pressure, mean 24 h diastolic blood pressure, SD 24 h diastolic blood pressure, mean 24 h heart rate, SD 24 h heart rate, pulse pressure 24 h, serum glucose, cholesterol, triglycerides, HDL, LDL, creatinine, urea, potassium and natrium did not statistically significant differ between the two groups. Furthermore, the left ventricular mass/BSA and IMT of both carotid arteries did not show a statistically significant difference between the groups. CONCLUSION: Systemic sclerosis is not associated with clinical blood pressure or the parameter of 24 h blood pressure monitoring.

Macrovascular disease of coronaries and cerebral arteries in streptozotocin-induced diabetic rats. A controlled, comparative study.

The aim of this study was to demonstrate the macrovascular disease in streptozotocin-induced diabetic rats and assess any possible differences between the histopatholological changes of the coronaries and cerebral arteries. Hearts and brains were obtained after 4 weeks (short-term experimental diabetes, 10 rats) and 12 weeks (long-term experimental diabetes, 10 rats) of streptozotocin injection. Sham injected, control rats were studied in parallel. Muscular-type arteries of 0.10-0.15 mm were examined and semiquantitatively classified either as normal, or slightly, or moderately, or severely thickened by light microscopy: While the arterial wall appeared normal in all sham-injected rats, a varying degree of hyperplasia of the muscular layer and deposition of fibrinoid material resulting in arterial stenosis was prominent in streptozotocin-injected rats. In the group of short-term diabetes there was a slight thickening of the cerebral arteries in the majority of the rats (8/10 rats), while thickening of the coronaries was moderate (9/10 rats). Further progression of arterial wall thickening in both cerebral and coronary arteries was observed in the long-term diabetic group. The mean severity of lesions was significantly higher in the coronaries than in cerebral arteries, both in the short-term (p < 0.0005) and long-term diabetes (p < 0.02). Moreover, by paired statistics within individual animals, we confirmed that wall thickening was significantly more severe in coronaries than cerebral arteries in both groups. These findings suggest an accelerated progress of macrovascular disease in the heart as compared to the brain in the streptozotocin-induced diabetic rat. Although histopathological changes in humans do not always mirror clinical severity, the differences in the macrovascular disease between heart and brain in experimental diabetes may be relevant to the higher relative risk of myocardial infarction compared to stroke for people with diabetes, as compared to people without diabetes.

Plasma renin activity as a marker of renovascular injury in patients with rheumatoid arthritis.

OBJECTIVES: To assess whether plasma renin activity (PRA) in patients with rheumatoid arthritis (RA) and evidence of renal involvement (microhematuria) can serve as potential marker of renovascular injury. METHODS: PRA was measured at rest and following exercise. All nonsteroidal antiinflammatory drugs or other medications that might affect renin release were stopped at least ten days prior to PRA measurements. PRA was correlated with the number of dysmorphic erythrocytes present in the urine sediment as indicators of glomerular capillary injury (microhematuria). RESULTS: All patients with RA had a higher mean PRA than controls. Moreover, all patients with RA in whom microhematuria was present had a higher PRA than those without microhematuria. Simple and multiple regression analysis revealed a significant correlation between: a) PRA and rheumatoid factor levels; b) rheumatoid factor levels and the number of erythrocytes in the urine sediment; and c) PRA levels and the number of erythrocytes in the urine sediment. CONCLUSIONS: The observations indicate that increased PRA may occur in normotensive patients with RA and no clinical or biochemical evidence of renal involvement. This may reflect activation of the renin-angiotensin system. The positive correlation between enhanced PRA, rheumatoid factor levels and microhematuria in RA patients may indicate inflammatory injury of the glomerular microvasculature involving the juxtaglomerular apparatus.

Cardiac involvement in collagen diseases.

The purpose of this study is to evaluate the early morphological and functional abnormalities of the heart in patients with collagen disease. The study population was free of risk factors for coronary artery disease and without any clinically evident cardiac manifestations. In 62 patients with collagen disease (25 with progressive systemic sclerosis, 19 with systemic lupus erythematosus, 15 with rheumatoid arthritis, three with dermatomyositis) and in 40 healthy subjects an echocardiographic study was performed. Echocardiographic examination from the apical four-chamber view was performed at rest and during the end of a 3 min isometric exercise with handgrip. Global and regional ejection fraction of the left ventricle were calculated. In the group with progressive systemic sclerosis the left ventricular mass index was significantly higher than in the control group (110.78 +/- 48.61 vs 82.18 +/- 28.46 g.m-2) and the ejection fraction (53.61 +/- 7.95%) was the lowest of all groups (control: 61.47 +/- 8.52%, systemic lupus erythematosus: 59.04 +/- 8.58%, rheumatoid arthritis: 62.38 +/- 6.88%). Regional ejection fraction analysis revealed a major dysfunction of the proximal segment of the interventricular septum, in all groups. During isometric exercise, the global and regional ejection fraction did not change significantly, although differences between groups disappeared. In rheumatoid arthritis, mitral and aortic valve leaflet separation appeared to be reduced. In the group with systemic lupus erythematosus, mild abnormalities were noticed, although the mean age and duration of the disease were the smallest compared with the other groups. In conclusion, patients with progressive systemic sclerosis mainly present left ventricular hypertrophy with a reduced ejection fraction while rheumatoid arthritis patients show a predominant valve dysfunction.(ABSTRACT TRUNCATED AT 250 WORDS)

CPPD crystal deposition disease as a cause of unrecognised pyrexia.

We describe three cases of CPPD crystal deposition disease in elderly patients whose main symptom was fever. Misdiagnosis of such cases is possible because of the similarity of the clinical picture to that of septic fever. The probable mechanisms causing the fever are discussed. There was spectacular improvement in these patients after a high dose of oral colchicine and loperamide and no relapse was observed during the long term administration of colchicine in a conservative dose together with supplementary magnesium.

Organic brain syndrome with psychosis as an initial manifestation of systemic lupus erythematosus in an elderly woman.

This paper describes a rare case of organic brain syndrome with psychosis and clinically transverse myelopathy, as initial manifestations of systemic lupus erythematosus in an elderly woman. The identification and evaluation of antibodies to ribosome P in the serum and cerebrospinal fluid may be of help in such cases for differential diagnosis. The patient was treated successfully with 30 mg prednisone daily.

Clinical and laboratory parameters in adult diabetics with and without calcific shoulder periarthritis.

The clinical and laboratory parameters of calcific shoulder periarthritis (CSP) were examined in 900 patients with type II diabetes mellitus as well as in 350 age- and sex-matched control subjects. A threefold increased prevalence of CSP in diabetics compared with the control group was associated with the presence of longstanding and poorly controlled diabetes, hypercholesterolemia, and hypertriglyceridemia suggesting pronounced diabetic angiopathy, as well as with minor trauma and hypomagnesemia. Aging and serum calcium concentrations were not related to the presence of CSP. Thirty-two percent of diabetics with CSP were symptomatic; 15% of them presented with severe pain and restriction of shoulder movement. These findings confirm a close pathogenetic interrelation between CSP and diabetes mellitus.

Cardiopulmonary hemodynamics in systemic sclerosis and response to nifedipine and captopril.

PURPOSE: This prospective study was performed to evaluate the response of the cardiopulmonary vasculature to two vasodilators in patients with systemic sclerosis and either minimal or no central hemodynamic abnormalities. PATIENTS AND METHODS: Twenty patients with systemic sclerosis, Raynaud's phenomenon (19 of 20 patients), and clinically normal cardiac function underwent right heart catheterization. Rest and exercise hemodynamic measurements, including cardiac output by thermodilution, were performed before and after oral administration of nifedipine 20 mg and captopril 25 mg. RESULTS: Half of the patients had normal hemodynamics (Group A); the other half (Group B) had abnormal baseline elevations in pulmonary vascular resistance and four of them showed "borderline" pulmonary arterial hypertension. Group A, with significantly shorter disease duration compared with Group B, responded poorly to nifedipine and captopril. However, Group B had significant decreases in pulmonary vascular resistance (from 148 +/- 20 to normal levels of 94 +/- 21 dynes.second.cm-5) and pulmonary mean pressure in response to nifedipine treatment but not to captopril. CONCLUSION: These observations show a short-term beneficial effect of nifedipine in the cardiopulmonary vasculature of patients with systemic sclerosis and suggest that a potentially reversible vasoconstrictive element is included in the vascular lesion of this disorder.

Gout and neurological abnormalities associated with cardiomyopathy in a young man.

A 21 year old man with a family history of gout and neurological deficits, developed severe idiopathic congestive cardiomyopathy after a long history of typical gouty attacks and neurological abnormalities. Clinical and laboratory evaluations showed borderline mental retardation, ataxia, sensorineural deafness, marked hyperuricaemia, and excessive uric acid excretion in the presence of impaired renal function. None of the known causes of cardiomyopathy was found. Even though red cell hypoxanthine guanine phosphoribosyltransferase enzyme activity was normal, this case probably represents an inborn error of purine metabolism. The association of cardiomyopathy with gout is very unusual. Previously it has been only once described in a single case.

Glycosaminoglycans in urine, articular and periarticular tissues in streptozotocin diabetes in rats.

Experimental data support the hypothesis that insulin deficiency increases the degradation of glycosaminoglycans (GAGs) and decreases their synthesis. The purpose of this study was to investigate the effect of streptozotocin-induced experimental diabetes on mucopolysaccharide content of the articular and periarticular tissues as well as on the urine GAGs excretion. In two groups (A and B) of ten white Wistar rats, experimental diabetes was induced after administration of streptozotocin in a dose of 65 and 45 mg/kg of body weight, respectively. The animals of group A (short term diabetes) and B (long term diabetes) were sacrificed on the 8th and 87th day of the experimental procedure respectively. GAGs were determined in urine specimens collected before and on the 8th day in group A and before and on 3rd, 8th, 16th, 23rd, 36th, 52nd, 71st and the 87th day in group B. At the end of the experiment articular and periarticular tissue specimens were examined for changes in their mucopolysaccharides content and compared to a group of five animals of the same age and dietary conditions (control group, C). We found a marked increase (P less than 0.001) of urine GAGs excretion during streptozotocin-induced diabetes as compared to the respective values before the experiment in both groups, while in group B a positive relationship (P less than 0.001) between 24 hour urine GASGs excretion, and the duration of diabetes was observed. The histochemistry of the articular and periarticular tissues revealed marked decrease in GAGs content, as well as hardening of the tendons.

Calcific shoulder periarthritis (tendinitis) in adult onset diabetes mellitus: a controlled study.

Two groups, one of 824 adult diabetics and one of 320 age and sex matched non-diabetics, were examined for abnormal glucose metabolism and calcifications on anteroposterior shoulder x rays. Two hundred and sixty two (31.8%) of the diabetics had shoulder calcification compared with 33 (10.3%) of the control group, with a preponderant localisation in the right shoulder. Diabetes of long duration treated with insulin for a long time was associated with a larger percentage of shoulder calcifications. These data and previous laboratory findings suggest a possible pathogenetic correlation between the prevalence of calcific shoulder tendinitis and diabetes.

Cardiac performance in collagen diseases estimated by non-invasive methods.

The myocardial performance was studied in 3 collagen diseases, i.e. rheumatoid arthritis (RA), systemic lupus erythematosus (SLE) and progressive systemic sclerosis (PSS). Sixteen patients with SLE, 12 with RA and 11 with PSS were examined, measuring the systolic time intervals from the first derivative of the carotid pulse in all cases. The ejection fraction (EF), was evaluated in 33 patients using radionuclide left ventricular angiography. The systolic time intervals were compared to those of 103 normal persons and the EF to that of 22 normal controls. In the SLE group the pre-ejection period was significantly shorter, while the ejection period was longer than normally expected. In the same group, the EF was significantly higher that the EF of the control group. These differences could not be related to age, blood pressure, disease duration, coronary risk factors, heart rate or blood values. Most findings in the RA group tended to be opposite to those of SLE. It is concluded that in SLE before any direct involvement of the heart the systolic time intervals and the EF present features similar to those of increased cardiac performance.

Paget's bone disease, anaemia and vitiligo: a case report.

The coexistence of Paget's bone disease, pernicious anaemia and vitiligo is very rare. The pathogenesis of Paget's bone disease remains still unknown. We report here a patient having these three entities simultaneously and we suggest that some cases of Paget's bone disease be due to autoimmunity.

Clinical and laboratory studies of levamisole in patients with rheumatoid arthritis.

Two studies with levamisole in rheumatoid arthritis are reported. In the first study of 30 patients levamisole was superior to placebo in terms of pain relief, reduction in articular tenderness, duration of morning stiffness, erythrocyte sedimentation rate (ESR), and radioisotope uptake in knee and wrist joints. There were no consistent differences in lymphocyte function, immunoglobin or complement concentration, or polymorphonuclear granulocytic function. In the second study forty patients were commenced on treatment with either gold or levamisole. At the end of one year there were significant improvements with both regimens in pain score, joint tenderness and ESR. The patients on levamisole showed significant improvement of duration of morning stiffness, while the patients on gold showed significant improvement of rheumatoid factor titre and left hand grip, but there were no significant differences between the two regimens.

The treatment of gout: a case of medical audit?

An assessment of quality of drug control of hyperuricaemia in gouty subjects is reported. Twenty seven of 40 patients were found to be hyperuricaemic. Factors influencing compliance with drug therapy included awareness of the nature of gout and excessive alcohol consumption.

Mefenamic acid: an under-rated antirheumatic?

A double-blind crossover study was carried out in 18 patients with classical rheumatoid arthritis to assess the effectiveness of mefenamic acid (500 mg t.d.s.) compared with placebo or indomethacin (25 mg q.d.s.). Each treatment was given for 1 week and subjective and objective assessments were carried out at the end of each period. Results showed that both mefenamic acid and indomethacin were significantly better than placebo in most parameters. Although duration of morning stiffness and patient preference favoured indomethacin it was considered that there was no demonstrable difference of any clinical significance between the two active medications.