Esophagitis in scleroderma. Prevalence and risk factors

Of 53 patients with scleroderma (43 women and 10 men) evaluated by esophagoscopy and biopsy, 32 (60%) had erosive esophagitis. Symptoms of heartburn and dysphagia were significantly more frequent in the patients who had erosive esophagitis but often were present in those without this condition. Abnormal motility characterized by loss of peristalsis in the distal esophagus was present in all patients with erosive esophagitis, including the 5 who were asymptomatic. No patient with normal esophageal motility had erosive esophagitis at endoscopy. The patients with erosive esophagitis also had significantly diminished lower esophageal sphincter pressures and increased frequency and duration of gastroesophageal reflux episodes. Stricture was present in 13 of 32 patients with erosive esophagitis and was absent in the other 21 patients. The duration of disease, rate of gastric emptying, and fungal smear and culture were not significantly different in those with or without esophagitis. Treatment of fungal infection for a month had little beneficial effect. The pattern of esophageal motility in scleroderma identifies high and low risk groups for esophagitis and stricture, and can be used to select those who require further investigation, irrespective of symptoms.     

Decreased blood fluidity in progressive systemic scleroderma

The aim was to define blood rheology in progressive systemic scleroderma (PSS). 55 patients were compared to controls. Blood and plasma viscosity, hematocrit, red cell aggregation, and deformability were measured. Except for hematocrit, all these variables are significantly altered, indicating a loss of blood fluidity in PSS. Drugs had no obvious effect on blood rheology, but the clinical picture did. The loss of blood fluidity in PSS is suggested to play a pathophysiological role in the initiation of Raynaud phenomena, from which all patients suffered.     

Problems and observations on progressive systemic scleroderma

At first the pathogenesis of sclerodermia is discussed. Disturbed immunological control mechanisms for the activity of fibroblasts with an increased humoral activity are mentioned as causes. Then relevant clinical changes in connection with own investigations are outlined and finally some therapeutic possibilities are mentioned. The pathobiochemical changes of the collagen metabolism of sclerodermia are analysed and own investigations about the laboratory parameters, hydroxyprolin and N-acetyl-neuraminic acid which summarizingly reflect the collagen metabolism in patients with sclerodermia follow. Finally diagnostic possibilities of a renal participation are shown with the help of a quantitative and qualitative protein analysis in the collective urine, in which case a particular point is to be made of the distinction of selectively and unselectively excreted proteins.     

Cardiovascular function in patients with progressive systemic sclerosis (scleroderma)

Sixteen patients with progressive systemic sclerosis (PSS), including 3 with the "CREST" (calcinosis, Raynaud's, esophageal dysfunction, sclerodactyly, and/or telangiectasias) variant, were evaluated with resting M-mode echocardiography and noninvasive measurements of cardiac output at rest and during submaximal exercise to determine the nature and extent of any cardiovascular impairment. No patient had arterial hypertension, significant renal impairment, clinical evidence of large vessel coronary artery disease, or severe pulmonary dysfunction. The duration of disease was 1 to 12 years (9 to 30 for patients with the CREST variant). Echocardiographic abnormalities included increased right ventricular dimension (3 patients), reduced left ventricular ejection fraction (3 patients), and pericardial effusion (3 patients). Cardiac index (CI) and stroke volume index (SVI) at rest were similar for patients and controls. Patients and controls were exercised to similar heart rates (130 +/- 3 vs 124 +/- 4; p, NS). Total peripheral resistance (TPR) was higher for patients (1123 +/- 81 vs 810 +/- 44 dyn X s X cm-5) and their mean SVI failed to increase significantly compared with sitting rest values (30 +/- 2 vs 35 +/- 3 ml/m2). The control subjects had the expected increase in SVI (36 +/- 2 vs 51 +/- 5; p less than 0.01). Ten patients with an abnormal hemodynamic response to exercise had a normal echocardiographic circumferential fiber shortening (VCF) or ejection fraction (EF) at rest. The data indicate that PSS patients have a greater degree of cardiovascular dysfunction than would be predicted from clinical data and laboratory evaluation of cardiovascular and pulmonary function at rest. Multiple mechanisms, including right and left ventricular dysfunction and abnormal vasoconstrictor activity, are likely to contribute to the reduction in exercise capacity seen in patients with PSS.     

Sj?gren's syndrome in patients with the CREST variant of progressive systemic scleroderma.

Twenty-three patients with the CREST (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia) variant of progressive systemic sclerosis, were clinically, histopathologically and serologically examined for the presence of Sj?gren's syndrome (SS). Fourteen were found to be positive. No significant difference could be demonstrated between them and the remaining 9. Characteristics of patients with CREST were compared with those of 29 randomly chosen patients with primary SS. Parotid gland enlargement was more frequently present (p less than 0.01) in the latter than in the former. Virtually no patients with CREST with SS had antibodies to Ro(SSA)/La(SSB).     

Skin thickness and collagen content in progressive systemic sclerosis and localized scleroderma.

Skin biopsies of uniform location and surface area (7 mm diameter) were obtained from the extensor aspect of the forearm of 147 patients with progressive systemic sclerosis (PSS) (107 with diffuse scleroderma, 40 with the CREST syndrome variant) and 58 individuals with normal skin. After careful removal of all subcutaneous fatty tissue, the skin cores were weighed and their water and hydroxyproline content determined. Despite recent claims to the contrary, it was found that there is a marked and highly significant increase in the thickness of the skin during the indurative phase of PSS, and that this is associated with a proportionate increase in total dermal collagen content. A similar degree of thickening was found in the skin of patients with eosinophilic fasciitis and acromegaly. A close correlation was observed between clinical estimation of the degree of skin thickening and the weight of the skin biopsy cores. Change in the weight of skin cores was observed during the course of illness of the patients with PSS and may serve as a useful measurement of alteration in the degree of skin thickening.     

Immune complexes in progressive systemic sclerosis (scleroderma)

Serum immune complexes were measured in 92 patients with progressive systemic sclerosis, and elevated levels were found as follows: Raji cell assay 72% (59% after pronase treatment of Raji cells), agarose gel electrophoresis 52%, and C1q binding 24%. Fortythree (47%) had abnormal results on two or more of these tests, but only 17 (18%) had normal results by all three assays. Computer-assisted analysis of immune complex results and extensive clinical and laboratory data compiled on these patients revealed that the patients with abnormal Raji cell assays more often had diffuse scleroderma, tendon friction rubs, and positive serum antinuclear antibody tests than did patients with negative results on Raji cell assays. Individuals with immune complexes detected by C1q binding had evidence of pulmonary involvement and positive serum rheumatoid factor more frequently than did patients whose C1q tests were negative.     

Prevalence and risk factors of vertebral fracture in female Japanese patients with systemic lupus erythematosus

Abstract

Objective We examined the prevalence and risk factors of vertebral fracture in female Japanese patients with systemic lupus erythematosus (SLE).

Methods We performed lateral radiographs of the thoracic and lumbar spine and bone mineral density (BMD) measurements and collected demographic, lifestyle, clinical, and treatment characteristics of 52 SLE patients. Vertebral fractures were defined as a >20 % reduction of vertebral body height. Odds ratios (ORs) and their 95 % confidence intervals (CIs) were computed to assess the strength of associations between vertebral fractures and selected factors among SLE patients.

Results At least one vertebral fracture was detected in 50 % of SLE patients. A history of previous bone fracture was significantly associated with an increased risk of vertebral fractures among SLE patients (adjusted OR = 14.8, 95 % CI = 1.62–134; P = 0.017). Daily use of tea or coffee was marginally associated with a decreased risk of vertebral fractures among SLE patients (adjusted OR = 0.11, 95 % CI = 0.01–1.01; P = 0.051).

Conclusion The high prevalence of vertebral fracture in SLE patients (50 %) indicates that we need to assess the lateral spine radiograph in more female Japanese SLE patients regardless of BMD and use of corticosteroids, although additional studies are warranted to confirm the findings suggested in this study.     

Heart involvement in progressive systemic sclerosis (generalized scleroderma)

17 patients with progressive systemic sclerosis were examined for cardiac involvement using standard ECG, chest X-ray, 24-h Holter ECG, and echocardiography. The control groups consisted of 7 patients with mixed connective tissue disease, 10 patients with systemic lupus erythematosus, and 10 patients with rheumatoid arthritis. The results disclosed a cardiac involvement of 70% using all 4 methods, which is more than is reported in the literature. The data also show that all 4 methods are necessary to diagnose the cardiac involvement. Conduction and rhythm disorders especially are more frequent than in other collagen vascular diseases. The use of calcium channel blockers is discussed as a new therapeutic possibility.     

Spontaneous pneumoperitoneum in progressive systemic sclerosis (generalized scleroderma)

In a twenty-nine year old female patient with progressive systemic sclerosis (scleroderma) (PSS) and an impressing involvement of the intestine (disturbance of the oesophageal motility, pseudo-obstruction, malabsorption) a spontaneous, asymptomatic pneumoperitoneum is observed. Perforation or pneumatosis cystoides intestinalis (PCI) could not be proved. The possible pathogenesis, course, prognosis and therapy of this rare complication are discussed.     

Skin thickness and collagen content in progressive systemic sclerosis and localized scleroderma

Skin biopsies of uniform location and surface area (7 mm diameter) were obtained from the extensor aspect of the forearm of 147 patients with progressive systemic sclerosis (PSS) (107 with diffuse scleroderma, 40 with the CREST syndrome variant) and 58 individuals with normal skin. After careful removal of all subcutaneous fatty tissue, the skin cores were weighed and their water and hydroxyproline content determined. Despite recent claims to the contrary, it was found that there is a marked and highly significant increase in the thickness of the skin during the indurative phase of PSS, and that this is associated with a proportionate increase in total dermal collagen content. A similar degree of thickening was found in the skin of patients with eosinophilic fasciitis and acromegaly. A close correlation was observed between clinical estimation of the degree of skin thickening and the weight of the skin biopsy cores. Change in the weight of skin cores was observed during the course of illness of the patients with PSS and may serve as a useful measurement of alteration in the degree of skin thickening.