Corrugated mucosal pattern in the esophagus associated with progressive systemic sclerosis

Three cases of unusual corrugated mucosal pattern in the esophagus accompanying the typical motility disturbance of progressive systemic sclerosis (PSS) are reported. The changes were rarely seen in a group of patients with esophageal involvement by PSS.     

Conjugal progressive systemic sclerosis

INTRODUCTION: Familial occurrence of progressive systemic sclerosis is unusual. The occurrence of conjugal scleroderma is exceptional. EXEGESIS: We report here a case of systemic sclerosis in a wife and husband who both developed the onset of illness within a 10-year period. Solvent exposure was noted. CONCLUSION: The etiology of systemic sclerosis remains unknown. Environmental factors may play role in its pathogenesis.     

The arthropathy of advanced progressive systemic sclerosis

The peripheral joint radiographs of 55 carefully selected patients with classic progressive systemic sclerosis (PSS) and 10 patients with CREST syndrome were reviewed in a longitudinal fashion. Thirty-three PSS and 7 CREST patients were available for followup (mean followup period 28.7 and 29.8 months, respectively). The high frequency of conventionally recognized abnormalities such as flexion contractures, digital tuft resorption, sclerodactyly, and subcutaneous calcinosis was reconfirmed. Additional hand findings included joint space narrowing (13%), juxtaarticular demineralization (4%), ankylosis (2%), marginal erosions (9%), and previously undescribed dorsal erosions (15%). All PSS findings showed progression, although isolated reversibility was noted. The CREST group showed a similar frequency and distribution of findings but with less tendency to progression. With the exception of an increased active joint count in PSS patients with erosions, computer-assisted analysis of multiple clinical and laboratory variables showed no correlation with any radiographic abnormality. These results confirm the presence of an erosive arthropathy in PSS not attributable to overlap with either rheumatoid arthritis or systemic lupus erythematosus.     

Intra-articular calcification in progressive systemic sclerosis

Summary Subcutaneous and periarticular dystrophic calcifications are well known to be associated with progressive systemic sclerosis (PSS). On the other hand, calcifications inside the joints are very rarely reported. We report a new case of this unusual complication of PSS. The observation of the complete radiological follow-up led us to propose a mechanical rather than a purely inflammatory pathogenetic mechanism of this complication.     

Cardiovascular manifestations in progressive systemic sclerosis

We studied 20 consecutive patients with progressive systemic sclerosis from the cardiological point of view through non invasive methods. Sixteen (80%) patients had some kind of cardiovascular complications as shown by any of the used methods. a) Symptoms: fourteen (70%) referred some type of cardiac symptoms. b) Physical examination: eleven (55%) had an abnormal cardiac examination and 10 (50%) had arterial hypertension. c) Electrocardiogram: sixteen (80%) were abnormal. Among them, three cases (15%) had bifascicular block, complication considered up till now as rare. d) Cardiac X Ray Series: Fourteen (70%) were abnormal mainly due to pulmonary fibrosis (55%). e) Echocardiogram: 45% of them showed some kind of abnormality.     

Antinuclear antibodies in progressive systemic sclerosis

Sera from 84 patients with progressive systemic sclerosis (PSS) were tested for the presence of antinuclear antibodies by immunofluorescence on HEp2 cells and gel immunodiffusion. Fluorescent antinuclear antibodies were detected in 80 subjects with PSS (95%). Ninety-three percent of patients with CREST syndrome and 3% of those with diffuse scleroderma had a centromere staining. Precipitating antibodies were found in 57% of PSS sera and identified as anti-Scl 70 in 42 cases (50%). This specificity was found in 42 of 70 subjects with diffuse scleroderma (60%); another patient was positive for anti-nRNP antibodies, and 5 more sera from PSS patients showed precipitin lines of unknown specificity. No serum from 14 patients with CREST syndrome was positive for anti-Scl 70 antibodies. Significant relationships have been found between centromere staining and CREST syndrome (p less than 0.0005) and between the presence of anti-Scl 70 antibodies and the diffuse form of scleroderma (p less than 0.0005). The latter specificity is strongly associated with grainy speckled pattern on HEp2 fluorescence (p less than 0.0005). These data suggest that anti-Scl 70 antibodies and anti-centromere antibodies are useful markers for different subgroups of patients with PSS.     

Secondary amyloidosis in progressive systemic sclerosis

Progressive systemic sclerosis (PSS) is a connective tissue disease that may affect many organs, including the kidneys. It is quite rare to see secondary amyloidosis due to PSS. We present a patient with a 9-year history of PSS who developed nephrotic syndrome, and whose renal biopsy was compatible with secondary amyloidosis. He died from massive upper gastrointestinal bleeding caused by oesophageal telangiectasia. Received: 23 August 2000 / Accepted: 26 February 2001     

系统性红斑狼疮及系统性硬化症的血液学异常分析

目的 :研究系统性红斑狼疮 (SL E)及系统性硬化症 (PSS)的血液学异常情况 ,进一步指导诊断及鉴别诊断。方法 :回顾性分析 6 2例 SL E及 39例 PSS患者的血液学资料 ,率比较采用 χ2 检验 ,均值比较采用 t检验。结果 :以贫血最常见 ,SL E者占 77.4% ,PSS者占 17.9% ,两者比较差异有显著性意义 (P <0 .0 5 )。 SL E者血红蛋白均数显著低于 PSS者。 2 8例 SL E患者行骨髓检查 ,大部分患者骨髓增生活跃或明显活跃 ,主要表现为增生性贫血骨髓像。 18例 SL E患者行抗人球蛋白试验 ,阳性率为 6 6 .7%。结论 :风湿类疾病相关性血液学异常较常见 ,其中以贫血最为多见 ,尤以 SL E者最突出 ;骨髓主要表现为增生性贫血 ;其抗人球蛋白试验阳性率高     

Esophageal disease in progressive systemic sclerosis

Opinion statement Progressive systemic sclerosis (PSS) or scleroderma is characterized by fibrosis of the skin and visceral organs. Gastrointestinal disease occurs in up to 90% of patients, with the esophagus being the most commonly affected organ. Heartburn, dysphagia, and regurgitation occur in most patients. Esophageal manometry aids in diagnosing PSS. Endoscopy rules out complications, such as Barrett’s esophagus, Candida esophagitis, and cancer. Lifestyle modifications should be implemented, including avoidance of alcohol, nicotine, and NSAIDs. Proton pump inhibitor therapy should be instituted, although it is unclear whether the dose should be adjusted according to symptoms or to 24-hour pH monitoring. Prokinetic agents are useful in the early stages of PSS when gastrointestinal musculature is still intact. Metoclopramide improves reflux, lower esophageal sphincter pressure, and gastric emptying but has an inconsistent effect on esophageal peristalsis. A decision on when to perform antireflux surgery, if at all, is controversial. Esophageal disease in PSS is a common and difficult-to-treat problem.     

Digestive involvement in progressive systemic sclerosis

We studied 14 patients with PSS, 12 females and 2 males with a mean age of 43.6 and a medium of 8 years disease. All of the patients were selected for this study according to updated ARA criteria and were included in a prospective protocol to investigate digestive involvement. This protocol consists of a complete medical history, physical examination, radiologic and endoscopic studies, parasitological and microbial flora investigation. The symptoms more frequently seen were: pyrosis (78%), gastroesophageal regurgitation (50%), flatulence (50%), dysphagia (42%) and chronic diarrhea (21%). The radiologic findings commonly seen were: distal esophageal aperistalsis (78%), gastroesophageal reflux (57%), dilatation of intestinal loops (35%), changes of the mucosal folds (35%). A mild esophagitis was seen endoscopically in 64% of the patients, moderate and severe in 7% respectively. The study of the microbial flora showed contaminations with enterobacteria in 5 patients (35%). After statistical analysis we concluded that the digestive compromise by PSS is frequent, being the esophagus more commonly affected (80%), at the beginning in the form of reflux esophagitis and later in esophageal stenosis, the compromise of the small intestine (40%) is manifested by chronic diarrhea or dyspeptic flatulence, which correlates well the radiologic findings and the bacterial overgrowth in this organ. The colonic compromise generally is asymptomatic, and the common finding is dilatation os the colonic loops. Finally, the bacterial overgrowth in the small intestine is a secondary involvement to the intestinal compromise of Progressive Systemic Sclerosis.     

Fibrinogen turnover in progressive systemic sclerosis

Large amounts of fibrin are seen in the intima of the renal arterioles in progressive systemic sclerosis (scleroderma). The half-life or half disappearance time of plasma fibrinogen in 15 patients with scleroderma was studied using 125I fibrinogen to find whether there is an increased turnover of plasma fibrinogen paralleling this morphologic abnormality. Patients had a more rapid fibrinogen turnover than normal controls (60.7 versus 90.6 hours); the subgroup of patients with "progressive" scleroderma had a more rapid fibrinogen half-life than those with "stable" scleroderma (56.5 versus 73.2 hours). The mean fibrinogen half-life of 8 patients given intravenous heparin increased to within one standard deviation of normal, a finding that suggested that the fibrinogen molecule in these patients was capable of normal survival. There was a considerable variation of normal survival. There was a considerable variation of fibrinogen half-lives in individual scleroderma patients over time (not seen in the normal controls) which may be the result of intermittently increased fibrinogen consumption.     

Anorectal abnormalities in progressive systemic sclerosis

Seventeen patients with progressive systemic sclerosis (PSS) were evaluated with manometry for anorectal function, and an additional 36 age-matched normal subjects were collected as a control group. The study group had a significant decrement of maximum basal pressure (MBP), 42.6±27.0 mm Hg, in PSS as compared with the control group, 71.2±24.9 mm Hg (P=.0004). The difference in the functional length (FL) of the anal canal, PSS∶control=2.4±1.0 cm∶3.7±0.5 cm (P=.0001); the volume of first defecating sensation, PSS∶control=66.3 ±35.2 ml∶125.1±43.8 ml; the voluntary component, the difference between maximum squeeze pressure (MSP) and MBP, PSS∶control=116.6±73.6 mm Hg∶61.8±35.9 mm Hg (P=.0087), were also found to be statistically significant. Nevertheless, the MSP and maximal tolerable capacity (Vmax) showed no difference in these two groups (MSP, PSS∶control=159.3±88.1 mm Hg∶132.9±44.9 mm Hg,P=.259), (Vmax, PSS∶control=193.1±67.7 ml∶230.0±60.9 ml,P=.0526), Twelve (71 percent) of 17 patients did not have rectoanal inhibitory reflex, and paradoxical contraction during rectal balloon inflation was noted in ten patients. Nine patients had different degrees of anal incontinence and abnormal anometric profiles were found in six of eight asymptomatic patients. Therefore, only two patients (12 percent) had neither symptoms nor anometric evidence of anorectal involvement in PSS. Two patients with long-standing disease received posterior anal repair for stool incontinence, the postoperative results were satisfactory both subjectively and objectively. The average MBP increased from 0 to 20 mm Hg, average FL from 0 to 1.5 cm. Patients complained less frequently about stool incontinence or soiling, and their daily life is now more comfortable. The analysis indicates that anorectal function in PSS is affected much more frequently and earlier than thought. Anorectal manometry can be used as an adjuvant in diagnosing controversial cases. Once anal incontinence occurs, posterior anal repair can achieve good results after six months of follow-up.