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PubMed1991

Antinuclear antibodies in systemic sclerosis. Clinical and ethnic associations.

Antinuclear antibodies were detected, using a sensitive immunofluorescence test, in 96% of 160 patients with systemic sclerosis. Analysis of the patterns of staining revealed that a speckled pattern was commonest (66%), followed by nucleolar staining (37%). In 15% these two co-existed, and in 26% the speckling was of the fine, grainy type, indicative of Scl 70 antibody. Centromere antibody was found in 12%. Black patients made up 54% of the series, and centromere antibody was notably absent from this group, which also had more Scl 70 and nucleolar antibodies than did Indian patients. These serological findings correspond with the clinical features, there being far fewer blacks than Indians with limited cutaneous involvement. The ethnic differences found suggest a genetic influence on the expression of the disease and its auto-antibodies.

Adolescent↗
PubMed1988

Esophageal scintigraphy: applications and limitations in the study of esophageal disorders.

This study examines the scintigraphic transit pattern in a variety of esophageal disorders. Scintigraphy was performed with a semi solid bolus and the patient in an upright position. Condensed esophageal images were obtained from which we derived the esophageal transit time. The pattern of bolus transit was graded by the duration of transit and by the presence of hold up or retrograde motion. Scintigrams were performed in 11 volunteers and 88 patients whose esophageal function had been confirmed by conventional gastroesophageal techniques. Esophageal disorders examined included achalasia (20), scleroderma (9), esophageal carcinoma (8), Barrett esophagus (5), and reflux esophagitis (27). We also examined the effects of gastroesophageal surgery on esophageal function. Transit times distinguished grossly abnormal esophageal function from normal but did not distinguish between different esophageal disorders. Graded transit patterns were a more sensitive indicator of esophageal function and permitted some differentiation between esophageal disorders and allowed evaluation of the effects of gastroesophageal surgery.

Esophageal Diseases↗
PubMed2002

Scleroderma 'en coup de sabre' and progressive facial hemiatrophy. Is it possible to differentiate them?

The aim was to be able to evaluate the diagnosis of two diseases by a consensus of clinical opinion used in the Department of Dermatology of the National Institute of Paediatrics in Mexico City. To differentiate between scleroderma 'en coup de sabre' (SCS) and progressive facial hemiatrophy (PFH), colour slides of 13 patients diagnosed as SCS and nine as PFH were examined by two dermatologists and microscopic slides by two pathologists. In both cases, the slides were randomly presented and no clinical information was given. The clinical and histopathological findings were statistically compared with two-tailed tests and alpha = 0.05. Kappa coefficients were obtained to evaluate the concordance between dermatologists, pathologists, and in terms of the consensus diagnosis. The usefulness of photographic assessment is limited by the inability to palpate the consistency of lesions. The most important clinical feature that differentiated both conditions was cutaneous sclerosis present in eight of 13 patients with SCS and in none of the PFH patients (P < 0.005). Other clinical features more frequently found in SCS were cutaneous hyperpigmentation and alopecia. The more frequent clinical features in PFH were total hemifacial involvement and ocular changes. Statistically significant histopathological features were: connective tissue fibrosis present in all cases with SCS and two of nine patients with PFH (P < 0.0002); adnexal atrophy present in 11 of 13 patients with SCS, and in three of nine with PFH (P < 0.02), and mononuclear cell infiltrates in all patients with SCS cf. six with PFH (P < 0.05). Our results suggest that in most cases it is possible to differentiate SCS from PFH based on clinicopathological findings.

Adolescent↗
PubMed1991

Current options for the treatment of systemic scleroderma.

The epidemiology, pathology, diagnosis, and clinical manifestations of systemic scleroderma (SSc) are described, and therapeutic options are discussed. SSc is a rare condition of unknown etiology that occurs in a subset of scleroderma patients. It is distinguished by involvement of the small arteries, microvessels, and diffuse connective tissue. The degree of internal organ involvement is the main determinant of morbidity and mortality. Management of SSc may entail supportive, palliative, remittive, and immunosuppressive therapies. Supportive therapy involves maintaining the affected extremities at warm temperatures and the use of emollient creams. Results of palliative treatment are mixed. Toxic reactions may be associated with many of these medications. Dermatologic manifestations have been treated with nonsteroidal anti-inflammatory agents, low-dose corticosteroids, dimethyl sulfoxide, and edetate disodium; peripheral and internal-organ vascular obstruction, with alpha-adrenergic blockers, angiotensin-converting-enzyme inhibitors, and calcium-channel blockers. Antacids with alginic acid, histamine H2-receptor antagonists, sucralfate, and cholinergic-acting agents may be used to relieve GI symptoms. Lung infections should be treated promptly with antibiotics. No drug therapy has been successful in reducing the incidence of fatal cardiac arrhythmias or in preventing cardiac fibrosis. Captopril and enalapril are essential in the control of SSc renal crisis. Penicillamine may hold promise as a remittive therapy. The immunosuppressive agents fluorouracil, cyclosporine, and methotrexate, which have shown limited effectiveness in preliminary studies, merit further investigation. No therapeutic agent has yet been shown to alter the course of SSc on a consistent or long-term basis. Toxicity and drug interactions remain a major concern in patient management, and aggressive monitoring is essential.

Adult↗
PubMedDate not supplied

[Study of bone density in systemic scleroderma].

BACKGROUND: Osteopenia in systemic sclerosis (scleroderma) patients was reported in X-ray studies of hands and by proximal and distal forearm bone mass measurement. It has been suggested that bone loss in these patients might be due to chronic ischemia, immobilization and early menopause. Nevertheless, it is not established if these patients present generalized osteopenia. To shed light into this point we studied bone mineral density in the spine, proximal femur and total body in patients with systemic sclerosis. PATIENTS AND METHOD: Twenty-five Caucasian women were evaluated. Mean age of patients was 48 +/- 12 years and mean time of disease was 7 +/- 7 years; 13 were postmenopausal (8 +/- 8 years). Bone mass was measured in the spine, proximal femur and total body by using a dual-photon absorptiometry with X rays source (Lunar-Model DPX). RESULTS: Bone mass in different sites was not statistically different from the age-matched control healthy women. Mean bone mass of patients with limited form was not different from patients with diffuse form of systemic sclerosis. Patients with calcinosis had lower bone mass at proximal femur than those without this alteration. CONCLUSIONS: Patients with systemic sclerosis do not present bone loss and this disease in not a risk factor for generalized osteoporosis.

Adult↗
PubMedDate not supplied

Bronchiectasis in systemic sclerosis. A study using high resolution computed tomography.

OBJECTIVE: To detect noninvasively the presence of bronchiectasis in patients with systemic sclerosis (SSc), through the use of high resolution chest computed tomography (HRCT). METHODS: Twenty two patients with SSc, of whom 13 with diffuse and 9 with limited disease, besides a complete history, physical and routine laboratory and immunologic profile, were evaluated by pulmonary function testing and HRCT. The chi square test with Yates' correction, the Fisher's exact test, the Fisher's test (F test) and the "t" test were used for statistical analysis of the results. RESULTS: Eleven patients (50.0%) had decreased carbon monoxide diffusing lung capacity (DLCO) and, out of these, four had restrictive lung disease, based on a combined decrease of forced vital capacity (FVC) and total lung capacity (TLC). Another two patients exhibited this pattern without DLCO impairment. HRCT revealed a ground glass picture in 15 patients (68.2%), fibrosis in 9 (40.9%) (of which 5 with ground glass as well), and cylindrical bronchiectasis in 13 (59.1%). Bronchiectasis was more common in diffuse than in limited SSc, and the difference approached but did not reach the level of statistical significance. On the other hand, it was not correlated with either decreased DLCO, presence of ground glass and fibrosis, or with patients' age and disease duration. CONCLUSION: Although the number of patients included in our study is relatively small, our data, for the first time in the literature, indicate a significant association between scleroderma and bronchiectasis. Bronchiectasis should be included in the list of pulmonary manifestations of SSc, and SSc in the list of conditions causing bronchiectasis.

Adult↗
PubMed1992

Anticentromere antibody as a predictor of digital ischemic loss in patients with systemic sclerosis.

OBJECTIVE: To determine the clinical and serologic risk factors for digital ischemic events in patients with systemic sclerosis (SSc). METHODS: Retrospective review of clinical and laboratory data and review of current clinical status of 98 patients with SSc, seen between 1985 and 1990. RESULTS: Amputation of 1 or more digits due to ischemia occurred in 20.4% of the patients; 9.2% had multiple digit loss. Sclerodactyly alone and anticentromere antibody (ACA) were associated with loss of 1 or more digits. Age, smoking status, duration of disease, or duration of Raynaud's phenomenon were not predictive for loss of digits. CONCLUSION: Patients with limited SSc who are positive for ACA have an increased risk of major peripheral vascular occlusive disease.

Adult↗
PubMed2004

Resting and exercise physiology in interstitial lung diseases.

Interstitial lung diseases (ILDs) are a heterogeneous group of parenchymal pulmonary disorders with varying histologic appearances. Pulmonary function tests have gained a prominent role in the diagnosis and management of patients with these disorders. Although their role in the differential diagnosis of the various ILDs is limited, physiologic measurements are pivotal studies providing clues regarding disease severity, prognosis,and response to therapy.

Diagnosis, Differential↗
PubMed2002

Anorectal involvement is frequent in limited systemic sclerosis.

The gastrointestinal tract, particularly the oesophagus, is affected in about half of all patients with systemic sclerosis. Only a few studies so far have dealt with the anorectal tract. We studied the anal function using anorectal manometry in 12 patients with limited systemic sclerosis. We also studied the oesophageal function. For the oesophagus, we measured the difference between intragastric and oesophageal pressure, while for the anorectal tract we investigated the maximum resting pressure, the maximum voluntary squeeze effort and the rectoanal inhibitory reflex. Maximum resting pressure and maximum voluntary squeeze effort were found to be decreased in all patients. The rectoanal inhibitory reflex was abnormal in four patients. Statistical analysis showed a significant correlation between maximum resting pressure and maximum voluntary squeeze effort. No correlation was found between oesophageal and anorectal involvement. Anorectal dysfunction is common in patients with limited systemic sclerosis. We suggest that these patients should have an evaluation of their anorectal function including anorectal manometry.

Anal Canal↗
PubMed2006

Bosentan for severe pulmonary arterial hypertension related to systemic sclerosis with interstitial lung disease.

BACKGROUND: The oral dual endothelin (ET) antagonist bosentan has been established as a cornerstone in the treatment of pulmonary arterial hypertension (PAH). ET is believed to be a key pathogenic mediator in systemic sclerosis (scleroderma, SSc), causing fibrotic, hypertrophic and inflammatory processes. PAH is one of the resulting deleterious effects in approximately 15% of SSc patients. MATERIALS AND METHODS: This was an open-label prospective observational study of 8 patients aged 34-73 years with symptomatic, severe PAH related to SSc (WHO class III or IV) and mostly, lung fibrosis. PAH diagnosis was ascertained with echocardiography or right heart catheterization. Patients were treated on top of diuretics and anticoagulants with bosentan 62.5 mg twice daily for 4 weeks followed by a maintenance dose of 125 mg twice daily. RESULTS: The mean 6-minute walk distance (data available in 7 patients) increased from 71.9 (+/- 54.7) m at baseline to 191.9 (+/- 104.6) m after 3 months (P = 0.012) and to 202.6 (+/- 108.1) m after 6 months (P = 0.011), respectively. Six of 8 patients improved in the 6-minute walk test, and these 6 patients also improved in World Health Organization functional class. Two patients did not sufficiently respond to bosentan therapy; one of them died. The tolerability of bosentan was good, and there were no discontinuations. Elevations of hepatic aminotransferases above 3 times the upper limit of normal were not recorded. CONCLUSION: Bosentan treatment was well tolerated in this cohort of SSc patients with interstitial lung disease and was effective for treatment of severe PAH in the majority of patients.

Adult↗
PubMed1995

Small bowel bacterial overgrowth in systemic sclerosis: detection using direct and indirect methods and treatment outcome.

Twenty-four patients with proven systemic sclerosis and with symptoms suggestive of malabsorption (i.e. chronic diarrhoea and weight loss) were investigated for small bowel bacterial overgrowth. Of the patients selected, six were suffering from the diffuse form of the disease. Jejunal aspiration was performed in all patients, and in nine normal volunteers. A specially designed double-lumen sterile catheter was used for this purpose and was introduced via a gastroscope. Twenty of these patients underwent a glucose hydrogen breath test. Eight patients (33%) had significant bacterial counts: > 10(5) colony forming units per ml (cfu/ml) of jejunal fluid. Less than 10(2) cfu/ml were found in the jejunal fluid from the nine control subjects. Glucose hydrogen breath testing was positive in seven patients, all of whom had significant jejunal bacterial growth. Diarrhoea rather than weight loss was shown to be the symptom which correlated best with the presence of bacterial overgrowth. Ciprofloxacin was used in six patients whose diarrhoeal symptoms ceased dramatically within 48 h of commencing the antibiotic. Trimethoprim produced a partial response despite bacterial sensitivity. A disadvantage of the hydrogen breath test is that subsequent antibacterial therapy cannot be specific, as bacterial species, antibiotic sensitivity and resistance are unknown. Systemic sclerosis involving the small intestine in the past has been said to more prevalent in patients with diffuse disease, whereas this study showed a preponderance of patients with long-standing limited cutaneous systemic sclerosis and small bowel involvement.

Adult↗
PubMedDate not supplied

Stiff-skin syndrome.

It is the purpose of this paper to present the case of a patient who came to our observation because of the occurrence of non-elastic hardening of the skin (stone hard skin) and of the subcutaneous tissue that began during early childhood. The parents reported having observed the gradual functional limitation of numerous joints with asymmetrical distribution, more evident in the elbow and the right scapulohumeral joint. There were no signs of involvement of the internal organs; hematologic testing was normal. Described for the first time in 1971 by Esterly nd McKusik and called stiff skin syndrome (SSS), this is a rare genetic alteration characterized by hardening of the skin and subcutaneous tissue associated with gradual joint stiffness and, at times, hirsutism. Generally, there are no modifications in the internal organs or in the musculoskeletal structures; anomalies of the immune system are not described. Modifications are manifested during early childhood causing gradual functional limitation of the hips and knees, with consequent difficulty related to posture and walking. Deficit in the upper limbs is less important. The literature describes about 25 cases with little incidence of familiarity; etiopathogenesis is unknown.

Adolescent↗
PubMed2006

Clinical and serological characteristics of progressive facial hemiatrophy: a case series of 12 patients.

BACKGROUND: Progressive facial hemiatrophy (PFH) is characterized by a slowly progressive atrophy of soft tissues and in some cases bony structures. Coexisting features of localized scleroderma (LS) are commonly observed, indicating the close nature of both disorders. OBJECTIVE: We sought to investigate clinical, serological, and radiographic findings in PFH from 278 patients with LS and to discuss the relationship to linear scleroderma en coup de sabre (LSCS). METHOD: A total of 12 patients with PFH were retrospectively evaluated on the basis of clinical, serological, and radiographic findings. RESULTS: Five patients (42%) presented with complete PFH, and 7 patients (58%) with partial PFH involving either cheek or forehead. Five of the patients (42%) had a coexisting LSCS lesion, and 3 of them (25%) had concomitant LS of the trunk. Intraoral involvement was present in 6 cases. Neurological involvement was common, in particular, epileptic seizures. Serologic investigations showed neither evidence for infection with Borrelia burgdorferi nor any other indication of underlying systemic immunological disorders. LIMITATIONS: There were a relatively small number of patients in a retrospective study. CONCLUSION: The reported cases suggest a close relationship between PFH and LSCS. PFH might appear as two different subtypes, one involving cutaneous structures and presenting with clinical features similar to LSCS, one being strictly restricted to subcutaneous structures, primarily affecting the cheek area. The manifold clinical features of central nervous system involvement indicate the pathogenetic importance of neurological involvement in the development of PFH. Magnetic resonance imaging should be included in the tools of standard diagnostic procedures in patients with PFH. The etiologic relevance of autoimmunity as well as preceding trauma should be investigated in larger collective studies.

Adolescent↗
PubMed2004

Involvement of skeletal muscle in dialysis-associated systemic fibrosis (nephrogenic fibrosing dermopathy).

Nephrogenic fibrosing dermopathy (NFD), a newly recognized scleroderma-like disease, was originally described as a purely cutaneous disorder. More widespread involvement, including fibrosis of pulmonary and cardiac tissues, has been documented only recently, and it has been suggested that a more appropriate designation is dialysis-associated systemic fibrosis. We report five cases of this novel disorder, spanning a spectrum of primarily skin to primarily muscle involvement. Clinical, radiological, electrophysiological, and pathological studies revealed moderate to severe fibrosis of striated muscles. All patients had end-stage renal failure on chronic dialysis, subacute to chronic hardening of the skin and muscles, restriction of limb movements with joint contractures, but normal to only mildly weak muscle strength. Limitation of movements was caused predominantly by skin tightness and induration, and by joint contractures rather than muscle weakness. Computerized tomography showed fibrosis of the fascia and muscles in the most severely affected patients, and electromyography showed mild to severe myopathic changes. Histopathology of affected muscles revealed a spectrum of mild to severe fibrosis, degenerating fibers, and chronic inflammatory cells. These results further support the contention that NFD is not a purely cutaneous disease, but is part of a larger systemic fibrotic process that may involve muscles.

Adult↗
PubMed1991

Altered T cell subpopulations and lymphocytes expressing natural killer cell phenotypes in patients with progressive systemic sclerosis.

Scleroderma (progressive systemic sclerosis [PSS]) is known to be associated with abnormal T cell immunoregulation. In the present study, we evaluated lymphocyte phenotypes in patients with PSS and normal control subjects by flow cytometry and monoclonal antibodies for total T (CD3), T suppressor (CD8), T helper (CD4), T helper-inducer (CDw29), T suppressor-inducer (CD45R), human leukocyte antigen, DR+B (CD19), DR+T, and natural killer subsets, HNK-1 (CD57) and NKH-1 (CD56) cells. Patients with PSS compared to normal subjects had significantly lower percentages of CD3+ (p less than 0.005) and CD8+ (p less than 0.05) (similar to several patients with rheumatoid arthritis also evaluated), as well as CD45R (p less than 0.05), T+DR+ (p less than 0.05), and NKH-1 (CD56) (p less than 0.0005) cells. Patients with PSS with late-limited or generalized disease had lower percentages of CD8+, CD19, NKH-1+, and CDw29, but higher percentages of CD4+, HNK-1, and CD45R cells compared to patients with early stage disease, but these results were not statistically significant. These unique alterations in patients with PSS may prove to be useful in monitoring the stage of disease activity for therapy and further define immunologic defects.

Adult↗
PubMed1991

Cervical esophageal motility: evaluation with US in progressive systemic sclerosis.

High-resolution ultrasound (US) showed that initial peristalsis propelled ingested soda smoothly and rapidly in 20 volunteers without symptoms who met both manometric and radionuclide esophageal scintigraphic (RES) criteria for normal motility. Twenty-eight patients with progressive systemic sclerosis were classified according to results of RES as follows: group 1, normal esophageal motility (three patients [11%]); group 2, hypomotility of the esophagus, excluding the cervical esophagus (18 patients [64%]); and group 3, hypomotility of the cervical esophagus (seven patients [25%]). In the seven patients of group 3, US demonstrated that an incomplete peristalsis sequence or a feeble peristalsis propelled the soda in a slow and/or to-and-fro motion with low velocities. In the other 21 patients (75%), the soda passed through the esophagus smoothly and rapidly. Retention of soda in the cervical esophagus was not limited to patients with hypomotility of the cervical esophagus. It is concluded that US is useful in evaluation of cervical esophageal motility.

Adult↗
PubMed2001

European multicentre study to define disease activity criteria for systemic sclerosis. I. Clinical and epidemiological features of 290 patients from 19 centres.

OBJECTIVE: To investigate the existence of differences among European referral centres for systemic sclerosis (SSc) in the pattern of attendance and referral and in the clinical and therapeutical approaches. METHODS: In 1995 the European Scleroderma Study Group initiated a multicentre prospective one year study whose aim was to define the disease activity criteria in SSc. During the study period each participating European centre was asked to enroll consecutive patients satisfying American College of Rheumatology criteria for SSc and to fill out for each of them a standardised clinical chart. Patients from various centres were compared and differences in epidemiological, clinical, and therapeutical aspects were analysed. RESULTS: Nineteen different medical research centres consecutively recruited 290 patients. The patients could be divided into two subgroups: 173 with the limited (lSSc) and 117 with the diffuse (dSSc) form of the disease. The clinical and serological findings for the series of 290 patients seemed to be similar to data previously reported. However, when the data were analysed to elicit any differences between the participating centres, a high degree of variability emerged, in both epidemiological and clinical features and in the diagnostic and therapeutic approaches to the disease. CONCLUSIONS: The clinical approach to SSc, not only in different countries but also in different centres within the same country, is not yet standardised. To overcome this problem, it will be necessary for the scientific community to draw up a standardised procedure for the management of patients with SSc. This would provide a common research tool for different centres engaged in research on this complex disease.

Adolescent↗
PubMed1999

Toward a multidimensional Health Assessment Questionnaire (MDHAQ): assessment of advanced activities of daily living and psychological status in the patient-friendly health assessment questionnaire format.

OBJECTIVE: To develop components of a multidimensional Health Assessment Questionnaire (MDHAQ) through the addition of new items in the "patient-friendly" HAQ format, including advanced activities of daily living (ADL), designed to overcome "floor effects" of the HAQ and modified HAQ (MHAQ) in which patients may report normal scores although they experience meaningful functional limitations, and psychological items, designed to screen efficiently for psychological distress in routine care. METHODS: The new MDHAQ items, as well as scales for pain, fatigue, helplessness, and global health status on a 2-page questionnaire, were completed by 688 consecutive patients with various rheumatic diseases, including 162 with rheumatoid arthritis (RA), 114 with fibromyalgia, 63 with osteoarthritis, 34 with systemic lupus erythematosus, 20 with vasculitis, 18 with psoriatic arthritis, 16 with scleroderma, and 261 with various other rheumatic diseases, over 2 years at a weekly academic rheumatology clinic. RESULTS: The new MDHAQ items have good test-retest reliability and face validity. MHAQ scores were highest in patients with RA, and scores for other scales were highest in patients with fibromyalgia. On the advanced ADL, 58% of patients reported difficulty with errands, 68% with climbing stairs, 79% with walking two miles, 87% with participating in sports and games, and 94% with running or jogging two miles. On the psychological items, 75% of patients reported difficulty with sleep, 63% with stress, 61% with anxiety, and 57% with depression. Normal MHAQ scores were reported by 23% of patients and normal HAQ scores by 16% of patients who completed these questionnaires, while fewer than 5% had normal scores on the MDHAQ. CONCLUSION: The MDHAQ items overcome in large part the "floor effects" seen on the HAQ and MHAQ, and are useful to screen for problems with sleep, stress, anxiety, and depression in the "patient-friendly" HAQ format. These data support the value of completion of a simple 2-page patient questionnaire by each patient at each visit to a rheumatologist.

Female↗