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PubMed1996

Psoriasiform and sclerodermoid dermatitis of the fingers with apparent shortening of the nail plate: a distinct entity?

A Caucasian boy, born to unrelated parents, and with no family history of psoriasis or atopy, developed chronic psoriasiform dermatitis on the palmar surface of the fingers when he was a year old. At the age of 6, the dorsal surface acquired a sclerodermoid appearance and the nail plates appeared to be shortened. The disease was unsuccessfully treated topically with emollients, keratolytic agents and corticosteroids. At the age of 9, when he was hospitalized, finger motility was limited and painful, and movements of the hands were impaired. The feet were normal. All the investigations were within normal limits. Histology of a biopsy revealed parakeratosis, acanthosis with papillomatosis, exocytosis and spongiosis. He was treated with a new oral non-steroidal anti-inflammatory drug, nimesulide (100 mg/day for 20 days, and then 50 mg/day for 20 days), and emollients. Marked improvement, and restoration of finger motility, were achieved after the first month of treatment; the nail plates reappeared because of regression of the eponychium.

Anti-Inflammatory Agents, Non-Steroidal↗
PubMed2004

[Patient-centered assessment of functional health in systemic sclerosis -- where are we now?].

Self-administered patient-centered questionnaires have been shown to be practical, reliable and valid in terms of evaluating functional limitations in rheumatic diseases. In systemic sclerosis a modified version of the HAQ and condition-specific questionnaires have been used. The Health Assessment Questionnaire (HAQ) does not comprehensively cover functional limitations in patients with systemic sclerosis. Visual Analogue Scales added to the modified HAQ reflect general and organ-specific symptoms only to some extent. The Self-administered Systemic Sclerosis Questionnaire (SySQ) includes general, organ-specific and musculoskeletal symptoms with a focus on functional limitations of the upper and lower extremities. The SySQ has not been examined longitudinally nor has it been validated cross-culturally. WHO's International Classification of Functioning, Disability and Health (ICF) could serve as a future reference framework and common language in terms of the design of new disease-specific, patient-centered, comprehensive questionnaires for systemic sclerosis as well as in the further improvement of established questionnaires.

Activities of Daily Living↗
PubMed2000

Increased nitric oxide production in patients with systemic sclerosis.

Nitric oxide (NO, nitrogen monoxide) is a messenger molecule whose synthesis can be induced by proinflammatory cytokines. Increased production of NO has been reported in various inflammatory and autoimmune diseases. We studied serum nitrite and citrulline as surrogate markers for NO production in patients with systemic sclerosis (SSc) and looked for correlation with extent of disease, disease duration, age, and systemic involvement. Thirty-four patients were studied against 20 controls. The nitrite levels were significantly higher in the disease group (1588.4 +/- 998.2 nmol/ml compared to 327.8 +/- 137.7 nmol/ml; P < 0.001). The citrulline levels of the disease group were also significantly higher (5490.1 +/- 2518.3 nmol/ml compared to 3264.5 +/- 2509.7 nmol/ml in the controls; P = 0.005). There was no significant difference among limited and diffuse subgroups. There was no significant difference in patients with or without arthritis or interstitial lung disease or with other systemic involvement. On multivariate analysis there was a trend toward a rising level of nitrite with worsening lung functions (P = 0.07). Hence, there is evidence of increased NO production in patients with SSc. There is no difference between NO levels in disease subgroups or those with systemic involvement.

Biomarkers↗
PubMed2002

Autoantibodies to NOR 90/hUBF: longterm clinical and serological followup in a patient with limited systemic sclerosis suggests an antigen driven immune response.

We describe the clinical and serological followup of a 9-year-old girl with anti-nucleolar organizing region 90/human upstream-binding factor (anti-NOR 90/hUBF) who had features of systemic sclerosis over a period of 17 years, from childhood into adulthood. We review the associations of anti-UBF autoantibodies, and provide evidence that anti-NOR 90/UBF immune response is antigen driven.

Autoantibodies↗
PubMed2006

[High-resolution ultrasonography: utility in diagnosis, treatment, and monitoring dermatologic diseases].

Although less precise than histological examination, high-resolution skin imaging is a noninvasive technique that provides complementary information to the clinical examination: the lesion's US structure as well as its lateral and depth extension. Ultrasonography helps in diagnosis and initial management by measuring melanoma thickness (so as to remove this lesion with safety margins) before destructive treatment (cryosurgery or radiotherapy), by showing tumor limits, or by detecting subclinical recurrence during the follow-up. In this paper, ultrasound images will be correlated with clinical and histological data in various clinical situations such as tumors, edema, cutaneous infiltration, sclerous tissues, hyperlaxity, and cutaneous aging.

Adolescent↗
PubMed2006

Vasoactive therapies in systemic sclerosis.

In systemic sclerosis (SSc), vasculopathy is a central mechanism and is a major initial event in the process of sclerosis and causing different complications such as Raynaud's phenomenon, ulcer(s) or pulmonary hypertension, the latter being life threatening. Therefore, vasoactive therapies are important when taking care of patients with SSc. However, as treatment has been difficult, numerous therapeutic modalities have been suggested. Until now, the interpretation of most studies is limited due to the heterogeneity of patient groups, the low number of patients, the short duration of the treatments and, possibly, further pathogenic mechanisms such as autoimmunity. Several drugs are now available with effects on vasculopathy and, furthermore, on specific pathogenic mechanisms in SSc. Prostacyclins, endothelin receptor antagonists and phosphodiesterase-5 inhibitors have potential effects on fibrosis, inflammation and endothelial cells, suggesting a disease-modifying capacity in systemic sclerosis. This review summarizes evidence-based therapy recommendations.

Endothelin Receptor Antagonists↗
PubMed2002

Measuring disease activity and functional status in patients with scleroderma and Raynaud's phenomenon.

OBJECTIVE: To document disease activity and functional status in patients with scleroderma (systemic sclerosis [SSc]) and Raynaud's phenomenon (RP) and to determine the sensitivity to change, reliability, ease of use, and validity of various outcome measures in these patients. METHODS: Patients with SSc and moderate-to-severe RP participating in a multicenter RP treatment trial completed daily diaries documenting the frequency and duration of RP attacks and recorded a daily Raynaud's Condition Score (RCS). Mean scores for the 2-week periods prior to baseline (week 0), end of trial (week 6), and posttrial followup (week 12) were calculated. At weeks 0, 6, and 12, physicians completed 3 global assessment scales and performed clinical assessments of digital ulcers and infarcts; patients completed the Health Assessment Questionnaire (HAQ), the Arthritis Impact Measurement Scales 2 (AIMS2) mood and tension subscales, 5 specific SSc/RP-related visual analog scales (VAS), and 3 other VAS global assessments. We used these measures to document baseline disease activity and to assess their construct validity, sensitivity to change, and reliability in trial data. RESULTS: Two hundred eighty-one patients (248 women, 33 men; mean age 50.4 years [range 18-82 years]) from 14 centers participated. Forty-eight percent had limited cutaneous SSc; 52% had diffuse cutaneous SSc. Fifty-nine patients (21%) had digital ulcers at baseline. Patients had 3.89 +/- 2.33 (mean +/- SD) daily RP attacks (range 0.8-14.6), with a duration of 82.1 +/- 91.6 minutes/attack. RCS for RP activity (possible range 0-10) was 4.30 +/- 1.92. HAQ scores (0-3 scale) indicated substantial disability at baseline (total disability 0.86, pain 1.19), especially among the subscales pertaining to hand function (grip, eating, dressing). AIMS2 mood and tension scores were fairly high, as were many of the VAS scores. Patients with digital ulcers had worse RCS, pain, HAQ disability (overall, grip, eating, and dressing), physician's global assessment, and tension, but no significant difference in the frequency of RP, duration of RP, patient's global assessment, or mood, compared with patients without digital ulcers. VAS scores for digital ulcers as rated by the patients were not consistent with the physician's ratings. Factor analysis of the 18 measures showed strong associations among variables in 4 distinct domains: disease activity, RP measures, digital ulcer measures, and mood/tension. Reliability of the RCS, HAQ pain and disability scales, and AIMS2 mood and tension subscales was high. The RP measures demonstrated good sensitivity to change (effect sizes 0.33-0.76). CONCLUSION: Our findings demonstrate that the significant activity, disability, pain, and psychological impact of RP and digital ulcers in SSc can be measured by a small set of valid and reliable outcome measures. These outcome measures provide information beyond the quantitative metrics of RP attacks. We propose a core set of measures for use in clinical trials of RP in SSc patients that includes the RCS, patient and physician VAS ratings of RP activity, a digital ulcer/infarct measure, measures of disability and pain (HAQ), and measures of psychological function (AIMS2).

Affect↗
PubMed2006

Angiotensin-converting enzyme gene insertion/deletion polymorphism in Korean patients with systemic sclerosis.

To determine whether angiotensin-converting enzyme (ACE) gene insertion/deletion (I/D) polymorphism is associated with the development and clinical features of systemic sclerosis (SSc) in Korean, we studied seventy two Korean patients with SSc fulfilling the ACR preliminary classification criteria. The controls were 114 healthy, disease free Koreans. ACE I/D genotypes were determined by PCR method using oligonucleotides. Sixty eight patients (94.4%) were women and age at diagnosis was 43.5+/-12.6 yr old (mean+/-SD). Thirty nine patients (54.2%) had a diffuse type of SSc. There were no statistical differences in the frequencies of all ACE I/D genotypes and D allele between patients and controls, and neither between diffuse and limited types of SSc. ACE I/D gene polymorphism was not associated with the development of SSc in Korea. The investigation for the pathogenesis of SSc requires more studies about the role of other candidate genes such as endothelin, TGF-beta, nitric oxide, or angiotensin II receptor in addition to the ACE genes.

Adult↗
PubMed2002

[New aspects in autoantibody diagnosis in collagen diseases].

Distinct, especially non-organ specific autoantibodies are closely associated with connective tissue diseases and in many cases are vital elements of the laboratory diagnostics of these disorders. Their inclusion into the common classification criteria is quite heterogeneous. None of the autoantibodies is 100% specific for a certain disease, and diagnostic specificity is usually reduced when application of the test is broadened and when more sensitive methods are used. In individual patients with scleroderma and/or myositis related diseases, typical autoantibodies usually exclude each other; however, there are characteristical exceptions from that rule. Evidence is accumulating that autoantibodies are detectable early during disease course and often even in preclinical stages. Variations of antibody levels during disease course are different in different systems and in some cases have been shown to correlate with disease activity. Negative results in sensitive screening assays are often essential to exclude a connective tissue disease. Although it has certain drawbacks and is being disputed, the indirect immunofluorescence assay with HEp-2 cells still serves as a standard first step in connective tissue disease-related antibody detection. In case of positive results in this assay, further steps should be performed carefully, considering the signs and symptoms of the suspected disease as well as the immunofluorescence pattern, and being aware of the peculiarities and limitations of the assay methods used.

Antibody Specificity↗
PubMed2002

Autoantibody to DNA binding protein B as a novel serologic marker in systemic sclerosis.

Systemic sclerosis is a systemic disease that is characterized by tissue fibrosis, small-vessel vasculopathy, and an autoimmune response associated with autoantibodies. We performed serological analysis of cDNA expression library (SEREX) to identify autoantibodies associated with systemic sclerosis. We identified 4 clones that react with sera of patients with SSc but not with those of healthy donors. These clones are phosphoglycerate mutase, centromere autoantigen C, U1 small nuclear ribonucleoprotein, and DNA binding protein B (dbpB). We chose to study autoantibody to DNA binding protein B. Immunoreactivity against recombinant dbpB was detected in 40.5% (15/37) of patients with SSc, 14.6% (6/41) of patents with systemic lupus erythematosus, 6.7% (1/15) of patients with rheumatoid arthritis, 0% (0/12) of patients with Sjogren syndrome, and 5.9% (1/17) of patients with polymyositis/dermatomyositis. The frequency of anti-dbpB was significantly higher in the SSc patients (15/37, 40.5%) compared to the healthy controls (3/41, 7.3%, p=0.0005 by chi(2) test). Eleven patients (11/20, 55%) with the diffuse cutaneous type of SSc had anti-dbpB and 4 patients (4/17, 23.5%) with the limited cutaneous type had anti-dbpB. The presence of anti-dbpB was significantly associated with the diffuse cutaneous type (p=0.00003 by chi(2) test). This is the first report to suggest that autoantibody to dbpB can be used as a serologic marker of systemic sclerosis.

Adult↗
PubMed2004

A study of the prevalence of systemic sclerosis in northeast England.

OBJECTIVES: We aimed to obtain an estimate of the prevalence and demographics of systemic sclerosis (SSc) and its subtypes at the turn of the millennium. METHODS: Case finding from multiple sources from a defined geographical area. Diagnosis confirmed by clinical examination. RESULTS: The crude prevalence of SSc in northeast England was 8.8 (95% CI: 6.8-10.8) per 100,000. The prevalence when adjusted for the entire UK is 8.2 (95% CI: 6.2-9.8) per 100,000. The ratio of women to men was 5.2:1. The median age of patients was 57.1 yr. The ratio of limited cutaneous SSc to diffuse cutaneous SSc was 4.7:1. Limited cutaneous SSc is associated with the presence of anticentromere antibodies; diffuse cutaneous SSc is associated with anti-Scl 70 antibodies, but either antibody was found in either form of SSc. CONCLUSIONS: SSc appears to be more common in northeast England than was found in the West Midlands in 1986. This may reflect changes in the diagnostic definition of SSc.

Adolescent↗
PubMed2003

Clinical improvement in systemic sclerosis resulting from urokinase therapy explained by light and electron microscopy skin examination.

OBJECTIVE: In our previous work, patients affected by SSc were treated with intravenous urokinase and showed clinical improvement. In this study we used microscopy to document ultrastructural alterations occurring in sclerodermic skin from SSc patients treated with urokinase. METHODS: Ten patients with SSc were selected for this study. Skin biopsies were taken from the medial side of the right forearm on the third proximal on the volar surface. The patients were then treated with urokinase for 7 consecutive days. At the end of the treatment, the patients were examined and a new skin biopsy was taken close to the above-mentioned zone of the forearm for optic and electron microscopy examination. RESULTS: The patients showed a gradual improvement of the skin after urokinase treatment. Raynaud's appeared to be less intense, and they had an increased articular range, with the restoration of movements that had previously been limited. Histological findings showed that, after treatment, skin alterations appeared attenuated, in particular the connective tissue showed a decreased density and inflammatory infiltrate was slight. Electron microscopy findings showed that collagen fibres appeared to have a more regular diameter, and the capillary vessels' lining was thicker, with fewer pinocytotic vesicles. CONCLUSION: These observations show that urokinase treatment seems to be an interesting therapeutic strategy to consider for the treatment of SSc.

Adult↗
PubMed1975

Autotransufsion of 3H-cytidine-labelled blood lymphocytes in patients with Hodgkin's disease and non-Hodgkin patients. I. Limitations of the method.

Autotransfusion of 3H-cytidine-labelled blood lymphocytes followed by autoradiographic evaluation as studied in 11 patients with Hodgkin's disease and pool sizes of lymphocytes. However, it is complicated by two major pitfalls; (1) The free (extracellular) activity of oH-cytidine transfused along with the cell-bound label is sufficient to cause DNA labelling of newly produced lymphocytes in the body. (2) The soluble intracellular pool of 3H-labelled compounds causes an increase of labelling intensity of lymphocyte RNA in the first hours after cell transfusion. Methods to correct for these sources of error are described.

Blood Transfusion, Autologous↗
PubMed2006

Radiological hand involvement in systemic sclerosis.

BACKGROUND: The osteoarticular and soft tissue structures of the hand may be involved in systemic sclerosis (SSc), causing functional disability. OBJECTIVE: To assess radiological hand features in a cross sectional study of SSc patients and in controls. METHODS: Hand radiology was done systematically in patients with SSc seen over a two year period and in unselected controls with rheumatoid arthritis or digital trauma. Two independent investigators blind to the diagnosis carried out the radiological assessment. RESULTS: 120 consecutive SSc patients (median (range) age, 56.5 (20 to 90) years; disease duration, 6 (0 to 42) years) and 42 controls (22 with rheumatoid arthritis and 20 with digital trauma) were studied. Radiological abnormalities in SSc patients included erosion (21%), joint space narrowing (28%), arthritis (defined by concomitant erosion and joint space narrowing) (18%), radiological demineralisation (23%), acro-osteolysis (22%), flexion contracture (27%), and calcinosis (23%). In univariate and multivariate analysis, the resorption of distal phalanges was significantly associated with digital ulcers, extra-articular calcification, and pulmonary arterial hypertension; flexion contracture was associated with the diffuse cutaneous form and high HAQ (Health Assessment Questionnaire) disability score. Calcinosis was most often seen in patients with digital ulcers, but was similarly observed in patients with the diffuse or limited cutaneous subtypes. CONCLUSIONS: Flexion contracture was associated with disability and occurred in patients with the diffuse cutaneous subtype of SSc, consistent with the tendency towards fibrosis and functional impairment of this subtype. Calcinosis and acro-osteolysis were both associated with vascular complications, highlighting a potential role of vascular injury in such lesions.

Adult↗
PubMed1996

Twenty-four hour heart period variability in systemic sclerosis.

OBJECTIVE: In patients with systemic sclerosis (SSc), an autonomic dysfunction has been implicated in the pathogenesis of Raynaud's phenomenon and gastrointestinal symptoms. To evaluate cardiovascular autonomic function we analyzed the heart rate variability (HRV) on 24 h electrocardiogram recordings. METHODS: In 50 patients diagnosed with SSc and 24 control subjects HRV was analyzed for both frequency and time domain. RESULTS: Frequency domain analysis showed reduction of low frequency and high frequency values in the patients with SSc compared to controls (5.52 +/- 0.74 vs 6.04 +/- 0.78 In(ms2), p = 0.007; and 4.17 +/- 0.97 vs 4.74 +/- 0.97 In(ms2), p = 0.021, respectively). Analysis in time domain showed no significant differences in any variable between groups. There were no significant differences between the diffuse and the limited form of SSc. Number of ventricular ectopic beats, prevalence of frequent ventricular ectopy, and supraventricular tachycardia episodes were increased in the patients with SSc compared to controls. CONCLUSION: Spectral analysis of heart period variability suggests cardiovascular autonomic dysfunction in patients with SSc. Better understanding of this complication could help delineate new strategies for the treatment of SSc.

Adult↗
PubMed1998

Estimates of the prevalence of arthritis and selected musculoskeletal disorders in the United States.

OBJECTIVE: To provide a single source for the best available estimates of the national prevalence of arthritis in general and of selected musculoskeletal disorders (osteoarthritis, rheumatoid arthritis, juvenile rheumatoid arthritis, the spondylarthropathies, systemic lupus erythematosus, scleroderma, polymyalgia rheumatica/giant cell arteritis, gout, fibromyalgia, and low back pain). METHODS: The National Arthritis Data Workgroup reviewed data from available surveys, such as the National Health and Nutrition Examination Survey series. For overall national estimates, we used surveys based on representative samples. Because data based on national population samples are unavailable for most specific musculoskeletal conditions, we derived data from various smaller survey samples from defined populations. Prevalence estimates from these surveys were linked to 1990 US Bureau of the Census population data to calculate national estimates. We also estimated the expected frequency of arthritis in the year 2020. RESULTS: Current national estimates are provided, with important caveats regarding their interpretation, for self-reported arthritis and selected conditions. An estimated 15% (40 million) of Americans had some form of arthritis in 1995. By the year 2020, an estimated 18.2% (59.4 million) will be affected. CONCLUSION: Given the limitations of the data on which they are based, this report provides the best available prevalence estimates for arthritis and other rheumatic conditions overall, and for selected musculoskeletal disorders, in the US population.

Adolescent↗
PubMed2002

Serum levels of tissue inhibitor of metalloproteinases 2 in systemic sclerosis: a preliminary study.

BACKGROUND: The etiopathogenesis of systemic sclerosis (SSc)--an autoimmunological disease characterized by excess collagen and other connective tissue components in skin and internal organs--remains unclear. Given the potential role of matrix enzymes, metalloproteinases, and their tissue inhibitors in pulmonary fibrosis, we assayed the serum concentration of tissue inhibitor of metalloproteinases 2 (TIMP-2) in patients with SSc and explored its possible correlation with the duration of Raynaud's phenomenon, the intensity of sclerosis of the skin, and pulmonary lesions. MATERIAL/METHODS: We studied 18 SSc patients, 8 with limited SSc and ten with diffuse. The degree of sclerosis of the skin was measured using the Total Skin Scale. Chest x-rays and spirometric examinations were also performed. The presence and type of antinuclear antibodies (ANA) were determined by indirect immunofluorescence and double immunodiffusion in agar gelatin. Serum TIMP-2 concentration was measured by ELISA. RESULTS: The average serum TIMP-2 concentration was not significantly higher in SSc patients than in controls. No statistically significant difference was found in this respect between limited and diffuse SSc. Despite the lack of correlation between the duration of Raynaud's phenomenon, the degree of sclerosis of the skin, and the serum concentration of TIMP-2, the latter concentration was higher in patients with restrictive pulmonary dysfunction in spirometric testing. CONCLUSIONS: Our research, though involving a small group of patients, points to the probable role of TIMP-2 in the development of pulmonary fibrosis.

Adolescent↗
PubMed1995

Reduced bone mass and normal calcium metabolism in systemic sclerosis with and without calcinosis.

Forty-three female patients with systemic sclerosis divided into subgroups based on the extent of skin involvement and the presence of calcinosis, and 50 sex and age-matched healthy controls were investigated for bone mineral density (BMD) on the basis of radial (dual photon absorptiometry, Osteograph, NIM), lumbar, and total body measurements (dual energy X-ray absorptiometry, Lunar DPX, Lunar Corp.), and for parameters of calcium metabolism. The patients showed a lower BMD (mean +/- SD; mg/cm2) than the controls at the radial (313 +/- 69 vs 347 +/- 73; p < 0.005), lumbar (974 +/- 143 vs 1081 +/- 154; p < 0.005), and total body (997 +/- 82 vs 1075 +/- 109; p < 0.05) determinations. The patients with the diffuse form of skin involvement had lower values than those with the limited form. There was a negative correlation between BMD and the duration of the disease. The presence of calcinosis was not found to have any effect on BMD. Calcium metabolism was found to be normal in each subgroup. It may be concluded that generalized osteoporosis is a feature of systemic sclerosis, with and without calcinosis. The extent and duration of the disease may play a role in determining bone loss.

Absorptiometry, Photon↗