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PubMed2004

Association of a functional CD19 polymorphism with susceptibility to systemic sclerosis.

OBJECTIVE: CD19 is overexpressed in B cells from patients with systemic sclerosis (SSc), and plays a crucial role not only for autoantibody production, but also for skin fibrosis in mouse models. We previously reported the association of a GT repeat polymorphism in the 3'-untranslated region (3'-UTR) of CD19 with human systemic lupus erythematosus. In this study, we examined whether CD19 polymorphisms are associated with genetic susceptibility to SSc. METHODS: A case-control association study was performed for CD19 polymorphisms, -499G>T in the promoter region and a GT repeat polymorphism in the 3'-UTR, in 134 patients with SSc and 96 healthy individuals recruited at Kanazawa University. CD19 expression levels in the peripheral blood naive and memory B cells from SSc patients were examined by 2-color flow cytometry. RESULTS: Carrier frequencies of the -499T allele in the promoter (odds ratio [OR] 2.18, 95% confidence interval [95% CI] 1.31-3.86, P = 0.003) and of the (GT)(14) allele in the 3'-UTR (OR 1.86, 95% CI 1.05-3.28, P = 0.03) were significantly increased in SSc patients compared with healthy controls. Association was particularly evident in patients with limited cutaneous SSc with anticentromere antibodies. These alleles were in linkage disequilibrium, but the -499T allele seemed to play a primary role. CD19 expression levels in peripheral blood B cells were significantly elevated in both naive (P = 0.0029) and memory (P = 0.0022) B cells from patients with SSc who had the -499T allele as compared with those without the -499T allele. CONCLUSION: The CD19 -499G>T polymorphism is associated with higher CD19 expression in B cells, and with susceptibility to SSc.

Adult↗
PubMed1984

High frequency ultrasonic imaging of skin: experimental results.

Experimental results are presented demonstrating the application of pulse echo ultrasound to imaging the skin. A laboratory prototype B-mode mechanical scanner was employed to obtain images of human skin, both in vitro and in vivo, using broadband pulsed ultrasound at 25 MHz. Images were formed by processing digitized A-mode waveforms and displaying the resulting two-dimensional cross sections using a digital imaging system. Images obtained by rectifying the A-modes are compared to those derived using a software-based cross-correlation technique. Scans of test targets demonstrate that an axial resolution of 100 m can be achieved at 25 MHz when the digital correlation method is employed. Lateral resolution is limited by the 0.25 mm half-power focal beamwidth of the transducer. Seventeen in vitro ultrasonic scans of human skin were compared to frozen section histology. Average skin depth was well correlated between the two techniques ( = 0.99, p less than 0.001). Application of cross-correlated processing to 25 MHz in vivo images produced good delineation of epidermis, papillary, and reticular dermis. Conversion to a 50 MHz transducer did not delineate skin layers as well as the 25 MHz transducer due to inherent difficulties with transducer reverberations.

Abdomen↗
PubMed2000

Altered diurnal rhythm of prolactin in systemic sclerosis.

OBJECTIVE: Mild hyperprolactinemia has been reported in systemic lupus erythematosus (SLE) and systemic sclerosis (SSc). We investigated whether the elevated serum level of prolactin (Prl) detected in SSc is due to a sustained increase over 24 h and/or a shift in the diurnal rhythm, and whether Prl autoantibodies--originally described in SLE--may interfere in the assay. METHODS: The serum level of Prl was measured by ELISA and compared between 73 patients with SSc and 73 age and sex matched controls (78% women, age 56 +/- 11 years). The diurnal rhythms of Prl and thyrotropin (thyroid stimulating hormone, TSH) were compared between 3 patients with SSc and 10 healthy controls. Blood was taken at 2-3, 6-7, 10-11 a.m., and 2-3, 6-7, 10-11 p.m. The serum level of Prl autoantibodies was measured by ELISA and compared between matched patients with SSc and SLE and controls (n = 42 each). Standard curves of the Prl ELISA were spiked with 10% sera containing high levels of Prl autoantibodies to test interference. RESULTS: Serum levels of Prl measured in the morning (8-10 a.m.) were significantly higher in patients with SSc (17.9 +/- 7.7 ng/ml), compared with controls (9.3 +/- 4.2 ng/ml; p < 0.05). In SSc, 40% of patients had Prl levels > 20 ng/ml, but no correlation was found with Scl-70 or Prl autoantibodies. Younger patients (< 50 years, n = 23/73) showed higher serum levels of Prl than older patients (21.3 +/- 10.3 vs 16.3 +/- 6.2 ng/ml; p < 0.05). The diurnal rhythm of Prl revealed that both a sustained increase over 24 h and some shift occurred in SSc. Peaks of secretion were detected between 6 and 11 a.m., instead of 2-6 a.m. The median levels of TSH over 24 h in patients with SSc ranged within the normal limits. Nevertheless, in SSc, a significant correlation (r = 0.59, p < 0.01) was found between diurnal rhythms of Prl and TSH. The prevalence of Prl autoantibodies in serum was 8% in SSc, 27% in SLE, and < 5% in controls. However, the presence of Prl autoantibodies did not interfere with our assay. CONCLUSION: Our data confirm that mild hyperprolactinemia occurs in a subgroup of patients with SSc, and showed that the elevated serum level of Prl is due to both a sustained increase over 24 h and a shift in the diurnal rhythm. The correlation between diurnal rhythms of Prl and TSH suggests common regulatory mechanisms.

Adult↗
PubMed2005

Digital ulcers in patients with systemic sclerosis.

Digital ulcers (DU), defined as necrotic lesions located at distal digits or overlying bony prominences, occur in up to 50% of patients with limited or diffuse systemic sclerosis (SSc). These lesions are extremely painful and lead to substantial functional disability. The pathogenesis of DU differs depending on their location. DU located at distal aspects of digits are thought to be related to tissue ischemia from several processes, including vasospasm secondary to Raynaud's phenomenon, intimal fibro-proliferation, and thrombosis of digital arteries. DU located over bony prominences, such as the phalangeal joints and elbows, are thought to be due to repetitive microtrauma and difficulty healing due to atrophic, avascular tissue overlying the joints. Management of DU include non-pharmacologic and pharmacologic modalities. This review summarizes the current available and investigational therapies for the treatment and prevention of DU in patients with SSc.

Anticoagulants↗
PubMed2003

A comparison between anti-Th/To- and anticentromere antibody-positive systemic sclerosis patients with limited cutaneous involvement.

OBJECTIVE: To compare anti-Th/To-positive and anticentromere antibody (ACA)-positive patients with limited cutaneous systemic sclerosis (lcSSc). METHODS: We reviewed the medical records of 107 anti-Th/To-positive patients and 365 ACA-positive patients who were first evaluated during 1985-2000. ACA was detected by indirect immunofluorescence on HEp-2 cell substrate, and anti-Th/To was detected by RNA immunoprecipitation with K562 cell extracts. Patients were included if they had a clinical diagnosis of lcSSc, and excluded if they had >1 SSc-associated serum autoantibody. RESULTS: The final study groups comprised 87 lcSSc patients with anti-Th/To antibodies and 306 with ACAs. Anti-Th/To-positive patients were younger (P < 0.04) and had a shorter disease duration at their first evaluation (P < 0.003). Patients with anti-Th/To antibodies had more subtle skin changes, less severe vascular involvement, and less frequent distal esophageal hypomotility. Both groups had a higher frequency of "intrinsic" pulmonary hypertension than has been previously reported in the literature (28% and 19% of anti-Th/To-positive and ACA-positive patients, respectively), perhaps due to referral bias. Patients in the anti-Th/To group more often had radiographic evidence of interstitial lung disease (48% versus 13% of the ACA group; P < 0.0001). Scleroderma renal crisis was uncommon (4 cases), but occurred exclusively in the anti-Th/To group. Survival among the anti-Th/To-positive patients was reduced compared with that in the ACA group (P < 0.02). CONCLUSION: Patients with anti-Th/To and those with ACA most often develop lcSSc and have a high frequency of "intrinsic" pulmonary hypertension. Compared with the ACA patients, anti-Th/To lcSSc patients have more subtle cutaneous, vascular, and gastrointestinal involvement, but more often have certain features typically seen in diffuse scleroderma, such as pulmonary fibrosis and scleroderma renal crisis, as well as reduced survival.

Adult↗
PubMed1998

Analysis of autoantibodies against RNA polymerases using immunoaffinity-purifed RNA polymerase I, II, and III antigen in an enzyme-linked immunosorbent assay.

Autoantibodies against RNA polymerases (RNAP) have been reported to occur in patients with a wide variety of connective tissue diseases (CTD), including systemic sclerosis (SSc), systemic lupus erythematosus (SLE), and mixed connective tissue disease (MCTD). The frequency of anti-RNAP antibodies has been reported to vary widely between different CTD diseases in studies examining different patient populations. Furthermore, these studies have been limited by the fact that methods have not previously been available for detecting antibodies against RNAP which are both rapid and quantitative. We have developed an enzyme-linked immunosorbent assay (ELISA) for rapidly quantitating antibodies against RNAP I, II, and III. We have utilized both the ELISA and the immunoprecipitation of 35S-labeled HeLa cells to analyze sera from a large cohort of well-characterized Caucasian CTD patients for the presence of anti-RNAP antibodies. We found excellent concordance for the presence of anti-RNAP antibodies using immunoprecipitation and ELISA. Anti-RNAP antibodies occurred predominantly among female patients with the diffuse form of SSc and were detected in 8/36 (22%) of Caucasian patients with diffuse SSc and 1/53 (2%) with limited SSc. Anti-RNAP antibodies occurred in 1/42 (2%) of patients with SLE. Anti-RNAP antibodies did not occur in MCTD (0/49). Antibodies against RNAP were rare among antinucleolar-reactive sera, occurring in only 3/200 (1.5%). The RNAP ELISA provides a validated method which can be rapidly utilized in a clinical diagnostic laboratory setting to identify SSc patients who are at risk for developing diffuse SSc with multiorgan involvement and hypertensive renal crisis.

Adult↗
PubMed2005

Epidemiology of systemic sclerosis in northwest Greece 1981 to 2002.

OBJECTIVES: To investigate the incidence and prevalence, as well as the mortality and survival rates, of systemic sclerosis (SSc) in a defined area of northwest Greece with a population of about 500,000 inhabitants. MATERIALS AND METHODS: Cases have been recorded from the following sources: (1) inpatients and outpatients referred to the Rheumatology Clinics of the Ioannina University Hospital and the Ioannina General Hospital; (2) patients referred to the private rheumatologists practicing in the study area. All patients recorded between 1/1/1981 and 31/12/2002, resident in the study area, were included in the study. Diagnosis was based on the American College of Rheumatology classification criteria for SSc. Incidence and prevalence rates were calculated as number of cases per 10(5) inhabitants. Population data were based on the National Census of 1981, 1991, and 2001. RESULTS: The age-adjusted prevalence of SSc was 15.40 cases/10(5) inhabitants on 31/12/2002. A total of 109 new cases were diagnosed during the study period, giving a mean annual age-adjusted incidence rate of 1.10 cases/10 5 inhabitants. There were 98 women and 11 men, giving a ratio of 8.9/1. Limited SSc was diagnosed in 75% and diffuse in 25% of the patients. Esophageal involvement was found in 59%, lung involvement in 56%, and renal disease in 5%. Thirty-six deaths were recorded during the study period in this incidence cohort. The 5-year survival rate was 83% and the 10-year survival rate was 70%. CONCLUSIONS: The incidence and prevalence of SSc in northwest Greece were found to be lower than those of the USA and Australia, and higher than those of northern European countries and Japan. The survival rates were similar to those reported by other studies.

Adolescent↗
PubMed2006

Arteriographic evaluation of vascular changes of the extremities in patients with systemic sclerosis.

BACKGROUND: Although digital ulcerations frequently occur in patients with systemic sclerosis (SSc), there have been few reports on the macrovascular involvement. OBJECTIVES: To evaluate the macrovascular involvement in patients with SSc exhibiting digital ulceration or gangrene. METHODS: Transfemoral catheter arteriography of the upper and/or lower extremities was performed in eight patients (one man and seven women, age range 42-71 years) with SSc exhibiting digital ulceration or gangrene. The background of the patients, such as autoantibody profiles and vascular risk factors including smoking habits, was also investigated. RESULTS: Macrovascular involvement was detected in seven of eight patients. In three of seven patients who underwent arteriography of the upper extremity, occlusion was limited to the digital arteries. Obliteration of the ulnar artery and superficial palmar arch was detected in three of seven patients, and the radial artery in one patient. Only one of five patients who underwent arteriography of the lower extremity showed the occlusion limited to digital arteries of the foot. Occlusion of the posterior tibial artery, dorsalis pedis artery and arcuate artery was detected, each in one patient. Two patients showed occlusion of the plantar arch. Overall, the occlusion of arteries proximal to the digits was demonstrated in four of eight patients. Three of the four patients were positive for antitopoisomerase-1 antibody and had diffuse cutaneous SSc (dcSSc) with multiple skin ulcers or gangrene. CONCLUSIONS: Macrovascular involvement as detected with arteriography is not rare in SSc patients with digital ulceration or gangrene. Moreover, the vascular occlusion proximal to the digits seemed to be frequent in antitopoisomerase-1 antibody-positive dcSSc patients with multiple skin ulcers or gangrene.

Adult↗
PubMed1995

Serum tissue inhibitor of metalloproteinases in patients with systemic sclerosis.

BACKGROUND: One of the suggested contributory factors to the development of dermal fibrosis is a decrease in collagenase activity, which may be related to levels of serum tissue inhibitors of metalloproteinase-1 (TIMP-1). OBJECTIVE: The aim of this study was to determine the clinical significance of serum TIMP-1 levels in systemic sclerosis (SSc). METHODS: We measured serum TIMP-1 concentration in 62 patients with SSc, 11 patients with systemic lupus erythematosus, 14 patients with rheumatoid arthritis, and 22 members of a normal control group. The clinical features of the patients with SSc and elevated TIMP levels were examined. RESULTS: The mean TIMP-1 level in the patients with SSc was significantly higher than that in the members of the control group or the patients with systemic lupus erythematosus or rheumatoid arthritis. In 44% of the patients with SSc the serum TIMP-1 level was elevated. The mean serum TIMP-1 level in patients with diffuse cutaneous SSc (dSSc) was significantly higher than that in those with limited cutaneous SSc. The patients with dSSc and elevated serum TIMP-1 levels showed a significantly greater incidence of lung fibrosis and anti-topoisomerase I antibody than those with normal serum TIMP-1 levels. The TIMP-1 level and diffusing capacity for carbon monoxide in the patients with SSc were negatively correlated. Increased mitogenic activity on dermal fibroblasts caused by serum from patients with dSSc was partially blocked by anti-TIMP-1 IgG. CONCLUSIONS: These findings suggest that serum TIMP-1 level is a useful indicator of disease activity in patients with SSc and that TIMP is involved in the pathogenesis of SSc.

Adolescent↗
PubMed1994

Aberrant expression of membrane cofactor protein and decay-accelerating factor in the endothelium of patients with systemic sclerosis. A possible mechanism of vascular damage.

BACKGROUND: One of the main features of systemic sclerosis (SSc) is vascular damage, the mechanism of which is not understood. The aim of this study was to investigate if complement (C) regulatory molecules, membrane cofactor protein (MCP), decay-accelerating factor (DAF), and CD59, which normally protect endothelial cells from damage by autologous C, are absent or down-regulated in vascular endothelium of patients with SSc. A deficiency or persistent down-regulation of the above molecules is likely to render vascular endothelium of these patients susceptible to damage by physiologically or pathologically activated C. From this point of view, expression of MCP, DAF, and CD59 was tested on endothelium of skin of normal subjects and patients with diffuse and limited forms of SSc. EXPERIMENTAL DESIGN: Punch biopsies of normal skin (N = 11) and lesional and non-lesional skin of patients with diffuse (N = 5) and limited (N = 5) forms of SSc were obtained and serial sections prepared. Immunoperoxidase staining of these sections was carried out using four monoclonal antibodies (MoAbs) directed to different epitopes of DAF, four to different epitopes of MCP, one to CD59 and one to von Willebrand factor. von Willebrand factor served as a marker of endothelial cells. Besides normal skin, lesional skin of systemic lupus erythematosus and several inflammatory and proliferative diseases, described below, served as controls. RESULTS: The endothelium of normal skin strongly expressed all the three proteins. However, the endothelium of lesional and nonlesional skin of all the 10 patients with diffuse or limited forms of the disease tested, expressed either decreased or undetectable amounts of MCP and DAF. CD59 expression was normal in some patients and lower than normal in others. Aberrant expression of MCP, DAF, or CD59 was not found in other control inflammatory, connective tissue and proliferative diseases studied. CONCLUSIONS: In view of the common function of MCP and DAF to protect self cells from autologous C, their decrease or virtual absence from the endothelium of patients with SSc strongly suggests that this deficiency may contribute to vascular damage, resulting in intima proliferation and, finally, fibrosis.

Antibodies, Monoclonal↗
PubMed1999

Oxidative stress in Systemic Sclerosis.

In 63 patients affected by Systemic Sclerosis (SSc) (limited subset: 40; diffuse subset: 23; early: 30; advanced: 33) the peroxidation product diene-conjugates (DC) and antibodies against oxidised low density lipoproteins (Ab oxLDL) were tested in serum by a spectrophotometer (absorbance 234 mn) and by a standard ELISA respectively. The data were compared with those obtained by 21 healthy subjects. DC was significantly higher in patients (73.3 +/- 37.2 microM/l; p < 0.0001) than in controls (48.4 +/- 16.7) as well as in the limited (80 +/- 48.8; p < 0.05) than in the diffuse subset (64.5 +/- 36.4); and in early (84.1 +/- 31.4; p < 0.05) than in advanced stage of the disease (67.9 +/- 42.5). The levels of Ab oxLDL were significantly higher in SSc patients (309.5 +/- 367.2 mU/ml; p < 0.0001) in all its subsets (limited: 351.9 +/- 351.1, p < 0.0001; diffuse: 207.7 +/- 316.1, p < 0.05; early: 428.9 +/- 417.1, p < 0.001; advanced: 302.7 +/- 89.9, p < 0.0001) than in controls (89.3 +/- 29.1). These antibodies levels were higher in limited subset than in diffuse (p < 0.05) and in early SSc than in advanced SSc (p < 0.05). The highest values of parameters of oxidative stress are found in the early stages, when the episodes of reperfusion after ischemic episodes (Raynaud's phenomenon) are very frequent. Moreover, the damage is higher in the early stages of SSc, with intact microvessels, than in late stages, when microvessels are very reduced in number, destroyed by the worsening of the disease. These data show that the respiratory burst deduced by the lipoperoxidation is higher in SSc than in controls, and may be an important pathogenetic factors involved in tissue changes in SSc.

Antibodies↗
PubMed1998

High seroprevalence of Helicobacter pylori infection in patients with systemic sclerosis: association with esophageal involvement.

OBJECTIVE: To determine the prevalence and clinical relevance of Helicobacter pylori infection in patients with systemic sclerosis (SSc). METHODS: Serum samples were obtained from 124 patients with SSc (67 with limited cutaneous SSc, 57 with diffuse cutaneous SSc). Fifty samples from age and sex matched individuals were used as controls. IgG antibodies to H. pylori infection were measured by ELISA. RESULTS: IgG antibodies to H. pylori were found in 55.6% (69 of 124) of the patients with SSc, significantly more than in the controls. There was a significant correlation of the presence of antibodies to H. pylori with the prevalence of esophageal hypomotility in the patients with SSc (p < 0.02). CONCLUSION: Patients with SSc have H. pylori infection at a higher prevalence than the general population. H. pylori might play a role in the development of esophageal dysfunction in SSc.

Adult↗
PubMed1991

Serum aminoterminal propeptide of type III procollagen in systemic sclerosis. A follow-up--investigations in subclasses and during therapy.

Fifty-seven patients with systemic sclerosis were investigated for connective tissue turn-over related to type III collagen. Sera from 13 patients with diffuse cutaneous systemic sclerosis and 44 patients with limited cutaneous systemic sclerosis were analysed for aminoterminal propeptide of type III procollagen (PIIINP) by a radioimmunoassay based on human propeptide. Increased levels of PIIINP in serum correlated with skin involvement and the clinical course. All patients with diffuse cutaneous systemic sclerosis had levels above the normal range, and in limited cutaneous systemic sclerosis elevated PIIINP levels seemed to be correlated with rapid progression and with extension of lesions. Immunosuppressive drugs, cyclosporin A, and prednisone with or without cyclophosphamide, which were given to patients with rapid disease progression, significantly reduced PIIINP. This was also the case with penicillamine, but to a lesser degree. Our data support the suggestion that immunosuppressive agents are justified in rapidly progressive, life-threatening or disabling disease, when used with the necessary precautions. Serum PIIINP may be utilized as a marker of type III collagen fibrogenesis in systemic sclerosis and be of prognostic value. PIIINP may also be of use in the differential diagnosis between diffuse cutaneous systemic sclerosis and scleredema.

Drug Therapy, Combination↗
PubMedDate not supplied

[Detection of antibodies to extractable nuclear antigens with counterimmunoelectrophoresis and the Western blot technic].

Detection of antibodies against extractable nuclear antigens (ENA) by counter immunoelectrophoresis (CIE) is currently used to screen sera from patients with collagen vascular disease. Although fast and reliable, the method has its limitations with regard to the differentiation of various antigen-antibody systems. In this study, results of the anti-ENA analysis by CIE and by Western-Blot technique are compared. Except for a higher frequency of Sm antibodies by Western-blot, the results of CIE were confirmed. The source of antigen and the higher sensitivity of the Western-Blot technique are responsible for the different results obtained. With respect to the relationship of antibody profile and illness, both methods demonstrated a high frequency of antibodies against U1-sn-RNP in mixed connective tissue disease and of anti-SS-B antibodies in Sjögren's syndrome.

Antibodies, Antinuclear↗
PubMed1993

Mechanisms of endothelial cell damage in systemic sclerosis and Raynaud's phenomenon.

OBJECTIVE: To investigate tumor necrosis factor (TNF), circulating immune complexes (CIC) and oxidized lipoproteins [measured as thiobarbituric acid reactive substances (TBARS)] as mediators of damage to endothelial cells (EC) in systemic sclerosis (SSc) and Raynaud's phenomenon (RP). METHODS: In addition to CIC, TBARS and TNF, von Willebrand factor (vWF), angiotensin converting enzyme (ACE) and EC cytotoxicity were measured as markers of EC damage. C-reactive protein (CRP) and rheumatoid factor (RF) were measured as indicators of inflammation. RESULTS: There were increases in TBARS and CIC, vWF and EC cytotoxicity, and CRP, with reduced levels of ACE. There were no correlations between any possible mediator of damage (TNF, TBARS, CIC) and indicators of such injury to the endothelium (vWF, ACE, EC cytotoxicity) except an inverse correlation between vWF and ACE. When patients with SSc were classified into those with limited disease (ISSc) or diffuse disease (dSSc), only wWF was different, being higher in patients with dSSc. In RP, vWF, RF and TBARS were again raised and ACE was lower. The only correlation was inversely between vWF and ACE. CONCLUSION: Our results are suggestive of injury to the endothelium in both SSc and RP, with greater damage in patients with dSSc. Although there were multiple abnormalities in biochemical and immunological indices, there were no consistent correlations to explain the clear damage to the vasculature. We conclude that additional mechanisms other than TNF, CIC and lipid peroxides may be responsible for the insult to the vascular tree so evident in these patients.

Adult↗
PubMed2002

[Obesity persistent scleredema: study of 49 cases].

BACKGROUND: Buschke sclerodema is a very rare disease. Our objective was to show that persistent scleredema is frequent in certain group of patients at risk. PATIENTS AND METHODS: We studied 49 patients, diagnosed between 1995 and 1999 in dermatology, pneumology and endocrinology departments in Martinique. Diagnosis was performed on classical clinical and histopathological aspects of sclerodema. Data studied were age, sex, mode of occurrence, clinical and histopathological aspects and associated diseases. RESULTS: The 49 patients presented with cutaneous infiltration of the upper part of the trunk, with thick dermis and large collagen bundles on histopathological examination. Forty-two had mucoid substance deposition, stained with Alcian Blue (this criteria was considered as inconstant by most authors in the literature). Sex ratio H/F was 0.06 (93 p. 100 females). Mediam age at onset was 50 years ranging from 20 to 79 years. The occurrence was insidious in 97 p. 100 of cases. All patients had neck and nuchae involvement. The disease involved the back in 93 p. 100, upper limbs in 50 p. 100 and lower limbs and face in 43 p. 100 of patients. Fifty-six percent of patients had limitation of shoulder movements, 16 p. 100 limitation of mouth opening, 20 p. 100 limitation of eyelid opening, 36 p. 100 had myalgia, 73 p. 100 had pruritus and 66 p. 100 had dyspnea. Obesity was present in 95 p. 100, diabetes in 79.5 p. 100, elevated blood pressure in 81.5 p. 100 and monoclonal dysglobulinemia in 46 p. 100 of patients. Twenty-five patients had a polysomnography showing severe obstructive sleep apnea syndrome. DISCUSSION: The large number of patients in our study can be explained by the search for sclerodema in patients with obesity, diabetes and high blood pressure. The disease is usually unknown by patients and physicians unless a systematic examination is performed. Association with obstructive sleep apnea syndrome was not previously reported and a larger study is ongoing.

Adult↗
PubMedDate not supplied

Treatment of early diffuse cutaneous systemic sclerosis patients in Japan by low-dose corticosteroids for skin involvement.

Systemic sclerosis (SSc) is a multiorgan systemic disorder characterized by excessive collagen deposition. This disease is usually classified into two clinical subsets - diffuse cutaneous SSc (dSSc) and limited cutaneous SSc (lSSc). We have proposed low-dose oral corticosteroid treatment with indication criteria consisting of the following three conditions: (1) early onset; (2) edematous changes; and (3) rapid progression. Cases satisfying 2 or 3 criteria have a definite indication, while those meeting 0 or 1 criterion have no indication. We treated 23 cases of dSSc by low-dose oral corticosteroid (prednisolone 20 mg/day as the initial dosage) and evaluated the effect using the modified Rodnan total skin thickness score (m Rodnan TSS). The mean initial TSS (20.3+/-9.3) decreased significantly to 12.8+/-7.0 after one year of treatment (p < 0.005) and to 8.7+/-6.1 at final evaluation (p < 0.001). Thus, we confirmed the usefulness of oral corticosteroid treatment for early dSSc in Japanese patients.

Adrenal Cortex Hormones↗
PubMed1998

The modified Rodnan skin score is an accurate reflection of skin biopsy thickness in systemic sclerosis.

OBJECTIVE: To test the ability of the modified Rodnan skin score to reflect skin thickness in skin biopsies from 141 patients with systemic sclerosis (SSc) obtained at entry during a prospective, double blind study of ketanserin versus placebo in SSc. METHODS: Punch skin biopsies (4 mm) were obtained from the dorsal surface of the distal forearm of 141 patients. Biopsy specimens were trimmed and weighed (wet weight) and then desiccated and reweighed (dry weight). Skin score was recorded for 17 areas, graded 0-4+, while edema was graded 0-4+ in 10 of the same sites using finger pressure. RESULTS: Total skin score correlated with wet weight (r = 0.553) and dry weight (r = 0.517) of the skin biopsies. Local skin score from the biopsied forearm also correlated with wet and dry weight (r = 0.536 and 0.530, respectively). Dry weight as a percentage of wet weight was the same for diffuse cutaneous SSc (dSSc) and limited cutaneous SSc (lSSc) (30.7% for both, NS), despite increased wet weight in patients with dSSc versus lSSc (17.75 vs 13.03 g; p < 0.001). Edema scores correlated poorly both with wet weight (r = 0.069) and dry weight (r = 0.169). CONCLUSION: Total and forearm skin score correlates well with both wet and dry forearm skin biopsy weight from forearm biopsies, indicating that skin score reflects the underlying pathology of SSc. Further, the percentage of dry to wet weight is similar for lSSc and dSSc, supporting the usefulness of skin score in differentiating SSc disease subtypes.

Adolescent↗