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PubMed2005

Evaluation of the effect of nifedipine upon myocardial perfusion and contractility using cardiac magnetic resonance imaging and tissue Doppler echocardiography in systemic sclerosis.

BACKGROUND: Primary myocardial involvement due to microcirculation impairment is common in systemic sclerosis (SSc). Cardiovascular magnetic resonance imaging (MRI) and tissue Doppler echocardiography (TDE) were recently shown to be more sensitive than conventional methods for the respective assessment of myocardial perfusion and contractility. Previous studies have suggested that dihydropyridine-type calcium channel blockers mitigate both myocardial perfusion and function abnormalities. OBJECTIVE: To investigate the effects of nifedipine on myocardial perfusion by MRI and on contractility by TDE, in patients with SSc. PATIENTS AND METHODS: 18 patients with SSc without clinical heart failure and with normal pulmonary arterial pressure (14 women, 4 men; mean (SD) age 59 (9) years; mean (SD) disease duration 7 (4) years, 10 with diffuse and 8 with limited cutaneous forms) were prospectively evaluated. The MRI perfusion index, determined from time-intensity curves, and systolic and diastolic strain rate determined by TDE were assessed at baseline, after a 72 hour vasodilator washout period, and after 14 days of oral treatment with nifedipine 60 mg/day. RESULTS: Nifedipine treatment led to a significant increase in the MRI perfusion index (mean (SD) 0.26 (0.07) v 0.19 (0.05) at baseline, p = 0.0003) and in systolic and diastolic strain rate (2.3 (0.6) v 1.5 (0.4) s(-1) at baseline, p = 0.0002, and 4.2 (1.6) v 3.0 (1.2) at baseline, p = 0.0003, respectively). CONCLUSION: Fourteen days of treatment with nifedipine simultaneously improves myocardial perfusion and function, as evaluated by highly sensitive and quantitative methods.

Adult↗
PubMed1996

Psychological adjustment to systemic sclerosis.

OBJECTIVE: To examine the role of demographic, disability, appraisal, and coping variables in predicting psychological adjustment in individuals with systemic sclerosis. METHODS: Two hundred forty-two individuals with systemic sclerosis (SSc; diffuse and limited) were surveyed by mail. Demographics, functional disability, pain, control appraisals, 8 types of coping, and individual psychosocial adjustment were assessed by self-report questionnaires with established reliability and validity. RESULTS: In regression analysis, 3 coping strategies emerged as significant predictors of adjustment: Wishful Thinking, Blaming Self, and Problem-Focused Coping. Self-reports of disability and control appraisals were also significant predictors. Collectively, 46% of the variance in adjustment was explained by these 5 predictor variables. The strongest predictor of overall adjustment was wishful thinking, explaining 22% of the variance in adjustment. CONCLUSIONS: Potentially modifiable appraisal and coping variables, along with disease-related disability, appear to play an important role in predicting adjustment to SSc, while demographic variables explained little of the variability in patient adjustment.

Activities of Daily Living↗
PubMed2002

[Alterations of rheological properties of blood in systemic sclerodermia].

AIM: To study hemorheological status in systemic sclerosis (SS) patients. MATERIAL AND METHODS: Macro- and microrheology of blood were investigated in 70 SS patients. The rate of spontaneous aggregation of red blood cells [T1(s)], durability of the largest units [Ia2.5(%)], general hydrodynamic durability of units [beta(s-1)], limit of fluidity [tau 0 (mPa)], Casson viscosity K (mPa/s) were determined. RESULTS: The total severity of hemorheological disturbances was 1.90 +/- 0.07 with T1 = 4.98 +/- 1.52s, beta = 62.76 +/- 19.32s, Ia 2.5 = -0.78 +/- 12.83%, tau 0 = 7.00 +/- 0.45 mPa. The Casson viscosity did not differ from normal levels. Plasma viscosity was 1.71 +/- 0.18 rel units, viscosity of sera 1.55 +/- 0.14 rel.units. The severity of hemorheological disturbances did not differ in cute and chronic courses but the parameter beta (72.0 +/- 4.5) was significantly higher (p < 0.05) in the acute course. The severity of hemorheological disturbances was highest in SS patients with cardiac disease (p < 0.05) and in 10 year SS duration. CONCLUSION: The course of SS is complicated by progressive hyperaggregation syndrome promoting development of visceral pathology. Active correction of hemorheology is pathogenetically validated in SS.

Adolescent↗
PubMed2002

Causes of death and poor survival prognostic factors in thai patients with systemic sclerosis.

Causes of death and poor prognostic factors for patients with systemic sclerosis (SSc) were studied in 222 cases. Their mean age at the onset and duration of disease was 48.9 +/- 12.0 years and 23.3 +/- 29.3 months, respectively. Fifty-three per cent were diffuse subtype. Patients with diffuse SSc had more digital pitting scars and more muscle, heart, lung, and esophageal involvement than those with limited subtypes (p < or = 0.02). One hundred and six patients were lost to follow-up. With a median follow-up duration of 25 months, 31 of the remaining 116 patients (26.7%) died. SSc related death occurred in 18 cases, in which the lung, heart and kidney (renal crisis) were the major causes. Infection contributed to the remaining 13 deaths. When compared with living patients, using a univariate analysis, factors associated with a reduced survival rate were age of > 45 years at the onset, diffuse skin thickness, and lung, gastrointestinal tract, heart, kidney and muscle involvement (p < or = 0.001). In the multivariate analysis, only age of > 45 years at onset and cardiac involvement remained poor prognostic factors (p = 0.04 and 0.001, respectively).

Adult↗
PubMed2006

A clue for telangiectasis in systemic sclerosis: elevated serum soluble endoglin levels in patients with the limited cutaneous form of the disease.

BACKGROUND: The transforming growth factor-beta (TGF-beta) system plays a critical role both in systemic sclerosis (SSc) and hereditary hemorrhagic telangiectasia (HHT). Endoglin, known as a gene responsible for HHT, is a TGF-beta receptor preferentially expressed on endothelial cells. The role of endoglin in SSc is potentially intriguing since limited cutaneous SSc (lcSSc) and HHT share several symptoms, including telangiectasia. OBJECTIVE: To determine serum levels of soluble endoglin (sEndoglin) and clinical associations in patients with SSc. METHODS: Serum sEndoglin levels were examined by ELISA in 70 patients with SSc, 20 patients with systemic lupus erythematosus and 20 healthy individuals. RESULTS: Serum sEndoglin levels were significantly elevated in patients with lcSSc compared with diffuse cutaneous SSc and systemic lupus erythematosus patients as well as normal controls. Patients with elevated sEndoglin levels had telangiectasia more frequently than those with normal sEndoglin levels. Furthermore, pulmonary artery pressure was positively correlated with sEndoglin levels in patients with lcSSc. CONCLUSION: Abnormal expression/function of endoglin may be linked to lcSSc-specific manifestations.

Adolescent↗
PubMed2001

Association of HLA class II genes with systemic sclerosis in Koreans.

OBJECTIVE: To investigate HLA class II associations with systemic sclerosis (SSc) in Koreans according to anti-topoisomerase I (anti-topo I), anti-centromere antibody (ACA), and anti-U1 ribonucleoprotein (RNP) status. METHODS: HLA class II alleles (DRB1, DRB5, DQB1) were determined by DNA typing in 200 healthy control subjects and 74 patients with SSc: 35 anti-topo I positive, 3 ACA positive, and 19 anti-U1 RNP positive; among them were 34 diffuse and 40 limited subtypes, and 16 overlap syndrome. RESULTS: Anti-topo I positive SSc was strongly associated with DRB1*1502 compared with both controls (23% vs 5%; Pcorr = 0.003) and anti-topo I negative patients (23% vs 3%; p = 0.009); our study confirms observations in Japanese. HLA-DR 67FLEDR71, especially 38V-67FLEDR71 sequence (carried on DRB5*0102 in linkage disequilibrium with DRB1*1502, DRB1*0802, DRBI*1101), showed the strongest association with anti-topo I response (46% vs 16% in controls; Pcorr = 0.001), and 38L-67FLEDR71 group alleles were not associated with anti-topo I response. Anti-topo I response was not significantly associated with HLA-DQB1 alleles in Koreans. There were only 3 ACA positive patients, and all patients had DRB1*1501 and DQB1*0602 as heterozygotes. Anti-U1 RNP occurred at a high frequency (63%) among patients with overlap syndrome, and was not associated with HLA-DR or DQ genes. Among anti-topo I negative patients, diffuse and limited subtypes of SSc were significantly associated with DRB1*0803 (47% vs 15% in controls; Pcorr < 0.05) and DRB1*1501 (50% vs 17% in controls; Pcorr < 0.01), respectively. These HLA associations have not been reported in other ethnic groups and possible associations with certain autoantibody subsets remain to be confirmed. CONCLUSION: HLA-DR gene has a primary association with anti-topo I response, and HLA-DR 38V-67FLEDR71 group alleles including DRB5*0102 (in linkage disequilibrium with DRB1*1502) show the strongest association with anti-topo I response in Korean patients with SSc.

Adult↗
PubMed2002

Systemic sclerosis therapy with iloprost: a prospective observational study of 30 patients treated for a median of 3 years.

Iloprost is useful in the short-term treatment of severe Raynaud's phenomenon and ischaemic ulcers in patients with systemic sclerosis (SSc), but its long-term effects are largely unknown. The aim of this study was to report long-term outcome (median follow-up 36 months) in a prospective observational study of a cohort of 30 consecutive patients with SSc who received iloprost therapy with maintenance infusions every 3 weeks after an initial cycle of 5 consecutive days. At the end of the observation, compared to the pretreatment point, we observed complete healing of digital ulcers in 19/21 patients (90%), a decrease of the Raynaud's phenomenon visual analogue score from 10/10 (25th-75th percentile 7-10) to 5/10 (4-6.75) ( P <0.001) and, in patients with diffuse cutaneous involvement, of the modified Rodnan skin thickness score from 25.5 (16.5-31.5) to 16 (13.5-20) ( P = 0.02), minimal improvement of the Health Assessment Questionnaire from 0.87 (0.68-1.37) to 0.75 (0.62-1.25), which was neither statistically nor clinically significant. The forced vital capacity was not significantly changed, but the diffusion capacity corrected for the alveolar volume decreased from 71% (54-76.7) of the expected value to 62% (51.5-71) ( P = 0.02). In one patient with limited SSc a positive effect on pulmonary hypertension was observed. Six patients, after a median of 25 months of treatment and healing of digital ulcers, discontinued the therapy; after a median of 10 months ulcers did not recur in five of these six. Other reasons for discontinuation were: tolerability (1), disease progression (normotensive renal crisis: 1), and death due to intracranial haemorrhage (1). This same patient had previously suffered a central retinal vein thrombosis. In conclusion, long-term therapy with iloprost in patients with SSc has a durable effectiveness on ischaemic ulcers and Raynaud's phenomenon, but it is not possible to conclude that the natural history of the disease was modified.

Adult↗
PubMed1996

Percutaneous needle biopsy and synovial histology.

Percutaneous needle biopsies of synovium are successfully used for diagnosis and investigation of joint disease by an increasing number of groups around the world. This procedure can be done in the office with little morbidity; a large number of samples can minimize the potential limitation of sampling error. Clinical indications for 'imaging the joint' by looking at morphological and other features of the actual tissue include undiagnosed acute or chronic mono- or oligoarthritis, haemarthrosis, suspected deposition diseases, new developments in previous stable disease and less often unexplained polyarthritis. Research into any joint disease can be helped by study of synovium especially using newer immunohistochemical, EM and molecular techniques. This report has reviewed other methods used for obtaining synovium, described the different percutaneous biopsy needles, detailed the methods used for biopsy with the Parker-Pearson needle and described how our group handles tissue so as to obtain maximal impact. The very few side effects of needle biopsy include haemarthrosis and, rarely, needle breakage. Finally, we have provided a brief overview of normal synovium and some aspects of synovium in a variety of joint diseases.

Arthritis↗
PubMed2001

Treating systemic sclerosis in 2001.

Systemic sclerosis (SS) is characterized by sclerosis of the dermis and internal organs and by vascular abnormalities. Although the pathophysiology of the disease has been partly elucidated, the efficacy of long-term treatments remains limited, with no significant increase in survival in prospective studies. Conventional drug treatments are disappointing in clinical practice, and in a recent prospective randomized study standard-dose D-penicillamine was not more effective than mini-dose D-penicillamine. New long-term treatments are emerging for diffuse SS, including cyclophosphamide for patients with progressive interstitial lung disease or stem cell transplantation for those with early organ involvement. The most effective treatments remain symptomatic, such as angiotensin-converting enzyme inhibitors for acute renal crisis, calcium channel antagonists for Raynaud's phenomenon, and proton pump inhibitors for the complications of gastroesophageal reflux. This review article focuses on long-term treatments that are most likely to be effective and suggests symptomatic treatment strategies tailored to specific organ involvements.

Antineoplastic Agents↗
PubMed1992

Long-term follow-up study of 164 patients with definite systemic sclerosis: classification considerations.

To evaluate the usefulness of recently proposed schemes of classification for systemic sclerosis an extensive cross-sectional study of a series of 164 consecutive patients with long-term systemic sclerosis was undertaken. There were 47 cases of proximal sclerosis, 93 of distal sclerosis and 24 of complete CREST syndrome. The study included clinical, visceral, immunological and follow-up data. In addition, a quantitative clinical score was calculated for each patient, thus providing indications for prognosis. Data were expressed according to three conventional systems of classification: The ARA system, the diffuse versus limited systemic sclerosis system and the early cutaneous involvement system. The most reliable indications of severe outcome were: proximal sclerosis, trunk skin involvement, presence of anti Scl 70 autoantibody, pulmonary and/or heart involvement and age. Diagnosis and prognosis were not generated by the same items. Prognosis indicators proved more accurate for groups than for individuals. Mortality was 1 death per 149 patient X years of follow-up from diagnosis. We conclude that the ARA criteria for classification should be recognized as a standard, but patients with complete CREST syndrome should be included in the distal group. Other systems of classification, principally 2-way versus 3-way criteria, allow different subsets of patients that correlate with prognosis and the severity of the disease, and could be used for therapeutic purposes.

Adolescent↗
PubMed2005

Polymorphism of the fractalkine receptor CX3CR1 and systemic sclerosis-associated pulmonary arterial hypertension.

Fractalkine (FKN) and its receptor CX3CR1 are critical mediators in the vascular and tissue damage of several chronic diseases, including systemic sclerosis (SSc) and pulmonary arterial hypertension (PAH). Interestingly, the V249I and T280M genetic polymorphisms influence CX3CR1 expression and function. We investigated whether these polymorphisms are associated with PAH secondary to SSc. CX3CR1 genotypes were analyzed by PCR and sequencing in 76 patients with limited SSc and 204 healthy controls. PAH was defined by colorDoppler echocardiography. Homozygosity for 249II as well as the combined presence of 249II and 280MM were significantly more frequent in patients with SSc compared to controls (17 vs 6%, p = 0.0034 and 5 vs 1%, p = 0.0027, respectively). The 249I and 280M alleles were associated with PAH (odd ratio [OR] 2.2, 95% confidence interval [CI] 1.01-4.75, p = 0.028 and OR 7.37, 95%CI: 2.45-24.60, p = 0.0001, respectively). In conclusion, the increased frequencies of 249I and 280M CX3CR1 alleles in a subgroup of patients with SSc-associated PAH suggest a role for the fractalkine system in the pathogenesis of this condition. Further, the 249I allele might be associated with susceptibility to SSc.

CX3C Chemokine Receptor 1↗
PubMed2005

Increased cutaneous T-cell-attracting chemokine levels in sera from patients with systemic sclerosis.

OBJECTIVE: We sought to assess serum cutaneous T-cell-attracting chemokine (CTACK) levels and CTACK expression levels in skin from patients with systemic sclerosis (SSc), and determine whether serum CTACK levels correlate with clinical features in SSc patients. METHODS: Serum samples were obtained from 73 SSc patients, 32 patients with systemic lupus erythematosus and 26 patients with dermatomyositis. Serum CTACK levels were determined by enzyme-linked immunoabsorbent assay. CTACK mRNA expression in sclerotic skin was assessed by real-time reverse transcription-polymerase chain reaction. RESULTS: Serum CTACK levels were significantly increased in patients with diffuse cutaneous SSc (dSSc; n=32) and those with limited cutaneous SSc (lSSc; n=41) compared with normal controls (n=31; P<0.05 and P<0.0005, respectively). The presence of calcinosis and muscle involvement was more frequently detected in SSc patients with elevated CTACK levels (P<0.05 and P<0.05, respectively). Elevated C-reactive protein levels were also observed more frequently in SSc patients with increased CTACK levels (P<0.05). CTACK mRNA expression levels in the sclerotic skin of SSc patients were augmented. In a longitudinal study, serum CTACK levels were generally decreased during the follow-up. CONCLUSIONS: The increased serum CTACK levels and enhanced skin CTACK expression in SSc patients suggest that CTACK is related to the inflammation associated with SSc.

Adult↗
PubMed1993

Demonstration of interleukin 8 and autoantibodies to interleukin 8 in the serum of patients with systemic sclerosis and related disorders.

BACKGROUND AND DESIGN: Interleukin 8 (IL-8), a chemotactic cytokine produced by various cell types, displays structural homology to the connective tissue-activating peptide III. Little is known of the possible role of IL-8 in connective tissue disorders. We therefore determined serum concentrations of IL-8 and autoantibodies to IL-8 in 134 patients with systemic sclerosis (SSc) and related connective tissue disorders, as well as in pooled serum from 28 healthy control subjects by a sensitive enzyme-linked immunosorbent assay. RESULTS: Interleukin 8 was undetectable in the pooled serum from 28 healthy controls, but detectable in serum samples from 24 of the 134 patients described above. It was detected in 13 of 60 patients with limited SSc and in eight of 48 patients with diffuse SSc. It was also detectable in one of three patients with eosinophilic fasciitis and in two of 10 patients with Raynaud's syndrome without skin involvement. In contrast, none of the three patients with morphea or the 10 patients with eosinophilia-myalgia syndrome had detectable IL-8 levels. We further determined the concentration of autoantibodies to IL-8 in the same serum samples. The values in healthy controls were 6.7 +/- 0.2 ng/mL (mean +/- SEM). Significantly elevated autoantibody levels were detected in patients with limited SSc (21.5 +/- 1.7), diffuse SSc (23.4 +/- 2.2), and Raynaud's syndrome (20.5 +/- 3.7). Elevated levels were also detected in patients with eosinophilic fasciitis (43.7 +/- 8.6) and morphea (14.7 +/- 3.2). Normal levels (7.5 +/- 2.0) were found in patients with eosinophilia-myalgia syndrome. Analysis of variance between the levels of autoantibodies to IL-8 and duration of the disease, extent of skin involvement, drug therapy, or serologic findings failed to show a significant correlation. CONCLUSIONS: These results suggest that increased production of IL-8 may relate to activation of mononuclear phagocytes, fibroblasts, or endothelial cells, among other cell types, in patients with SSc, but not in those with eosinophilia-myalgia syndrome. This activation could be related to the production of autoantibodies to IL-8.

Adult↗
PubMed2006

Evaluation of interleukin 13 polymorphisms in systemic sclerosis.

Systemic sclerosis (SSc) is a multisystem disease of unknown etiology. It is characterized by excessive cutaneous and visceral fibrosis, damage to small blood vessels, and production of autoantibodies. Interleukin-13 (IL-13) has been shown to be involved in abnormal fibrosis in other diseases. Therefore, we have evaluated its possible involvement in SSc. We analyzed four IL13 gene polymorphisms, rs1800925 (IL13-1055), rs20541 (Arg130Gln), rs847, and rs2243204 in 107 unrelated SSc patients (40 patients having diffuse cutaneous form and 67 patients having limited cutaneous form) and in 170 controls. All subjects were Caucasians. In the total patient population and in the diffuse cutaneous subset, we observed an association between two IL13 polymorphisms, IL13 rs1800925 (IL13-1055), and IL13 rs2243204, and disease (p=0.03-0.04). The IL13 rs2243204T allele was more common in SSc patients (p=0.01, OR=2.3 CI 1.21-4.38) and in the diffuse cutaneous form (p=0.01, OR=2.95, CI 1.35-6.49) than in control subjects. Our result supports the suggestion that polymorphisms in IL13 are associated to SSc and skin fibrosis process. However, further studies on larger and independent population and functional analyses are needed to confirm these findings.

Aged↗
PubMed2004

Blink reflex discloses CNS dysfunction in neurologically asymptomatic patients with systemic sclerosis.

OBJECTIVE: To investigate trigeminal-nerve, brain-stem, and brain function, in order to disclose a possible nervous system involvement in neurologically asymptomatic systemic sclerosis (SSc) patients. METHODS: Using a standard electromyographic (EMG) technique, we recorded the early (R1) and late (R2) components of the blink reflex in 35 SSc patients with no history or signs of cranial-nerve impairment and 20 control subjects. SSc patients were classified as limited or diffuse SSc and also evaluated for disease duration, autoantibody pattern (ANA, ACA, Scl70) and skin score (by Rodnan modified method). RESULTS: Whereas no SSc patients had an abnormal R1, six (18%) had delayed R2 responses. We found no correlation between R2 latency and clinical or laboratory data. CONCLUSIONS: Whereas previous studies reported both R1 and R2 abnormalities (reasonably due to trigeminal neuropathy) in symptomatic SSc patients, we found selective abnormalities of the R2 components in asymptomatic patients. The selective R2 abnormality is secondary to a central dysfunction, either because of a direct impairment of the polysynaptic circuits in the medulla, or because of a decreased cortico-reticular drive on these circuits. The lack of overt trigeminal symptoms in our patients favours a suprasegmental dysfunction, possibly due to microvascular lesions disseminated in the subcortical white matter.

Adult↗
PubMed2003

Epstein-Barr virus-associated posttransplantation lymphoproliferative disorder after high-dose immunosuppressive therapy and autologous CD34-selected hematopoietic stem cell transplantation for severe autoimmune diseases.

High-dose immunosuppressive therapy followed by autologous hematopoietic stem cell transplantation (HSCT) is currently being evaluated for the control of severe autoimmune diseases. The addition of antithymocyte globulin (ATG) to high-dose chemoradiotherapy in the high-dose immunosuppressive therapy regimen and CD34 selection of the autologous graft may induce a higher degree of immunosuppression compared with conventional autologous HSCT for malignant diseases. Patients may be at higher risk of transplant-related complications secondary to the immunosuppressed state, including Epstein-Barr virus (EBV)-associated posttransplantation lymphoproliferative disorder (PTLD), but this is an unusual complication after autologous HSCT. Fifty-six patients (median age, 42 years; range, 23-61 years) with either multiple sclerosis (n = 26) or systemic sclerosis (n = 30) have been treated. The median follow-up has been 24 months (range, 2-60 months). Two patients (multiple sclerosis, n = 1; systemic sclerosis, n = 1) had significant reactivations of herpesvirus infections early after HSCT and then developed aggressive EBV-PTLD and died on days +53 and +64. Multiorgan clonal B-cell infiltrates that were EBV positive by molecular studies or immunohistology were identified at both autopsies. Both patients had positive screening skin tests for equine ATG (Atgam) and had been converted to rabbit ATG (Thymoglobulin) from the first dose. Of the other 54 patients, 2 of whom had partial courses of rabbit ATG because of a reaction to the intravenous infusion of equine ATG, only 1 patient had a significant clinical reactivation of a herpesvirus infection (herpes simplex virus 2) early after HSCT, and none developed EBV-PTLD. The T-cell count in the peripheral blood on day 28 was 0/microL in all 4 patients who received rabbit ATG; this was significantly less than in patients who received equine ATG (median, 174/microL; P =.001; Mann-Whitney ranked sum test). Although the numbers are limited, the time course and similarity of the 2 cases of EBV-PTLD and the effect on day 28 T-cell counts support a relationship between the development of EBV-PTLD and the administration of rabbit ATG. The differences between equine and rabbit ATG are not yet clearly defined, and they should not be considered interchangeable in this regimen without further study.

Adult↗
PubMed2002

Correlation between plasma concentrations of calcitonin gene related peptide and pulmonary pressure in patients with systemic sclerosis.

OBJECTIVE: To examine plasma levels of calcitonin gene related peptide (p-CGRP) in patients with systemic sclerosis (SSc) and pulmonary hypertension (PH). MATERIAL AND METHODS: Twenty nine patients with SSc, 10 with diffuse form, 18 with limited form and one with overlapping systemic lupus erythematosus were examined. Twelve patients displayed normal systolic pulmonary artery pressure (PAPsyst) < or =30 mm Hg and 17 increased PAPsyst >30 mm Hg. Eight patients had isolated PH without interstitial lung disease (ILD) and nine had PH and ILD (secondary PH). PAPsyst was measured non-invasively by Doppler cardiography. CGRP was determined by radioimmunoassay. RESULTS: Patients with PH had higher p-CGRP than patients with normal pressure. A positive relation was found between p-CGRP and PAPsyst and between p-CGRP and erythrocyte sedimentation rate (ESR), particularly in patients with isolated PH. CONCLUSION: In patients with SSc p-CGRP correlates with pulmonary pressure and with ESR. Whether CGRP reflects disease activity or is released secondary to pulmonary vasoconstriction needs to be investigated further.

Adult↗
PubMed2006

[Therapeutic management of systemic sclerosis].

UNLABELLED: Improved understanding of the pathophysiology of systemic sclerosis (SSc) opens new therapeutic avenues in its treatment. The efficacy of disease-modifying agents remains limited however, and none has yet demonstrated its ability to improve survival in a prospective randomized trial. RESULTS: of traditional antifibrotic agents such as colchicine and D-penicillamine are disappointing. Cyclophosphamide (CYC) seems to be beneficial in interstitial lung disease associated with SSc. Organ-specific therapies may produce dramatic benefits. Examples include angiotensin-converting enzyme inhibitors for renal failure and epoprostenol for primary pulmonary hypertension. Several new therapeutic approaches are currently under evaluation, including high-dose CYC followed by peripheral stem cell transplantation, vasodilators, and antiinflammatory and antifibrotic agents. Physical therapy and rehabilitation may help to treat disability and loss of function in SSc patients.

Acute Kidney Injury↗