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PubMed2005

Cytokine profile and proteome analysis in bronchoalveolar lavage of patients with sarcoidosis, pulmonary fibrosis associated with systemic sclerosis and idiopathic pulmonary fibrosis.

The aim of this study was to analyze the type of immune response (Th1, Th2) and protein composition of bronchoalveolar lavage (BAL) of patients with sarcoidosis, pulmonary fibrosis associated with systemic sclerosis (SSc) and idiopathic pulmonary fibrosis (IPF). Flow cytometry analysis of intracellular cytokines revealed different patterns: in IPF and SSc Th2 profiles were predominant, whereas in sarcoidosis Th1 prevailed. The proteomic analysis of BAL fluid (BALF) showed that there were quantitative differences between the three diseases. These were more evident between sarcoidosis and IPF, confirming our previous observations, whereas SSc had an intermediate profile between the two, however with some peculiarities. Comparison of BALF protein maps, constructed with the same quantity of total proteins, enabled us to identify the main profiles of the three diseases: an increase in plasma protein prevalent in sarcoidosis and also present in SSc, though for fewer proteins with respect to IPF and a greater abundance of low molecular weight proteins, mainly locally produced, in IPF. These findings are in line with the different pathogenesis of these diseases: IPF is considered a prevalently fibrotic disorder limited to the lung, with intense local production of functionally different proteins, whereas sarcoidosis and SSc are systemic immunoinflammatory diseases.

Adult↗
PubMed1995

Elevated plasma endothelin levels in systemic sclerosis.

Endothelin is a novel potent vasoconstrictor peptide produced mainly by endothelial cells. Thrombomodulin is a high-affinity thrombin receptor on vascular endothelial cells that plays an important role as a natural anticoagulant. In this study, we measured plasma levels of endothelin and thrombomodulin in patients with systemic sclerosis or Raynaud's disease. Plasma levels of endothelin and the ratio of thrombomodulin to creatinine were significantly increased in patients with systemic sclerosis compared with normal controls, and there was a positive correlation between these two indicators (r = 0.615, P = 0.004). Moreover, plasma levels of endothelin were significantly higher in patients with diffuse systemic sclerosis than in patients with limited systemic sclerosis. In contrast, plasma levels of endothelin in patients with Raynaud's disease were not significantly increased. These results suggest that increased plasma levels of endothelin and thrombomodulin may reflect microvascular damage in systemic sclerosis.

Adult↗
PubMed1996

Measurement of skin elastic properties with a new suction device (II): systemic sclerosis.

We previously reported the skin elastic properties of normal subjects as measured by a newly developed suction device (6). In the present report, we examined the skin elastic properties of 62 patients with systemic sclerosis (SSc), clinically diagnosed as limited or diffuse type. We measured the skin elastic properties on the forearm and chest and investigated correlations with disease type and clinical skin score. Patients with the diffuse type SSc showed significantly lower values than those with the limited type SSc or normal subjects in distention and retraction ability at the chest and forearm. This suction device is clinically useful for the objective evaluation of skin elastic properties in diffuse systemic sclerosis.

Adult↗
PubMed2005

Treprostinil for the treatment of severe digital necrosis in systemic sclerosis.

We report a case of severe digital ulcerations associated with systemic sclerosis, successfully treated with treprostinil (Remodulin). There was improvement within days of the treatment initiation; complete healing was accomplished after 16 weeks of therapy. Patients with systemic sclerosis and peripheral small vessel disease have limited therapeutic options. Treprostinil is a prostacyclin analogue that can be delivered by subcutaneous infusion and is approved in the USA only for treatment of primary pulmonary hypertension. This report provides an impressive example of an alternative, complementary indication for the use of treprostinil.

Adult↗
PubMed2000

Nailfold videocapillaroscopy assessment of microvascular damage in systemic sclerosis.

OBJECTIVE: To correlate microvascular abnormalities, evaluated by nailfold videocapillaroscopy (NVC), with the duration of both Raynaud's phenomenon (RP) and systemic sclerosis (SSc) from the date of diagnosis, in a large number of patients with SSc. METHODS: Ninety-seven consecutive patients were recruited and distributed into 3 groups on the basis of the morphological NVC patterns observed: "early" (E), "active" (A), and "late" (L). In each group the age of patients, age at onset, and the duration of RP as well as of overt SSc were investigated and correlated with the different NVC pattern variables. RESULTS: The early appearance of giant capillaries and hemorrhages (E pattern) is of great relevance for the early diagnosis of SSc. Therefore, these alterations are more evident in the active phase of the disease (A pattern). Conversely, the NVC observation of loss of capillaries and vascular architectural disorganization and the presence of ramified/bushy capillaries (L pattern) represents the clearest aspect of advanced SSc microvascular damage. These morphological alterations were found to correlate significantly with the duration of both RP and SSc, as well as with age of patients (p = 0.0001). No significant differences were observed when the variables were analyzed in the patients classified as having limited cutaneous SSc or diffuse cutaneous SSc. CONCLUSION: Classification of defined major nailfold patterns may be useful in assessing the appearance and progression of sclerodermic microangiopathy. As well, nailfold changes might represent a morphological reproduction of the evolution of SSc.

Female↗
PubMed2001

Relationship between parity and clinical and biological features in patients with systemic sclerosis.

OBJECTIVE: To assess the influence of parity on the clinical and biological features of systemic sclerosis (SSc). METHODS: We recorded the following clinical and biological data of 100 consecutive women with SSc: age, disease duration before diagnosis, cutaneous extension of sclerosis according to LeRoy's classification, pulmonary involvement, and antinuclear antibodies. We compared these features to the number and sex of children who were born before SSc onset. Date of birth of the first children was systematically recorded. RESULTS: Patients with limited SSc had more children before SSc onset than patients with diffuse SSc (2.4 +/- 1.8 vs 1.7 +/- 1.5; p < 0.05). The interval between first birth and SSc onset was shorter for patients with limited SSc than for patients with diffuse SSc (11.0 +/- 9.9 vs 23.5 +/- 14.5 yrs; p < 0.01). Patients with pulmonary fibrosis had more children than patients without pulmonary fibrosis (2.5 +/- 1.9 vs 2.0 +/- 1.6; p < 0.05). Age at first birth was significantly higher when the child was a girl than a boy (26.8 +/- 7.5 vs 22.9 +/- 5.3 yrs; p < 0.05). The interval between the first birth and SSc onset was shorter when the child was a girl than a boy (16.2 +/- 9.6 vs 25.4 +/- 13.4 yrs; p < 0.05). CONCLUSION: Pregnancy related microchimerism could be preferentially associated with limited SSc and pulmonary fibrosis. Microchimerism may be facilitated in cases in which the fetus is female.

Adult↗
PubMed1991

Cutaneous and serologic subsets of systemic sclerosis.

The relevance of the extent of skin sclerosis and of other clinicoserological features in diagnosis, severity and prognosis of disease was studied in a large number of unselected patients with systemic sclerosis (SSc). One hundred and fifty-one patients with SSc (126 F and 25 M, mean age 48 +/- 14 SD) followed for 5.3 +/- 3.2 years were included. Patients were divided into 3 cutaneous subsets: limited (68), intermediate (46) and diffuse SSc (37). Serological markers were detected in 288 patients with Raynaud's phenomenon and other connective tissue diseases (CTD). Limited and intermediate SSC prevailed in female patients while the diffuse subset was more frequent in males (p less than 0.0001). Duration of Raynaud's phenomenon before disease onset was shorter in the diffuse variant (p less than 0.0001). A wider cutaneous involvement was associated with more severe forms of SSc. Diffuse subset showed the poorest prognosis at 10 years of followup compared with intermediate (p less than 0.05) and limited variant (p less than 0.001). Intermediate SSc seems a distinct variant of SSc on the basis of clinical manifestations and survival. Among serological markers, anticentromere, anti-Scl-70 and antinucleolar antibodies were found in 21, 40 and 27% of the cases, respectively; these were statistically less frequent (p less than 0.0001) in other CTD. In 83.5% of patients with SSc at least one of these specific markers was recorded. Anticentromere antibodies were correlated to sex (female), limited SSc, calcinosis and telangiectasia. On the contrary anti-Scl-70 was associated with diffuse and intermediate subsets and with more severe SSc manifestations.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed2006

[Rare autoimmune rheumatic illnesses during pregnancy. Systemic sclerosis, polymyositis/dermatomyositis and vasculitis].

Autoimmune rheumatic diseases (ARD) affect young females during childbearing age. Over the last decades, improvements in survival as well as quality of life in patients affected with ARD have led to an increased number of pregnancies observed during the course of such diseases. Systemic lupus erythematosus (SLE) is the most frequently observed ARD during pregnancy, and the immunoendocrine changes occurring during pregnancy may influence the course of this disease. Pregnancy can also occur in patients with rare ARD, namely systemic sclerosis, polymyositis/dermatomyositis, systemic vasculitis including Wegener's granulomatosis, Churg-Strauss syndrome, polyarteritis nodosa, microscopic polyangiitis, Takayasu arteritis and Behçet disease. This review focuses on the complications during pregnancy caused by these rare ARD, and we briefly discuss the data published on these disorders. Some guidelines for the management of these conditions during pregnancy will also be provided. However, it is important to note that data on pregnancy outcome are very limited and, in the absence of prospective studies, most of the information derives from case reports and retrospective studies.

Adult↗
PubMed2004

Overview of treprostinil sodium for the treatment of pulmonary arterial hypertension.

Pulmonary arterial hypertension is a life-threatening disorder that refers to a group of diseases characterized by an abnormal elevation of the blood pressure within the pulmonary circulation due to a vasculopathy of the pulmonary microcirculation (1). If left untreated, the overall prognosis of pulmonary arterial hypertension is poor, with a 5-year survival rate of 34%. The most common cause of death is progressive right-sided heart failure (2). There is no pharmacologic cure for pulmonary arterial hypertension, and a team approach is required for the proper care of these patients (3). Treatment is directed at improving clinical symptoms, increasing exercise tolerance and extending survival. Until just a few years ago, standard treatment options for the treatment of pulmonary arterial hypertension were limited and included coumarin derivatives, calcium channel blockers, diuretics, digoxin and oxygen supplementation (4). In the last decade, the therapeutic options for pulmonary arterial hypertension have made considerable advances. In at least three major randomized controlled trials, the continuous intravenous infusion of epoprostenol, a synthetic salt of prostacyclin with potent vasodilatory ability, has been shown to improve exercise tolerance, hemodynamics, survival and quality of life in patients with New York Heart Association (NYHA) functional classes III and IV with either primary pulmonary hypertension or pulmonary arterial hypertension associated with scleroderma (5-9). Epoprostenol has now become a mainstay therapy in the long-term treatment of primary pulmonary hypertension or pulmonary arterial hypertension associated with scleroderma. Nevertheless, epoprostenol is far from an ideal form of therapy. Issues such as the short half-life of the drug, need for continuous central intravenous access and significant side effects have led to the development of more stable prostacyclin analogues as alternative therapies for primary pulmonary hypertension. These alternative therapies include iloprost (inhaled delivery) and treprostinil (subcutaneous delivery). As a result, the Food and Drug Administration (FDA) recently approved treprostinil for the treatment of pulmonary arterial hypertension in patients with NYHA classes II-IV. This review will offer a discussion of the basic pharmacology of treprostinil, its similarities to and differences from epoprostenol, the animal studies as well as the initial investigational studies leading to its FDA approval, and clinical uses of the drug alone or in combination with newer therapies directed at pulmonary arterial hypertension developed over the last decade.

Antihypertensive Agents↗
PubMed1995

Thoracoscopy. A prospective study of safety and outcome.

OBJECTIVE: To assess a standard classification of adverse events and evaluate the safety and long-term outcome of thoracoscopy in patients with pleural disease. DESIGN: Prospective nonrandomized cohort study. SETTING: The Pulmonary Special Procedures and Nd:YAG Laser Unit of the University of California San Diego Medical Center, San Diego. PATIENTS: Fifty consecutive patients undergoing thoracoscopy for diagnosis of pleural effusion or thickening, pleurodesis, or empyema drainage. INTERVENTION: A list of major and minor adverse events that could be temporally related to thoracoscopy performed for diagnosis or treatment of pleural processes was established before beginning this prospective study. Procedures were performed using multiple point-of-entry techniques and either local or general anesthesia. Most procedures were performed in the operating room using double-lumen intubation. Patients were seen daily during hospitalization and at least 7, 30, and 90 days after thoracoscopy. Long-term follow-up data were obtained by telephone calls, clinical visits, or medical chart reviews or all of these, until 12 months after procedures. MEASUREMENTS: Occurrence of major and minor adverse events possibly related to thoracoscopy was recorded prospectively. Demographic and clinical data, as well as efficacy and outcome after thoracoscopy, were also noted. RESULTS: Fifty-two procedures were performed in 50 patients. Median age was 60 years (range, 18 to 88 years). Thoracoscopy provided a diagnosis in 93% of patients with pleural disease of unclear origin. Pleurodesis by thoracoscopic talc insufflation was successful in 95% of cases and in 91% of patients with malignant pleural effusions still available for evaluation and follow-up examination 3 months after pleurodesis. Thoracoscopic drainage of empyema was successful in six of seven patients and led to referral for open decortication in one. There were no procedure-related deaths or intraoperative accidents. Open-chest surgery intervention was never required. Only 1 major adverse event occurred: a patient with scleroderma and trapped lung had recurrent pleural effusion requiring chest tube drainage 1 week after hospital discharge. Minor adverse events, however, were noted in ten instances (19%). These included fever after talc pleurodesis, asymptomatic pneumothorax after chest tube removal, and minor would infection in a patient with empyema. CONCLUSION: Using the proposed classification of major and minor adverse events, prospective evaluation demonstrated the safety, diagnostic utility, and long-term efficacy of thoracoscopy performed for diagnosis and management of pleural processes. Potential dangers, however, of thoracoscopy are acknowledged, and limitations of the procedure are addressed.

Adolescent↗
PubMed2005

Bosentan: new preparation. Pulmonary hypertension: an option before epoprostenol infusion.

(1) Pulmonary hypertension is a progressive disorder characterised by abnormally high pulmonary artery pressure, leading to right ventricular failure and death, generally after a few years. (2) Treatment usually combines an anticoagulant, oxygen therapy, a diuretic and, sometimes a calcium channel blocker. Efficacy is poorly documented but appears to be limited. Long-term treatment with epoprostenol (prostacyclin), given as a continuous intravenous infusion via a central catheter and pump improves the quality of life of patients with severe pulmonary hypertension (NYHA classes III and IV, associated with discomfort during daily activities or even at rest). The impact of this treatment on survival seems to depend on the type of pulmonary hypertension: benefits were seen in a trial in patients with idiopathic pulmonary hypertension, while there was no effect in another trial in patients with pulmonary hypertension associated with scleroderma. Epoprostenol causes numerous adverse effects, some of which can be severe. (3) Marketing authorisation was recently granted in Europe for oral bosentan therapy for pulmonary arterial hypertension severe enough to restrict daily activities. (4) Two placebo-controlled trials lasting 12 to 16 weeks included 32 and 213 patients who responded inadequately to calcium channel blockers. They showed that the 6-minute walking distance improved by a median of 76 metres and 44 metres, respectively. In the trial with 213 patients, 42% of patients in the bosentan group and 30% of those in the placebo group were improved according to the NHYA scale. No impact on survival was observed in these short trials. (5) A trial in 33 patients showed no difference in symptom improvement between groups treated with intravenous epoprostenol + oral bosentan or intravenous epoprostenol + oral placebo. (6) Two adverse effects require monitoring, namely anemia and elevated transaminase activity. Bosentan carries a risk of multiple pharmacokinetic interactions and is teratogenic in animals. Women taking bosentan cannot use hormone-based contraception, because of a pharmacokinetic interaction. (7) In practice, oral bosentan improves symptoms in patients whose daily activities are restricted by pulmonary arterial hypertension. Bosentan is easy to use, making it an option before continuous intravenous epoprostenol infusion, even though it may not be as effective.

Administration, Oral↗
PubMed1989

Pulmonary arterial histology and morphometry in systemic sclerosis: a case-control autopsy study.

Morphometric measurements were performed on pulmonary arteries in 58 patients with systemic sclerosis (20 limited cutaneous and 38 diffuse cutaneous involvement [21 with and 17 without renal crisis]) and age, race, and sex matched autopsy controls. Matched pairs analysis was employed. For arteries of all sizes, the area of the intima and percent luminal occlusion were greater in the limited and diffuse (no renal crisis) groups than in controls, and these differences were statistically significant for large and medium sized vessels. The greatest luminal occlusion was found in limited cutaneous patients, and especially those with clinical evidence of pulmonary arterial hypertension, providing a rationale for the poor response to vasodilator therapy in these patients.

Constriction, Pathologic↗
PubMed1997

[Anticentromere antibodies and lung disease without skin involvement].

Anticentromere antibodies are closely related to systemic sclerosis and basically to limited cutaneous form. Two patients with different forms of lung disease, positive anticentromere antibodies and absence of the characteristic skin involvement of the systemic sclerosis are presented.

Aged↗
PubMed2006

Endothelin axis polymorphisms in patients with scleroderma.

OBJECTIVE: To evaluate the distribution of polymorphisms in the endothelin 1 (EDN1), endothelin receptor A (EDNRA) and endothelin receptor B (EDNRB) genes in systemic sclerosis (SSc; scleroderma) and SSc subsets. METHODS: Two hundred five patients with SSc and 255 healthy controls were screened for polymorphisms in EDN1, EDNRA, and EDNRB, using sequence-specific primer-polymerase chain reaction. The polymorphisms studied were at the following positions: for EDN1, -1370 (T-1370G) of the promoter, +138 of exon 1 (+138 A/-), +85 of exon 3 (E106E), and +23 of exon 5 (K198N); for EDNRA, -231 of exon 1 (G-231A), and +69(H323H) and +105 (E335E) of exon 6; for EDNRB, +2841 of exon 2 (EDNRB-3), -2547 of exon 3 (EDNRB-2), and -2446 of exon 3 (EDNRB-1). RESULTS: No significant differences between the SSc group as a whole and control subjects were observed for any of the investigated polymorphisms in EDN1, EDNRA, and EDNRB. However, compared with patients with limited cutaneous SSc, patients with diffuse skin involvement had an increased frequency of allele carriage of EDNRB-1A (76.8% versus 54.4%; P = 0.002), EDNRB-2A (79.7% versus 60.2%; P = 0.006), and EDNRB-3G (79.7% versus 56.6%; P = 0.001). Significantly increased carriage frequencies for EDNRA alleles H323H/C and E335E/A were observed in SSc patients with anti-RNA polymerase (anti-RNAP) antibodies, compared with both anti-RNAP-negative SSc patients (P < 0.05) and control subjects (P < 0.005). CONCLUSION: The finding of associations between endothelin receptors A and B and distinct clinical and immunologic SSc subsets supports the role of endothelin and its receptors in the pathogenesis of SSc. However, these findings and their functional significance need to be confirmed and investigated in future studies.

Endothelins↗
PubMed1993

Cyclosporine in systemic sclerosis. Results of a forty-eight-week open safety study in ten patients.

OBJECTIVE: To evaluate safety and efficacy of cyclosporin A (CSA) treatment in systemic sclerosis (SSc). METHODS: Ten patients with < or = 60 months of SSc were entered into a 48-week open study of CSA. Patients with hypertension or azotemia were excluded. Concurrent use of nonsteroidal antiinflammatory agents or diuretics was not permitted. The extent of cutaneous and visceral involvement at 48 weeks and at study entry were compared. RESULTS: Adverse reactions (especially nephrotoxicity) were frequent, usually transient, often dose-limiting, and usually associated with CSA doses > or = 3-4 mg/kg/day. Skin thickening decreased significantly (P < 0.001), while pulmonary and cardiac involvement remained unchanged. CONCLUSION: CSA should undergo blinded controlled study in patients with SSc.

Adult↗
PubMed2003

New insights into the pathogenesis of systemic sclerosis.

Systemic sclerosis (SSc) is a connective tissue disorder characterized by vascular abnormalities and excessive collagen synthesis. Extracellular matrix overproduction by fibroblasts results from abnormal interactions among endothelial cells, mononuclear cells (lymphocytes and monocytes) and fibroblasts, in a setting of vascular hyperreactivity and tissue hypoxia. Many autoantibodies have been identified in the sera of SSc patients; some of them are specific to the disease, such as anti-centromere antibodies in limited SSc, anti-topoisomerase 1 and anti-RNA polymerase I/III antibodies in diffuse SSc. Their pathogenetic role(s) remains uncertain. However, genetic, environmental and possibly alloreactive factors might also contribute to disease susceptibility.

Autoantibodies↗
PubMed1979

Identification of specific antibodies to extractable nuclear antigens by passive immunodiffusion.

Extractable nuclear antigens have been identified in a number of systemic rheumatic diseases and may be useful in differentiating those with overlapping clinical features. A number of detection systems have been described but technical feasibility and sensitivity have limited widespread application. We describe a simple immunodiffusion method to identify extractable nuclear antigens with high specificity and sensitivity that compares favorably with parallel hemagglutination studies. This method can be applied easily to routine analysis of antinuclear antigens.

Antibodies, Antinuclear↗