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PubMed1987

Frequency, levels, and significance of blood eosinophilia in systemic sclerosis, localized scleroderma, and eosinophilic fasciitis.

Blood eosinophilia is a common feature of eosinophilic fasciitis and is variably reported in systemic sclerosis and localized scleroderma. Since these diseases share cutaneous fibrosis as the final outcome and have other clinical and pathologic features that are difficult to differentiate, the presence of blood eosinophilia may be a further source of confusion. In this study, we examined the frequency and level of blood eosinophilia in 715 patients with systemic sclerosis, 72 patients with localized scleroderma, and 22 patients with clinically active eosinophilic fasciitis. When defined as greater than 400 cells/mm3, eosinophilia was present in 7% of patients with systemic sclerosis, 31% of patients with localized scleroderma, and 83% of patients with eosinophilic fasciitis. Greater than 1000 eosinophils/mm3 were present less frequently in systemic sclerosis (1%) and localized scleroderma (8%) than in eosinophilic fasciitis (61%). No difference in the frequency of eosinophilia was present in patients with the limited cutaneous CREST syndrome or the diffuse cutaneous variety of systemic sclerosis, and in these patients the presence of eosinophilia did not correlate with the extent of cutaneous or internal organ involvement or with other laboratory abnormalities. Among patients with localized scleroderma, eosinophilia was more common in those with linear scleroderma and generalized morphea than in those with morphea, and both the frequency and level of eosinophilia were greater in individuals with clinically active disease (p less than 0.02). Eosinophilia was a persistent feature in untreated patients with active eosinophilic fasciitis, even up to 30 months of disease duration.

Eosinophilia↗
PubMed1996

A placebo-controlled study on urokinase therapy in systemic sclerosis.

A 4-month randomized placebo controlled trial on urokinase therapy in 36 consecutive systemic sclerosis patients randomly treated with urokinase or placebo was conducted. While patients on placebo did not show any significant improvement, in those following urokinase therapy there was a noticeable improvement in skin sclerosis observed via hand-print and ultrasonography of the skin. Vascular involvement improved: this was demonstrated by capillaroscopy results, showing an improvement in pattern and signs of revascularization and the resolution of skin ulcers. Vascular damage is a typical occurrence in systemic sclerosis cases and various vasoactive drugs are used symptoms for some such as Raynaud's syndrome or skin ulcers. At the moment these drugs seem to constitute the most effective therapy, and have few side effects. We have found only one previous study utilizing urokinase therapy for acute digital ischemia in systemic sclerosis. Our study is the first in which urokinase therapy has been used for the treatment of systemic sclerosis in a large number of patients.

Adult↗
PubMed1985

Mesangial function and glomerular sclerosis in rats with aminonucleoside nephrosis.

The possible relationship between mesangial dysfunction and development of glomerular sclerosis was studied in the puromycin aminonucleoside (PAN) model. Five male Wistar rats received repeated subcutaneous PAN injections; five controls received saline only. After 4 weeks the PAN rats were severely proteinuric (190 +/- 80 mg/24 hr), and all rats were given colloidal carbon (CC) intravenously. At 5 months glomerular sclerosis was found in 7.6 +/- 3.4% of the glomeruli of PAN rats; glomeruli of the controls were normal. Glomeruli of PAN rats contained significantly more CC than glomeruli of controls. Glomeruli with sclerosis contained significantly more CC than non-sclerotic glomeruli in the same kidneys. CC was preferentially localized within the sclerotic areas of the affected glomeruli. Since mesangial CC clearance from the mesangium did not change during chronic PAN treatment, we conclude that this preferential CC localization within the lesions is caused by an increased CC uptake shortly after injection in apparent vulnerable areas where sclerosis will develop subsequently. Cluster analysis showed a random distribution of lesions in the PAN glomeruli in concordance with the random localization of mesangial areas with dysfunction in this model. Similar to the remnant kidney model in PAN nephrosis the development of glomerular sclerosis may be related to "mesangial overloading."

Animals↗
PubMed1994

Insulin-like growth factor-I is partially responsible for fibroblast proliferation induced by bronchoalveolar lavage fluid from patients with systemic sclerosis.

1. Interstitial lung disease is a common complication of systemic sclerosis. The mechanism by which excess collagen is deposited in the lung is poorly understood, but is thought to involve release of mediators which activate lung fibroblasts. In this study we investigated and partially characterized the fibroblast proliferative activity of bronchoalveolar lavage fluid from 29 patients with systemic sclerosis, 19 with and 10 without evidence of lung disease assessed by thin-section computed tomography. 2. Bronchoalveolar lavage fluid from both groups of patients stimulated fibroblast proliferation compared with control subjects: systemic sclerosis with normal computed tomography, 27.7 (range 10.5-57.9)% above control; systemic sclerosis with abnormal computed tomography, 26.7 (range 5.0-47.8)% above control, P < 0.02 in both cases. 3. The activity was reduced by about one-third by neutralizing antibodies to insulin-like growth factor-1 but not platelet-derived growth factor. Levels of insulin-like growth factor-1 of bronchoalveolar fluid were increased in patients with systemic sclerosis [2.10 (range 1.10-3.48) ng/ml of bronchoalveolar lavage fluid] compared with controls [1.45 (range 1.10-2.05) ng/ml; P < 0.01]. When patients were subdivided into those with abnormal computed tomography [2.10 (range 1.20-3.48) ng/ml] and those with normal computed tomography [1.85 (range 1.10-2.90) ng/ml] only the values for the group with evidence of lung disease were increased compared with control subjects (P < 0.02). Platelet-derived growth factor could not be detected in bronchoalveolar lavage fluid from any group. Fractionation of bronchoalveolar lavage fluid demonstrated activity in several fractions consistent with the molecular masses of insulin-like growth factor-1 associated with binding proteins.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1998

Interferon-gamma in the treatment of systemic sclerosis: a randomized controlled multicentre trial.

We report the results of a randomized controlled multicentre study on interferon-gamma (IFN-gamma) treatment of systemic sclerosis as determined by skin sclerosis, renal and other organ involvement, global assessment, subjective symptoms and quality of life. Forty-four patients were enrolled into the trial, 27 in the treatment group and 17 in the control group. All patients presented with type I or type II scleroderma. Twenty-nine patients (64%) finished the study. The mean duration of Raynaud's phenomenon and skin sclerosis was 15.3 and 10.8 years, respectively. The skin scores tended to improve in the treatment group (P > 0.05). Mouth aperture increased significantly from 38.5 to 47.7 mm in the treatment group (P < 0.001). Subanalysis of IFN-gamma treated patients with normalized skin sclerosis scores >/=1 showed significant improvement in both skin involvement and subjective symptoms (P < 0.05). Organ involvement improved in eight of 18 treatment patients and in three of 11 control patients. It worsened in three of 18 treatment patients and in four of 11 control patients. One control patient died due to cardiorespiratory failure during the study. No deterioration of renal function occurred during IFN-gamma treatment. There was a significant improvement in quality of life parameters in the control group but not in the treatment group. Plasma levels of neopterin increased significantly during IFN-gamma treatment but not in the control group, whereas N-terminal procollagen III peptide levels did not change in either group. There was a high frequency of mild to moderate influenza-like adverse events during IFN-gamma treatment. Only four of nine drop-out patients, however, experienced symptoms most probably associated with IFN-gamma treatment. We conclude that IFN-gamma therapy has mild beneficial effects on skin sclerosis and disease-associated symptoms in type I and II scleroderma. IFN-gamma treatment was associated with acceptable tolerability and did not induce major renal dysfunction in our patients.

Adolescent↗
PubMed2003

Elevated exhalation of hydrogen peroxide in patients with systemic sclerosis.

BACKGROUND: Systemic sclerosis is accompanied by an influx of activated phagocytes into distal airways. These cells release H2O2, which may evaporate from the airways surface and be detected in expired breath condensate. We tested whether patients with systemic sclerosis exhale more H2O2 than healthy subjects and whether breath condensate H2O2 levels correlate with some clinical parameters. MATERIAL AND METHODS: H2O2 was measured fluorimetrically in the expired breath condensate of 27 patients (22 women, five men, mean age 49 +/- 13.1 years) with systemic sclerosis and 27 age- and sex- matched healthy controls. RESULTS: Exhaled H2O2 levels were 3.5-fold higher (0.88 +/- 0.62 microM vs. 0.25 +/- 0.17 microM, P < 0.001) in the patients with systemic sclerosis than in the controls. Treatment with cyclophosphamide and/or prednisone (29 +/- 50 months, range 3-168 months) did not significantly decrease H2O2 exhalation (0.78 +/- 0.50 microM, n= 10 vs. 0.94 +/- 0.67 microM, n= 17, P > 0.05). No significant difference was found between patients with limited and diffuse scleroderma (1.03 +/- 0.69 microM, n= 17 vs. 0.63 +/- 0.41 microM, n= 10, P > 0.05). H2O2 levels correlated with disease duration (r = 0.38, P < 0.05) and time from the first Raynaud's episode (r = 0.44, P < 0.05). CONCLUSIONS: Patients with systemic sclerosis exhale more H2O2 than healthy controls, suggesting involvement of reactive oxygen species in disease processes. Lack of significant intergroups differences in H2O2 levels may have resulted from the small number of patients analyzed.

Breath Tests↗
PubMed2000

Identification of Gal(beta 1-3)GalNAc bearing glycoproteins in cerebrospinal fluid of amyotrophic lateral sclerosis (ALS) patients.

Glycoproteins in cerebrospinal fluid of 55 patients with amyotrophic lateral sclerosis (ALS), six disease controls (multifocal motor neuropathy, sensorimotor neuropathy, Guillain-Barré syndrome, spinal muscular atrophy type II, motor neuropathy with monoclonal gammopathy) and 20 healthy controls were separated by PAGE electrophoresis and then detected immunochemically with peanut agglutinin (PNA). In 36 amyotrophic lateral sclerosis patients the 262 kDa glycoprotein was significantly increased (over the normal mean +/- SD x 2), which was associated with a decrease in the 114 kDa fraction. In the remaining patients, both fractions were either equal in concentration or the 114 kDa glycoprotein predominated. In normal cerebrospinal fluid, the 114 kDa glycoprotein predominated over the other glycoproteins. The total amount of separated glycoproteins was increased in 15 amyotrophic lateral sclerosis patients. In 12 of them it was followed by an increase in the percentage of the 262 kDa glycoprotein. There was no correlation between the content of the peanut agglutinin-labelled glycoproteins and the patients' age, duration and severity of the disease. There was a correlation between the 262 kDa glycoprotein being increased in cerebrospinal fluid and the electrophysiological pattern of denervation seen in electromyographic study. The glycoproteins change, similar to that occurring in amyotrophic lateral sclerosis patients, was also observed in one case of multifocal motor neuropathy (MMN). We suggest that in amyotrophic lateral sclerosis and multifocal motor neuropathy, the peanut agglutinin-labelled glycoproteins are released in excess from the nervous tissues into the cerebrospinal fluid as a result of neuronal degeneration. The question to be answered is, whether the released glycoproteins are becoming targets for auto-antibodies.

Adult↗
PubMed1982

[Morphological findings in the distal end of the oesophagus after sclerosis of bleeding varices (author's transl)].

The morphological changes in the distal oesophagus occurring after tube treatment, sclerosis and/or shunt operation for acutely bleeding oesophageal varices were investigated in 29 post mortems between March 1979 and April 1980. In addition three oesophagi were investigated after sclerosis several months previously. As was to be expected the oesophagi showed no specific changes after recent porto-caval shunts. In contrast there were definite histological differences after tube treatment as opposed to sclerosis. After compression tubes the oesophagi showed focal pressure necroses and ulcers. In the sclerosed oesophagi there frequent widespread, partially transmural, necroses, suppurative inflammation of the oesophageal wall and mediastinitis. Serious complications occurring after sclerosis were perforation with mediastinal abscess, pleuritis and severe paraoesophageal haemorrhage. In the prevention of complications after sclerosis both the injection technique and the choice of sclerosing agent are important.

Adult↗
PubMed1999

Association of aortic-valve sclerosis with cardiovascular mortality and morbidity in the elderly.

BACKGROUND: Although aortic-valve stenosis is clearly associated with adverse cardiovascular outcomes, it is unclear whether valve sclerosis increases the risk of cardiovascular events. METHODS: We assessed echocardiograms obtained at base line from 5621 men and women 65 years of age or older who were enrolled in a population-based prospective study. On echocardiography, the aortic valve was normal in 70 percent (3919 subjects), sclerotic without outflow obstruction in 29 percent (1610), and stenotic in 2 percent (92). The subjects were followed for a mean of 5.0 years to assess the risk of death from any cause and of death from cardiovascular causes. Cardiovascular morbidity was defined as new episodes of myocardial infarction, angina pectoris, congestive heart failure, or stroke. RESULTS: There was a stepwise increase in deaths from any cause (P for trend, <0.001) and deaths from cardiovascular causes (P for trend, <0.001) with increasing aortic-valve abnormality; the respective rates were 14.9 and 6.1 percent in the group with normal aortic valves, 21.9 and 10.1 percent in the group with aortic sclerosis, and 41.3 and 19.6 percent in the group with aortic stenosis. The relative risk of death from cardiovascular causes among subjects without coronary heart disease at base line was 1.66 (95 percent confidence interval, 1.23 to 2.23) for those with sclerotic valves as compared with those with normal valves, after adjustment for age and sex. The relative risk remained elevated after further adjustment for clinical factors associated with sclerosis (relative risk, 1.52; 95 percent confidence interval, 1.12 to 2.05). The relative risk of myocardial infarction was 1.40 (95 percent confidence interval, 1.07 to 1.83) among subjects with aortic sclerosis, as compared with those with normal aortic valves. CONCLUSIONS: Aortic sclerosis is common in the elderly and is associated with an increase of approximately 50 percent in the risk of death from cardiovascular causes and the risk of myocardial infarction, even in the absence of hemodynamically significant obstruction of left ventricular outflow.

Aged↗
PubMed2000

Ultraviolet A1 (340-400 nm) phototherapy for scleroderma in systemic sclerosis.

BACKGROUND: The presence of an inflammatory infiltrate consisting of helper T cells and a dysregulated matrix metabolism leading to excessive deposition of collagen are two pathogenetic factors responsible for the developments of fibrosis and sclerosis in patients with systemic sclerosis. In previous studies, ultraviolet A1 (UVA1) radiation phototherapy was shown to deplete skin-infiltrating T cells through the induction of T-cell apoptosis and to up-regulate the expression of matrixmetalloproteinase-1 (collagenase-1) in dermal fibroblasts. OBJECTIVE: Our purpose was to determine whether UVA1 phototherapy is effective for systemic sclerosis. METHODS: Lesional skin on the forearms of patients with systemic sclerosis (diffuse type, n =3; limited type, n =1) was exposed to medium-dose UVA1 radiation (60 J/cm(2)) daily. RESULTS: In all patients studied, UVA1 phototherapy-treated skin lesions were markedly softened after 9 to 29 exposures. Clinical improvement was associated with an increase in (1) joint passive range of motion values (P <.05), (2) skin temperature (thermography, P <.05), and (3) cutaneous elasticity (cutaneous elastometry, P <.05). Histologic evaluation of skin specimens obtained before and after UVA1 phototherapy revealed loosening of collagen bundles and the appearance of small collagen fibers. CONCLUSION: These studies indicate that UVA1 phototherapy is effective for patients with systemic sclerosis.

Aged↗
PubMed2000

Management of leg ulcers in patients with rheumatoid arthritis or systemic sclerosis: the importance of concomitant arterial and venous disease.

PURPOSE: We assessed the etiology and the prevalence of peripheral arterial and venous disease in leg ulcers in patients with rheumatoid arthritis and systemic sclerosis and analyzed the outcome after treatment of macrovascular disease. METHODS: A clinical study on 15 consecutive patients with chronic leg ulcers in collagen vascular disease (nine patients with rheumatoid arthritis, six patients with systemic sclerosis) was carried out in a referral center. Angiography was used when the ankle-arm index was less than 0.8; venography was used when venous reflux was detectable by means of a hand-held Doppler examination. Therapies included percutaneous transluminal angioplasty (seven patients), femoropopliteal bypass grafting surgery (one patient), saphenectomy of the greater saphenous vein (six patients), and split skin graft (11 patients). RESULTS: All patients with rheumatoid arthritis exhibited a multifactorial etiology of their ulcers: four of nine patients had peripheral arterial disease, and five of nine patients had venous insufficiency. In one of these patients, arterial and venous disease was combined. Five of six patients with systemic sclerosis exhibited a multifactorial etiology of their ulcers: three of six patients had peripheral arterial disease, and three of six patients had venous insufficiency. One of these patients had both arterial and venous disease. In patients with rheumatoid arthritis, healing was achieved in six of nine patients, and marked improvement occurred in two of nine patients. A below-knee amputation was necessary in one patient with rheumatoid vasculitis. In patients with systemic sclerosis, healing was achieved in three of six patients, and marked improvement occurred in the other three patients. CONCLUSION: Most leg ulcers in patients with rheumatoid arthritis and systemic sclerosis disclose a multifactorial etiology. Relevant arterial and venous disease can be found in approximately half the patients. Our study suggests that revascularization and vein surgery improve the healing of leg ulcers in patients with collagen vascular disease. A prospective trial is now required to confirm these results.

Aged↗
PubMed2004

Treatment of systemic sclerosis with the 5-HT3 receptor antagonist tropisetron.

BACKGROUND: There is no known disease-modifying therapy for progressive systemic sclerosis. OBJECTIVES: It was shown that a patient with secondary fibromyalgia syndrome for whom the development of systemic sclerosis was suspected because of a Raynaud's phenomenon and the presence of SCL-70 antibodies in the serum had experienced a clear pain reduction under treatment with tropisteron, which is the reason why this drug was also used with established systemic sclerosis. METHOD: Two patients with progressive systemic sclerosis and positive SCL-70 antibodies were treated for 6 weeks with 5 mg tropisetron daily. Both patients had clear skin symptoms, functional impairments of the locomotor system, and a secondary fibromyalgia syndrome. The skin score and joint motion were checked before, during, and after treatment. In addition, the patients filled in the visual analog scale for pain at these times. At the end of the 6 weeks, the patients showed a clear improvement of the skin score and the movability of various joints as well as a clear reduction of pain. The medication was well-tolerated. Constipation developed in the patients; it could be controlled with laxatives. Follow-up questioning of the patients after 3 months showed that their condition had remained stable. CONCLUSION: Two patients with progressive systemic sclerosis showed an improvement of various symptoms under a blockade of the 5-HT3 receptors via tropisetron. The long-lasting effect pointed to immunomodulation. The two cases give cause for clarifying this by means of clinical studies, which should also investigate the question of dosage (possibly 5 mg tropisetron twice daily).

Adult↗
PubMed1998

Mortality and causes of death of 344 Danish patients with systemic sclerosis (scleroderma)

OBJECTIVE: To determine survival, mortality and causes of death in Danish patients with systemic sclerosis (scleroderma), and to analyse how these parameters are influenced by demographic variables and the extent of skin involvement. METHODS: A cohort of 344 patients with incident systemic sclerosis (SSc) after 1 January 1960 was retrospectively identified, representing 3716 patient-years of follow-up. The vital status at the end of the study ultimo 1996 was established by reviewing the clinical charts and by contacting the Danish Central Person Register. The causes of death were based on information obtained from the hospital charts and the autopsy reports (80%) and death certificates (20%). The extent of skin sclerosis was described by means of a two-subset model (limited and diffuse involvement) and a three-subset model (digital, extremity and truncal involvement) in all patients. Expected numbers of deaths were calculated by means of age- and sex-specific mortality rates of the general Danish population. RESULTS: Crude mortality rates were 6.5 and 3.9%/yr, respectively, in men and women. The standardized mortality ratio (SMR) was 2.9 (95% CI 2.5-3.4) and was not significantly influenced by sex or age at disease onset. Mortality rates were significantly increased in patients with diffuse skin sclerosis (SMR 4.5, 95% CI 3.5-5.7) and patients under the age of 35 yr (SMR 13, 95% CI 2.7-37). Of the 160 patients who died during the study, 41 deaths (26%) were SSc related, accounting for one-third of the excess mortality. CONCLUSION: We found a significantly increased mortality in patients with SSc, particularly in the subset of patients with diffuse skin sclerosis and in young patients. The excess mortality was due to an increase in both the SSc-related mortality and the unrelated mortality. However, all other subsets also had a significantly increased risk of death, mainly due to an increased risk of death due to causes unrelated to SSc, cancer among others. The fact that the unrelated mortality in SSc was found to be 2-fold increased suggests that the excess mortality that was classified as unrelated should more appropriately be termed indirectly SSc related.

Adolescent↗
PubMed1984

Glomerular sclerosis in a renal isograft and identical twin donor. A family study.

Loss of renal mass has been associated with the development of glomerular sclerosis in animals and human beings. The pathophysiology of this renal injury is unknown, but glomerular sclerosis in animals can be aggravated or accelerated following exposure to nephrotoxic antibodies, puromycin aminoglycoside or renal irradiation. We describe here the outcome of the first renal transplant performed in the British Commonwealth. Glomerular sclerosis occurred in identical twins who were kidney donor and recipient, renal failure occurring 14 and 16 years after transplantation, respectively. Examination of these twins and all living immediate family members showed that six of the seven family members (both twins, their mother, and three sisters) had increased concentrations of circulating immune complexes, decreased total hemolytic complement, and low or borderline concentrations of C4. Only twins with single kidneys had detectable renal disease. Other preexisting causes of renal disease in these twins that would account for the glomerular sclerosis could not be identified. We suggest that a familial immune defect contributed to the development of glomerular sclerosis in these twins who were predisposed to renal disease due to loss of renal mass.

Adolescent↗
PubMed1997

Do patients with systemic sclerosis have abnormal gallbladder function?

OBJECTIVE: To determine gallbladder motility in patients with systemic sclerosis. DESIGN: Case control study. SETTING: University hospital, out-patient department of rheumatology. PATIENTS: Ten patients with systemic sclerosis according to the criteria of the American Rheumatism Association with documented involvement of the gastrointestinal tract and 10 healthy controls matched for age, sex and body mass index. INTERVENTION: Cephalic vagal cholinergic simulation by modified sham feeding and hormonal stimulation by infusion of cholecystokinin. MEASUREMENTS: Gallbladder volume obtained by ultrasonography and determination of plasma cholecystokinin concentrations. RESULTS: Fasting gallbladder volumes were not significantly different between patients with systemic sclerosis and controls (19.6 +/- 1.9 cm3 and 23.3 +/- 2.9 cm3, respectively, mean plus or minus standard error of the mean). Neither were there significant differences in reduction of gallbladder volume in response to modified sham feeding (35 +/- 4% and 33 +/- 4%, respectively) nor during cholecystokinin infusion (56 +/- 4% and 60 +/- 6%, respectively). The increase in plasma cholecystokinin levels during infusion was not different in the two groups. CONCLUSION: Gallbladder motility in patients with systemic sclerosis is preserved in response to both cholinergic and hormonal stimulation, even when other gastrointestinal motor disturbances are present. These results suggest that patients with systemic sclerosis are not at increased risk for cholelithiasis because of gallbladder dysmotility.

Case-Control Studies↗
PubMed1990

A possible role for transforming growth factor-beta in systemic sclerosis.

The cause of systemic sclerosis remains unknown, but cellular and molecular mechanisms possibly responsible for the characteristic clinical manifestations of fibrosis and vascular damage (Raynaud's phenomenon, telangiectasis, digital infection, and renal arteriopathy) are becoming understood in greater detail. One possibly important cytokine is transforming growth factor-beta (TGF-beta); its involvement is reviewed here. With regard to vascular lesions, TGF-beta has variably been shown to inhibit endothelial cell growth in vitro but to promote angiogenesis in vivo, a paradox that remains unresolved. Nonetheless, an injurious activity of TGF-beta on microvascular endothelial cells could help to explain the intimal proliferation and microvascular obliteration seen. Whether as a result of or as a cause of endothelial cell damage, platelet activation has been well documented in systemic sclerosis and the platelet alpha granule pool contains a large quantity of TGF-beta. TGF-beta is also produced by activated macrophages and T cells, both of which are known to occur within systemic sclerosis lesions. An important effect of TGF-beta is its stimulation of fibroblast collagen and fibronectin synthesis and their deposition into the extracellular matrix. Stimulation by TGF-beta may therefore account for the fibrosis seen in the dermis and in the internal organs. Direct evidence of TGF-beta involvement in systemic sclerosis is scanty, and awaits discovery of either an abnormal expression of or response to TGF-beta. The biologic effects of TGF-beta appear to be regulated at the level of activation from a latent polypeptide precursor form. Descriptions of the importance of this cytokine in pathologic conditions will need to account for this activation and its regulation. Nonetheless, the physiologic effects so far attributed to TGF-beta make its involvement in systemic sclerosis an attractive possibility to explain some of the manifestations of this enigmatic disease.

Chemotactic Factors↗
PubMed2006

Urticarial vasculitis appearing in the progression of systemic sclerosis.

We report a case of urticarial vasculitis that appeared during the course of limited cutaneous systemic sclerosis. The urticarial lesions responded to systemic administration of prednisolone. After the appearance of urticarial vasculitis, the progression of scleroderma in distal sites of her elbow and knee joint became apparent. We consider this case to be consistent with limited cutaneous systemic sclerosis. The patient started treatment with prednisolone and her edema as well as scleroderma softened gradually. We analyzed, by immunohistochemistry, the number of tryptase-positive mast cells of this case in the lesions of urticarial vasculitis as well as systemic sclerosis. The number of tryptase-positive mast cells in the lesions of urticarial vasculitis as well as systemic sclerosis was significantly increased compared to normal skin (P < 0.05 and P < 0.005, respectively). We demonstrate that, in the present case, mast cells might be involved in both courses of urticarial vasculitis and systemic sclerosis as a common factor.

Diagnosis, Differential↗
PubMed1991

Hydroxyproline in the oesophageal mucosa of patients with progressive systemic sclerosis during omeprazole-induced healing of reflux oesophagitis.

Hydroxyproline concentration in oesophageal mucosal biopsies was used as an index of collagen in an attempt to evaluate the potential for stricture formation in patients with progressive systemic sclerosis. Eight patients suffering from progressive systemic sclerosis with complicating gastro-oesophageal reflux, 8 patients with idiopathic gastro-oesophageal reflux, and 7 normal controls were compared. Acid gastro-oesophageal reflux was assessed with 24-h pH-metry; degree of oesophagitis was evaluated both endoscopically and histopathologically. The patients with progressive systemic sclerosis were investigated at the start of the study and later when healing of oesophagitis was accomplished with omeprazole therapy. The hydroxyproline concentration was significantly increased (P less than 0.01) in the oesophageal mucosa from patients with progressive systemic sclerosis (median 21.8 nmol/mg) as compared to patients with idiopathic gastro-oesophageal reflux (median 6.4 nmol/mg) and normal controls (median 8.1 nmol/mg). Hydroxyproline concentration in oesophageal mucosa from patients with progressive systemic sclerosis decreased significantly and to a normal level (median 6.5 nmol/pg; P = 0.014) when healing of oesophagitis was achieved.

Adult↗