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PubMed1991

[Changes in the lungs in progressive systemic scleroderma].

In 24 patients with progressive systemic scleroderma the respiratory disturbances, X-ray, scintigraphic and clinical deviations as well as the statistical correlations between them are described. The functional examination of respiration revealed lowered diffusion capacity, restrictive type of ventilatory failure and hypoxemia as the most frequent disturbances. The functional examination of respiration is correlated with the X-ray changes in the lungs in these patients.

Acid-Base Equilibrium↗
PubMed2003

Tuberculous fasciitis in scleroderma.

A 25-year old woman visited the hospital because of a painful swelling for 20 days in the midposterior portion of her right thigh. She had been diagnosed with systemic sclerosis and treated for 10 months. An MRI scan of the right thigh showed diffuse fascial thickening and involved the superficial portion of thigh muscles, which were hyperintense on the T(2)-weighted image. A biopsy of the involved muscles revealed chronic granulomatous inflammation with several acid-fast bacilli on a Ziehl-Neelsen stain. We here report a case of tuberculous fasciitis manifest with painful swelling of the midposterior muscles of the right thigh without pulmonary tuberculosis in a patient with scleroderma.

Adult↗
PubMed2003

Evaluation of caspase 1 and sFas serum levels in patients with systemic sclerosis: correlation with lung dysfunction, joint and bone involvement.

Recent studies point out at the role of apoptosis disturbances in the development of systemic sclerosis(SSc). The aim of our study was to examine caspase 1 and sFas serum levels in scleroderma patients and correlate the obtained results with skin involvement and internal organ changes. We studied 29 patients (14 with limited and 15 with diffuse SSc). The extension of skin involvement was measured using Total Skin Score (TSS). Internal organ involvement was assessed by specialist procedures. Serum caspase 1 and sFas levels were measured by enzyme-linked immunosorbent assay. We found correlation between sFas serum level and duration of Raynaud's phenomenon and TSS; caspase 1 serum level correlated only with TSS. Correlations between caspase 1 and lung dysfunction and sFas levels with joint and bone involvement in SSc patients were also observed. The obtained results revealed that disturbances of apoptosis might play a role in SSc pathogenesis. Caspase 1 and sFas serum levels correlate with the skin involvement severity, lung dysfunction, joint and bone changes.

Adolescent↗
PubMed1989

Immunohistologic demonstration of platelet-derived growth factor (PDGF) and sis-oncogene expression in scleroderma.

Although the pathogenesis and mechanisms responsible for excessive connective tissue deposition are not known, it has been thought that specific growth factors may have an effect on scar formation by increasing the fibroblast population and by affecting the amount and types of matrix synthesized. In this regard, we explored the appearance and localization of TGF alpha, TGF beta, PDGF, and sis-onc expression in situ. Sections of skin biopsies from eight scleroderma patients were investigated using specific antibodies to TGF alpha, TGF beta, human PDGF, and sis-onc products for immunohistochemistry. Most significantly, deposition of PDGF was detected in the endothelial lining of small capillaries in association with certain mononuclear cells of the perivascular infiltrates. In particular, strong labeling was observed in the cytoplasm of macrophages. Smooth muscle also appeared to be specifically labeled. Similarly, sis-onc product localized in the same areas. No significant staining was observed with antibodies to TGF alpha. TGF beta was found rather diffusely throughout the dermal connective tissue and was only occasionally observed in capillaries of lesions. We conclude that the PDGF may play an important role in the pathogenesis of scleroderma.

Humans↗
PubMed1975

Tryptophan metabolism in skin diseases.

In a number of skin disturbances conditioned or aggravated by sunlight and/or mainly diffused to the cutaneous connective tissue (present acquired pellagra, lupus erythematosus, porphyria cutanea tarda, actinic reticuloid, Rothmund-Thomson syndrome, lymphocytoma cutis, scleroderma, dermatomyositis, burns, linphomas, parapsoriasis, acrodermatitis enteropathica) excretive changes were found only in the "via kynurenine" metabolites. As a rule kynureniase activity was reduced and tryptophan-pyrrolase activity was increased. In the epidermis tryptophan leads to niacin pathway was found to be present and sometimes autonomous.

Animals↗
PubMed2005

Severe gastrointestinal involvement in systemic sclerosis: report of five cases and review of the literature.

OBJECTIVE: To review current literature on the gastrointestinal tract (GIT) manifestations of systemic sclerosis (SSc) and to report on 5 patients with severe gastrointestinal SSc. MATERIALS AND METHODS: The clinical course and histopathology of 5 patients are described. A review of the medical literature registered in MedLine and PreMedLine databases from 1996 through mid-2004 was performed using the keywords systemic sclerosis and scleroderma and combining them with text words such as gastric, gastrointestinal, anorectal, colonic, and hepatic. RESULTS: All 5 patients had severe GIT involvement: 4 with diffuse cutaneous SSc (dcSSc) and 1 with limited cutaneous SSc (lcSSc). Autopsy results of 2 patients who died from severe malnutrition and aspiration pneumonia are presented. Literature review includes involvement from oral cavity to anus with varying degrees of severity. Most GIT manifestations result from dysmotility secondary to infiltration of the gastrointestinal wall with fibrous tissue and can cause life-threatening malabsorption and malnutrition. Diagnostic tests, pathology, and treatments of GIT SSc are reviewed. CONCLUSIONS: GIT involvement in SSc can be severely debilitating and even life-threatening. Although morbidity is inevitable, appropriate supportive treatment can prolong survival. RELEVANCE: GI complications of SSc cause significant morbidity and mortality.

Adult↗
PubMed2004

[Long-term effects of sildenafil in a patient with scleroderma-associated pulmonary hypertension and Raynaud's syndrome].

HISTORY: A 65-year-old woman was admitted because of dyspnea at rest and peripheral edema due to scleroderma-associated pulmonary fibrosis and hypertension, as well as Raynaud's phenomenon. DIAGNOSTIC FEATURES, TREATMENT AND COURSE: She had a marked restrictive ventilatory disorder with severe impairment of diffusion capacity. Right heart catheterization demonstrated a mean pulmonary artery pressure of 50 mmHg. She was able to walk only 220 m. All usual methods of treatment failed to give satisfactory results so that sildenafil (phospherodiesterase type-5 |PDE-5| inhibitor; Viagra ((R)) was given, even though it is not licensed for this indications ("off-label", as a therapeutic attempt. This achieved definite reduction in pulmonary arterial pressure and significantly improved the clinical symptoms. In particular, it drastically reduced the level of atrial natriuretic peptide, an important prognostic marker in right heart failure. Sildenafil also significantly raised peripheral perfusion and the signs of Raynaud's syndrome. CONCLUSION: PDE-5 inhibitors are efficacious in scleroderma-associated pulmonary hypertension and may also provide a new option in the treatment of Raynaud's disease.

3',5'-Cyclic-GMP Phosphodiesterases↗
PubMed1986

Scleroderma in childhood.

Scleroderma is a rare disease in children. Heterogeneous in its many forms, it ranges from circumscribed and self-limited pigmentary disorders to disabling and disfiguring involvement of an extremity. It also may include diffuse skin disease, multiple internal organ derangements, and a rapidly fatal outcome. Outcome varies widely and is related to the location and extent of cutaneous involvement, evaluation of disfigurement or impairment, and which treatment resources are available.

Child↗
PubMed1998

[Progressive systemic scleroderma--prognosis determining involvement of internal organ systems].

Prognosis of systemic sclerosis (scleroderma, Ssc) is largely depending on involvement of internal organs. Abnormalities of the gastrointestinal tract are found most frequently (85%), especially decreased motility of the oesophagus, which has little impact on the longterm clinical course of Ssc. Pulmonary manifestations can be demonstrated in 40-90% of patients; one must distinguish between pulmonary hypertension or fibrotic lung disease. The heart is affected in 50% of cases. Patchy or diffuse myocardial fibrosis, as well as pericarditis and pericardial effusions can induce symptoms of arrhythmia or congestive heart failure. Renal involvement is associated with increased mortality and occurs in 45% of Ssc, producing proteinuria, hypertension, scleroderma renal crisis and renal failure. In conclusion, involvement of the lungs, heart and kidneys are determining factors for the longterm course of systemic sclerosis.

Heart Diseases↗
PubMed1986

Clinical manifestations of acrodermatitis chronica atrophicans in 50 Swedish patients.

A study was made of 50 consecutive patients with untreated acrodermatitis chronica atrophicans (ACA). In all patients elevated anti-spirochetal antibody titers were found at indirect immunofluorescence and enzyme-linked immunosorbent assays, and histologically biopsies exhibited a dermal lymphocytic infiltrate with a moderate to rich admixture of plasma cells and telangiectases. Nine patients had a history consistent with spontaneously healing erythema chronicum migrans Afzelius (ECMA) on the extremity on which, after 0.5-8 years, ACA lesions developed. Eight patients had a history indicating previous cranial nerve involvement and nine had had periods of severe pains in the cervical or lumbar region. Two patients had developed ECMA, facial palsy and ACA in chronological order. In 15 patients radiographic abnormalities of joints and/or bone tissue were found. Besides ACA lesions, lichen sclerosus et atrophicus- or scleroderma-like lesions were found in six patients. The inflammatory ACA lesions were sometimes discrete and had been overlooked. Joint deformities, sclerotic lesions, diffuse edema or pain were the cardinal symptoms in some patients. The findings indicate that clinical recognition of ACA may be difficult and that a combination of clinical, histopathologic and serologic findings may be necessary to establish the diagnosis. The results are consistent with the concept that ACA is a late manifestation of infection by the same spirochete as causes ECMA and Bannwarth's syndrome.

Acrodermatitis↗
PubMed1995

Endoscopic detection of ischaemia with a new probe indicates low oxygenation of gastric epithelium in portal hypertensive gastropathy.

Changes in mucosal blood flow may be important in the pathogenesis of many conditions. Study of mucosal blood perfusion is difficult, and available methods have significant technical limitations. This study describes the development of an instrument for endoscopy, which indicates blood flow indirectly, by measuring the quantity of tissue oxygen that can diffuse from the mucosa to a luminal surface electrode. The instrument was used through an endoscope in patients with portal hypertension (n = 14), scleroderma (n = 3), disease controls (n = 7), and normal controls (n = 11). In portal hypertension readings were one quarter that in normal controls in both antrum (geometric mean (SEM) 35 (1.1)), nanoamps v 137 (1.1), and upper corpus 34 (1.1) v 125 (1.1)). Scleroderma patients showed greatly reduced oxygen readings in both antrum (18 (1.2)) and corpus (24 (1.2)), an expected but hitherto undiscovered result. These differences are highly significant (p = 0.0001), and the findings suggest that tissue hypoxia may contribute to mucosal changes in portal hypertensive gastropathy and in scleroderma.

Epithelium↗
PubMed2006

Oral cyclophosphamide improves pulmonary function in scleroderma patients with fibrosing alveolitis: experience in one centre.

Lung involvement constitutes nowadays the major cause of morbidity and mortality in scleroderma patients. Pulmonary fibrosis in systemic sclerosis (SSc) is thought to be the consequence of interstitial inflammation. Early diagnosis and treatment of active alveolitis is essential to prevent the deterioration of pulmonary function, improving outcome in SSc patients. The aim of the study was to investigate the effect of 1-year treatment with oral cyclophosphamide (CYC) on the evolution of interstitial lung disease in scleroderma patients with a diagnosis of active alveolitis. An open-label one-arm monocenteric study was conducted on 33 scleroderma patients with active alveolitis--defined as the presence of areas of 'ground-glass attenuation' on high-resolution computed tomography and a recent deterioration in lung function-treated with oral CYC 2 mg kg-1 day-1 for 1 year and medium-low dose steroids (prednisone 25 mg for 3 months and then tapered to 5 mg/day). Results showed that diffusing capacity for carbon monoxide (DLco) values remained stable after 6 months of treatment and significantly increased after 12 months (2.06+/-1.38, 2.21+/-1.62 and 2.39+/-1.64 mmol/min/kPa, at baseline/6/12 months, respectively; p<0.001 12th month vs baseline) vital capacity (VC) values slightly increased (i.e. stabilised) in the same time frame (2.46+/-0.71, 2.41+/-0.76 and 2.56+/-0.75 l). Accordingly, the vast majority of our patients (n=29, 87.9%) presented a DLco and/or a VC improvement or stabilisation with respect to baseline. Favourable results were more likely to be observed in patients with a lower Wells' radiological grade (grade I). In 25 patients followed up for further 12 months after the interruption of therapy, VC and DLco remained stable. Thus, long-term therapy with oral CYC is effective in ameliorating and/or stabilising lung function in scleroderma patients with active alveolitis, with beneficial effects lasting up to 1 year after interruption. The higher efficacy in those patients with an early pulmonary disease stage and a lower radiological grade underlies the importance of an early diagnosis and intervention.

Administration, Oral↗
PubMedDate not supplied

[An electro- and vectorcardiographic study of ventricular activation in scleroderma].

Electrical ventricular activation was studied by means of electrocardiogram and vectorcardiogram in 62 patients, 55 women and 7 men, affected by systemic sclerosis of average disease duration of 10.3 years. 45% of the patients showed the "diffused form" of the disease and 55% the "limited form". The vectorcardiogram showed some alterations of the ventricular activation in 55% of the recorded cases. The previous electrocardiogram found only 18%. The alterations, which are more frequent in the diffuse form than in the limited one, are the presence of loss of forces in septal, lateral or inferior or combined areas and intraventricular conduction disorders as complete and incomplete right bundle branch block, left anterior subdivision block, left posterior subdivision block, or combination between incomplete right bundle branch block and left anterior or posterior subdivision block. The vectorcardiogram was more sensitive than the electrocardiogram in showing loss of forces, also in patients with the limited form of systemic sclerosis, when the electrocardiogram was normal. Moreover the vectorcardiogram is helpful to identify left posterior subdivision block. The vectorcardiographic investigation seems to be a useful complement of the electrocardiogram especially for the identification of cases with myocardial involvement that is clinically silent.

Adult↗
PubMed1998

Current uses of diagnostic high-frequency US in dermatology.

OBJECTIVE: The diagnosis of most skin diseases, both focal and diffuse, has long relied mainly on physical examination findings. The recent introduction of technologically advanced ultrasound equipment using 20 MHz probes has permitted the specific application of ultrasound to dermatology. Accordingly, we investigated whether the findings at very high frequencies can represent a valid adjunct to clinical assessment in many skin conditions, including neoplasms, inflammatory states and diseases of unknown origin. MATERIALS AND METHODS: Skin lesions are studied using high frequency probes, which very clearly detail the three layers (epidermis, dermis and subcutaneous tissues) forming the normal skin. The choice of the probes frequency should depend mainly on the lesion diameters and site. Electronic 7.5-13 MHz linear probes depict flat and regular surfaces effectively and provide a wider field of surface vision and, therefore, a wider view than sectorial probes. Water bath sectorial mechanical probes with 10-20 MHz frequency have very superficial focusing and are excellent to study irregular surfaces. RESULTS: High frequency ultrasound can be usefully correlated with clinical tests to study focal skin lesions. The diagnosis of most benign skin cancers is usually made on clinical bases. Ultrasound examinations are performed preoperatively in questionable cases. Malignant neoplasms appear at ultrasound as hypoechoic focal lesions, generally with no specific features in relation to the histologic type; nevertheless, preoperative ultrasound may play an important role in that it measures the thickness of cutaneous melanoma, which is a very important prognostic factor. In particular, 20 MHz probes permit to assess the depth of melanoma invasion. The sonographic evaluation of melanoma thickness is usually in agreement with histologic findings. 'Satellite' neoplastic lesions growing near the main tumor can also be revealed. Color and power Doppler studies may be combined with gray-scale imaging: the identification of abnormal intra- or peritumoral low-resistance pulsatile flow signals suggests the malignant nature of the cutaneous lesion. High frequency ultrasound can also be used to study diffuse cutaneous conditions. Among them, ultrasound can provide a valid morphologic representation of psoriatic skin lesions and it is also a noninvasive and accurate method for evaluating the therapeutic efficacy of antipsoriatic drugs. In scleroderma, sonographic findings vary depending on disease activity and the patterns vary; therefore, 20 MHz probes may also prove useful over the other instrumental tools to monitor the disease course and treatment efficacy in focal scleroderma. Other potential applications include allergic dermatitis, nodular erythema, dermatomyosis, sarcoidosis, lymphedema of the limbs and allergologic conditions. Ultrasound can also be used in monitoring the response to or complications of topic drugs administration, and in the follow-up of focal burns. CONCLUSION: High frequency ultrasound can provide a reliable morphologic representation of skin lesions but it is also an accurate noninvasive tool for monitoring the therapeutic efficacy of drugs administration in focal or diffuse diseases. The application of high frequency studies to dermatology is very challenging. Indeed, the very high frequency probes up to 20 MHz currently available are particularly useful for reliable studies. Contrast-enhanced color and power doppler are very promising techniques. Advancements in technology will improve the correlation of clinical with high frequency ultrasound findings in the assessment of several skin diseases.

Humans↗