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PubMed2006

Natural remedies for scleroderma.

Scleroderma is an autoimmune disease of the connective tissue characterized by fibrosis and thickening of various tissues. It can be limited to the skin or affect multiple organs, and its course ranges from slowly to rapidly progressive. Penicillamine, glucocorticoids, and other drugs are used to treat scleroderma, but none of these treatments has a high degree of efficacy. This article reviews several promising natural treatments for scleroderma, including para-aminobenzoic acid, vitamin E, vitamin D, evening primrose oil, estriol, N-acetylcysteine, bromelain, and an avocado/soybean extract.

Humans↗

PubMed1997

The palpable tendon friction rub: an important physical examination finding in patients with systemic sclerosis.

OBJECTIVE: To evaluate the clinical and prognostic significance of palpable tendon friction rubs in patients with systemic sclerosis (SSc). METHODS: SSc patients evaluated prospectively at the University of Pittsburgh were examined serially for the presence of tendon friction rubs on physical examination. Demographic, clinical, and laboratory features of disease were obtained by patient examination, annual patient questionnaire, and medical record review. Patients were classified as having limited or diffuse scleroderma according to standard definitions. The prognostic significance of the presence of tendon friction rubs was determined using this comprehensive database. RESULTS: The SSc patients (n = 1,305) were first evaluated during 1972 through 1991 and were followed up for a mean of 6.3 years. Tendon friction rubs were detected most frequently in patients who had or who developed diffuse cutaneous involvement. There were strong correlations between the presence of tendon friction rubs and symptoms and signs typical of diffuse scleroderma, including more severe skin thickening, more frequent heart and kidney involvement, and decreased survival. In multiple regression analyses, the presence of 1 or more tendon friction rubs was one of the best predictors of both evolution to diffuse scleroderma and reduced survival. CONCLUSION: The palpable tendon friction rub is an easily detected, inexpensively obtained physical examination finding which is highly associated with diffuse cutaneous scleroderma and decreased survival. This observation should lead to the early diagnosis of diffuse scleroderma and should identify patients at high risk for serious visceral involvement who are thus candidates for potential disease-modifying therapy.

Ankle Joint↗

PubMed1994

Gastrointestinal manifestations of systemic sclerosis.

Gastrointestinal (GI) manifestations of systemic sclerosis (SSc) were found in 82% of 262 patients followed up prospectively. Esophageal dysmotility, lower esophageal sphincter laxity, bacterial overgrowth, and wide mouth diverticuli were the most common findings. The disease is usually diffuse with multiple levels of involvement. Gastrointestinal involvement was not significantly correlated with gender, age at SSc diagnosis or disease type (limited or diffuse scleroderma). Upper GI symptoms develop early in the course of SSc and may not correlate with objective findings. Various investigations, treatment regimens, and less frequent disease manifestations are reviewed and discussed.

Adult↗

PubMed1990

Cutaneous manifestations of the L-tryptophan-associated eosinophilia-myalgia syndrome: a spectrum of sclerodermatous skin disease.

The natural history of the clinical and pathologic features of skin disease was reviewed prospectively in 30 patients with the L-tryptophan-associated eosinophilia-myalgia syndrome. Overall, cutaneous manifestations developed in 26 patients (87%). Early lesions were nonspecific and characterized predominantly by an erythematous macular eruption on the trunk and extremities. The most characteristic abnormality noted was the spectrum of sclerodermatous disease in 15 patients (50%) often after a subacute stage of peripheral or truncal edema. Clinical and/or biopsy evidence of eosinophilic fasciitis was seen in nine patients (30%). Findings consistent with diffuse, limited, or localized scleroderma were subsequently observed in nine patients (33%). Small mucinous papules, similar to those seen in scleromyxedema, were found in five patients (17%). Alopecia, frequently a late sequela, developed in 11 (37%). Common histologic features included papillary dermal fibrosis, dermal and fascial infiltrates consisting of mononuclear cells and eosinophils, deposition of glycosaminoglycans in the dermis, and, in some patients, numerous mast cells.

Adult↗

PubMed2002

[Pulmonary hypertension associated with connective tissue diseases].

PURPOSE: Pulmonary hypertension is a rare but well-known life-threatening complication of connective tissue diseases. The aim of this article is to analyse the available literature and to report the experience of a pulmonary vascular diseases centre about this complication. CURRENT KNOWLEDGE AND KEY POINTS: Scleroderma and its limited variant, the CREST syndrome (calcification, Raynaud phenomenon, esophageal dysmotility, sclerodactily, telangiectasia), is the most common connective tissue disease affected by pulmonary hypertension. Dyspnea is the main symptom and is frequently severe. Echocardiography is an excellent exam to detect pulmonary hypertension. However, right heart catheterization is necessary to confirm the diagnosis of pulmonary hypertension and to test vasoreactivity with a potent vasodilator such as nitric oxide. Pulmonary hypertension is less severe in patients with connective tissue diseases perhaps because of an earlier diagnosis. A significantly lower proportion of patients presents an acute vasodilator response, suggesting an early constitution of irreversible pulmonary vascular lesions. Continuous intravenous epoprostenol therapy seems to be less effective as compared with patients with primitive pulmonary hypertension and does not improve survival. So, we observed dramatic improvement in rare cases after immunosuppressive therapy. FUTURE PROSPECTS AND PROJECTS: New treatments with oral, subcutaneous or inhaled stable prostacyclin analogs or with an endothelin receptor antagonist are currently being evaluated. The role of immunosuppressive therapy has to be defined.

Antihypertensive Agents↗

PubMed2000

Autoepitopes on autoantigen centromere protein-A (CENP-A) are restricted to the N-terminal region, which has no homology with histone H3.

Anti-centromere autoantibodies (ACA) are commonly found in the serum of patients with a limited type of scleroderma and other systemic autoimmune diseases. CENP-A is one of the major antigens against ACA and a histone H3-like protein. To analyse the autoantigenic epitopes of CENP-A, a series of truncated peptides of human CENP-A were expressed in Escherichia coli and immunoblotting analysis was performed with 91 ACA+ sera. Eighty sera (88%) with the ACA reacted to the 52-amino acids N-terminal region which is not homologous to H3, while no sera reacted to the C-terminus which has a sequence similarity with H3. Moreover, ELISA was also employed in this study using two synthetic peptides corresponding to the amino acid sequences 3-17 (peptide A) and 25-38 (peptide B). Peptides A and B were reactive to 78 (86%) and 79 (87%) of ACA, respectively. Core antigens of hepatitis B virus (HBV) and hepatitis C virus (HCV) have similar sequences to peptide A and/or peptide B, but three sera containing HBV without ACA and five sera containing HCV without ACA were found to be reactive to neither peptide. Centromere localization of CENP-A is dependent on the H3-like C-terminal domain which is not autoantigenic, while the antigenic N-terminal domain, which might play unidentified functional roles, should be an important region for the induction of ACA.

Amino Acid Sequence↗

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↗

PubMed1996

Achalasia-like syndrome as the first manifestation in a patient with CREST syndrome.

We report a case of oesophageal disease as the first manifestation in a patient with CREST syndrome. A 46-year-old man with achalasia-like syndrome developed CREST syndrome 4 years later. A pneumatic dilatation of the cardia was performed. After pneumatic dilatation the dysphagia and regurgitation disappeared but the patient developed reflux oesophagitis. Four years after diagnosis of oesophageal disease he presented with a clinical picture of CREST syndrome. An acute ileus and constipation developed later. After receiving medical therapy with omeprazole and cisapride the patient is free of oesophageal symptoms and bowel movements are normal. Oesophageal disease is common in patients with limited and diffuse scleroderma, but to our knowledge achalasia-like syndrome has not been previously described as the first manifestation of the systemic disease.

Anti-Ulcer Agents↗

PubMed2001

Cytomegalovirus esophagitis as a treatable complication of systemic sclerosis.

We report the case of a 51-year-old woman with a connective tissue disease of 8 years duration. She had been taking corticosteroids at a dose of 1 mg/kg/day and azathioprine at a dose of 3 mg/kg/day for 1 month. Given the clinical suspicion of systemic sclerosis (limited form of scleroderma), she was studied according to a protocol including endoscopy to assess the degree to which the underlying disease had affected the gastrointestinal tract. Endoscopy revealed a asymptomatic severe esophagitis and a subsequent biopsy disclosed the presence of cytomegalovirus. Cytomegalovirus pneumonia was also detected. Both processes were successfully managed with intravenous ganciclovir (5 mg/kg/12 hr) for 21 days. This report is a case of cytomegalovirus involving the esophagus in association with systemic sclerosis in a patient immunosuppressed because of drugs that she had been taking. This complication can be asymptomatic and is amenable to treatment.

Journal Article↗

PubMed1998

Cutaneous metastases from adenocarcinoma of unknown primary.

Cutaneous manifestations of malignancy are not uncommon, especially in advanced disease. They may also occur early in malignant disease or they may even signify recurrence particularly if they are paraneoplastic in nature. Clinical diagnosis can be difficult because of the wide spectrum of appearance of these lesions, and, in many cases, because of the lack of an identifiable underlying primary. Presented here is the case of a 65-year-old woman with multiple inflammatory cutaneous metastases, which were sclerodermoid in nature. These appeared 14 months after initial diagnosis of adenocarcinoma of unknown primary (ACUP) and signified the beginning of a rapid deterioration in her condition. The coexistence of limited systemic sclerosis (scleroderma) and ACUP initially raised several interesting diagnostic possibilities. Adenocarcinoma of unknown primary and the sclerodermoid reaction in malignancy are discussed.

Adenocarcinoma↗

PubMed1983

Anticentromere antibody. Clinical Correlations and association with favorable prognosis in patients with scleroderma variants.

The presence of antibody to the chromosomal centromere appears to be associated with a subset of patients with the limited CREST form of scleroderma. To further define the prognostic value of this autoantibody, 27 patients, who were identified as having anticentromere antibody by screening antinuclear antibody tests using HEp-2 cell substrates, were followed clinically and serologically for 2 years. The presence of anticentromere antibody is common in the limited CREST forms of systemic sclerosis, and it is often the only autoantibody specificity present in the sera of patients with the CREST variant. When compared with other patients who exhibit speckled or nucleolar antinuclear antibody patterns, those with anticentromere antibody had significantly less major organ system involvement.

Adult↗

PubMed2004

Long-term evaluation of lung function in patients affected by scleroderma treated with cyclic iloprost infusions.

OBJECTIVE: The aim of this study was to evaluate pulmonary function in patients suffering from scleroderma treated for 3 years with cyclic iloprost infusions. METHODS: Thirty patients affected by scleroderma (five men and 25 women, mean age 49.3 years, mean disease duration 8 years, 19 with limited and 11 with diffuse disease patterns) were treated for 5 consecutive days every 4 months for 3 years with iloprost. Pulmonary function was evaluated at baseline and after 3 years. At the end of the trial, color Doppler echocardiography was performed in 26 of the patients. RESULTS: Spirometric parameters did not significantly change after 3 years of therapy when expressed as percentage of the predicted normal value. Diffusing lung capacity for carbon monoxide decreased at the limit of statistical significance. No patients developed clinical pictures suggestive of severe pulmonary arterial hypertension, which was confirmed in 26 subjects by evaluation of estimated pulmonary artery systolic pressure. CONCLUSIONS: This long-term study showed that lung function remained substantially stable after 3 years of treatment with cyclic iloprost infusions. No cases of severe pulmonary hypertension were observed.

Adult↗

PubMed2003

Human scleroderma sera contain autoantibodies to protein components specific to the U3 small nucleolar RNP complex.

OBJECTIVE: To determine whether antifibrillarin autoantibodies in scleroderma patients are associated with autoantibodies to protein components specific for U3 small nucleolar RNP (U3 snoRNP). METHODS: Sera from 220 scleroderma patients were examined for antinucleolar autoantibodies (ANoA) and for antibodies to fibrillarin and the U3 snoRNP-specific proteins Mpp10 and hU3-55K. Clinical correlates were determined for the different autoantibody specificities. RESULTS: Fifty-nine of the 220 patients were positive for ANoA, and 31 of these patients were antifibrillarin positive. Anti-hU3-55K was found in 10 patients, all of whom were antifibrillarin positive. Twenty-nine patients had anti-Mpp10 antibodies; 23 of these were antifibrillarin positive and 6 were antifibrillarin negative. ANoA, including antifibrillarin, anti-hU3-55K, and anti-Mpp10, were associated with diffuse, rather than limited, systemic or localized scleroderma. Esophageal and lung involvement were more common in patients with antifibrillarin and anti-Mpp10 antibodies, and the highest frequency was in patients with anti-Mpp10 alone. CONCLUSION: Antifibrillarin autoantibodies are associated with autoantibodies to protein components specific to U3 snoRNP, particularly Mpp10. The prevalence of anti-Mpp10 antibodies in antifibrillarin-positive patients suggests that the U3 snoRNP particle is a source of immunogenic/antigenic material for the anti-snoRNP response in scleroderma. Autoantibodies to snoRNP components were more frequent in patients with diffuse scleroderma than in those with either the limited systemic or localized forms. The increased expression of these antibodies in patients with the more severe form of scleroderma, coupled with the observations that fibrillarin expression is positively linked to collagen expression in fibroblasts and that fibrillarin is overexpressed in scleroderma fibroblasts, suggests a source of snoRNP to initiate and maintain these autoantibody responses.

Adult↗

PubMed1999

Bleomycin-induced scleroderma: report of a case with a chronic course rather than the typical acute/subacute self-limiting form.

We report a case of bleomycin-induced scleroderma in a 35-year-old woman treated with chemotherapy for Hodgkin's disease. Approximately 6 months after the first chemotherapy cycle, the patient developed skin sclerosis in both arms. The lesion showed no signs of spontaneous clinical amelioration and treatment with steroids was unsuccessful. A partial remission of the skin sclerosis was instead obtained by the administration of D-penicillamine. A family history revealed other cases of autoimmune diseases and HLA typing showed the presence of antigens associated with scleroderma. The association between bleomycin therapy and scleroderma is discussed.

Adult↗

PubMed1995

Scleroderma and fasciitis in children.

Scleroderma is a spectrum of disorders, all of which may occur in childhood. Childhood disease differs from adult disease in that localized forms predominate, with major problems confined to the skin and underlying soft tissues; generalized scleroderma, whether diffuse or limited, is less common. Childhood eosinophilic fasciitis is rare, and the literature is scant. The pattern appears to be similar to that in adults, and as in the adult form, the overlap between eosinophilic fasciitis, morphea, and linear scleroderma is blurred. The etiopathogenesis of juvenile-onset scleroderma is unknown but almost certainly multifactorial. The heterogeneity of its clinical expression may argue for it being more than one disease. This review attempts to document the expression of scleroderma in childhood and relate it to etiologic, immunologic, and pathogenic considerations.

Autoimmunity↗

PubMed1995

High resolution computed tomography for the evaluation of lung involvement in 101 patients with scleroderma.

The aim of the study was to investigate the pulmonary parenchymal abnormalities by high resolution computed tomography (HRCT) in patients with various forms of scleroderma. Three scans were performed sequentially in all cases: one at the level of aortic arch, one at the tracheal carina and one 1-2 cm above the diaphragm. Seventy cases with limited, 21 patients with diffuse cutaneous systemic sclerosis and 10 cases with circumscribed scleroderma were investigated. The 21 patients with diffuse scleroderma included three normal HRCT scan (14%), three with ground glass attenuation (14%), one with ground glass attenuation with fibrosis, three with fibrosis (14%), six with subpleural and five with diffuse honeycombing. The majority of cases with extensive honeycombing of the lungs had anti-Sc! 70 autoantibody which was the characteristic antibody of the diffuse scleroderma subset. The 70 cases with limited cutaneous systemic sclerosis were characterized by normal HRCT (43%), ground glass opacity with or without fibrosis (18.6%), whereas fibrosis was detected in 22.9%, subpleural or diffuse honeycombing in 15.7% of the patients. Six of the ten cases with circumscribed scleroderma also showed a fibrosis. Cases with diffuse scleroderma are characterized by the presence of advanced fibrosis, whereas the extent of fibrosis is far less pronounced in limited scleroderma. Mild fibrosis may also be present in circumscribed scleroderma forms.

Adult↗

PubMed1999

Microvascular abnormalities in Sjögren's syndrome: nailfold capillaroscopy.

OBJECTIVE: To describe microvascular abnormalities by nailfold capillaroscopy in patients with primary Sjögren's syndrome (SS) with or without Raynaud's phenomenon (RP) and those with anticentromere antibodies (ACA). METHODS: Forty patients with SS (14 without RP, 16 with RP, 10 with ACA), 20 patients with scleroderma (SSc) (10 with limited and 10 with diffuse disease) (disease control group) and 40 healthy controls (control group) were evaluated by nailfold capillaroscopy. RESULTS: Capillaroscopic abnormalities in SS ranged from non-specific findings (crossed capillaries) to more specific findings (confluent haemorrhages and pericapillary haemorrhages) or scleroderma-type findings. SS patients with RP presented capillary abnormalities in higher frequency than patients without RP. The majority of SS patients with ACA (80%) presented scleroderma-type findings. CONCLUSION: Nailfold capillaroscopy can be used as a simple non-invasive method to evaluate the microvascular abnormalities in SS patients, especially in those with RP and those with ACA.

Capillaries↗

PubMed1998

Classification and epidemiology of scleroderma.

Scleroderma is classified as two separate but related entities, a localized form and a systemic form. The classification scheme for morphea presented here is that of Peterson et al, which divides morphea into five categories: plaque, generalized, bullous, linear, and deep. Using this system, these authors estimated the incidence rate of localized scleroderma to be 27 new cases per million population per year. Overall survival was similar to that of the general population. There was a preponderance of female cases (approximately 3:1) for all forms of morphea except for linear scleroderma, which had an even sex distribution. Systemic scleroderma is divided into limited and diffuse disease based on the extent of skin involvement. Recent estimates have placed the incidence rate of systemic sclerosis in the United States at 19 new cases per million adults per year, with an overall prevalence of 240/million adults. The female-to-male ratio is approximately 5:1. The prevalence of scleroderma varies by geographic region and ethnic background and is higher in the United States than in Europe or Japan. Although systemic sclerosis survival has improved over the past two decades, with 5-year survival over 80%, long-term survival is significantly lower than expected, and morbidity is considerable.

Adult↗