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PubMed1998

African-American race and antibodies to topoisomerase I are associated with increased severity of scleroderma lung disease.

STUDY OBJECTIVES: To determine whether African-American race is independently associated with lung disease in scleroderma. DESIGN: Retrospective review. SETTING: University medical center in Baltimore. PATIENTS: One hundred one patients with diffuse cutaneous scleroderma with available serum samples. MEASUREMENTS: Patients underwent lung function testing as part of their routine clinical care. Percent predicted values adjusted for race were calculated for FVC, single-breath carbon monoxide diffusing capacity (Dco), and FEV1. Serum samples were assayed for the presence of antibodies to topoisomerase I and RNA polymerase II. RESULTS: Scleroderma patients of African-American race had lower percent predicted values than white patients for FVC (p<0.002), Dco (p<0.0001), and FEV1 (p<0.0001). Antibodies to topoisomerase I but not antibodies to RNA polymerase II were also associated with lung function. African-American scleroderma patients were distinct from white patients in having younger age of onset and higher prevalence of antibodies to topoisomerase I. In multivariate analyses accounting for sex, age, smoking history, years of scleroderma symptoms, and RNA polymerase II antibody status, African-American race and topoisomerase I antibody status independently predicted lower lung function. CONCLUSION: African-American race and antibodies to topoisomerase I are independent risk factors for scleroderma lung disease.

Autoantibodies↗
PubMed1976

Skin capillary changes in early systemic scleroderma. Electron microscopy and "in vitro" autoradiography with tritiated thymidine.

Skin biopsy specimens obtained from involved and noninvolved areas in a patient with early diffuse systemic scleroderma were processed for histology, electron microscopy, and "in vitro" autoradiography with tritiated thymidine. The affected area revealed cellular infiltrates around the eccrine sweat glands, consisting of plasma cells and lymphocytes. The capillaries showed thickening of the basement lamina, damage of endothelial cells, and obstruction of their lumens. However, in some vessels, endothelial cells were preserved and appeared in prophase. Autoradiography with tritiated thymidine showed a marked increase in endothelial and periendothelial cell labeling. Blood immunological studies revealed an increase in B-lymphocytes, IgG, and IgA and the presence of antinuclear and antismooth muscle antibodies.

Adult↗
PubMed1995

Circulating Fc gamma receptor-specific autoantibodies in localized and systemic scleroderma.

BACKGROUND: Anti-Fc gamma receptor (anti-Fc gamma R) autoantibodies occur in patients with systemic scleroderma. Their clinical significance is unknown. OBJECTIVE: Our purpose was to determine the incidence of anti-Fc gamma R autoantibodies in patients with localized and systemic scleroderma and to examine the relation between these autoantibodies, the severity of the disease, and the presence of other autoantibodies. METHODS: Patients were placed into three clinical groups: three had diffuse systemic scleroderma, 47 had limited systemic scleroderma, and nine had localized systemic scleroderma. Antinuclear antibody titer and pattern were measured by indirect immunofluorescence with human epithelial (HEp)-2 cells and tissue sections, whereas anti-Scl-70 antibodies were measured by gel diffusion technique. Anti-Fc gamma R autoantibodies were measured in serum from patients and from 25 healthy persons by enzyme-linked immunosorbent assay with human recombinant Fc gamma RII (CD32) and Fc gamma RIII (CD16). RESULTS: Anti-Fc gamma R autoantibodies were detected in 54% of patients and in none of the healthy control subjects. Autoantibodies were present in all three clinical groups and were most frequently directed against Fc gamma RIII. Correlation between patients' clinical and laboratory data and anti-Fc gamma R autoantibodies could not be demonstrated. CONCLUSION: The presence of anti-Fc gamma R autoantibodies in the serum of patients with either systemic or localized scleroderma and the lack of these autoantibodies in healthy persons suggest that they may play a role in the pathogenesis of these diseases.

Adolescent↗
PubMed1997

Autoantibodies: diagnostic fingerprints and etiologic perplexities.

Autoantibodies are a hallmark of systemic rheumatic diseases, organ-specific autoimmune diseases and paraneoplastic syndromes. Cell biologists have used autoantibodies as probes to define the structure and function of novel macromolecules and to determine the chromosomal location of their respective genes. The observation that many autoantibodies appear before the clinical expression of disease suggests that they are not epiphenomena. Some autoantibodies are disease-specific markers and are in aid to establishing a diagnosis. Although it has been difficult to link autoantibodies to pathogenesis, they can be used to predict disease progression and outcome. For example, autoantibodies directed against topoisomerase are associated with progression of scleroderma to diffuse skin involvement and severe systemic disease, whereas antibodies to centromere proteins predict a more slowly progressive disease and development of a limited variant of scleroderma. Certain models of autoantibody production hold promise of a clearer understanding of the mechanisms that underlie autoimmunity. Drugs such as procainamide and hydralazine induce the production of chromatin autoantibodies. Exposure to heavy metals (e.g., mercury) is also linked to the development of autoantibodies. The data provide evidence that the autoimmune response is driven by autoantigens, which are multimolecular complexes involved in essential cellular functions.

Animals↗
PubMed1977

Diffuse eosinophilic fasciitis. A new syndrome or variant of Scleroderma?

Recently, attention has been drawn to a scleroderma-like illness, characterized by transient eosinophilia, which is commonly antedated by unusual physical exertion, is apparently free from significant systemic changes, and in which the primary pathological alterations, consisting of intense inflammation and thickening with or without eosinophils, occur initially in the fascia, not the skin. These patients are said to respond well to oral corticosteroids and an occasional one may undergo spontaneous resolution. Clinicopathologic study of a patient with this syndrome, suggests an even deeper tissue genesis than that recently proposed for some of the cutaneoindurative disorders. Eosinophilic fasciitis probably represents an impressive, but perhaps relatively benign variant of diffuse scleroderma, according to reported cases.

Diagnosis, Differential↗
PubMed2003

Interstitial lung disease in scleroderma.

Pulmonary fibrosis occurs most patients who have scleroderma. It progresses to severe restrictive lung disease in about 15% of patients and remains a major cause of death in this disease. Risks for developing pulmonary fibrosis include diffuse cutaneous scleroderma and anti-Scl-70 antibodies, and risks for developing progressive pulmonary fibrosis and death include low pulmonary function test results at presentation and lung inflammation. Early evaluation of the extent and severity of pulmonary fibrosis and the presence of lung inflammation is key, so that therapy can be given to patients who are at higher risk of progressive pulmonary fibrosis before they develop severe functional impairment occurs. Evaluation often includes pulmonary function tests, HRCT of the lungs, Doppler echocardiogram, and bronchoalveolar lavage. For patients who are unwilling or unable to participate in therapeutic trials that target pulmonary fibrosis in scleroderma, therapy with oral or intravenous cyclophosphamide is often given.

Humans↗
PubMed1994

Localized scleroderma: imaging features.

Localized scleroderma is distinct from the diffuse form of scleroderma and does not show Raynaud's phenomenon and visceral involvement. The imaging features in 23 patients ranging from 2 to 17 years of age (mean 11.1 years) were reviewed. Leg length discrepancy and muscle atrophy were the most common findings (five patients), with two patients also showing modelling deformity of the fibula. One patient with lower extremity involvement showed abnormal bone marrow signals on MR. Disabling joint contracture requiring orthopedic intervention was noted in one patient. In two patients with "en coup de sabre" facial deformity, CT and MR scans revealed intracranial calcifications and white matter abnormality in the ipsilateral frontal lobes, with one also showing migrational abnormality. In a third patient, CT revealed white matter abnormality in the ipsilateral parietal lobe. In one patient with progressive facial hemiatrophy, CT and MR scans showed the underlying hypoplastic left maxillary antrum and cheek. Imaging studies of areas of clinical concern revealed positive findings in half our patients.

Adolescent↗
PubMed2004

[Collagen diseases with pulmonary involvement].

Pulmonary involvement is a common feature in patients with various collagen diseases. Some types of the pulmonary involvement are resistant to currently available treatment regimens and thus considered as intractable conditions. These include acute/subacute interstitial pneumonia in dermatomyositis, pulmonary interstitial fibrosis in scleroderma, and diffuse alveolar hemorrhage. Acute/subacute interstitial pneumonia with the histology of diffuse alveolar damage (DAD) is mainly occurred in patients with amyopathic or hypomyopathic dermatomyositis who lack autoantibodies to aminoacyl tRNA synthetases. Intensive immunosuppressive treatment in the early phase of the disease may be effective for this intractable complication. Nearly one-third of patients with scleroderma have slowly progressive pulmonary interstitial fibrosis, leading to end-stage respiratory failure. Non-specific interstitial pneumonia (NSIP) with an excessive fibrotic change is a major histology of these patients. There are accumulating evidences showing the effectiveness of cyclophosphamide in patients with this intractable condition, especially those with active alveolitis. Diffuse alveolar hemorrhage is a fatal complication mainly occurred in patients with systemic lupus erythematosus and those with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, including microscopic polyangitis (MPA) and Wegener's granulomatosus. Immediate diagnosis and introduction of intensive treatment are necessary to save the patients with this complication.

Collagen Diseases↗
PubMed1992

The absence of factor B activation products in scleroderma.

Previous investigators have reported the presence of complement activation products in scleroderma. Using an assay consisting of isoelectric focussing followed by immunofixation (IEF/IF) for alternative pathway activation product Ba, and an ELISA for C3d that we developed for evaluating lupus plasmas, we assayed 48 plasmas from patients with diffuse cutaneous scleroderma, 16 patients with the limited CREST variant, 2 patients with mixed connective tissue disease (MCTD) and 4 patients with Raynaud's disease. Ba was not detected in any patient's plasma. Only one plasma from a patient with CREST contained elevated levels of C3d. We cannot reconcile the absence of B activation products in our patients with scleroderma with the results of the previous report, and conclude that further studies are necessary.

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↗
PubMed2002

Development of histologic features of scleroderma in congenital lesions.

BACKGROUND: Localized scleroderma has been reported after radiation therapy, but has never been reported to occur at the site of a congenital lesion. CASE REPORT: We present two patients, both with family histories of autoimmune disease, who reported unilateral hypopigmented areas on the trunk since birth. The areas remained asymptomatic and grew with the patients until adulthood when the areas became indurated then firm and showed hyperpigmentation. HISTOLOGY: Histologically, both lesions showed features of localized scleroderma with diffuse sclerosis of collagen and loss of periadnexal fat. There was a perivascular lymphoplasmocytic infiltrate with occasional eosinophils extending into the subcutaneous fat predominantly along fat septae, and diffuse loss of CD34+ stromal cell populations within the lesions. CONCLUSION: We propose that somatic mutations affecting vessels may predispose to increased endothelial cell apoptosis. This could lead to the development of an autoimmune response in some individuals, and the areas of localized scleroderma may be markers of susceptibility to autoimmune disease.

Adult↗
PubMed1999

Correlates of the disability index of the health assessment questionnaire: a measure of functional impairment in systemic sclerosis.

OBJECTIVE: To evaluate functional impairment in systemic sclerosis (SSc) patients with diffuse cutaneous scleroderma at the time of entry into a trial of a therapeutic intervention (D-penicillamine). METHODS: The 20-item Disability Index of the Health Assessment Questionnaire (HAQ-DI) was administered to 134 patients as they entered a multicenter trial of high-dose versus low-dose D-penicillamine. All patients had diffuse SSc of < 18 months' duration. SSc patients who had severe organ system involvement and recent renal crisis and who were receiving prednisone > 10 mg/day were excluded from entry. Logistic regression modeling was used to examine the relationship of HAQ-DI scores to SSc skin and organ system involvement. Odds ratios (OR) and 95% confidence intervals (95% CI) were used to estimate effects. RESULTS: The mean (+/-SD) HAQ-DI score at entry was 1.04 +/- 0.67. Fifty-three percent of patients had HAQ-DI scores > or = 1.0 (signifying moderate-to-severe functional impairment). Multivariate logistic regression demonstrated that impaired fist closure > or = 23 mm (OR 4.24, 95% CI 1.68-10.70), reduced handspread < or = 175 mm (OR 4.5, 95% CI 1.80-11.24), joint tenderness count > or = 1.0 (OR 2.93, 95% CI 1.16-7.40), age > or = 43 years (OR 2.44, 95% CI 1.01-5.95), platelet count > or = 330,000/mm3 (OR 2.30, 95% CI 0.96-5.57), and female sex (OR 2.43, 95% CI 0.77-7.73) were the most important correlates of HAQ-DI scores > or = 1.0. CONCLUSION: Increased HAQ-DI scores at baseline were correlated with reduced fist closure, reduced hand-spread, elevated platelet count, presence of tender joints, older age, and female sex. The most important contributor to functional impairment was hand dysfunction. Even within the first 18 months after SSc onset, moderate-severe functional impairment (HAQ-DI scores > or = 1.0) was frequent (53%) in this group of diffuse SSc patients. In early diffuse SSc, the self-administered HAQ-DI is therefore a valuable assessment of function that correlates with objective physical and laboratory measures of SSc disease involvement. Abnormal HAQ-DI scores may support patient claims of functional impairment, help to focus physician attention on implementing measures to reduce functional impairment, and be useful in reflecting the disease course over time.

Adolescent↗
PubMedDate not supplied

Scleroderma in association with the use of docetaxel (taxotere) for breast cancer.

The taxanes, paclitaxel (Taxol) and docetaxal (Taxotere), are a new class of anti-microtubule agents which have shown cytotoxic activity in a number of solid tumours. Phase I and II trials confirm that docetaxal is highly active in the treatment of metastatic breast cancer. Reported toxicities of docetaxel include, dose limiting neutropenia, alopecia, skin reactions and fluid retention. Here we report the first case of rapid onset, diffuse scleroderma-like illness, which occurred in a 59-year-old female receiving treatment with docetaxel for locally invasive and advanced metastatic breast cancer.

Antineoplastic Agents, Phytogenic↗
PubMed2005

Reticulate hyperpigmented scleroderma: a new pigmentary manifestation.

Scleroderma is a systemic disease with a myriad of cutaneous manifestations. Pigmentary disturbances have been described in scleroderma. While diffuse generalized hyperpigmentation and localized hypopigmentation with perifollicular hyper-pigmentation are well recognized, a vascular induced hyperpigmentation has only been recently described. In this paper, we report another unrecognized pigmentary abnormality in a 48-year-old patient who presented with a reticulate hyper-pigmentation affecting the trunk, upper and lower limbs. To our knowledge, cutaneous hyperpigmented reticulate scleroderma has not been reported previously.

Female↗
PubMed1984

Diffuse fasciitis with hypergammaglobulinemia and eosinophilia: a new syndrome?

Two male patients (53 and 19 years old) have been seen with a diffuse scleroderma-like illness with firm taut skin bound down to underlying structures (sparing the face in both patients and the hands and feet in one). Flexion contractures of elbows and knees and limitation of abduction at the shoulders developed in a few weeks after onset. Raynaud's phenomenon was absent and thorough evaluation failed to reveal any evidence of myositis or the visceral manifestations of systemic sclerosis. There was no loss of skin appendages. Both had circulating eosinophils (12% and 37%) without drug ingestion or parasitic infestation. Both had elevated sedimentation rates and hypergammaglobulinemia (in one, IgG = 4.1 g.%). Serologic tests for syphilis, rheumatoid factor, LE cells, antinuclear antibodies, complement and cryoglobulins were negative or normal in both patients. Bone marrow examination revealed plasmacytosis and eosinophilia. Biopsies revealed striking thickening of the fascia between the subcutis and muscle. Within the thickened connective tissue there was intense infiltration with plasma cells and lymphocytes, at times in follicles, both about and separate from vessels; eosinophils were absent. Skin biopsies revealed no changes of scleroderma; and muscle biopsies, no evidence of myositis. Prednisone therapy over 15 months in decreasing dosage, using laboratory parameters, induced in the first patient a full remission which has persisted for 5 years without therapy. In the second (more severely involved) patient prednisone therapy has reversed laboratory abnormalities; but clinical improvement to date has been minimal. The pathogenesis of this diffuse fasciitis is obscure, although unusual physical exertion antedated the onset of illness in each case.

Adolescent↗
PubMed2005

Acute exacerbation of systemic scleroderma in Borrelia burgdorferi infection.

In recent years a possible aetiological connection between skin sclerosis and an infection with Borrelia burgdorferi has been discussed, but this association has not yet been reported for systemic scleroderma. Several treatment modalities are suggested for systemic scleroderma, but no treatment has yet been found to alter the overall course of the disease. This report describes a 61-year-old woman with Raynaud's phenomenon, nail-fold changes and circulating anticentromere antibodies, who showed an abrupt onset of erythemas and doughy swellings involving the face and upper trunk, followed by thickening and induration of the skin mimicking diffuse systemic scleroderma. Laboratory tests including enzyme-linked immunosorbent assay (ELISA), immunoblot and urine polymerase chain reaction (PCR) showed an infection with B. burgdorferi sensu lato that was successfully treated with intravenous ceftriaxone, an antibiotic recommended for Lyme borreliosis. Fourteen days after the end of treatment the skin was no longer stiff and indurated and had returned to its normal predisease state. This case suggests that Lyme disease should be considered in atypical cases of skin sclerosis in patients predisposed to the development of systemic scleroderma.

Acute Disease↗
PubMed2003

Open lung biopsy for diffuse parenchymal lung disease in children.

An open lung biopsy was performed in 12 children with diffuse parenchymal lung disease. A definitive histopathological diagnosis was obtained from all procedures but determined treatment options in only 10 children (83%). Three (25%) children were ventilated for respiratory failure prior to the procedure. Four (44%) of the other 9 children required ventilatory support after the procedure. Three (25%) children developed post-op pneumothorax that resolved fully with chest tube drainage. There were no deaths as a direct result of the procedure. Open lung biopsy is useful in providing a definitive diagnosis in children with diffuse parenchymal lung disease and determining treatment in the majority of cases. The procedure was well-tolerated with minimal complications.

Biopsy↗