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PubMed2003

Goodpasture-like syndrome induced by D-penicillamine in a patient with systemic sclerosis: report and review of the literature.

We report a case of Goodpasture-like syndrome developing in a patient who was treated with D-penicillamine for the diffuse form of systemic sclerosis. This unusual pulmonary-renal syndrome has been described on rare occasions in patients receiving D-penicillamine. This complication appeared to be uniformly fatal unless treated with aggressive immunotherapy. We review the cases reported to date in the literature and describe the clinical characteristics, therapy, and outcome of this group of patients.

Aged↗
PubMedDate not supplied

Severe ankylosing spondylitis and diffuse systemic sclerosis: case report of a genetic trap.

A rare association of ankylosing spondylitis (AS) and systemic sclerosis (SSc) is reported in a Brazilian Mestizo patient presenting a human leucocyte antigen (HLA) genotype that included HLA SSc-susceptibility genes previously reported in different ethnic groups and HLA-B27 associated with AS. The underlying genetic background supporting the full expression of both SSc- and AS-susceptibility alleles and the pertinent literature are discussed.

Adult↗
PubMedDate not supplied

Renal involvement in systemic sclerosis.

Renal crisis occurs in systemic sclerosis patients with rapidly progressive diffuse cutaneous thickening early in their disease course. SRC is characterized by malignant hypertension, hyperreninemia, azotemia, and microangiopathic hemolytic anemia. This complication was almost uniformly fatal but can now be treated successfully in most cases with ACE inhibitors. The result has been improved survival, reduced requirement for dialysis, and even discontinuation of dialysis after 6 to 18 months of treatment. Prompt diagnosis and early aggressive treatment of SRC with ACE inhibitors will result in the most optimal outcome.

Humans↗
PubMed2004

A study of the prevalence of systemic sclerosis in northeast England.

OBJECTIVES: We aimed to obtain an estimate of the prevalence and demographics of systemic sclerosis (SSc) and its subtypes at the turn of the millennium. METHODS: Case finding from multiple sources from a defined geographical area. Diagnosis confirmed by clinical examination. RESULTS: The crude prevalence of SSc in northeast England was 8.8 (95% CI: 6.8-10.8) per 100,000. The prevalence when adjusted for the entire UK is 8.2 (95% CI: 6.2-9.8) per 100,000. The ratio of women to men was 5.2:1. The median age of patients was 57.1 yr. The ratio of limited cutaneous SSc to diffuse cutaneous SSc was 4.7:1. Limited cutaneous SSc is associated with the presence of anticentromere antibodies; diffuse cutaneous SSc is associated with anti-Scl 70 antibodies, but either antibody was found in either form of SSc. CONCLUSIONS: SSc appears to be more common in northeast England than was found in the West Midlands in 1986. This may reflect changes in the diagnostic definition of SSc.

Adolescent↗
PubMed1996

Beneficial effect of granulocyte-colony stimulating factor in scleromyxoedema associated with severe idiopathic neutropenia.

The treatment of scleromyxoedema is notoriously difficult. We present a patient with long-standing diffuse scleromyxoedema associated with functional impairment who developed chronic idiopathic neutropenia complicated by recurrent life-threatening infections. Treatment with recombinant granulocyte--colony stimulating factor led to normalization of the neutrophil count, prevented further systemic infections, and unexpectedly was associated with a striking clinical improvement of her skin disorder and decrease in mucin deposition in the dermis.

Chronic Disease↗
PubMed2002

Decreased MMP-9 activity in the serum of patients with diffuse cutaneous systemic sclerosis.

Matrix metalloproteinase-9 (MMP-9) and its inhibitor, tissue inhibitors of metalloproteinases-1 (TIMP-1) are involved in tissue inflammation and fibrotic processes. We previously reported an elevated serum TIMP-1 level in patients with systemic sclerosis (SSc). We measured serum TIMP-1 and active MMP-9 levels in 62 patients with SSc, 10 patients with rheumatoid arthritis (RA) and 15 normal controls using a modified enzyme-linked immunosorbent assay. The active MMP-9 level in the serum of the patients with RA or SSc was not significantly different from that of controls. Serum MMP-9 activity in patients with diffuse cutaneous SSc was significantly decreased compared with that of limited cutaneous SSc or normal controls. The MMP-9 activity and modified-Rodnan total skin thickness score in patients with SSc were negatively correlated. Serum MMP-9 activity and TIMP-1 level in patients with SSc were not correlated. Serum MMP-9 activity might be a useful indicator of disease activity, especially skin severity, in SSc patients.

Adolescent↗
PubMed2001

[Evidence-based therapy of systemic sclerosis].

For the treatment of different forms of systemic sclerosis (SSc), drugs play a predominant role. Depending on disease activity as well as type and severity of cutaneous, vascular and internal organ manifestations, different systemic (antiinflammatory, immunosuppressive, antifibrotic) or organ-specific therapies are used. The scientific basis of most treatment modalities is insufficient and incomplete. There is sufficient evidence for an antiinflammatory and antiproliferative efficacy of glucocorticosteroids, methotrexate, cyclophosphamide and cyclosporine A in the treatment of diffuse cutaneous systemic sclerosis. Vasoactive therapies play an important role in treating Raynaud's phenomenon (nifedipine or other dihydropyridines, prostaglandin analogs, losartan, prazosine), and arterial (ACE blockers, AT-1 antagonists) or pulmonary (epoprostenol) hypertension. Cyclophosphamide is effective in fibrosing alveolitis, prokinetic substances (metoclopramid, domperidone) in gastroesophageal dysmotility or octreotide in intestinal pseudoobstruction. Physical therapies (e.g., massage) are poorly studied. In particular cases, surgical measures (e.g., removal of calcifications) are necessary.

Antirheumatic Agents↗
PubMed1982

Association of antinuclear and antinucleolar antibodies in progressive systemic sclerosis.

Antinuclear and/or antinucleolar antibodies were demonstrated in the sera of 74 of 76 patients (97%) with progressive systemic sclerosis, using tissue culture cells (HEp-2) as substrate in the indirect immunofluorescent method. Six patterns of nuclear staining and three nucleolar patterns were recognized. The nuclear patterns were centromere, fine speckles, coarse speckles, diffusely grainy, homogeneous and nuclear dots. The nucleolar patterns were speckled, homogeneous and clumpy. The results of digestion studies with ribonuclease, deoxyribonuclease and trypsin suggested that the nuclear antigens are proteins, some of which may be associated with chromatin. The nucleolar antigens appeared to be nucleic acid in nature. Certain characteristic serologic and clinical features associated with staining patterns were observed. The diffusely grainy pattern was seen only in sera containing antibody to Scl-70 antigen. Centromere staining was confirmed to be highly selective for the CREST (Calcinosis, Raynaud's phenomenon, esophageal involvement, sclerodactyly and telangiectasis) variant of progressive systemic sclerosis with rheumatoid factor titres higher in these patients with anti-centromere antibodies.

Adult↗
PubMed1987

Longitudinal changes in lung function and respiratory symptoms in progressive systemic sclerosis. Prospective study.

Most patients with progressive systemic sclerosis (PSS) exhibit lung involvement. However, the natural history of lung disease in PSS remains poorly defined. To evaluate lung function over time in PSS, a battery of lung function tests were prospectively performed serially between 1973 and 1982 in 61 patients with PSS. Functional indexes of restriction (vital capacity and total lung capacity) and diffusion impairment (diffusing capacity) showed greater-than-expected annual rates of change. Male subjects showed a trend toward faster declines in forced vital capacity, forced expired volume in one second, total lung capacity, and functional residual capacity and a more rapid increase in static recoil pressure at 90 percent of total lung capacity than did female subjects. Nonsmokers had greater rates of decline in total lung capacity and static lung compliance (but not in forced vital capacity or diffusing capacity) and a greater rate of increase in static recoil pressure than did current and former smokers. Level of lung function at initial study visit, age, race, and chlorambucil therapy had no significant effect on the annual rates of change in lung function, whereas longer duration of disease prior to study entry was associated with a slower annual decrease in lung volumes. Between the first and last visits (mean interval 3.1 years, maximum nine years), the frequency of abnormality in pulmonary function test results showed significant change only in the diffusing capacity (60 percent increasing to 82 percent) and static lung compliance (40 percent increasing to 54 percent), whereas the frequency of respiratory symptoms showed little change. These findings indicate an overall indolent progression of PSS-related lung disease, with substantial individual variability.

Adult↗
PubMed2002

[Systemic sclerosis and pregnancy].

PURPOSE: Pregnancy in a patient with systemic sclerosis (SSc) may pose a double problem to the medical team: influence of SSc on pregnancy and consequences of pregnancy to SSc manifestations. CURRENT KNOWLEDGE AND KEY POINTS: Concepts have evolved. SSc was considered for a long time not only as not very propitious for pregnancy but also as a strict contraindication for procreation because risks for the mother and the baby were thought to be major. Currently, fertility is thought to be normal. Miscarriages and small-for-gestation age infants rate do not seem to be higher in SSc. Maternal and perinatal mortality is also not higher in SSc without severe visceral manifestations, i.e. without either pulmonary hypertension, or cardiac or respiratory insufficiency. Conversely, there is a significantly higher frequency of premature infants in SSc. As regards influence of pregnancy on SSc, the greatest fear is the occurrence of renal crisis, which may be life threatening for both mother and child. Each elevation of blood pressure, even if this increase is mild, should be considered as potentially very serious. However, pregnancy itself does not seem to increase the risk of renal crisis. Consequences of pregnancy to SSc manifestations are various but usually mild. FUTURE PROSPECTS AND PROJECTS: SSc is not a strict contraindication for pregnancy only if severe organ involvement, diffuse subset of SSc or recent onset of the disease has been ruled out. Physicians should be aware of specific problems, which SSc is possibly posing during pregnancy. Finally, it has been recently suggested that pregnancies could be involved in the pathogenesis of SSc through persisting microchimerism of fetal origin.

Acute Disease↗
PubMed1979

Measurement of clinical change in progressive systemic sclerosis: a 1 year double-blind placebo-controlled trial of N-acetylcysteine.

N-acetylcysteine was employed as a therapeutic agent in a 1-year, parallel, double-blind, placebo-controlled, prospective study of 22 patients with progressive systemic sclerosis (PSS). Patients were closely followed-up and were extensively evaluated at baseline, 6 and 12 months. Most parameters remained unchanged during 1 year of study in both the NAC and placebo groups, which showed the need for prolonged studies of drugs in PSS if definitive results are to be obtained. Over the year of the study the diffusing capacity decreased in both groups, while several other parameters measured both increased and decreased. Several simple measures of function and skin distensibility were tested and two (oral aperature and handspread) were reproducible and sensitive enough to measure moderate changes of disease activity. They may be useful in future trials of drug effect in PSS.

Acetylcysteine↗
PubMed1996

Antiphospholipid antibodies and retinal vascular disease.

BACKGROUND: Antiphospholipid antibody retinopathy is a rare and poorly defined clinical entity in which the proper diagnosis has important ocular and systemic implications. The clinical course of five patients with antiphospholipid antibody retinopathy is described and the role of such antibodies in idiopathic retinal venous occlusive disease is investigated. METHODS: Five case reports are presented highlighting the presentation, course, therapy, associations and outcomes of patients with antiphospholipid antibody retinopathy. In addition, twenty additional patients presenting with idiopathic retinal venous occlusive disease were tested for the presence of antiphospholipid antibodies. RESULTS: All five patients with antiphospholipid antibody retinopathy had diffuse retinal vascular occlusion. All five patients presented with associated rheumatologic disease, including three with lupus or lupus-like disease. Antibodies to antiphospholipid were not detected in any of the twenty patients with idiopathic retinal venous occlusive disease. Prompt panretinal photocoagulation together with varying regimens of corticosteroids, immunosuppressives, or warfarin was partially successful in stabilizing the ocular and systemic disease. CONCLUSION: The diagnosis of antiphospholipid antibody retinopathy should be suspected in patients with diffuse retinal vaso-occlusion, particularly when characterized by involvement of both arteries and veins, neovascularization at presentation, and symptoms of symptoms of systemic rheumatologic disease. Antiphospholipid antibodies do not appear to play an important role in idiopathic retinal vein occlusions.

Adult↗
PubMed1997

Association of progressive systemic sclerosis with pulmonary sarcoidosis. Just a chance occurrence?

A 46-year-old Japanese woman has been followed up for 3 years due to interstitial pneumonia associated with progressive systemic sclerosis (PSS). During this follow-up period, chest roentgenogram revealed additional diffuse nodular shadows. She was diagnosed as having pulmonary sarcoidosis, which was confirmed by the presence of epitheloid granulomas within the alveolar septa. She was successfully treated with corticosteroids and recovered almost completely without worsening pulmonary abnormalities caused by her PSS. The independent clinical courses of these two diseases in the present case suggest that the complication of PSS and sarcoidosis in this patient may be coincidental.

Adrenal Cortex Hormones↗
PubMedDate not supplied

[Autologous stem cell transplantation in a patient with diffuse systemic sclerosis].

Systemic Sclerosis (SSc) is a systemic disease of unknown etiology presenting with disseminated skin thickening and fibrotic impairment of various organs including lung and kidney. According to the rate and degree of skin involvement, SSc can be classified in a limited and a diffuse form, the latter showing a severe and progressive lung involvement, which is responsible for its high related morbidity and mortality along with resistance to standard therapeutic protocols. High dose chemotherapy, followed by autologous stem cell transplantation, is a standard therapeutic regimen for haematological diseases: re-infusion of mobilised peripheral blood progenitor cells overcomes the myeloablative effect of super-maximal eradicative doses of chemotherapeutic agents. Recently, this therapeutic approach has been applied in some cases of resistant SSc and, albeit the low number of cases, it has been proven effective in early diagnosed and rapidly progressive forms of the disease showing a clinical improvement and an instrumentally detectable decrease of fibrosis extent. We report the case of a young woman affected by diffuse SSc with a rapid progression of clinical signs and instrumentally detectable lesions who underwent a conditioning regimen with fludarabine, cyclophosphamide and anti-thymoglobulines followed by re-infusion of autologous peripheral blood stem cells. Two years after transplantation a clinical and instrumental evidence of treatment was observed, with good control of disease evolution. The only sign of disease resumption was a slow worsening of skin involvement.

Adult↗
PubMed2001

Influence of clinical features, serum antinuclear antibodies, and lung function on survival of patients with systemic sclerosis.

OBJECTIVE: To evaluate the independent contribution of several clinical and laboratory variables to the mortality of a cohort of Danish patients with systemic sclerosis (SSc). METHODS: A cohort of 174 patients with incident SSc was retrospectively identified using clinical charts and study records of all new patients with SSc. Disease onset was defined as the time of onset of cutaneous sclerosis. Vital status and causes of death were determined at the end of the observation period. Data on clinical status and pulmonary function were obtained. Antitopoisomerase I (anti-topo I), anticentromere, anti-U1-RNP, anti-U3-RNP, anti-Th-RNP, and anti-RNA polymerase (anti-RNAP) antibodies were determined by means of double immunodiffusion, immunofluorescence, hemagglutination technique, radioactively labelled antisense riboprobes, and ELISA, respectively. RESULTS: Patients were followed for a mean period of 13.3 yrs; 16 died of an SSc related condition and 50 of other causes. Pulmonary fibrosis, DLCO reduction < 40% of the expected, diffuse cutaneous involvement, SSc nephropathy, cardiac disease, and anti-topo I and anti-RNAP antibody were related to decreased survival due to SSc. Variables that entered a Cox regression model of SSc related mortality were right heart failure (RR 12.4, 95% CI 2.5-60), diffuse SSc (RR 7.8, 95% CI 1.8-35), SSc nephropathy (RR 6.1, 95% CI 1.8-21), and DLCO < 40% (RR 4.8, 95% CI 1.1-20). The relative risk of developing right heart failure and diffuse SSc given the presence of anti-RNAP antibody was 14 (p = 0.0001) and 1.9 (p = 0.01), respectively. The corresponding figures for anti-topo I antibody were 4.6 (p = 0.02) and 2.0 (p = 0.01). CONCLUSION: SSc related mortality was associated with right heart failure and diffuse SSc, both of which were also associated with the presence of anti-topo I and anti-RNAP antibody. The prognostic value of these autoantibodies may lie in the early course of the disease when specific morbidity has not yet evolved.

Adolescent↗
PubMed1989

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

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

Constriction, Pathologic↗
PubMed2004

Circulating endothelial cells as a marker of ongoing vascular disease in systemic sclerosis.

OBJECTIVE: Circulating endothelial cells (CECs) have been described in different conditions involving vascular injury. Vascular abnormalities play a key role in the pathogenesis of systemic sclerosis (SSc). The aim of this study was to search for the presence of CECs in patients with SSc and to evaluate their clinical associations and possible pathogenic role. METHODS: The study cohort included 46 patients with SSc and 40 healthy controls. Five-parameter, 3-color flow cytometry was performed with a FACScan. CECs were defined as CD45 negative, CD34 positive, and P1H12 positive, and activated CECs were defined as CD45 negative and P1H12 positive, CD62 positive, or CD106 positive. Progenitors were identified as CD34 positive and CD133 positive. RESULTS: Total and activated CEC counts were significantly higher in SSc patients compared with healthy controls and were positively correlated with the disease activity score. With respect to visceral involvement, significant correlation was observed between the CEC number and the severity of pulmonary hypertension. High levels of endothelial progenitors were observed in patients with SSc, and the counts were higher in the early stages of disease. CONCLUSION: The presence of CECs in patients with SSc may represent direct evidence of endothelial disease and may be a promising new clinical marker for active SSc. Notably, the association between CECs and pulmonary hypertension and impaired carbon monoxide diffusing capacity was evident in patients with limited cutaneous SSc only, suggesting an important role for CECs in this disease subset with prominent vascular changes. Detection of circulating endothelial progenitors may represent a response to vascular ischemia in early SSc, as an attempt at revascularization.

Adult↗
PubMed1986

Microangiopathy of progressive systemic sclerosis. Evaluation by dynamic fluorescence videomicroscopy.

Morphologic changes of the nail-fold capillaries have been studied extensively in progressive systemic sclerosis (PSS). With videomicroscopy after intravenous injection of fluorescein sodium as a tracer, a more accurate morphologic description became possible, including data on the pericapillary interstitial space and the measurement of transcapillary diffusion of dye (videodensitometry). The results in 13 patients with PSS and Raynaud's phenomenon were compared with those obtained in 12 healthy volunteers. The diameters of the patients' nail-fold capillaries were significantly increased. The normal barriers for diffusion of the dye are located at the capillary wall and at the outer border of the skin papilla (halo). Both were less effective in PSS, with significantly enhanced fluorescent light intensities in the juxtacapillary region and outside of the halo. "Streetlike" or "lakelike" areas are stained in the remote parts, demonstrating abnormal architecture of the interstitial space. The technique could facilitate early detection of PSS.

Absorptiometry, Photon↗