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PubMed1990

Adult respiratory distress syndrome after limited resection of adenocarcinoma of the lung.

Two cases of the adult respiratory distress syndrome developed after limited resection for lung carcinoma. No other known precipitants were evident. The adult respiratory distress syndrome is a clinical expression of acute lung injury that may arise from various insults and include air and blood borne factors. Tumour related blood borne factors may have contributed to lung injury in these cases.

Adenocarcinoma↗
PubMed1992

Anti-interleukin-6 autoantibodies in rheumatic diseases. Increased frequency in the sera of patients with systemic sclerosis.

OBJECTIVE: To investigate the presence and the roles of anti-interleukin-6 (anti-IL-6) autoantibodies in rheumatic diseases, and to further elucidate clinical and pathophysiologic significance of anticytokine autoantibodies. METHODS: Anti-IL-6 IgG autoantibodies were measured by the 125I-IL-6 binding activity of IgG, which was isolated from serum by protein A-Sepharose. RESULTS: Nine of 52 sera (17.3%) from patients with systemic sclerosis (SSc) contained anti-IL-6 antibodies, whereas only 1.9% of sera from normal subjects and 0-5% of sera from patients with other rheumatic diseases were positive for the antibodies. Moreover, anti-IL-6 autoantibodies were found predominantly among patients with the limited form of SSc (42.9%), compared with those with the diffuse form (7.9%). CONCLUSION: Anti-IL-6 IgG autoantibodies were detected in patients with SSc, particularly those with the limited form of the disease, at a significantly increased frequency compared with normal subjects and patients with other rheumatic diseases. These results suggest that the development of anti-IL-6 autoantibodies and IL-6 may have a role in the pathophysiology of SSc.

Adult↗
PubMed1996

Comprehensive noninvasive assessment of cardiac involvement in limited systemic sclerosis.

OBJECTIVE: To assess cardiovascular abnormalities in patients with limited systemic sclerosis (SSc), using noninvasive cardiac techniques. METHODS: Sixty-three patients with limited SSc were prospectively evaluated with Doppler echocardiography and thallium-201 perfusion scintigraphy after a cold-stress test and radionuclide ventriculography. RESULTS: In the patients with limited SSc, there was a significantly high prevalence of abnormal left- and right-diastolic function parameters (P = 0.001 and P = 0.0002, respectively), thickening of papillary muscles (46%; P = 0.003), and mild mitral regurgitation (49%; P < 0.0001), compared with controls. Systolic pulmonary arterial hypertension was detected in 9 patients (14%), and pericardial effusion in 11 patients (18%). In 64% of patients with limited SSc, an ischemic response was detected on the thallium cold-stress scan; similarly, an ischemic response was detected in 57% of patients with primary Raynaud's phenomenon (P < 0.0001 versus controls). CONCLUSION: Although the frequency of cardiovascular symptoms was low in patients with limited SSc, a significant rate of cardiovascular abnormalities was found by noninvasive cardiac techniques.

Adult↗
PubMed1997

Characteristic epitope recognition pattern of autoantibodies against eukaryotic ribosomal protein L7 in systemic autoimmune diseases.

OBJECTIVE: To define the epitope-recognition pattern and the fine specificity of the autoantibody response to protein L7 in patients with rheumatic diseases. METHODS: The epitope-recognition pattern was studied by enzyme-linked immunosorbent assay utilizing overlapping fragments of L7. The fine specificity was examined by binding inhibition and isoelectric focusing. RESULTS: We observed a disease-specific epitope-recognition pattern of anti-L7 autoantibodies. There was one immunodominant epitope that was recognized by all anti-L7-positive sera from patients with systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), and systemic sclerosis (SSc). Additional recognition of minor epitopes was observed; it arises by intramolecular epitope spreading and was correlated with disease activity in SLE patients. SSc patients differed from SLE and RA patients in that their sera did not recognize certain minor epitopes. The major epitope was recognized by high-affinity autoantibodies of limited heterogeneity. Minor epitopes were recognized by heterogeneous low-affinity autoantibodies. CONCLUSION: The anti-L7 autoantibody response is oligoclonal. Additional B cell clones are activated by antigen during active phases of disease.

Adolescent↗
PubMed1990

Pericardial tamponade and limited cutaneous systemic sclerosis (CREST syndrome)

A patient with limited cutaneous systemic sclerosis (ISSc) is described who presented with acute chest pain, dyspnoea and signs of cardiac tamponade requiring pericardiocentesis although the quantity of effusion was moderate. The haemodynamic data recorded from this patient suggested that pericardial fibrosis may be a major contributing predisposing factor to pericardial tamponade, while the cause of pericarditis and effusion remain obscure.

Cardiac Tamponade↗
PubMed1997

Limited microsurgical arteriolysis for complications of digital vasospasm.

Twenty-two digits in seven patients with chronic digital vasospasm were surgically treated after failed medical management. All patients complained of severe ischaemic pain. Chronic digital tip ulceration was present in seven digits and dry gangrene in one. Following surgical microarteriolysis all digital ulcers healed completely. Severe digital ischaemic pain was significantly improved in all digits and completely resolved in 19 of 22 digits.

Adult↗
PubMed2005

Thymus alterations and systemic sclerosis.

OBJECTIVES: The pathogenesis of systemic sclerosis (SSc) includes complex alterations to the immune system, possibly responsible for diffuse microvasculature and fibroblast dysfunction. Previous anecdotal observations suggest a possible role for thymus alterations in some autoimmune rheumatic diseases, including SSc. This study aimed to investigate the prevalence of radiological thymus alterations in SSc patients. METHODS: Thirthy-four unselected patients [28 female and 6 male, mean age (+/- S.D.) 49.7 +/- 9.5 yr, range 33-67 yr] and 34 age- and sex-matched controls were included in the study. The presence of major radiological thymus alterations, i.e. an abnormally enlarged or nodular thymus, were blindly investigated by means of unenhanced multidetector computed tomography. RESULTS: Abnormally enlarged or nodular thymuses were detected in a statistically significant percentage of SSc patients compared with controls (21 vs 0%, P = 0.011). More interestingly, radiological thymus alterations were invariably observed in patients with shorter disease duration (< or =5 yr, 41% vs >5 yr, 0%; P = 0.007), frequently associated with serum anti-Scl70 antibodies (P = 0.017). Among patients with thymus alterations one developed myasthenia gravis while two others showed thymus hyperplasia at histopathological evaluation after thymectomy. CONCLUSIONS: The present study suggests a possible role of thymic disorders, mainly thymus hyperplasia, in a significant number of SSc patients. Due to the limitations of radiological evaluation, the actual relevance of such an association might be underestimated. The relationship of thymus alterations with shorter disease duration, as well as with serum anti-Scl70, suggests that thymic dysfunction could play a pathogenetic role mostly in the early phases of the disease, and possibly in specific SSc patient subsets.

Adult↗
PubMed1992

Intravenous iloprost treatment of Raynaud's phenomenon and ischemic ulcers secondary to systemic sclerosis.

OBJECTIVE: We conducted this study to assess the clinical usefulness and physiologic effects of intravenous iloprost in patients with Raynaud's phenomenon secondary to systemic sclerosis. METHODS: Thirty-five patients with Raynaud's phenomenon secondary to systemic sclerosis, including 11 with digital ischemic ulcerations, were enrolled in a double blind placebo controlled parallel study in 2 centers. Following a 2 week washout, subjects received intravenous iloprost (0.5-2.0 ng/kg/min) or saline by continuous infusion for 6 h on 5 consecutive days. Clinical assessments, status of digital ulcers, measures of in vivo platelet activation and detailed studies of peripheral vascular response to cold challenge, were performed at entry, at 5 days of therapy and at biweekly intervals for 10 weeks. RESULTS: Complete healing of all cutaneous lesions (ulcers, fissures, and paronychia) was observed 10 weeks after treatment in 6 of 7 patients receiving iloprost versus none of 4 receiving placebo (p = 0.015). Ischemic digital tip ulcers completely healed in all 4 patients with ulcers in the iloprost group, but none in the placebo group (p = 0.029). Patient diaries of frequency, duration and symptoms of Raynaud's phenomenon showed improvement in both groups. Critical ischemic temperature (finger temperature during controlled cold challenge at which Raynaud's or loss of detectable digital blood flow occurred) progressively decreased in the iloprost group from 21.3 +/- 7.3 degrees C at baseline to a minimum of 16.1 +/- 3.2 degrees C at 8 weeks after treatment (p = 0.076), whereas no consistent changes were observed in the placebo group. Treatment was associated with improvement in the rate of skin temperature recovery following cold challenge. No changes were noted in ambient digital skin temperature, total digital blood flow, finger systolic pressure or in measures of in vivo platelet activation. One subject dropped out with chest pain, but adverse effects of nausea, vomiting, headache and jaw pain were otherwise limited to the 5 days of drug infusion. CONCLUSION: Iloprost appears useful for the treatment of digital ulcers in systemic sclerosis and is associated with evidence of prolonged physiologic improvement although the mechanism of this effect remains unclear.

Adult↗
PubMed1992

Alterations in tryptophan metabolism in the toxic oil syndrome and in the eosinophilia-myalgia syndrome.

The eosinophilia-myalgia syndrome (EMS) was associated with ingestion of L-tryptophan containing products and was accompanied by altered metabolism of L-tryptophan during the active phase. Many patients with EMS exhibited clinical and histopathological features similar to another epidemic, the toxic oil syndrome (TOS), associated with ingestion of adulterated rapeseed oil. We hypothesized that patients with TOS, like patients with EMS, may have had altered metabolism of L-tryptophan during the acute phase of the illness. Therefore, we quantitated the tryptophan metabolites, L-kynurenine and quinolinic acid, and we measured neopterin, a marker of interferon-gamma (IFN-gamma), in blood obtained during the acute phase of each syndrome. Patients with TOS or EMS had significantly higher L-kynurenine and quinolinic acid than healthy control subjects or rheumatic disease control subjects. Neopterin was also elevated in patients with untreated TOS and EMS, and correlated strongly with L-kynurenine and quinolinic acid. Our data suggest that indoleamine-2,3-dioxygenase (IDO), the rate limiting enzyme of the kynurenine pathway of L-tryptophan metabolism, was activated in both syndromes by cytokines including IFN-gamma, and that perhaps products of tryptophan metabolism played a role in the pathogenesis of EMS and TOS.

Arthritis, Rheumatoid↗
PubMedDate not supplied

Bowed fingers. A helpful sign in the early diagnosis of systemic sclerosis.

Thirteen patients who had in common the clinical finding of shortened forearm flexors were investigated. Additional findings were Raynaud's phenomenon, arthralgia, reduced pulmonary CO diffusion capacity, abnormal EMG, and histological evidence of myopathy. Clinical and serological features in 2 patients were consistent with features described in the mixed connective tissue disease syndrome. The remainder were regarded as having a limited connective tissue disease syndrome, in all probability, a minimal expression of systemic sclerosis. We suggest that the finding of bowed fingers is a more reliable clinical sign than the assessment of skin texture in determining the presence of minimal connective tissue disease.

Adult↗
PubMed1995

[A case of melorheostosis with linear sclerodermatous skin changes].

A 69-years old Japanese woman complained of pain in the left elbow joint and thickened skin over the left upper limb. The pain had been present for 20 years, and the thickened area of the skin gradually enlarged during this period. Her left elbow joint showed some limitation of motion. There was no record of any similar condition in her family history. Radiographs of the left limb showed cortical hyperostosis extending from the middle of the left humerus to the distal end of the radius. Radiographs of the other limbs were normal. A technetium 99m-methylene diphosphonate bone scintigraphy revealed increased uptake in the areas of radiographic hyperostosis. The diagnosis of melorheostosis was made. Skin biopsy of thickened area was performed. The epidermis was normal, and proliferation of normal-appearing collagen fibers into the subcutaneous fat was noted. No inflammatory changes were found. The cause of sclerodermatous skin changes was thought to be not by linear scleroderma but by melorheostosis. In cases of linear sclerodermatous changes, melorheostosis as its origin should be considered.

Aged↗
PubMed1978

Sclerodermatous graft-versus-host disease limited to an area of measles exanthem.

A female patient with severe idiopathic aplastic anaemia received a successful bone-marrow transplant from her HLA-identical, mixed-lymphocyte-culture-compatible, brother. 8 months after transplantation she had localised cutaneous measles. Chronic sclerodermatous changes developed which were indistinguishable from chronic graft-versus-host disease and were limited to the areas of the original exanthem. Interaction between viral infection and minor histocompatibility differences probably resulted in graft-versus-host disease in this patient.

Adolescent↗
PubMed1992

The evolution of a case of overlap syndrome with systemic sclerosis, rheumatoid arthritis and systemic lupus erythematosus.

An unusual case of overlap syndrome which evolved over a 12-year period is described. The patient initially presented with limited cutaneous systemic sclerosis. She then developed seropositive erosive rheumatoid arthritis and subsequently vasculitis with positive lupus serology. There was no evidence that she had mixed or undifferentiated connective tissue disease, and antibody to ribonuclearprotein was negative. This unusual combination of connective tissue disorders in one patient is reported and the literature is reviewed.

Arthritis, Rheumatoid↗
PubMedDate not supplied

Postirradiation morphea in a breast cancer patient.

We present the case of a 52-year-old woman who underwent lumpectomy and radiation therapy for breast carcinoma and 2 years later presented with changes limited to the breast radiation field including hyperpigmentation, retraction, and skin thickening. These clinical features, along with pathologic findings, were diagnostic of postirradiation morphea. It is important to be aware of this rare complication of radiation therapy because it clinically presents with symptoms mimicking malignancy and may be misinterpreted as recurrent carcinoma or even angiosarcoma.

Breast Neoplasms↗
PubMed1994

Plasma TGF beta in systemic sclerosis: a cross-sectional study.

OBJECTIVES: To determine whether the active 25 kDa form of the fibrogenic cytokine transforming growth factor beta (TGF beta) can be detected in plasma from patients with systemic sclerosis and to examine the relationship between plasma TGF beta and clinical markers of disease severity and serum concentrations of the aminoterminal peptide of type III procollagen (PIIINP) (a laboratory marker of the fibrotic process). METHODS: A cross sectional study was made of 39 patients with systemic sclerosis (11 diffuse and 28 limited), nine patients with primary Raynaud's disease and 60 healthy controls. TGF beta 1 and TGF beta 2 were measured by enzyme linked immunosorbent assay (ELISA) (sensitivity 100 pg/ml) and PIIINP by radioimmunoassay. RESULTS: TGF beta 1 was detected in plasma from six of 39 patients with systemic sclerosis but not in any patient with primary Raynaud's disease or healthy controls. TGF beta 2 was not detected in plasma from patients or controls. No clear relationship was demonstrated between TGF beta 1, clinical features or PIIINP concentrations. CONCLUSIONS: The 25 kDa form of TGF beta 1 can be detected in the plasma of some patients with systemic sclerosis. This provides limited support for the hypothesis that this cytokine plays a role in the pathogenesis of this disease. However, longitudinal studies, particularly in early diffuse disease, are required to clarify the relationship between circulating TGF beta 1 and disease activity.

Adult↗
PubMed1998

Molecular heterogeneity of second and fourth components of complement and their genes in systemic sclerosis and association of HLA alleles A1, B8 and DR3 with limited and DR5 with diffuse systemic sclerosis.

Deficiency of the complement component C4 at the functional, protein and gene level and deficiency of complement component C2 at the functional level were investigated and HLA analysis was performed on patients with limited and diffuse systemic sclerosis (SSc). One of the patients with limited SSc (n = 15) had subnormal C4, 1 subnormal C2 and 1 subnormal C4 and C2 activities; the latter patient had HLA alleles A11;B35;Dw1 associated with type II C2 deficiency and therefore most likely had a defect at the C2 locus. One of the patients with diffuse SSC (n = 12) had subnormal C4 and 1 subnormal C4 and C2 activities. C2 deficiencies in patients other than the one with the haplotype associated with C2 deficiency appeared not to be determined by the gene at the C2 locus. The incidence of partial C2 deficiency in a normal Caucasian population is reported to be 16 in 10,000, and that of partial C4 deficiency also appears to be very low. The percentages of C4A*Q0 and C4B*Q0 alleles in normal controls (n = 45) were within the reported range. Seven patients with limited SSc (n = 14) had one or two C4A*Q0 alleles and 2 with diffuse SSc (n = 13) had one C4A*Q0 allele. Thus, the incidence of C4A*Q0 was higher than normal in limited SSc and within the normal range in diffuse SSc. The two-sided Fisher's exact test applied on these data revealed that the association of C4A*Q0 with limited SSc did not reach a significant level (p = 0.10). Two of the 3 patients with limited SSc, who had two C4A*Q0 alleles, carried a heterozygous C4A-21-hydroxylase A (OHA) gene segment deletion as detected by Southern blotting. There was no correlation between the subnormal activity of C4 and the occurrence of one or two C4A*Q0 (and C4A-21-OHA segment deletion). HLA alleles A1, B8 and DR3 (p = 0.002) were associated with limited SSc (n = 23) and DR5(w11) (p = 0.018) with diffuse SSc (n = 17).

Alleles↗
PubMed2004

Eosinophilia in rheumatologic diseases: a prospective study of 1000 cases.

The role of eosinophilia in connective tissue diseases and the relationship between symptoms of rheumatic disease and eosinophilia have not been clearly established. The purpose of the present study was to explore the prevalence of eosinophilia in rheumatologic disease and determine its relationship to the symptoms. One thousand patients who applied to our rheumatology outpatient clinic between 2001 and 2002 were prospectively studied. The upper limit of normal blood eosinophil numbers was determined as 500 cells/microl of blood. A detailed history was obtained from all patients and careful physical examination was done. A negative correlation was observed between eosinophilia and dryness of the mouth, vitiligo, and fatigue (P < 0.05). Nonsteroidal anti-inflammatory drug usage correlated positively with eosinophilia, which was also statistically meaningful (P < 0.05). Twenty-six of our patients with fibromyalgia (n = 293), three of our subjects with rheumatoid arthritis who were using methotrexate (n = 182), 15 of whom who were not on methotrexate therapy, and one of the 26 with vasculitis had eosinophilia, which was not statistically significant (P > 0.05). None of the patients with scleroderma (n = 12) had eosinophilia. Eleven of the patients with gout had eosinophilia, and only one of them was using allopurinol. We conclude that eosinophilia can be seen in various rheumatologic conditions but, as corticosteroids are one of the most common medications used in collagen tissue diseases, the eosinophil numbers found may be lower than expected and eosinophilia may be more frequent than reported.

Adult↗
PubMed1998

Diversity of myocardial involvement in systemic sclerosis: an 8-year study of 95 Japanese patients.

BACKGROUND: Myocardial fibrosis has been reported to be an important complication of systemic sclerosis (SSc). Left ventricular (LV) hypertrophy has also been reported, but its significance is unclear. Correlations between cardiac complications and clinical or serologic subsets of SSc have not been previously evaluated by long-term observational studies. METHODS: We correlated the findings of echocardiography, electrocardiography, and autopsy with clinical and serologic features in 95 patients with SSc who were monitored for up to 8 years. RESULTS: Septal thickness determined by echocardiography was significantly greater in patients with anti-nuclear ribonucleoprotein (nRNP) antibodies than in patients with anti-Scl70 or anticentromere antibodies. Fractional shortening was reduced in patients with diffuse cutaneous involvement or anti-Scl70 antibodies. Ten patients had cor pulmonale, which was confirmed by echocardiography. Four of them had diffuse cutaneous involvement and advanced pulmonary fibrosis, whereas four had anti-nRNP antibodies and mild or no fibrosis. Septal hypertrophy was detected in 12 patients, 10 of whom had limited cutaneous involvement and six had anti-nRNP antibodies. Diffuse or regional LV wall hypokinesia was seen in seven patients, of whom six had anti-Scl70 antibodies and five had diffuse cutaneous involvement. Six patients with cor pulmonale, five with abnormal LV wall motion, and three with septal hypertrophy died during the follow-up period. Autopsy revealed LV hypertrophy in three with septal hypertrophy, disarray and irregular hypertrophy of myocytes in one of them, and extensive myocardial fibrosis in one with diffuse LV wall hypokinesia. CONCLUSIONS: Septal hypertrophy and cor pulmonale caused by pulmonary vasculopathy were associated with limited cutaneous involvement or anti-nRNP antibodies, whereas abnormal LV wall motion and cor pulmonale caused by advanced pulmonary fibrosis were mostly seen in patients with diffuse cutaneous involvement or anti-Scl70 antibodies.

Adult↗