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PubMed1995

[The concept of the pathogenesis of occlusive disorders in diseases of the temporomandibular joint].

Abnormal occlusion developing in adult patients is always caused by involvement of the temporomandibular joints. Destructive diseases of an infectious origin are characterized by unilateral involvement of the joints, unilateral reduction of the interocclusal height, and increased abrasion of teeth due to increased masticatory loading. Occlusive contact is preserved in all teeth. The second group of destructive diseases of the temporomandibular joint includes rheumatoid arthritis and systemic scleroderma and is characterized by symmetrical involvement, polyarthritis, and rapid progress. Clinically frontal occlusion develops, which is caused by deformation of articular processes, mandibular body, angle, and branch.

Adolescent↗
PubMed2003

[Stem cell transplantation in primary systemic vasculitis].

BACKGROUND: Many patients with various autoimmune diseases fail to respond to conventional immunosuppressive therapy and develop irreversible organ damage. In some cases the complications might be fatal. Furthermore, prolonged immunosuppression with cyclophosphamide or corticosteroids often leads to long-term side effects, cumulative organ damage, and development of secondary malignancies. THERAPY: Thus, short-term, high-dose immunosuppressive therapy with autologous stem cell transplantation might be an alternative for otherwise refractory patients. This concept has been used mainly in patients with scleroderma, systemic lupus erythematosus, rheumatoid arthritis, and multiple sclerosis. PATIENTS: Patients with primary systemic vasculitis (PSV) seem to be suitable candidates for high-dose chemotherapy (HDCT). Although complete, long-term remissions are possible with standard immunosuppressive therapy, there is a subgroup of patients who cannot be cured by standard therapy. They need long-term immunosuppression and eventually die due to progression of the disease or cumulative therapy-related toxicity. In large series, the 5-year mortality rate was > 20%. The severity of vasculitis rather than the distinction between subgroups should determine whether HDCT might be a life-saving treatment. RESULTS AND CONCLUSION: Careful patient selection with the help of scoring systems and determination of the optimal time in the course of disease are now the major goals in the approach to HDCT. First reports as well as our own single-center experience in HDCT with a limited number of patients with severe PSV have shown that long-term remissions are possible even in patients refractory to conventional immunosuppression.

Adrenal Cortex Hormones↗
PubMed1997

Mitochondrial abnormalities and peripheral neuropathy in inflammatory myopathy, especially inclusion body myositis.

Computer retrieval in a database, comprising 7,225 muscle cases, revealed that mitochondrial myopathies do not occur more frequently in inflammatory myopathies (3.74%) than in the whole series (3.69%). A more detailed study of inclusion body myositis (IBM), however, showed that severe mitochondrial alterations were apparent in about twice as many IBM cases as expected. This confirms recent studies of others although a causal relationship has thus far not been established. Identification of mitochondrial deletions by Southern blotting corresponded to the presence of severe structural abnormalities of mitochondria. Peripheral neuropathy of variable severity was noted in all cases of IBM and mitochondrial myopathy. By contrast, the association of severe mitochondrial abnormalities with polymyositis, systemic scleroderma, and vasculitis observed in some cases of the present series may be incidental or age dependent.

Adult↗
PubMedDate not supplied

[The clinical significance of disorders of humoral immunity in Sjögren's disease and syndrome].

Data on 100 patients: 50 with Sjögren's disease (SD) and 50 with Sjögren's syndrome (SS) accompanied by systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), systemic scleroderma (SSD) were analysed. It was characteristic of SD to have hyperproteinemia, hypergammaglobulinemia, high levels of the rheumatoid factor (RF), circulating immune complexes (CIC), and cryoglobulins, anti-Ro and anti-La; SD + RA was characterized by RF, hypercomplementemia; SS + SLE was characterized by various antibodies to nucleic acids (nDNA, DNP (and anti-Sm, antinuclear factor (ANF) of homogenous and peripheral types of glow, hypercomplementemia. The main systemic manifestations of SD (arthritis, vasculitis) and a high activity of the process was associated with the presence of anti-Ro/La, and marked local manifestations of the dry syndrome--with antibodies to nDNA and dDNA. And vice versa in SS + SLE anti-nDNA antibodies correlated with anemia, serositis, nephritis, vasculitis and the presence of anti-Ro/La--with a comparatively mild renal pathology. The presence of high titres of RF in SB correlated with the absence of the adequate therapy, the presence of hypergammaglobulinemic purpura, weakly pronounced local manifestations; but a decrease in the titres of RF and an increase in the titres of ANF; anti-nDNA, anti-DNP in young patients with SD proved to be a prognostically unfavourable factor. The presence of CIC was associated with hypergammaglobulinemic purpura and affection of the eyes, and hypocomplementemia--with affection of the kidneys, joints and salivary glands in patients with SD.

Adolescent↗
PubMed1997

[Chronic edema, monoclonal dysglobulinemia and profuse telangiectasia: a distinct entity?].

INTRODUCTION: Monoclonal gammopathy and edema are features encountered during several diseases, especially systemic capillary leak syndrome. The diagnoses of POEMS syndrome, edematous systemic scleroderma and a fortuitous association may be also discussed. We report the cases of two patients which did not fulfill the criteria for such diagnoses. CASE REPORT: Although the 2 cases share some discrepancies, they have also similar and particular features: association of chronic edema, monoclonal gammopathy and profuse and acquired telangiectasias. DISCUSSION: The meaning of these cases remains to be clarified. It might be an entity close to the systemic capillary leak syndrome but characterized by the chronicity of edema and by a distinct cutaneous sign, the occurrence of numerous telangiectasias. These cases emphasize that the acquired and profuse telangiectasias belong to the wide range of cutaneous abnormalities which may be associated with monoclonal paraproteinemia.

Capillary Leak Syndrome↗
PubMed2005

Advanced formulation and pharmacological activity of hydrogel of the titrated extract of C. asiatica.

Titrated extract of Centella asiatica (TECA) contains three principal ingredients, asiaticoside (AS), asiatic acid (AA), and madecassic acid (MA). These components are known to be clinically effective on systemic scleroderma, abnormal scar formation, and keloids. However, one problem associated with administration of TECA is its low solubility in aqueous as well as oil medium. In this study, various nonionic surfactants and bile salts as anionic surfactant were tested and screened for solubilizing TECA with a view to developing topical hydrogel type of ointment which is stable physicochemically, and has better pharmacological effects. When TECA was incorporated into various nonionic surfactant systems, labrasol had the most potent capacity for solubilizing TECA. In cases of bile salt systems, Na-deoxycholate (Na-DOC) had foremost solubilizing capacity, even more than labrasol. In differential scanning calorimetric study, the peaks of AA, MA, AS and Na-DOC disappeared at the coprecipitate of 1% TECA and 1% Na-DOC, suggesting the optimum condition of Na-DOC for solubilizing TECA. When the physicochemical stability of hydrogel containing this mixture was assessed, it was stable at room temperature for at least one month. Pharmacologically it significantly decreased the size of wound area at the 9th day when applied to the wound area of rat dorsal skin. Taken together, solubility of TECA was dramatically improved by using nonionic and anionic surfactant systems, and Na-DOC was found to be the most effective solubilizer of TECA in formulating a TECA-containing hydrogel typed ointment. Moreover this gel was considered to be applicable to clinical use for wound healing effect.

Animals↗
PubMed1996

[Deep morphea-type lesions, first manifestations of lymphocytic lymphoma].

INTRODUCTION: The association between scleroderma and lymphoma is uncommon and can sometimes query an fortuitous association. More particular are observations where chronological and histological features show an evident link between a tumorous process and sclerosis. OBSERVATION: This case concerns a man 69 years old who was treated for one year for partial subcutaneous sclerosis present on his legs, thighs, fore-arms, and on the upper part of the trunk. No signs were present of visceral involvement evoking a systemic scleroderma and histology showed an intrication of a deep sclerosis, fasciitis and a tumoral lymphoma process. Diagnosis of lymphocytic lymphoma was confirmed. Initiating a chemotherapy (CHOP) allowed for a reduction of the sclerosis which didn't respond to the only corticotherapy. DISCUSSION: This observation can be linked to the association of fasciitis and lymphoma identified by Naschitz et al, but is different by: 1) the clinical aspect which correspond more to deep morphea lesions; 2) the histological link between sclerosis and lymphoma. This last point suggest that it is the tumoral population which have induced the sclerosis process linked to the secretion of pro-inflammatory factors. In adverse cases with longer delay between lymphoma and scleroderma this association may be fortuitous.

Aged↗
PubMedDate not supplied

[Studies of the role of cellular immunity and angiogenetic changes in the pathogenesis of circumscribed scleroderma].

The studies were performed in 48 patients with morphea and included evaluation of 1) antinuclear antibodies 2) lymphocyte induced angiogenesis 3) natural killer (NK) cell activity and 4) T cell subpopulations in peripheral blood. The presence of antinuclear antibodies was found in 44.4% (8/18) patients with scleroderma linearis and in 21% (4/19) patients with morphea disseminata. Lymphocyte induced angiogenesis was increased in 41.5% (17/41) morphea patients, mainly in cases with pronounced vascular changes. The E rosette forming test showed a decreased percentage of active rosette forming cells (ARFC) and total rosette forming cells (TRFC) in peripheral blood and the NK cell activity was lowered in patients with morphea. These results obtained in patients with morphea show some similarities and differences in comparison to cellular immunity disturbances in patients with systemic scleroderma.

Angiogenesis Inducing Agents↗
PubMedDate not supplied

[The Ha(La) antibody as a serologic marker of systemic lupus erythematosus].

Ha(La)-antibodies are detected by counterimmunoelectrophoresis (CIE) in sera of systemic lupus erythematosus (SLE) patients, but not in sera of patients with progressive systemic scleroderma (PSS) (n = 38), rheumatoid arthritis (n = 91), ankylosing spondylitis (n = 97), and other rheumatic diseases (n = 452). This antibody specificity was found in 27 out of 103 SLE patients (26%). However, 50% of SLE patients with Sjögren's syndrome (n = 12) showed the Ha(La)-antibody, suggesting a strong association in this clinical subgroup. Thus the Ha(La)-antibody can serve as a serological marker and helpful diagnostic tool for SLE. Additionally, the Ha(La)-antibody reacts with specific puffs in polytene chromosome preparations of Drosophila melanogaster. Therefore, this heterologous antigen system is suitable for the identification of Ha(La) sera.

Antibodies, Antinuclear↗
PubMed1994

Selective induction of anti-fibrillarin autoantibodies by silver nitrate in mice.

Female SJL (H-2s) mice developed serum IgG anti-nucleolar antibodies (ANoA) after 5 weeks treatment with 0.05% or 0.01% silver nitrate (AgNO3) in drinking water. Five more weeks of treatment increased the ANoA titre to 3410 +/- 853 and 640 +/- 175 (reciprocal mean +/- s.e.m.), respectively. Controls receiving ordinary tap water and mice given 0.002% AgNO3 showed no antinucleolar antibodies. The high-titre ANoA targeted a 34-kD nucleolar protein identified as fibrillarin, the major autoantigen in murine mercury-induced autoimmunity and in a fraction of patients with systemic scleroderma. Serum autoantibodies to chromatin or histones, kidney, spleen, stomach, thyroid, or skin antigens (except the nucleolus) were not found in any of the mice. There was no consistent significant increase of serum IgG1, IgG2a, IgG2b, or IgG3 concentrations after AgNO3 treatment compared with controls. Mice treated with 0.05% AgNO3 for 10 weeks showed a slight decrease in serum IgG1, IgG2b and IgG3 concentrations. These mice also showed a small but statistically significant increase in renal, mesangial IgM deposits, which was not accompanied by any increase in C3c deposits, whereas mice given lower doses of silver nitrate showed no significant increase in mesangial immunoglobulin immune deposits. Systemic vessel wall immune deposits were not found in any of the mice. In mice given 0.05% silver nitrate, the kidney showed the highest concentration of silver (12.2 +/- 0.09 micrograms Ag/g wet weight; mean +/- s.e.m.), followed by the spleen (8.7 +/- 1.3), and the liver (3.9 +/- 0.4). Treatment with 0.01% silver nitrate caused a different distribution of silver, with the highest concentration in the spleen (2.1 +/- 0.16 micrograms Ag/g), followed by the kidney (0.63 +/- 0.037), and the liver (< 0.29 micrograms Ag/g; mean). Silver seems to be a more specific inducer of antinucleolar/anti-fibrillarin autoantibodies than mercury and gold, lacks the general immune stimulating potential of mercury, and has only a weak tendency to induce renal immune deposits. These observations suggest that the autoimmune sequelae induced in mice by metals is dependent, not only upon the genetic haplotype of the murine strain, but also on the metal under investigation.

Animals↗
PubMed1999

Clinical features and IgG subclass distribution of anti-p80 coilin antibodies.

We examined the clinical features of patients presenting antinuclear autoantibodies against p80-coilin and the IgG subclass distribution of anti- p80-coilin antibodies. Sera from 365 Japanese patients were analysed. Immunoblotting and indirect immunofluorescence microscopy techniques were used with a polyclonal rabbit antiserum against p80-coilin. Eleven patients with anti-p80-coilin antibodies were found. All the patients were female and nine were in their twenties. None could be diagnosed with differentiated rheumatic disease except for one case of systemic scleroderma and another of Sjögren's syndrome. Most patients had general fatigue, arthralgia, headaches, dysmenorrhea, lymph node swelling and/or low grade fever such as chronic fatigue syndrome (CFS), and showed low complement. One patient fulfilled the criteria for CFS. All were younger females than those often diagnosed with rheumatic disease in previous reports. Patients' sera had a predominant distribution of subclass IgG(1)anti-p80-coilin antibodies and five sera had concomitant subclass IgG(2). Two rheumatic disease patients had a relatively high titer of IgG(2)anti-p80-coilin antibodies. The IgG(2)subclass of anti-p80-coilin antibodies may be a specific marker for systemic autoimmune disease.

Adult↗
PubMed1997

Prevalence of scleroderma spectrum disorders in the general population of Estonia.

The purpose of this study was to estimate the prevalence of scleroderma spectrum disorders (SDS) in the general population of Estonia while taking into account environmental and ethnic factors. A random sample of 14,467 subjects from the general population were surveyed by mail questionnaire to detect those with suspected Raynaud phenomenon (RP) and SDS. A subsample of 2,154 participants was then seen during the field study to confirm the RP diagnosis and to perform a short clinical examination. Of 581 subjects with RP, 13 cases (all women) were diagnosed as having SDS; based on these findings, the estimated prevalence of SDS in the adult population is 228 per 100,000 (95% CL 121; 391), while in women alone it is 354 per 100,000 (95% CL 188; 605). The best estimate of SD (systemic scleroderma), based on ACR criteria, is 35 per 100,000 (95% CL 4; 127). No significant difference in SDS prevalence was found between the two environmentally different regions we studied. Although statistically not significant, the prevalence of SDS, especially of SD and CREST syndrome, among Russians was higher than among Estonians, as we had hypothesized. The distribution of SDS subtypes also suggests an ethnic difference. The overall prevalence of SD/CREST that we found is higher than that reported in studies not based on a random sample of the general population.

Adult↗
PubMed1994

[Pathology of rheumatoid lung].

In the framework of rheumatic illnesses, the lungs and pleura are often observed to be involved. Dependent on the basic illness in question, conditions of the bronchoalveolar system, vessels, lymphatic system, and pleura that have developed differently inter- and intra-individually can be diagnosed in topo-regionally variable forms and combinations. In the causal pathogenesis, the initial (auto-) immunological alteration of the alveolar septa plays a role. When the monocytes, macrophages, and fibroblasts are activated, as a result of a chronic inflammatory process there is increasing reparative proliferation, which finally leads to the non-specific end stage of irreversible interstitial fibrosis. The incidence and manifestation forms of lung changes show certain differences dependent on the underlying illness (e.g., rheumatoid arthritis, systemic lupus erythematosus, progressive systemic scleroderma, dermatomyositis and polymyositis, Sjögren syndrome, and mixed connective tissue disease). In addition to vasculitis and granulomatosis of the lung, therapy-induced lung changes must also be considered in the differential diagnosis since almost all basic therapeutic agents can cause this type of lung condition. Good knowledge of the parameters from the viewpoint of possible therapeutic measures is indispensable, as is close cooperation between the treating physician and the pathologist.

Arthritis, Rheumatoid↗
PubMed1988

Evaluation of muscular lesions in connective tissue diseases: thallium 201 muscular scans.

We performed thallium 201 muscle scans to assess muscular involvement in 40 patients with different connective tissue diseases (7 with dermatomyositis, 7 with systemic lupus erythematosus, 12 with progressive systemic scleroderma, 2 with calcinosis, Raynaud's phenomenon, esophageal involvement, sclerodactyly, and telangiectasia (CREST) syndrome, 3 with monomelic scleroderma, 6 with morphea, and 3 with Raynaud's disease). Only 12 of these patients complained of fatigability and/or myalgia. Electromyography was performed and serum levels of muscle enzymes were measured in all patients. Comparison of thallium 201 exercise recording with the other tests revealed that scan sensitivity is greater than electromyographic and serum muscle enzymes levels. Thallium 201 scans showed abnormal findings in 32 patients and revealed subclinical lesions in 18 patients, while electromyography findings were abnormal in 25 of these 32 patients. Serum enzyme levels were raised in only 8 patients. Thallium 201 scanning proved to be a useful guide for modifying therapy when laboratory data were conflicting. It was useful to evaluate treatment efficacy. Because our data indicate a 100% positive predictive value, we believe that thallium 201 scanning should be advised for severe systemic connective tissue diseases with discordant test results.

Connective Tissue Diseases↗
PubMed2003

Carotid and femoral arterial wall mechanics in scleroderma.

OBJECTIVE: Large-vessel arterial disease is increasingly recognized as a major cause of morbidity in autoimmune rheumatic disorders. Recent evidence suggests that scleroderma (systemic sclerosis, SSc) may be linked to altered fibrillin-1 metabolism associated with a defect in chromosome 15q. If this is the case, we may expect to see changes in the arterial wall mechanics of large vessels not clinically involved in the disease process. We undertook a study to determine whether the biomechanical properties and intima-media thickness (IMT) of the elastic carotid artery and the muscular femoral artery are altered in subjects with limited (lcSSc) and diffuse (dcSSc) cutaneous SSc. METHODS: Measurements of carotid and femoral wall mechanics were made in 33 patients with lcSSc, 19 patients with dcSSc and 21 control subjects, using a duplex scanner coupled to a Wall Track system. Their age, gender, body mass index, heart rate, systolic and diastolic blood pressures, presumed cardiovascular load, and plasma creatinine, fasting cholesterol, triglyceride and glucose concentrations were also measured. RESULTS: There was a progressive and significant reduction (P < 0.001) in the elastic properties of the carotid artery from the control group (compliance, 16.24 +/- 4.39 %mmHg(-1) x 10(-2)) to the lcSSc group (10.89 +/- 2.43 %mmHg(-1) x 10(-2)) to the dcSSc group (7.65 +/- 2.08 %mmHg(-1) x 10(-2)), even after adjustment for the systemic physiological and biochemical variables studied, which are known to influence the mechanics of arterial walls. There was no apparent difference between the groups in the mean elastic indices of the femoral artery and the IMT of the carotid and femoral arteries. CONCLUSION: The elastic properties of the carotid artery are significantly altered in SSc, and the two major subsets of SSc may be distinguished by their carotid artery biomechanics. This suggests that connective tissue abnormality occurs at sites not previously assessed.

Carotid Arteries↗