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ID PMID Title PublicationDate abstract
2349839 [The induction of an acquired storage pool deficiency of the thrombocytes by IgG antibodie 1990 Mar Sera of patients with RA and SLE contain platelet-reactive IgG antibodies, which bring about the release of serotonin from platelets. They can also induce the aggregation of normal human platelets. In addition, such antibodies cause a decreased in vitro disaggregation of platelets after ADP exposure, as well as a significantly reduced collagen-induced aggregation. Thus, IgG platelet antibodies in RA and SLE can cause a functional impairment of patients' platelets, and may also be instrumental in systemic inflammatory rheumatoid disease by releasing proinflammatory mediators.
2379360 Hindfoot arthrodesis in adults utilizing a dowel graft technique. 1990 Aug Surgical fusion may be indicated to stabilize deformed or painful hindfoot joints. Although more commonly performed in children or adolescents, it is the salvage procedure available for adults. Twenty-six patients (30 feet; 20 women, six men) followed for an average of 44.1 months after hindfoot arthrodesis were investigated in a prospective study. Most (20 patients, 24 feet) had inflammatory arthritis, usually rheumatoid, two had previous fractures, and four patients had other diagnoses. All had fusions done using a dowel graft technique. Twenty-four patients (92%) were completely satisfied with the operation, one had mild reservations, and the one dissatisfied patient was a technical failure. All joints were fused utilizing clinical and roentgenographic evaluation. A detailed roentgenographic assessment indicated that the talometatarsal angle was the most effective measurement to assess the clinical deformity. Anteroposterior weight-bearing roentgenograms of the ankle were also important in evaluating these patients preoperatively and postoperatively. Postoperatively, only nine ankles appeared normal roentgenographically, while the others worsened, and four required ankle arthrodesis.
3382310 Diphosphonate bone scans in patients with polyarthralgias. 1988 Jul Early detection of inflammatory arthropathy is notoriously difficult with standard radiographic techniques. We therefore assessed bone turnover with technetium Tc 99m medronate in 16 patients with persistent polyarthralgias who had no clinical synovitis, normal radiographs, and nondiagnostic results from laboratory evaluations. Abnormal scans were found in 11 of 16; five were unremarkable. Scan abnormality corresponded with symptomatic joints (11 of 11 patients). These 11 patients had normal test results for rheumatoid factor, antinuclear antibody, and HLA-B27. Patients with abnormal scans were treated with nonsteroidal antiinflammatory drugs or analgesics (11 of 11), hydroxychloroquine sulfate (four), or gold salts (one), with improvement (nine of 11); patients with normal scans (five of five) were treated successfully with nonsteroidal antiinflammatory drugs or analgesics and reassurance. One patient with a normal scan developed sarcoidosis; one, hypermobility syndrome; and one, a viral syndrome. Two patients had no diagnosis. Abnormal technetium Tc 99m medronate scans in patients with previously undiagnosed polyarthralgias suggested inflammatory arthropathy and influenced management decisions with favorable therapeutic outcomes.
2171012 [Sonography of the wrist and the hand]. 1990 Aug Sonographic examination of the hand requires high-frequency linear transducers. As the relevant structures are located very close to the surface, water stand-off pads are mandatory. Owing to the high sensitivity of sonography in the detection of fluid, exudative synovitis, tenosynovitis and ganglia can be easily diagnosed. Sonographic information on muscle atrophy and alterations of the shape and echogenicity of the median nerve in patients with carpal tunnel syndrome may be useful in evaluating the extent of disease. Further indications for the sonographic examination of the hand include suspected tumors, foreign bodies and synovial proliferation. Osseous destruction can be visualized in patients with rheumatoid disease, but the precise extent is hard to determine.
2008382 Popliteal cyst: a surgical approach. 1991 Feb Twenty-nine adult patients (30 knees) who failed conservative treatment had surgical excision of a non-rheumatoid cyst. To determine the effectiveness of this surgical procedure, we reviewed the cases of 24 patients (25 knees) who were available for subjective and objective follow up. Twenty of the 25 knees (80%) were rated excellent or good. Three knees had fair results. Two knees continued to have problems and ultimately underwent total knee replacement for degenerative arthritis.
1940802 High frequency of t(14;18) translocation in salivary gland lymphomas from Sjögren's syndr 1991 Nov 1 Sjögren's syndrome (SS) is a chronic autoimmune disorder characterized by lymphocytic infiltration of the salivary and lacrimal glands. These patients have a markedly increased frequency of developing non-Hodgkin's lymphoma in their salivary glands and cervical lymph nodes. Translocations of proto-oncogene bcl-2 t(14;18) were observed in five of seven SS-associated lymphomas by Southern blot analysis. Using primers specific for chromosomes 14 and 18, translocation of the proto-oncogene bcl-2 was detected by polymerase chain reaction (PCR) in all five lymphomas positive by Southern blot analysis. Among SS patients lacking clinical evidence of coexistent lymphoma, no bcl-2 translocations were detected in 50 consecutive salivary gland biopsies. Of particular interest, pre-lymphoma biopsies were available from the seven SS patients who subsequently developed lymphoma and these DNA samples lacked detectable t(14;18) translocations even though they exhibited oligoclonal rearrangements of their immunoglobulin genes. We conclude that the great sensitivity of PCR can help us in detecting early onset of lymphoma in SS patients and aid in understanding the transition from autoimmunity to lymphoma.
2403122 Autoimmune neutropenia with anti-neutrophil autoantibody associated with Sjögren's syndro 1990 Aug A 74-year-old man developed neutropenia in association with Sjögren's syndrome. The peripheral neutrophils in his blood decreased to 210/mm3 (total white blood cell count 2,100/mm3). Bone marrow examination showed an increase in the number of neutrophil precursors. The presence of anti-neutrophil autoantibody (ANAB) in his plasma was determined by an enzyme-linked immunosorbent assay. Prednisolone therapy resulted in an increase in the neutrophil count and a decrease in the ANAB titer. However, when the daily dose of prednisolone was decreased, the neutrophil count gradually decreased, and the ANAB titer increased again. These results suggest that neutropenia in this patient was caused by ANAB, and ANAB could be the result of autoimmune disorders associated with Sjögren's syndrome.
2267448 [Dacie syndrome and other splenomegalies without apparent cause]. 1990 Apr Some enlarged spleens do not seem to be related with known pathogenetic mechanisms (passive congestion, functional workload, malignant infiltration and inflammatory or storage disorders). Non-tropical idiopathic splenomegaly (Dacie's syndrome) is a form of hypersplenism of unknown origin that evolves into a non-Hodgkin lymphoma, after a variable interval, in 20% of the patients. Tropical idiopathic splenomegaly (or hyperreactive malarial splenomegaly) develops when a chronic malarial challenge triggers an abnormal immunological response consisting in decreased suppressor T lymphocytes and increased amounts of circulating immunoglobulin M and immunocomplexes, which are cleared by the splenic macrophages. This peculiar response to malaria seems to be linked to particular HLA antigens. Other confusing splenomegalies are seen in Felty's syndrome, in populations subjected to recurrent infections, and in some families. Overlapping findings and diseases suggest chronic antigenic stimulation as a common feature, with diverse responses depending on the host. A small percentage (probably less than 3%) of normal individuals has minimal splenomegaly without any clinical significance.
2840228 High levels of complement fixing antibodies against cytomegalovirus in patients with prima 1988 Jan Antibodies to cytomegalovirus (CMV) were examined in sera from 21 consecutive patients with primary Sjögren's syndrome (SS) and 19 consecutive patients with secondary SS, using a complement fixation (CF) test and an antibody capture enzyme-linked immunosorbent assay (ELISA). Sera from 15 CMV-negative subjects, 15 CMV-positive subjects, 3 patients with primary CMV infection and 3 patients with recurrent CMV infection served as controls. The prevalence of CMV antibodies in the patients with primary and secondary SS was found similar to the prevalence known to occur in the normal adult population. Unrelated to clinical parameters, 5 patients with primary SS (24%) had high levels of CF antibodies against CMV. Ig class antibodies to CMV were not elevated in these 5 patients. Preferential production of CF antibodies to CMV may be a pathogenetic factor in some patients with primary SS.
3307296 Immunohistochemical characterization of intraepidermal in vivo IgG deposits in patients wi 1987 Sep 68% of patients with primary Sjögren's syndrome have previously been found to have intra-epidermal in vivo IgG deposits in clinically unaffected skin. In this investigation, we examined immunohistologically skin biopsies from 5 patients with primary Sjögren's syndrome and from 5 normal controls in order to characterize further the intra-epidermal IgG deposits. Employing direct immunofluorescence and peroxidase-antiperoxidase (PAP) techniques, IgG was localized to epidermal cell surfaces. Double-labelling immunofluorescence experiments showed IgG to be bound to OKT6-positive Langerhans cells, and to some degree also to keratinocytes. Only IgG1, IgG3 and in one patient IgA were deposited, whereas IgG2, IgG4, IgM, IgD, IgE, C1q, C3c, C3d, C4, beta-2 microglobulin, albumin, fibrinogen and C-reactive protein were not found deposited in the intra-epidermal area. The epidermal IgG deposits were reactive with anti-Fc-fragment antibodies and with staphylococcal protein-A. These results are compatible with, although not definitive proofs of, the hypothesis that the in vivo deposited IgG is found in the form of IgG-containing immune complexes.
3531358 Discordance of SSA/Ro and SSB/La cellular antigens in synchronized cells. 1986 Oct SSA/Ro and SSB/La are soluble cellular proteins to which antibodies are frequently produced in patients with Sjögren's syndrome and systemic lupus erythematosus. In this investigation, we examined anti-SSA/Ro and anti-SSB/La staining patterns on synchronized WiL2 cells and mixed lymphocyte culture cells using monospecific antisera. In addition to its presence in the nucleoplasm, the SSB/La antigen was highly concentrated in the nucleolus of cells during the late G1 and early S phase and is thus cell cycle-related. In contrast, the SSA/Ro antigen was found to be independent of cell cycle, showing a nuclear speckled pattern in all phases. Blocking experiments indicated that free SSB/La is responsible for the nucleolar staining, whereas the combination of both SSA/Ro and SSB/La determines the nucleoplasmic speckled staining pattern.
1994921 Association of the complement allele C4AQ0 with primary Sjögren's syndrome in Japanese pa 1991 Feb We studied allotypes of the fourth component of complement (C4) and factor B in 76 patients with Sjögren's syndrome (SS) and in 63 normal subjects. C4A-null (C4AQ0) was found in 10 of 28 patients who had primary SS, compared with 1 of 63 control subjects (P less than 0.005). In contrast, no significant difference in the frequency of any C4 allotype was observed between patients with secondary SS and control subjects. An association of HLA-DRw53 with primary SS in Japanese patients has been reported. Since there is no linkage disequilibrium between DRw53 and C4AQ0, it is possible that at least 2 genes in the major histocompatibility complex may determine susceptibility to the development of primary SS in the Japanese population.
2790331 Autoantibody analysis in chronic graft-versus-host disease. 1989 Sep Chronic graft-versus-host disease (cGVHD) bears clinical similarities to connective tissue diseases which are characterized by a spectrum of autoantibody formation. A wide range of autoantibody analyses in 19 allogeneic and 16 autologous bone marrow transplant (BMT) patients has determined that the most commonly detected antibody is IgM anti-cytoplasmic factor (ACF) occurring in 37% and 20% of allogeneic and autologous group respectively and of a type not normally seen in connective tissue disease. The antigen is as yet unidentified. These results suggest that autoantibody formation post-BMT is unrelated to the graft-versus-host process. Ophthalmic examination revealed evidence of a Sjögren-like syndrome in 20% autologous and 47% allogeneic patients, suggesting that the development of dry eyes post-BMT is not uniquely a feature of cGVHD.
3260691 [Monoclonal cryoglobulins in primary Sjögren's syndrome]. 1988 Cryoglobulins were studied in the sera of 40 unselected pSS patients. The nature of the cryoglobulins was defined using a very fast and sensitive resolution electrophoresis technique combined with immunofixation. Thirty-five per cent of SS patients were shown to have circulating IgMk mixed monoclonal cryoglobulins. On the contrary, all the other tested cryoprecipitates were found to be mixed polyclonal, with the exception of one patient with SLE. The presence of cryoglobulins in the sera of SS patients correlated with extraglandular disease and with autoantibodies to Ro (SSA) cellular antigen and rheumatoid factor. Patients with cryoglobulins had lower serum C4 levels compared to patients without cryoglobulins. These findings suggest that SS in addition to polyclonal B-cell hyperreactivity expresses a monoclonal process preceding the development of lymphoid neoplasia. Extraglandular manifestations of the syndrome may be associated with an immune complex mediated pathology.
3307295 Circulating IgG from patients with primary Sjögren's syndrome deposited in the epidermis 1987 Sep Sera from 7 patients with primary Sjögren's syndrome and from two control persons were administered intraperitoneally to athymic nude mice transplanted with normal human skin. Seven days after transfer of serum from 5 of the patients, intra-epidermal IgG1 and IgG3 deposits were demonstrated in the skin grafts by immunofluorescence. The deposits closely resembled in vivo deposits found in the skin of these patients. No correlation was found between the presence of epidermal deposits and levels of IgG1 and IgG3 in serum. No IgG deposits were found in skin grafts on animals given control serum, and neither could human IgG be detected in mouse skin adjacent to the grafts. Epidermal deposits of human-IgM, -IgA, -fibrinogen, -C3c and mouse-Ig were not demonstrated in biopsies from grafts or mouse skin. The results support the hypothesis that epidermal in vivo deposits of IgG in patients with primary Sjögren's syndrome are the result of Fc-receptor-mediated binding to epidermal cells.
3079684 Subclass restriction of anti-SS-B (La) autoantibodies. 1986 Jan Antibodies to the SS-B (La) nuclear ribonucleoprotein particle are relatively specific for the diagnoses of Sjögren's syndrome or systemic lupus erythematosus. The formation of such autoantibodies is likely, then, to reflect the basic immunopathogenesis of these disorders. We have studied the isotype distribution of anti-SS-B antibodies as a clue to their immunoregulation. Using specific ELISA assays, we found that nearly all anti-SS-B antibodies in 39 patients were IgG, and, of these, only the IgG1 and, to a much lesser extent, IgG3 subclasses were represented. Both kappa and lambda light chain antibodies were found in most sera, and the overall kappa/lambda ratio approximated that of normal serum immunoglobulin. These results suggest that the formation of anti-SS-B antibodies is T-cell dependent and that the response is polyclonal in most patients.
1787489 Sjögren's syndrome in patients with the CREST variant of progressive systemic scleroderma 1991 Nov Twenty-three patients with the CREST (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia) variant of progressive systemic sclerosis, were clinically, histopathologically and serologically examined for the presence of Sjögren's syndrome (SS). Fourteen were found to be positive. No significant difference could be demonstrated between them and the remaining 9. Characteristics of patients with CREST were compared with those of 29 randomly chosen patients with primary SS. Parotid gland enlargement was more frequently present (p less than 0.01) in the latter than in the former. Virtually no patients with CREST with SS had antibodies to Ro(SSA)/La(SSB).
2173499 Pure sensory neuropathy in patients with primary Sjögren's syndrome: clinical, immunologi 1990 Oct A pure sensory neuropathy caused by lymphocytic infiltration of the dorsal root ganglia has been reported in a few patients with Sjögren's syndrome. The clinical, immunological, and electromyographic findings of five patients with this type of neuropathy and primary Sjögren's syndrome were reviewed. Typical clinical indications were the presence of a chronic asymmetrical sensory deficit, initial disease in the hands with a predominant loss of the vibratory and joint position senses, and an association with Adie's pupil syndrome or trigeminal sensory neuropathy. The simultaneous impairment of the central and peripheral evoked cortical potentials suggested that there was a lesion of the neuronal cell body. The neuropathy preceded the diagnosis of Sjögren's syndrome in four patients. Four patients were positive for Ro antibodies, but systemic vasculitis or malignancy was not found after a mean follow up of six years. These findings indicate that in patients with a sensory neuropathy the diagnosis of Sjögren's syndrome has to be considered, even if the patient denies the presence of sicca symptoms, and that appropriate tests must be carried out.
2680309 Epithelial immunoglobulin deposits and Langerhans cells in patients with primary Sjögren' 1989 Sep (1a + 1b) The purpose of this study was initially to examine whether in vivo Ig deposits in unaffected skin are a feature of patients with primary and secondary SS. A novel deposition pattern of IgG in the intercellular epidermal area was found in 68% of the patients with primary SS, but in only 13% of the patients with secondary SS. DEJ Ig deposits were observed in none of the patients. (2) Different stratified squamous epithelia from patients with primary SS were examined for intraepithelial in vivo Ig deposits. In the total of 3 investigations, epidermal intercellularly located IgG was found in 57 of 84 (68%) patients with primary SS. Intraepithelial Ig deposits were also demonstrated in the oral labial and uterine cervical mucous membranes of patients with primary SS. (3) The underlying pathomechanism(s) of the intraepidermal in vivo IgG deposits was (were) examined by investigations of skin and sera from patients with primary SS. The intraepidermal IgG deposits were found located to cell surfaces of both LC and keratinocytes. Circulating IgG from patients with primary SS was shown to bind in vivo to the intercellular area of normal human epidermis, when examined in the athymic nude mouse/human skin model. However, no similar in vitro binding of circulating IgG to normal human epidermis was found. Based on these observations and on further IH skin examinations, it was hypothesized that IgG-containing IC, bound to Fc-receptors on LC and keratinocytes, may be responsible for the in vivo deposits in skin of patients with primary SS. (4) The diagnostic significance of the intraepidermal in vivo IgG deposits for primary SS was examined in studies including normal controls and patients with other CTD. Intraepidermal IgG deposits were found to be significantly more common in patients with primary SS than in normal controls (p less than 0.001), in patients with RA (p less than 0.001), in patients with SLE (p less than 0.001), in patients with SS secondary to RA and SLE (p less than 0.001) and in the total group of other well-defined CTD (p less than 0.001). The frequency of intraepidermal IgG deposits in the patients with manifest primary SS did not differ from that of patients with incipient primary SS (0.1 less than p less than 0.2), patients with Wegener's granulomatosis (0.1 less than p less than 0.2), or patients with ill-defined CTD (0.05 less than p less than 0.1).(ABSTRACT TRUNCATED AT 400 WORDS)
3661643 Chronic anterior and posterior uveitis and primary Sjögren's syndrome. 1987 Oct 15 We examined eight patients with primary Sjögren's syndrome and uveitis. In all patients, the uveitis was bilateral, chronic, anterior and posterior, and not associated with active chorioretinitis. Seven of the eight patients were women. The diagnosis of primary Sjögren's syndrome was generally based on results of a Schirmer test, minor salivary gland biopsy, and serologic studies. Frequent findings included keratic precipitates, cataract, a pars plana exudate, an increased sedimentation rate, a positive test for antinuclear antibodies in a speckled pattern, and associated systemic disease compatible with the spectrum of Sjögren's syndrome.