bluuebunny/crossref_metadata_2025 huggingface dataset:
Purine Nucleoside Phosphorylase Deficiency
Authors: Wortmann Robert L. , Andres Catherine , Kaminska Janice , Mejias Edwin , Gelfand Erwin , Arnold William , Rich Kenneth , Fox Irving H.
Published: February 2007

The biochemical features of two families with purine nucleoside phosphorylase deficiency are compared. Laboratory studies and an evaluation of kinetic and physical properties of erythrocyte purine nucleoside phosphorylase give evidence that a) the degree of abnormality in uric acid and nucleoside concentrations in plasma and urine reflect the severity of the enzymatic deficiency and b) structural alterations of the mutant enzymes result from structural gene mutations and demonstrate genetic heterogeneity in the disease purine nucleoside phosphorylase deficiency.
Cardiac Involvement in Rheumatoid Arthritis
Authors: Nomeir Abdel Mohsen , Turner Robert A. , Watts Lester E.
Published: February 2007

Initial studies from Bowman Gray School of Medicine showed that 18 of 30 patients with classic rheumatoid arthritis (RA) had cardiac involvement from their disease. These abnormalities were detected by echocardiography and consisted of mitral valve and/or pericardial abnormalities. All patients were followed for 4 years from the initial workup. Mitral valve abnormalities were seen on followup in 63% of the patients who initially showed this abnormality, while pericardial effusion remained in 20%. Pericardial thickening persisted in 6 of 7 patients. None of the patients developed constrictive pericarditis or heart failure. There was no definite correlation between persistence of these abnormalities and other clinical data, but it was noticed that patients who had persistent pericardial effusion and mitral valve abnormalities showed a higher number of involved joints and a higher erythrocyte sedimentation rate. The cardiac abnormalities described in this study have remained clinically insignificant in this population of RA patients.
IgG Antibodies to Double‐Stranded DNA in Systemic Lupus Erythematosus Sera
Authors: Beaulieu Andre , Quismorio Francisco P. , Friou George J. , Vayuvegula Bharathi , Mirick Gary
Published: February 2007

Antibodies to double‐stranded deoxyribonucleic acid were studied using the kinetoplast of Crithidia luciliue. Titers were determined separately by conventional immunofluorescence and the complement fluorescent technique, and results by the two methods were compared. Complement fixing activity varied independently of antibody content in whole serum and in IgG fractions. The well established correlation of complement fixing activity of this antibody with activity of lupus nephritis appears related, therefore, to qualitative rather than solely quantitative differences. This finding has important implications for the clinical assessment of patients with lupus, and investigations on the relationship of anti‐DNA antibodies to lupus nephritis.
Chemotactic factor‐induced generation of superoxide radicals by human neutrophils
Authors: Simchowitz Louis , Mehta Jagdish , Spilberg Isaias
Published: February 2007

Human peripheral neutrophils generated superoxide radicals as assessed by ferricytochrome C reduction in response to activation by the synthetic chemotactic factor, N‐formyl‐methionyl‐leucyl‐phenylalanine. Superoxide generation was inhibited by 2‐deoxy‐D‐glucose (ID50 4X10−5M), 2‐iodoacetate (ID50 5X10−5M), and N‐ethyl‐maleimide (ID50 5X10−6M), suggesting a dependence on anaerobic glycolysis and sulfhydryl groups. Ouabain, microtubule‐disrupting agents, inhibitors of respiration, oxidative phosphorylation, and protein and nucleic acid synthesis were without appreciable effects. Indomethacin (ID50 1X10−4M), ibuprofen (ID50 9X10−4M), and phenylbutazone (ID50 1X10−5M) all caused dose‐dependent inhibition of superoxide generation at concentrations approximating those plasma and tissue levels obtained in human beings at therapeutic doses. Acetylsalicylic acid (125–500 ug/ml) and aurothioglucose (10−3−10−6M) were without appreciable effects. Superoxide generation was inhibited only by relatively high concentrations of hydrocortisone (ID50 > 10−3M). Because superoxide radicals have been implicated in the pathogenesis of tissue injury in several forms of inflammation and arthritis in vivo, these studies suggest that the production of a potential cytotoxic factor may be subject to pharmacologic manipulation and that at least some of the antiphlogistic effects of the nonsteroidal antiinflammatory agents may be mediated through effects on superoxide production.
The clinical significance of raynaud's phenomenon in systemic lupus erythematosus
Authors: Dimant Jacob , Ginzler Ellen , Schlesinger Michael , Diamond Gary Sterba Herbert , Kaplan Damond , Weiner Max
Published: February 2007

In a prospective study of 226 patients with systemic lupus erythematosus (SLE), 91 patients (40%) had Raynaud's phenomenon. These patients were compared to 135 patients without Raynaud's phenomenon. Patients with Raynaud's phenomenon had a greater incidence of arthritis (P < 0.02), malar rash (P < 0.003), and photosensitivity (P < 0.03), and a lesser incidence of severe renal disease as manifested by serum creatinine over 3.0 mg/dl (P < 0.007) or creatinine clearance below 60 ml/minute. Patients with Raynaud's phenomenon were less likely to have severe, life threatening disease and received a lower average monthly (P < 0.01). and a lower peak daily corticosteroid dose (P < 0.01). Fourteen patients (16%) with Raynaud's phenomenon died, compared to 41 without (30%) (P < 0.03). Raynaud's phenomenon in patients with SLE is associated with milder disease and may be regarded as a favorable prognostic sign.
Quantification of human plasma inorganic pyrophosphate
Authors: Ryan Lawrence M. , Kozin Franklin , Mccarty Daniel J.
Published: February 2007

The methodologic variables of the UDPG pyrophosphorylase method for analysis of inorganic pyrophosphate (PPi) levels in biologic fluids are described. Use of a tourniquet in collection of blood specimens elevated plasma PPi levels from 35% to 55% above control values and may explain the differences in published normal values. The sodium pyrophosphate decahydrate used to prepare the standard solution lost 8 waters of hydration after dessication, which could result in the calculation of spuriously elevated PPi levels. Normal plasma PPi concentration was 2.18 uM with a range (95% confidence limits) of 0.58‐3.78 uM. Comparison of plasma PPi in normal subjects, patients with primary osteoarthritis, and patients with calcium pyrophosphate dihydrate deposition disease revealed no significant intergroup differences.
T lymphocyte subpopulations in synovial fluid of patients with rheumatic disease
Authors: Biberfeld Gunnel , Nilsson Ethel , Biberfeld Peter
Published: February 2007

The proportion of T lymphocytes with receptors for the Fc portion of IgG (TG cells) or IgM (TM cells) was determined in synovial fluid and blood of 16 patients with various rheumatic diseases including rheumatoid arthritis, psoriatic arthritis, and ankylosing spondylitis. The percentage of TG cells was low in all synovial fluid samples, whereas in the patients' blood the percentage was higher than or equal to the level found in the blood of healthy subjects. Eight patients also had a lower level of TM cells in synovial fluid as compared to the percentage found in the blood of healthy donors. In the patients' blood the percentage of TM cells was usually within the normal range. Thus the proportion of T cells lacking either receptor (Tnull cells) was higher in synovial fluid than in blood. This pattern of low TGcell and high Tnull cell percentages was found in the synovial fluid of patients with various rheumatic diseases and thus seems to be a general feature of chronic inflammatory joint exudates.
The rarity of felty's syndrome in blacks
Authors: Termini Trudy Elsmore , Biundo Joseph J. , Ziff Morris
Published: February 2007

Evidence is presented that Felty's syndrome (FS) is rare among black patients with rheumatoid arthritis (RA). All of 12 patients with FS seen at Parkland Memorial Hospital, Dallas, Texas between 1964 and 1978 were white. During this period 52% of patients admitted to the Parkland medical service were black and 31% of patients discharged with a diagnosis of RA were black. The number of expected black cases of FS on the basis of the racial distribution of hospitalized patients with RA was 3.7 (P < 0.02 when the zero incidence in blacks was compared with the expected incidence). All 7 cases of FS observed at Charity Hospital, New Orleans, Louisiana between 1968 and 1978 were also white. During this period, 65%of patients discharged with a diagnosis of RA were black, and the number of expected black cases of FS was 4.5 (P < 0.001). These findings suggest a genetic basis for the development of leukopenia and splenomegaly in RA patients.
Mutilans‐like arthropathy in mixed connective tissue disease
Authors: Alarcón‐Segovia Donato , Uribe‐Uribe Oscar
Published: February 2007

Patients who combine clinical features of various connective tissue disorders have been found to have antibodies to ribonucleoprotein. This antigen is included as the ribonuclease‐sensitive portion of an extractable nuclear antigen (ENA) (1). These patients have been grouped under the term mixed connective tissue disease (MCTD), and there is increasing evidence that they constitute a distinct disease group. Articular manifestations in patients with MCTD are prominent and may be destructive to the point of closely mimicking rheumatoid arthritis (2–4). Herein we describe a unique patient with MCTD who developed severe arthritis, as well as middle and distant phalangeal bone resorption which resulted in opera‐glass hands akin to those found in arthritis mutilans.
Erythrocyte rosette inhibition as an assay for naturally occurring t lymphocytotoxic antibody in systemic lupus erythematosus
Authors: Koike Takao , Kobayashi Seiichi , Yoshiki Takashi , Itoh Tetsuo , Shirai Toshikazu
Published: February 2007

By means of a modified sheep erythrocyte rosette inhibition assay, we were able to detect naturally occurring lymphocytotoxic antibodies in sera from patients with systemic lupus erythematosus (SLE). The incidence of lymphocytotoxic antibodies was 86% in all SLE patients, and 100% in patients with active disease. Since this assay detects only the antibodies that react with the determinants on T cells or on both T and B cells, it has a great advantage of demonstrating in combination with appropriate absorptions the antibodies specific for T cells. When an appropriate panel of target cells was used, most of the antibodies in SLE sera as detected by this assay appeared to be analogous to a natural thymocytotoxic autoantibody (NTA) of New Zealand mice in its specificity and nature. The changes in the antibody titer of a patient with SLE during the course of disease correlated well with those in the total number of T cells in the blood, the antinuclear antibody titer, and some delayed skin hypersensitivities.
Serum‐serum interactions in autoimmune mice
Authors: Eisenberg Robert A. , Thor Lee T. , Dixon Frank J.
Published: February 2007

Sera from a majority of old, sick mice of the MRL/I strain interact with other MRL/I sera to form visible immunoprecipitates and fix complement. The mouse sera can be divided into two sets such that interset interactions are far more common than intraset ones. The reactive principle in each mouse serum is IgG in the form of an intermediate‐sized complex. Reactivity between sera is dependent on IgG anti‐IgG specificities.
Studies of the effects of sex hormones on autosomal and x‐linked genetic control of induced and spontaneous antibody production
Authors: Raveche E. S. , Tjio J. H. , Boegel W. , Steinberg A. D.
Published: February 2007

In these studies we investigated the modifying effect of sex hormones on both the levels of induced antibodies after immunization with single‐stranded DNA (ssDNA) and the levels of spontaneously produced anti‐T cell antibodies (NTA). To learn whether the responses were genetically determined or under hormonal regulation, we analyzed hybrids produced by crossing the autoimmune NZB strain with the nonautoimmune DBA/2 strain. For both anti‐ssDNA and NTA, males usually had a lower response than females; this difference could largely be removed by castration of the males. Females given testosterone implants also had decreased antibody levels. The higher responses in females and suppression by testosterone were true for all mice studied except NZB mice. NZB mice appear to have an insensitivity to the suppressive effects of testosterone.
Effect of estrogen on natural killer cells
Authors: Seaman William E. , Gindhart Thomas D.
Published: February 2007

Treatment of mice with sustained high levels of β‐estradiol leads to a reduction in natural killer cell activity and genetic resistance to bone marrow transplantation. The loss of natural killing does not seem to result from either humoral or immune suppression. Natural killer cells are thought to depend on the bone marrow, and it is notable that estrogens reduce natural killing at approximately the same time that they produce a loss of marrow due to osteoproliferation. Similarly, mice with congenital osteopetrosis are deficient in natural killing. However, changes in natural killing during and after treatment with estrogen do not correspond directly to changes in marrow volume. Estrogens are known to exacerbate spontaneous autoimmunity in NZB/NZW mice. The relationship between this effect and the effect of estrogen on natural killing is not clear. When natural killing is lowered in NZB/NZW mice by the in vivo administration of 89Sr, autoimmunity is reduced.
Sex differences in response to hepatitis b virus
Authors: London W. Thomas
Published: February 2007

We proposed the hypothesis that there is an antigen on the hepatitis B virus (HBV) that cross‐reacts with a male associated antigen to explain four observations: 1) sex differences in the responses to HBV infection, 2) sex differences in the prevalence of chronic liver diseases associated with hepatitis B, 3) the association of parental responses to HBV with the sex ratio (at birth) of their offspring, and 4) the relation of kidney graft survival with the combination of anti‐HBs in the recipients and the sex of the organ donor. Patients with aplastic anemia treated with bone marrow transplantation from HLA identical sibling donors were studied to further test this hypothesis and the results provide additional support. Cross reactivity of HBsAg with H‐Y antigen, however, has not been demonstrated.
Sex hormone production and action
Authors: Siiteri Pentti K.
Published: February 2007

The mechanisms of the production of estrogens and androgens in women and men are reviewed. Extraglandular estrogen formation from circulating androgens is the principal source of estrogen in men and postmenopausal women. The amount of estrogen formed is dependent upon precursor availability and metabolic factors, such as obesity and hepatic disease. Both androgens and estrogens in the circulation of humans are bound to sex hormone binding globulin which limits their availability to target cell receptors.
Placenta‐bound immunoglobulins
Authors: Jurjus Abdo , Wheeler David A. , Gallo Robert C. , Witz Isaac P.
Published: February 2007

Low pH eluates were prepared from trophoblasts derived from 8 term human placentas. A qualitative analysis for immunoglobulins revealed the presence of IgG, IgA, and IgM in these eluates. IgG‐rich fractions were obtained by DEAE‐cellulose chromatography of ammonium sulfate‐concentrated eluates. These fractions were able to neutralize, in vitro, the catalytic activity of reverse transcriptases (RT) from several retroviruses. RT from baboon endogenous virus (BEV) seemed to be more susceptible to the neutralizing activity of some eluates. This was in contrast to RT from feline leukemia virus (FeLV) which were neutralized by eluates of leukocytes from chronic myelogenous leukemia. In contrast to previous and present results with purified IgG from leukemic leukocytes, the purified IgG from placenta eluates was incapable of RT neutralization. However, such purified IgG fractions inhibited mixed lymphocyte reactions.
The effect of thoracic duct drainage on lymphocyte dynamics and clinical symptoms in patients with rheumatoid arthritis
Authors: Ueo Toyoji , Tanaka Seisuke , Tominaga Yoshinori , Ogawa Hiroyiki , Sakurami Takehiko
Published: February 2007

Thoracic duct drainage (TDD) was performed in 4 patients with severe rheumatoid arthritis. Clinical effects were apparent in all during drainage, but the term of TDD and the cumulative number of lymphocytes drained had no direct relation to the improvement of clinical symptoms. The number of lymphocytes in the peripheral blood increased despite discharge of lymphocytes from the thoracic duct in the very early stage of drainage, suggesting that lymph drainage from thoracic duct accelerates migration of lymphocytes from lymphocyte pools to the blood stream. Biopsy specimens of synovial membranes obtained post‐TDD showed marked decrease of mononuclear cell infiltration as compared to the specimens obtained preoperatively. These findings suggest that clinical effectiveness may be due not only to systemic immunosuppression induced by lymphocyte depletion but also to accelerated migration of inflammatory cells from the synovial tissues to the blood stream occurring with dynamic change of lymph flow during TDD.
Metabolic and secretory properties of peripheral and synovial granulocytes in rheumatoid arthritis
Authors: Van De Stadt R. J. , Voorde‐Vissers Els Van De , Feltkamp‐Vroom Thea M.
Published: February 2007

The metabolic and secretory properties of peripheral and synovial granulocytes of patients with rheumatoid arthritis were investigated with serum‐ or immunoglobulin‐treated zymosan as activators of cell metabolism. During isolation of the synovial cells precautions were taken to prevent in vitro phagocytosis of immune materials present in the synovial fluids. Oxygen uptake, extracellular release of lysosomal enzymes under resting and activated conditions, yield of the isolated granulocytes, and the granule enzyme content of peripheral and synovial cells did not differ significantly from those of peripheral granulocytes from healthy volunteers. In agreement with the biochemical results, intracellular inclusions could be detected in only a few synovial cells with a direct immunofluorescence technique. The possibility that formation of “ragocytes” may be an in vitro phenomenon is discussed.
Therapeutic studies in new zealand mice
Authors: Melez Kathleen A. , Boegel William A. , Steinberg Alfred D.
Published: February 2007

In view of the known inhibitory effects of androgens on the spontaneous production of autoantibodies in hybrids of NZB mice, as well as the dramatic effects of sex hormones on the modulation of autoimmunity in NZB/NZW F1 mice, we undertook a systematic study of treatment of female NZB/NZW mice with androgens. We initiated treatment at 0.5, 3, 5, 6, 7, or 8 months of life in castrated or intact mice by use of one of three steroid preparations at different doses. We found that danazol was not effective in suppressing proteinuria, reducing antibodies to ssDNA, or in prolonging survival. In contrast, both testosterone and 5 α‐dihydrotestosterone were capable of prolonging survival, suppressing proteinuria, and reducing anti‐ssDNA in both intact and castrated mice. Testosterone was effective at lower doses than was the 5 α hormone. Testosterone significantly prolonged survival even when started after the onset of clinical illness at 5, 7, and 8 months of age. Mice treated from 5 months of life with testosterone had a much more marked reduction in antibodies to ssDNA than to native DNA. However, treatment from 7 months of age significantly prolonged survival without altering the serum levels of either autoantibody, suggesting that some other mechanism of testosterone action may be contributing to therapeutic efficacy. These studies indicate that androgen therapy may be beneficial even after the development of clinical illness, that castration is not necessary for benefit, and that the effectiveness of a therapeutic regimen is dependent upon both the dose and the particular preparation of hormone.
Genetic studies in nzb mice
Authors: Raveche Elizabeth S , Tjio J H , Steinberg Alfred D
Published: February 2007

Hybrid NZB X NZW or NZB X DBA/2 females have markedly accelerated development of autoimmunity when compared with their respective male littermates. This difference is attributable to the ability of male sex hormones to retard the expression of autoimmunity. In contrast to the sex differences in expression of autoimmunity in F1 mice, parental NZB males and females have only minor differences in disease expression. We have been investigating the basis for the difference in anti‐T cell antibody production between NZB and F1 mice. In this study, the appearance of antibodies cytotoxic for T cells (NTA) was studied in NZB and DBA/2 mice and in their F1 hybrids and backcross progeny. A major sex difference in NTA production was observed in the F1 hybrids; females produced more NTA than did males. Castration of males led to a marked increase in NTA production. Furthermore, the NTA production of castrated male and female F1 mice was significantly suppressed by administration of testosterone in Silastic capsules. In contrast to the studies in F1 mice, we found little difference between intact male and female parental NZB mice at any age studied. Furthermore, NZB mice of both sexes who were given androgen‐containing capsules at 2 weeks of age failed to demonstrate a decrease in NTA production. This result suggested an androgen insensitivity in NZB mice with regard to NTA production. This insensitivity, which appears to be a recessive trait, may help to explain why NZB mice do not manifest the sex differences in disease expression observed in the F1 hybrids.
Ankylosing spondylitis
Authors: Kaprove Robert E. , Hugh Little A. , Graham Donald C. , Rosen Phillip S.
Published: February 2007

We investigated survival of a group of 151 Canadian war veterans with ankylosing spondylitis who were entered into a prospective study in 1947–1949. With 94% successful followup, total survival was 60.9%, significantly less than expected. However, a subgroup who had not been treated with radiotherapy had a survival not significantly different from the general population. Causes of death in both irradiated and nonirradiated subgroups were determined.
Identification of anaplasmataceae (haemobartonella) antigen and antibodies in systemic lupus erythematosus
Authors: Kallick Charles A. , Thadhani Kaloo C. , Rice Thomas W.
Published: February 2007

Free antigen related to Anaplasma marginale (AM) (Haemobartonella) was demonstrated in the glomeruli of one patient with lupus nephritis. Indirect fluorescent antibodies against this rickettsia were demonstrated in all of 22 lupus sera tested, with titers ranging from 1:20 to 1:1280. Geometric mean titer (GMT) was 116. Fifty‐eight percent of 102 controls did not react to AM by indirect fluorescent antibody technique, and GMT of all controls was 10.7 Immunofluorescence was eliminated by neutralization and blocking techniques.
Studies of immune functions of patients with systemic lupus erythematosus
Authors: Sakane Tsuyoshi , Steinberg Alfred D. , Green Ira
Published: February 2007

Genetic, environmental, and immune factors have been implicated in the pathogenesis of active systemic lupus erythematosus (SLE). To investigate the basis for abnormalities in immune regulation, we studies lymphocytes from patients with SLE during the active and inactive phases of their disease to examine their ability to develop Con A induced suppressor function (5 patients) and to develop a normal autologous mixed lymphocyte reaction (MLR) (7 patients). In each case results of these in vitro tests using whole T cells were abnormal during the active phase of the disease and returned to normal when the disease activity decreased. The results of this study suggest that the abnormalities of suppressor cell generation and the autologous MLR observed in whole T cell populations from patients with active SLE are not solely due to intrinsic and invariant lymphocyte defects. They further suggest that loss of immune regulatory function is not a simple genetically determined trait since the regulatory function returned to normal when the disease became inactive. However, these results do not exclude an underlying genetic effect on the immune system which requires a second factor (such as an environmental trigger) for expression. Evidence in support of this latter possibility derives from studies of Tγ cells. Fractionation of the functionally normal whole T cells from some patients who were clinically inactive revealed impaired function of Tγ cells. This subset of T cells may be easily perturbed by stimuli not sufficient to cause clinically apparent disease.
A sensitive radioimmunoassay for quantitation of igm rheumatoid factor
Authors: Koopman William J. , Schrohenloher Ralph E.
Published: February 2007

A solid‐phase radioimmunoassay capable of detecting nanogram quantities of human IgM rheumatoid factor (RF) in biologic fluids has been developed. Binding curves for monoclonal IgM RF and polyclonal IgM rheumatoid factors were similar under the conditions utilized for the assay. Human IgG did not interfere with the detection of IgM RF by this method. Small quantities (≤ 0.2%) of nonspecific binding by nonRF IgM to the human IgG coated tubes utilized in the assay were corrected for by assaying samples in parallel bovine serum albumin coated control tubes. As expected, patients with seropositive rheumatoid arthritis (RA) had significantly higher concentrations of IgM RF than seronegative RA patients (mean ± 1 SD = 652 ± 553 μg/ml versus 11.3 ± 13.3 μg/ml, P < 0.001). In contrast, all normal control sera assayed to date contained < 0.1 μg/ml of IgM RF. The capacity of the assay to detect nanogram quantities of IgM RF should permit investigation of the cellular mechanisms underlying RF production.
Suppressor cell loss and dysfunction in mixed connective tissue disease
Authors: Alarcon‐Segovia Donato , Ruiz‐Arguelles Alejandro
Published: February 2007

Of 11 patients with untreated mixed connective tissue disease (MCTD) who were studied, all had decreased circulating Ty cells, and all but one had decreased suppressor cell function of T cells on B cells, determined by a reverse hemolytic plaque‐cell assay. Results in 21 normal subjects were significantly different. Incubation of mononuclear cells from MCTD patients in purified anti‐ribonucleoprotein (anti‐RNP) IgG resulted in further decrease in their suppressor function. Decrease of Ty cells and their suppressor function may be due to in vivo penetration of anti‐RNP antibody into Ty cells, causing their deletion.
The effect of synovial tissue on the synthesis of proteoglycan by the articular cartilage of young pigs
Authors: Jubb R. W. , Fell Honor B.
Published: February 2007

Explants of pig articular cartilage were grown in organ culture in the presence of synovial tissue; controls consisted of paired explants that were cultivated in isolation. To find whether the synovial tissue affected synthesis of sulfated proteoglycan by the cartilage, 35SO4 was added to the medium and its incorporation into the cartilage examined by both biochemical assay and autoradiography. The synovial tissue severely inhibited the uptake of 35SO4, but if the synovium was removed after 8 days cultivation and the cartilage was grown in isolation for a further 4 days, incorporation of 35SO4 equalled and sometimes surpassed that of controls which had been grown without synovium continuously for 12 days. Synovium did not prevent the formation of new cartilage on the cut surfaces of the explants, but it reduced the incidence of newly formed cartilage.
An epidemiologic study of households exposed to canine systemic lupus erythematosus
Authors: Reinertsen James L. , Kaslow Richard A. , Klippel John H. , Hurvitz Arthur I. , Lewis Robert M. , Rothfield Naomi F. , Zvaifler Nathan J. , Steinberg Alfred D. , Decker John L.
Published: February 2007

To determine whether exposure to canine lupus is a risk factor for human lupus, we studied 83 members of 23 households exposed to 19 dogs with high titer antinuclear antibodies and compared these contact households to 50 members of 18 control households matched for dog age, sex, and primary veterinarian. No differences were found between contacts and controls in titer of antinuclear, antiDNA, and antilymphocyte antibodies, frequency of positive rheumatoid factor, or elevated serum immunoglobulins. Further analysis of subgroups by age, sex, and intensity of dog exposure did not reveal any serologic differences between contacts and controls. No cases of lupus were identified in either group. Three contact households and no controls reported a family history (remote from the household) of lupus. This study did not detect any clinical or serologic effect of human household exposure to dogs with high titer antinuclear antibodies.
Diversity of antinuclear antibodies in progressive systemic sclerosis
Authors: Tan Eng M. , Rodnan Gerald P. , Garcia Ignacio , Moroi Yasuoki , Fritzler M. J. , Peebles Carol
Published: February 2007

Antinuclear antibodies wer demonstrated in the sera of 43 of 45 (96%) patients with progressive systemic sclerosis (22 of 24 with diffuse scleroderma and 21 of 21 with the CREST syndrome variant). This high percentage of positive reactions resulted from the use of a tissue culture substrate (Hep‐2) to detect antinuclear antibodies by the indirect immunofluorescent method. Patterns of nuclear staining included diffuse fine speckles, large coarse speckles, nucleolar and centromere staining. When organ sections such as mouse kidney were used as substrate for the detection of antinuclear antibodies, nucleolar staining and centromere staining were the two patterns most frequently overlooked. Three types of antibodies appeared to be highly specific for scleroderma: antibody to Sc1‐70 antigen, antibody to centromere, and antinucleolar antibody. The anti‐centromere antibody appeared to be highly selective for the CREST variant of progressive systemic sclerosis.
Absence of immunoglobulins in rheumatoid cartilage‐pannus junctions
Authors: Shiozawa Shunichi , Jasin Hugo E. , Ziff Morris
Published: February 2007

Immune complexes (1C) present in the superficial region of rheumatoid articular cartilage may be an important factor in the invasion of the cartilage by pan‐nus. The presence of such 1C at the cartilage‐pannus junction in rheumatoid articular cartilage invaded by pannus was investigated by using hnmunoelectron microscopy with horseradish peroxidase anti‐human im‐munoglobulin (Ig). It was observed that, although the pannus‐free surface of the cartilage contained easily detectable deposits of Ig, the areas covered by pannus failed to show any Ig in the 5 patients studied. This was true even in areas immediately adjacent to the advancing edge of the pannus which presumably had only recently been invaded by the granulation tissue. These results were confirmed by fluorescent antibody ultraviolet microscopy. It was concluded that rheumatoid pannus invasion of the free cartilage surface containing trapped 1C results in the disappearance of such complexes.
Heterogeneity of precipitating antibodies in polymyositis and dermatomyositis
Authors: Nishikai Masahiko , Reichlin Morris
Published: February 2007

Precipitating antibodies occurring in 26 “pure” polymyositis (PM) patients and 22 “pure” dermatomyositis (DM) patients were studied by double immunodiffusion using calf thymus nuclear extract and whole calf thymus extract. Seventeen of 26 (65%) PM sera and 13 of 22 (59%) DM sera showed positive reactions, and 18, 10, and 2 sera exhibited 1, 2, and 3 precipitin lines, respectively. Common specificities were established by immunodiffusion for the 11 PM and 6 DM sera with strong precipitin lines. These were classed into three groups of shared specificity and six reactions specific for individual sera. These 9 calf thymus antigens were shown to be different from the known soluble tisue antigens Sm, nuclear RNP, Ro, and La. A 600‐f old purification of the most frequently appearing system designated Jo‐1 was achieved and its clinical specificity for patients with PM syndromes was established.
Stimulation of prostaglandin e production by bacterial endotoxins in cultured human synovial fibroblasts
Authors: Yaron Michael , Yaron Ilana , Smetana Ophyra , Eylan Emanuel , Zor Uriel
Published: February 2007

E coli, shigella, salmonella, and cholera endotoxins stimulated prostaglandin E (PGE) production by cultured human synovial and foreskin fibroblasts. The minimal effective dose of Shigella endotoxin was 2 μg/ml and a maximal response was observed at 10 μg/ml. PGE stimulation was first detected 7 hours after addition of cholera endotoxin. Stimulation by shigelia endotoxin of both PGE and hyaluronic acid production was inhibited by indomethacin and aspirin. The present results suggest that PGE is a mediator of joint inflammation induced by endotoxins.
Carpal tunnel syndrome in chondrocalcinosis of the wrist
Authors: Gerster Jean C. , Lagier René , Boivin Georges , Scheider Claude
Published: February 2007

Carpal tunnel syndrome (CTS) defined electromyographically was found in 8 of 22 unselected patients with chondrocalcinosis of the wrists (bilateral in 6, unilateral in 2). Thenar atrophy was the most frequently encountered clinical sign. In the hands with CTS, calcifications of the wrist were more extensive than in the hands without CTS. Degenerative joint changes of the hands were significantly more frequent in the group suffering from CTS. Surgery was perfomed in 5 patients with CTS. Microscopic examination of the volar carpal ligament and the flexor synovial tendon sheath failed to reveal histologic signs of chondrocalcinosis. However, the presence of scarce crystals, as seen in polarized light as well as electron microscopy, suggests a minor form of chondrocalcinosis.
Gold does not alter the arthritic, humoral, or cellular responses in rats with type ii collagen‐i nduced arthritis
Authors: Joseph McCune W. , Trentham David E. , David John R.
Published: February 2007

We assessed the effects of administration of gold sodium thiomalate on the clinical and immunologic manifestations of type II collagen‐i nduced arthritis in rats. Injections of varying doses of gold did not alter these parameters significantly. Thus, there was no evidence in this animal model that gold possesses either antiinflammatory or immunosuppressive properties.
Enhanced in vitro synthesis of igm rheumatoid factor in rheumatoid arthritis
Authors: Koopman William J. , Schrohenloher Ralph E.
Published: February 2007

Peripheral blood mononuclear leukocytes (MNL) from 42 patients with classic/definite seropositive rheumatoid arthritis (RA) and 24 healthy adult controls were tested for their capacity to produce IgM rheumatoid factor (RF) in vitro in the presence and absence of pokeweed mitogen (PWM). In no instance was spontaneous elaboration of IgM RF from control MNL observed. In contrast, MNL from 16 of the 42 RA patients spontaneously synthesized IgM RF (22 ± 43 ng/ culture) which constituted a substantial fraction of the total IgM in these culture fluids (48 ± 26%). Pokeweed mitogen induced detectable quantities of IgM RF in MNL culture supernatants from 10 of 24 controls (12 ± 11 ng/culture) and 33 of 42 RA patients (60 ± 82 ng/ culture, p = 0.008). IgM RF constituted a significantly higher proportion of the total IgM in RA MNL supernatants than in control supernatants (11 ± 11% versus 1.01 ± 1.03%; P = 0.013). IgM RF production (spontaneous and PWM‐induced) was dependent upon de novo protein synthesis. The results indicate that B cells programmed to produce IgM RF are present in both normal and RA B cell repertoires, but are preferentially expressed in RA.
Correlation between anti‐rana and anti‐ebna titers in normal subjects with and without hla‐drw4
Authors: Catalano Michael A. , Carson Dennis A. , Stastny Peter , Freer Sofia , Vaughan John H.
Published: February 2007

We have determined the relationship of antibody titers to the rheumatoid arthritis nuclear antigen (RANA) and of antibody titers to the Epstein‐Barr nuclear antigen (EBNA) to each other and to the DRw4 B cell alloantigen in the sera of 34 normal white adults. By a sensitive indirect immunofluorescence (IF) assay, 76% had RANA antibody, compared to 23% by a micro‐immunodiffusion assay. The correlation coefficient for the tube dilution titers of anti‐RANA and anti‐EBNA was 0.61 (P < 10‐4). The 14 DRw4‐positive subjects and the 20 DRw4‐negative subjects did not differ with respect to anti‐RANA titers (P = 0.51) or anti‐EBNA titers (P = 0.89). We conclude that: 1) most normal adults have RANA antibody by IF; 2) anti‐RANA and anti‐EBNA titers are closely related; 3) the titers of these antibodies cannot be related to the presence of the DRw4 determinant in normal persons.
Synovial fluid in seronegative juvenile rheumatoid arthritis
Authors: Martin Christina L. , Pachman Lauren M.
Published: February 2007

Synovial fluid of 20 children with seronegative juvenile rheumatoid arthritis (JRA) and 20 patients with other joint pathology was examined for levels of total hemolytic complement and selected components, immune complexes, and α2‐macroglobulin (α2‐M). When adjusted for total protein, synovial levels of total hemolytic complement, C3, C4, and α2‐M did not differ significantly in the two groups. The concentrations of IgM and IgG were elevated in the JRA synovial fluid, but immune complexes were not increased when compared with the non‐JRA group as determined by the Raji cell technique. α2‐M activity against substrates of C1 esterase was absent. Therefore, evidence of specific increase in immune complexes and complement activation was sought but not found, suggesting that they may not be a major factor in the pathogonesis of seronegative, pauciarticular JRA.
The C1q binding assay in systemic lupus erythematosus
Authors: Inman Robert D. , Fong J. K. K. , Pussell B. A. , Ryan P. J. , Hughes Graham R. V.
Published: February 2007

To investigate the relationship of C1q binding assay (C1qBA) to disease activity in systemic lupus erythematosus (SLE), a retrospective study was carried out on 232 C1qBA performed in 33 patients with SLE. When initial values only were assessed (33 tests in 33 patients), there was no relationship between positive and negative C1qBA and abnormal renal function (P = 0.482, Fisher exact test). Of 87 tests performed during active renal disease, 34 (39%) were positive; of 48 tests during active non‐renal disease, 25(52%) were positive; of 83 tests when the disease was inactive, 45(54%) were positive; and of 14 tests during episodes of infection, 10 (71%) were positive. Corresponding means for the C1qBA were as follows: renal 65.66, non‐renal 78.02, in‐active 56.04, infection 135.79 (no significant differences by Student's t‐test). There was no significant relationship with the C1qBA when comparing active disease (renal and non‐renal) with inactive disease (X2 Yates = 1.875, P = 0.171). When renal function abnormalities were analyzed separately, the C1qBA values were independent of azotemia or proteinuria (X2 Yates = 1.399, P = 0.237). Patients were seen with progressive renal disease who had consistently negative C1qBA, as well as patients with benign clinical courses despite elevated C1qBA. The discordance of the C1qBA results with disease activity in SLE highlights the limitations of immune complex (IC) determinations by a single technique, and stresses the importance of evaluating such tests in terms of both their specificity and sensitivity. These data further suggest that the relationship of IC to the disease process in SLE may be a complex one.
Roman arches, human joints, and disease
Authors: Simkin Peter A. , Graney Daniel O. , Fiechtner Justus J.
Published: February 2007

The subchondral plate of bone underlying concave joint surfaces is significantly thicker, at most sites, than that under the corresponding convex mates. This morphologic difference may be related to parallel differences in loading stress and in the distribution of osteonecrosis and other joint lesions.
The spondylitis of inflammatory bowel disease
Authors: Enlow Roger W. , Bias Wilma B. , Arnett Frank C.
Published: February 2007

Of 12 patients with inflammatory bowel disease (IBD) and ankylosing spondylitis (AS) or sacroilitis (SI), only 4 (33%) had HLA‐B27. Family studies revealed 3 B27‐ negative relatives with AS, 1 with SI, 1 with SI and IBD, and 1 with IBD alone. HLA haplotypes did not segregate with disease. These data suggest a non‐HLA linked genetic predisposition to IBD which also confers susceptiblity to spondylitis, even in the absence of expression of bowel disease.
Frentizole therapy in systemic lupus erythematosus
Authors: Sabharwal Umesh K. , Vaughan John H. , Kaplan Roy A. , Robinson Charles A. , Curd John G.
Published: February 2007

Seven corticosteroid dependent patients with active systemic lupus erythematosus (SLE) were given frentizole in order to assess its effect on their clinical manifestations. Three patients exhibited slight clinical improvement, 2 patients did not change, and 2 deteriorated. Five of the 7 patients developed subclinical and reversible liver damage related to frentizole. This study, although preliminary and uncontrolled, suggests that frentizole is of limited value in the management of patients with SLE.
Frentizole Therapy of Active Systemic lupus Erythematosus
Authors: Kay Donald R. , Valentine Thomas V. , Walker Sara E. , Valentine Merilee H. , Bole Giles G.
Published: February 2007

Frentizole is a benzimidazoleurea that has immunosuppressive properties in mice. Eleven steroid‐treated patients with active systemic lupus erythematosus received frentizole (150‐350 mg/day) in combination with stable or decreasing doses of prednisone in an open label trial. Nine patients completed at least one 21‐to 75‐day course of therapy with this drug. Clinical parameters of disease improved in 8 of these 9 patients. Mean DNA binding decreased by 28%, mean CH50 increased by 20%, and mean absolute lymphocyte and T cell counts decreased by 25‐26%. Granulocytopenia was not observed. Three patients developed reversible hepatic toxicity. Clinical and serologic improvement was noted in 3 patients who accepted a second 90‐day course of frentizole therapy.
Characterization of the immunocompetent cells of rheumatoid synovium from tissue sections and eluates
Authors: T Konttinen Yrjö , Reitamo Sakari , Ranki Annamari , Häyry Pekka , Kankaanapää Uolevi , Wegelius Otto
Published: February 2007

T lymphocytes positively identified by alphanaphthyl acetate esterase (ANAE) (70%) were localized in perivascular “cuffs” in rheumatoid synovia. ANAE‐negative lymphocytes (B lymphocytes) were primarily within the reactive lymphoid centers, whereas intra‐cellular Ig‐expressing plasma cells were outside the centers. Lysozyme or diffuse ANAE expressing mono‐nuclear phagocytes (5–15%) were diffusely dispersed, but were seldom found in the lymphoid centers. There were few granulocytes (10%). On elution, plasma cells and lymphocytes were lost. Most granulocytes and mononuclear phagocytes in the eluate were immunoglobulin‐positive. The in situ architecture is compatible with active immunologic inflammation and T‐dependent immunglobulin synthesis.
Deficiency of c7 with systemic lupus erythematosus. solubilization of immune complexes in complement‐deficient sera
Authors: J Zeitz Howard , W Miller Gary , Lint Thomas F , Ali Mir A , Gewurz Henry
Published: February 2007

A woman with systemic lupus erythematosus (SLE) was found to have undetectable serum total complement (C) activity with marked reductions in levels of several C components. Recognition of rising C4, C3, and C5 levels coupled with sustained absence of hemolytic activity led to studies of individual C components that identified a persisting selective and complete absence of C7 hemolytic activity. Review of the literature indicated that the frequency of SLE is increased in patients with complete deficiency of a late‐acting C component (7% as compared to 0.05% in the general population); however, the disease frequency is less than that observed in patients with complete deficiency of an early‐acting C component (41% incidence in patients lacking C1, C4, or C2). In order to explore one possible basis for this association, the capacity of serum from the patient described herein and from patients with other C component deficiencies to mediate in vitro solubilization of immune complexes was studied by using a recently developed assay. The patient's serum had a markedly decreased capacity to induce solubilization of immune complexes, as did sera of other patients with active SLE and sera of individuals with inborn deficiencies of C1, C2, or C3 without SLE. By contrast, the sera of patients with a selective terminal C component deficiency (C5, C6, C7, or C8) but without SLE readily supported immune complex solubilization. These results suggest that patients lacking a terminal C component are predisposed to the development of SLE by a process which is independent of C‐mediated immune complex solubilization. Whether the compromised capacity for C‐mediated immune complex solubilization contributes to the even greater frequency of SLE in patients lacking an early‐acting C component remains to be established.
“Milwaukee shoulder”—association of microspheroids containing hydroxyapatite crystals, active collagenase, and neutral protease with rotator cuff defects. iii. morphologic and biochemical studies of an excised synovium showing chondromatosis
Authors: Garancis John C. , Cheung Herman S. , Halverson Paul B. , Mccarty Daniel J.
Published: February 2007

Synovial tissue excised from the unstable right shoulder joint of a patient with an absent rotator cuff, severe glenohumeral joint degeneration, and hydroxyapatite‐containing microspherules, collagen types I, II, and III, active collagenase, and neutral protease in the joint fluid showed extensive osteochondromatosis histologically. Electronmicroscopy revealed calcific foci in microvilli which could easily escape into the adjacent joint space through areas denuded of synovial cells. Fibrocytes demonstrated intensive pinocytotic activity of unknown significance. Energy dispersive analysis showed elemental ratios consistent with hydroxyapatite. A literature review suggested some relationships between the various pathologic lesions present in this joint. Whether similar synovial changes exist in the opposite shoulder joint of this patient and in 3 other subjects with nearly identical clinical, radiographic, and joint fluid findings is not known.