bluuebunny/crossref_metadata_2025 huggingface dataset:
Multiple interferons in the circulation of patients with systemic lupus erythematosus and vasculitis
Authors: Hooks John J. , Jordan George W. , Cupps Thomas , Moutsopoulos Haralampos M. , Fauci Anthony S. , Notkins Abner Louis
Published: February 2007

Recently, we found interferon in the sera of patients with systemic lupus erythematosus, rheumatoid arthritis, scleroderma, and Sjögren's syndrome. In this study, we surveyed a variety of other immunologically mediated diseases. We did not find interferon in the sera of patients with Wegener's granulomatosis, sarcoidosis, infectious mononucleosis, minimal change nephritis, kidney transplants, myasthenia gravis, or uveitis, but we did find this protein in the sera of patients with active systemic and cutaneous vasculitis. Attempts to characterize the interferon in the sera of patients with systemic lupus erythematosus and vasculitis revealed that antibody to alpha (leukocyte) interferon, but not to beta (fibroblast) interferon, partially or completely neutralized the antiviral activity. The failure of antibody to alpha interferon to completely neutralize the antiviral activity in certain specimens and the lability of the antiviral activity in some specimens to pH 2.0 treatment both suggest that more than one type of interferon was present.
High rate of death caused by systemic lupus erythematosus among U. S. residents of asian descent
Authors: Kaslow Richard A.
Published: February 2007

From 1968 through 1976, adjusted annual rates of death from systemic lupus erythematosus, based on National Center for Health Statistics data from the 12 states with most (88%) of the Asian population, were higher for Chinese, Japanese, and Filipinos than for whites in the United States. These Asian subgroups had a combined average annual mortality rate significantly higher than that of whites whether Hawaii was included (6.8/million for Asians versus 2.8 for whites) or excluded (6.0 versus 2.8).
In vitro cartilage degradation by escherichia coli and staphylococcus aureus
Authors: Smith R. Lane , Merchant Thomas C. , Schurman David J.
Published: February 2007

Effects of Escherichia coli and Staphylococcus aureus on cartilage and chondrocytes in culture are reported. Under these conditions, bacterial effects on cartilage degradation and cell viability are measured in the absence of inflammation. E coli causes a 28% loss and S aureus an 83% loss of cartilage glycosaminoglycan within 48 hours. Collagen content is unchanged. Both bacterial species induce chondrocyte death in explants and in monolayers within 48 hours. Bacterial effects on glycosaminoglycans and cell viability do not result from depletion of nutrients from the culture medium. Serum in the culture media inhibits the bacterial effects on cartilage degradation but does not prevent cell death.
The association of antinuclear antibodies, antilymphocyte antibodies, and c4 activation among the relatives of children with systemic lupus erythematosus. preferential activation of complement in sisters
Authors: Lehman THOMAS J. A. , Curd John G. , Zvaifler Nathan J. , Hanson Virgil
Published: February 2007

We studied 31 children with systemic lupus erythematosus (SLE) and 108 first‐degree relatives of the children to determine if HLA type, familial relationship to patient, or gender influenced the familial aggregation of SLE and the serologic and serum complement abnormalities associated with SLE. There were no significant relationships between the presence of antinuclear antibodies (ANA), antilymphocyte antibodies (ALA), or circulating immune complexes. Furthermore, there was no correlation between the occurrence of ANA, ALA, or circulating immune complexes with HLA type or relationship to patient. Both ANA and ALA were associated with a reduction in the mean serum C4 level, without a corresponding reduction in mean serum C3 or CH50. The reduction in mean serum C4 level with ALA occurred without regard to the subject's relationship to the patient, but the reduction in mean serum C4 level with ANA was significant only among the sisters. Because of these data, it seems that the sisters of patients with SLE are more likely than other relatives to have activation of the complement system in association with serologic abnormalities. Those sisters with ANA and ALA had the greatest amount of complement activation and may be the relatives at greatest risk for the subsequent development of SLE.
Controlled multicenter trial of tiopronin and D‐penicillamine for rheumatoid arthritis
Authors: Pasero Giampiero , Pellegrini Pietro , Ambanelli Umberto , Ciompi Maria Laura , Colamussi Vincenzo , Ferraccioli Gianfranco , Barbieri Paola , Mazzoni Maria Rosa , Menegale Germano , Trippi Donatella
Published: February 2007

Fifty‐seven patients took part in a controlled double‐blind trial between tiopronin and D‐penicillamine as basic treatment for rheumatoid arthritis. Thirty‐nine (19 receiving tiopronin, 20 receiving D‐penicillamine) completed the trial after 1 year. Both drugs resulted in a decrease of the erythrocyte sedimentation rate, Ritchie index, and Lee index and in a sparing effect on symptomatic antiinflammatory therapy. Improvement in these variables was statistically highly significant at any interval with tiopronin, but was sometimes less or not at all significant with D‐penicillamine. Nevertheless, the difference in effects between the 2 drugs never reached statistical significance. Six patients receiving tiopronin and 6 receiving D‐penicillamine were taken out of the experiment because of side effects.
Mechanisms of abnormal platelet aggregation in systemic lupus erythematosus
Authors: Dorsch Carole A. , Meyerhoff John
Published: February 2007

Platelet aggregation was measured in 14 patients with systemic lupus erythematosus (SLE) and 13 normal controls. Ten SLE patients (group I) showed decreased responsiveness to collagen, while aggregation was normal in 4 (group II). Group I patients also responded poorly to epinephrine. Platelets from SLE patients did not differ from controls in the production of malondialdehyde in response to N‐ethylmaleimide, suggesting intact prostaglandin synthetic pathways. However, a significant decrease (P < 0.005) in platelet levels of the dense granule constituent, serotonin, was noted in group I SLE patients. Treatment of SLE platelet‐rich plasma with deoxyribonuclease (DNase) resulted in enhancement of collagen‐induced aggregation in 4 group I SLE patients, but not in 1 group II or 8 normal individuals. These results suggest that defective aggregation in SLE platelets may be partially related to a storage pool deficiency state. However, the ability to restore aggregation to collagen by digestion of platelet‐rich plasma with DNase indicates that the defect is reversible and that it is perhaps mediated by plasma or platelet‐associated DNA.
Olecranon bursitis related to calcium pyrophosphate dihydrate crystal deposition disease. Clinical and pathologic study
Authors: Gerster Jean‐C. , Lagier René , Boivin Georges
Published: February 2007

A case of olecranon bursitis in an 81‐year‐old patient is presented. Analysis of the bursal fluid revealed positive birefringent crystals; radiographs showed calcifications in the distal triceps tendon. A bursectomy was performed. X‐ray diffraction analyses demonstrated calcium pyrophosphate dihydrate patterns in a subcutaneous “tophus”s and in a specimen of the tendon. On histologic examination, there was a bursitis with positive birefringent crystals on the bursa's inner surface; histologic images of “chondrocalcinosis's's were observed in and around the tendon. It is concluded that bursitis may be part of the extraarticular manifestations of calcium pyrophosphate dihydrate crystal deposition disease.
A syndrome of seronegative enthesopathy and arthropathy in children
Authors: Rosenberg Alan M. , Petty Ross E.
Published: February 2007

Thirty‐nine children with a syndrome of seronegative enthesopathy and arthropathy were evaluated. The group included 25 patients with no apparent underlying primary disease and 13 with either ankylosing spondylitis, inflammatory bowel disease, reactive arthritis, or Reiter's syndrome. Significant distinguishing characteristics of the group included male predominance, late age at onset, positive family histories of arthritis, oligoarthropathy, axial skeleton involvement, and the presence of the B27 histocompatibility antigen. This syndrome is distinguishable from other childhood rheumatic disorders, including juvenile rheumatoid arthritis. Its recognition may reliably identify children with the prodromal manifestations of seronegative spondylarthropathies.
The arthritis impact measurement scales. further investigations of a health status measure
Authors: Meenan Robert F. , Gertman Paul M. , Mason John H. , Dunaif Richard
Published: February 2007

The Arthritis Impact Measurement Scales (AIMS) have been developed to assess the health status of arthritis patients. In this study, the self‐administered AIMS questionnaire, which includes scales designed to measure the physical, psychologic, and social aspects of health status, was completed by 625 patients with various forms of arthritis. A comprehensive battery of analytic techniques was used to investigate the performance of these scales in this large sample. The results confirmed the reliability and validity of the AIMS instrument. They also showed that AIMS performs well in at least 4 major types of arthritis, in a range of sociodemographic groups, and across time. These findings emphasize the strengths of the AIMS approach and suggest that the instrument will prove useful as a tool to assess arthritis outcome in a wide variety of clinical settings.
Circulating immune complexes in eosinophilic fasciitis
Authors: Seibold James R. , Rodnan Gerald P. , Medsger Thomas A. , Winkelstein Alan
Published: February 2007

Serum immune complexes were measured in 22 patients with eosinophilic fasciitis, 8 of whom had serial determinations. Elevated levels were found by Raji cell radioimmunoassay in 14 (64%) patients, by agarose gel electrophoresis in 13 (59%), and by C1q agglutination‐inhibition in 9 (41%). Elevated levels by Raji cell assay were present more frequently at times of active disease [17 of 22 sera (77%)] than at times of inactive disease [2 of 24 (8%) (P<0.0005)] and were more closely correlated with disease activity than were eosinophilia, hypergammaglobulinemia, or increased erythrocyte sedimentation rate.
Hypertrophic osteoarthropathy in cyanotic congenital heart disease. its prevalence and relationship to bypass of the lung
Authors: Martínez‐Lavín Manuel , Bobadilla MaríA , Casanova José , Attié Fause , Martínez Marco
Published: February 2007

The frequency of hypertrophic osteoarthropathy in cyanotic congenital heart disease has previously been considered to be very low. Only a few isolated reports have described such an association. We studied 32 consecutive patients older than 6 years with various types of cyanotic congenital heart disease and examined each case for hypertrophic osteoarthropathy. We also assessed the role of the altered cardiopulmonary hemodynamics in the development of hypertrophic osteoarthropathy by means of cardiac catheterization. Our results showed that 31% of the patients had hypertrophic osteoarthropathy. When the hemodynamic parameters of the group of patients with hypertrophic osteoarthropathy were compared with those of the remaining patients, we found significant differences in the systemic blood flow (P ⩽ 0.05), right‐to‐left shunt (P ⩽ 0.05), and arterial oxygen unsaturation (P ⩽ 0.005). Thus, we found a much higher prevalence of hypertrophic osteoarthropathy in patients with cyanotic congenital heart disease than has previously been recognized. The development of hypertrophic osteoarthropathy is related to the degree of bypass of the lung. This is consistent with the concept that hypertrophic osteoarthropathy results from mediators in the systemic venous circulation that escape inactivation in the pulmonary capillary bed.
Immobilization of the knee prevents osteoarthritis after anterior cruciate ligament transection
Authors: Palmoski Marshall J. , Brandt Kenneth D.
Published: February 2007

The effect of immobilization of the knee in flexion on the development of osteoarthritis was studied in dogs that had undergone transection of the anterior cruciate ligament. Knees of dogs that were permitted ad libitum ambulation in a pen for 12 weeks after transection of the ligament showed osteophytes and fibrillation and a decrease in proteoglycan (uronic acid) content, although thickness of the articular cartilage was normal. Proteoglycan synthesis was 80% greater (P < 0.01) than that in cartilage from the contralateral knee, and more than twice the normal proportion of the total proteoglycans was present in the medium of cultures of cartilage from the unstable knee (P < 0.01). Similarly, the proportion of total tissue proteoglycans extracted with 0.4M guanidinium chloride was 3 times greater than normal (P < 0.01). In contrast, osteophytes were not seen when the leg was immobilized in flexion immediately after transection of the ligament, and the articular surface remained intact. The cartilage became atrophic, however, and its proteoglycan content (uronic acid) was diminished; proteoglycan synthesis was decreased to 61% of the level in cartilage from the contralateral knee, although proteoglycan extractability was normal. Knee cartilage from these legs that were immobilized after transection, therefore, resembled that from dogs whose leg had been immobilized without cruciate ligament transection. In cartilage from all operated and/or immobilized joints the proportion of proteoglycans that interacted with hyaluronic acid in vitro was diminished, because of a defect in the hyaluronate‐binding region of the proteoglycan.
Histoplasmosis presenting with joint pain and hilar adenopathy. “pseudosarcoidosis”
Authors: Thornberry Denise K. , Wheat L. Joseph , Brandt Kenneth D. , Rosenthal Jerome
Published: February 2007

During 2 recent epidemics of histoplasmosis in Indianapolis, 7 patients with hilar adenopathy and joint complaints were identified who were initially considered to have sarcoidosis, but in whom histoplasmosis was ultimately diagnosed on the basis of a positive culture for Histoplasma capsulatum or serologic studies. Articular features were the sole presenting complaints in 3 of these patients. All had arthralgias, which were generalized or confined to the lower extremities and were additive rather than migratory. Only 1 patient had joint swelling. Two had erythema nodosum. In all but 1 patient, the articular symptoms resolved rapidly and completely with minimal therapy. The data emphasize that sarcoidosis is a diagnosis of exclusion and that granulomatous infectious diseases must be excluded in patients with hilar adenopathy and joint disease.
Relationship of clinical findings in systemic lupus erythematosus to seroreactivity
Authors: Rothschild Bruce M. , Jones John Verrier , Chesney Carolyn , David Pifer D. , Thompson Lee D. , James Karen K. , Badger Harriette
Published: February 2007

We have characterized 52 consecutive patients fulfilling 4 or more of the American Rheumatism Association criteria for systemic lupus erythematosus in order to provide, for the first time, a homogeneous sample for statistical comparison of antinuclear antibody (ANA)‐positive and ANA‐negative groups. Ten patients (19%) were seronegative. There was no significant difference in age, disease activity, organ system involvement, erythrocyte sedimentation rate, immune complex levels, or C3 levels. The ANA‐negative group showed a higher incidence of involvement for whites and men. Leukopenia, lower levels of antibody to DNA, and higher C4 levels were also characteristic of the ANA‐negative group.
The glycosaminoglycans in menisci in experimental and natural osteoarthritis
Authors: Adams Mark E. , Billingham Michael E. J. , Muir Helen
Published: February 2007

The glycosaminoglycans in the menisci of beagles 5–7 years old were analyzed at various times after osteoarthritis was induced by sectioning the anterior cruciate ligament of one knee; the unoperated knee served as control. In the first month after induction, there were signs of inflammation in the operated joint. After 1 week, the water content was elevated and the glycosaminoglycan content (per dry weight) was reduced. The content of keratan sulfate decreased more than that of chondroitin sulfate, but the hyaluronic acid content did not change consistently. The relative proportions of chondroitin‐4‐sulfate, chondroitin‐6‐sulfate, and dermatan sulfate remained unchanged. After 3–18 months, the glycosaminoglycan levels reverted to normal, and there was some evidence that after 15–18 months, they were elevated above normal. These results, together with results obtained from single examples of mild and severe osteoarthritis in working foxhounds, suggest that, in contrast to articular cartilage, the meniscus is capable of some regeneration in response to injury.
Pancreatitis with arthropathy and subcutaneous fat necrosis
Authors: Alexander Wilson H. , Askari Ali D. , Neiderhiser Dewey H. , MyronJohnson A. , Andrews Brian S. , Hoskins Lansing C.
Published: February 2007

The occurrence of peripheral fat necrosis in exceptional cases of pancreatic disease is not well understood. We report studies on such a patient with arthropathy and subcutaneous nodules. Examination of serial serum samples demonstrated striking elevations of the pancreatic enzymes phospholipase A, 3–3.4 units/ml (normal 0.17–0.41); lipase, 7–39 Sigma‐Tietz units/ml (normal < 1); immunoreactive trypsin, 912–3,207 ng/ml (normal 12–41). The distinguishing characteristic of the patient's synovial fluid was a marked elevation of hydrolized fatty acids (680 mg/dl versus 19 ± 19 in control inflammatory joint fluids). Synovial fluid fatty acid distribution was identical to values for tissue fat. In contrast, serum fatty acid levels and distribution were normal. No associated proteinase inhibitor or significant immunologic abnormality was detected. Certain properties of adipose cells and lipolytic enzymes may help explain the characteristically selective necrosis of fat cells observed in this syndrome.
Free fatty acids in the pancreatitic arthritis syndrome
Authors: Simkin Peter A. , Brunzell John D. , Wisner David , Fiechtner Justus J. , Carlin Jeffrey S. , Willkens Robert F.
Published: February 2007

Fat necrosis and arthritis occur in some individuals with pancreatitis. The intraarticular free fatty acid concentration was found to be markedly elevated in 2 patients with this pancreatitic arthritis syndrome. Injection of free fatty acids into rabbit knees reproduced the inflammatory synovial response including intracellular fat globules within leukocytes. Free fatty acids exceeding the available binding sites of albumin appear to mediate the extrapancreatic inflammatory manifestations of this syndrome.
The natural disease course of ankylosing spondylitis
Authors: Carette Simon , Graham Donald , Little Hugh , Rubenstein Joel , Rosen Philip
Published: February 2007

One hundred fifty war veterans with ankylosing spondylitis were entered into a prospective study in 1947. In 1957, 142 were traced, and they have been reviewed periodically. Eighty‐one of these patients were still alive in 1980. Information was obtained from 67 (83%) of the survivors and 51 were reexamined. This report is based on the clinical findings in these 51 patients, who have a mean disease duration of 38 years. Forty‐seven (92%) were functioning well. The disease in 21 (41%) had progressed to cause severe spinal restriction. Of those, 12 had peripheral joint involvement early in their course and 9 had iritis. Seventy‐four percent of the patients who had mild spinal restriction after 10 years did not progress to having more severe restriction. Eighty‐one percent of the patients who had severe spinal restriction in 1980 were severely restricted within the first 10 years. Hips that were normal after 10 years of disease did not become diseased subsequently. This study suggests that a predictable pattern of ankylosing spondylitis emerges within the first 10 years of the disease.
Alizarin red s staining as a screening test to detect calcium compounds in synovial fluid
Authors: Paul Hernando , Reginato Antonio J. , Ralph Schumacher H.
Published: February 2007

A simple, rapid screening method using alizarin red S stain and ordinary light microscopy to detect microcrystalline or noncrystalline calcium phosphate salts was used on wet drop preparations of synovial fluids. This proved to be helpful in detecting apatite crystal clumps and small calcium pyrophosphate dihydrate (CPPD) crystals missed by polarized light. The staining was positive in 100% of synovial fluids from patients later proven to have apatite and/or CPPD deposition diseases. Apatite and CPPD crystals were commonly found together in the same fluids. In addition, some synovial fluids from patients with osteoarthritis, renal failure dialysis, rheumatoid arthritis, and gout also exhibited positive staining. The correlation of positive alizarin red S staining with radiologic evidence of osteoarthritis suggests that apatite crystals might be related to articular cartilage degeneration in different rheumatic diseases.
Clinical and serologic study of sjögren's syndrome in patients with progressive systemic sclerosis
Authors: Osial Thaddeus A. , Whiteside Theresa L. , Buckingham Robert B. , Singh Gurmukh , Barnes E. Leon , Pierce John M. , Rodnan Gerald P.
Published: February 2007

Fifty‐eight patients with progressive systemic sclerosis (PSS) were evaluated clinically and by biopsy of the minor salivary glands of the lips for the presence of Sjögren'S syndrome. Clinical findings included dry eyes in 38%, dry mouth in 32%, parotid enlargement in 4%, and an abnormal Schirmer'S test in 34%. Histologic changes in lip biopsies included lymphocytic infiltrates characteristic of Sjögren'S syndrome in 17 individuals (29%). In 19 (33%) there was periglandular and intraglandular fibrosis (PSS‐fibrosis) without significant inflammation, an alteration characteristic of PSS per se. In the remaining 22 patients (38%) with PSS, no abnormality was found. Of those with PSS and Sjögren'S syndrome, 53% had serum antibodies to SS‐A and/or SS‐B, while only 1 patient with a normal biopsy had either of these antibodies. Anti‐SS‐A and anti‐SS‐B were not detected in patients with glandular fibrosis alone. The mortality rate of the PSS‐fibrosis group was higher due to a variety of severe internal manifestations related to PSS. Antibodies to SS‐A and SS‐B are useful serologic markers of the presence of Sjögren'S syndrome in patients with PSS.
Connective tissue activation
Authors: Castor C. W. , Roberts D. J. , Hossler P. A. , Bignall M. C.
Published: February 2007

In this study, virtually all sulfated glycosaminoglycan (GAG) synthesized and secreted by human synovial cells, both normal and rheumatoid, was detected in the form of proteoglycans of monomeric size. Enzyme hydrolysis studies that were performed demonstrated dermatan sulfate to be the dominant GAG in the proteoglycan, with lesser amounts of chondroitin 4/6 sulfate. Exposure to β‐xyloside, used as a false “core protein,” resulted in marked enhancement of GAG chain formation, suggesting that the synthesis of the sulfated carbohydrate chain itself was not rate limiting. Proteoglycan synthesis and secretion were stimulated by several types of connective tissue activating peptides (CTAP); CTAP‐III stimulation of incremental core protein and glycosaminoglycan was shown to be of a similar magnitude. Since chain synthesis was not rate limiting, it is suggested that stimulated proteoglycan formation caused by the CTAP peptides may be primarily modulated through increased formation of core protein.
Serotonin content of platelets in inflammatory rheumatic diseases
Authors: Zeller J. , Weissbarth E. , Baruth B. , Mielke H. , Deicher H.
Published: February 2007

Significantly decreased platelet serotonin contents were measured in rheumatoid arthritis, systemic lupus erythematosus (SLE), progressive systemic sclerosis, and mixed connective tissue disease. An inverse relationship between platelet serotonin levels and clinical disease activity was observed in both rheumatoid arthritis and systemic lupus erythematosus. SLE patients with multiple organ involvement showed the lowest platelet serotonin values. No correlation was observed between platelet serotonin contents and nonsteroidal antiinflammatory drug treatment, presence of circulating platelet reactive IgG, or the amount of circulating immune complexes. The results are interpreted as indicating platelet release occurring in vivo during inflammatory episodes of the rheumatic disorders investigated.
Identification and partial purification of the major aspirin hydrolyzing enzyme in human blood
Authors: Costello Patrick B. , Green Floyd A.
Published: February 2007

In normal whole human blood in vitro, the source of the enzyme controlling the hydrolysis of aspirin (ASA) was found to be the erythrocyte (RBC). Experiments were carried out to determine whether this enzyme was membrane‐bound or free in the lysate. The mean rates of ASA hydrolysis in comparable concentrations of intact RBC (1.61 ± 0.20 γmole/liter/minute, 12 = n and 1.23 ± 0.17 γmole/liter/minute, n = 5) were much faster than that measured in isolated RBC membranes (0.23 ± 0.08 γmole/liter/minute, n = 6, P = ≤0.001). Detailed study showed that the RBC‐related ASA esterase is located intracellularly and is not related to membrane acetylcholinesterase. The ASA esterase from crude lysate was purified 900‐fold by means of DEAE Sephacel chromatography of active enzyme recovered from a 50% saturated ammonium sulfate fraction. Non‐SDS polyacrylamide gel electrophoresis (pH 8.3 and 9.0) resulted in one major band and one or more small minor protein bands. When esterase activity was assayed in a non‐stained gel, ASA depeletion and salicylate production corresponded exactly to the major stained band. This band was eluted from another unstained gel, concentrated, and applied to an SDS gel. This yielded a single band with a molecular weight of approximately 95,000. The partially purified enzyme had a mean Km of 66.6 ± 3.5 γM and a mean Vmax of 4.0 ± 0.9 γmole/liter/minute/mg under the assay conditions. The results of inhibition studies suggested that this enzyme'S activity is sulfhydryl group dependent, does not require divalent cations for activity, and is different from the RBC type D “nonspecific” esterases.
The Prevalence of Juvenile Arthritis
Authors: Gewanter Harry L. , Roghmann Klaus J. , Baum John
Published: February 2007

A secondary analysis of two new large practitioner surveys, one national and one local, showed the prevalence of juvenile arthritis to be between 0.16 and 0.43 cases per 1,000 children. These rates compare quite closely with the majority of reports both in the United States and the world. We believe the prevalence of juvenile arthritis is between 0.2 and 1 case per 1,000 children (13,000—63,000 children), with the best estimate being about 0.5 cases per 1,000 children. Based on the 1980 census data, this latter estimate means there are approximately 32,000 children with juvenile arthritis in the United States.
Antinuclear Antibodies in Localized Scleroderma
Authors: Takehara Kazuhiko , Moroi Yasuoki , Nakabayashi Yasuharu , Ishibashi Yasumasa
Published: February 2007

When HeLa cells were used as the substrate for detection by the indirect immunofluorescence method, antinuclear antibodies were demonstrated in 16 of 22 (72.7%) sera from patients with localized scleroderma. When mouse kidney sections were used, the positive rate for antinuclear antibodies was 50% (11 of 22). In the 3 subgroups of localized scleroderma, frequencies of anti‐nuclear antibodies on HeLa cells were as follows: morphea, 50% (2 of 4), generalized morphea, 100% (6 of 6), linear scleroderma, 67% (8 of 12). Antibodies to centromere, Scl‐70, nuclear RNP, Sm, and SS‐B antigens were not detected in any patient with localized scleroderma. The high frequency of antinuclear antibodies in localized scleroderma sera suggests that localized scleroderma is a disease which, though different from diffuse scleroderma, also involves an immunologic abnormality.
Acute Effects of Steroids On Immune Complex Profile of Patients with Systemic Lupus Erythematosus
Authors: Boyd Robert E. , Birchmore Daniel A. , Kaiser Donald L. , Young Alice C. , Davis John S.
Published: February 2007

Eleven patients with active systemic lupus erythematosus, previously untreated, were studied to 1) determine the acute effect of corticosteroids on circulating immune complex (CIC) levels and 2) correlate the initial CIC profile with the development of organ system involvement. Using serial measurements of CIC as detected by assays for cryoglobulins and binding to Clq, Raji cells, and rheumatoid factor, we found that levels of CIC change little during the first month of high dose daily steroid therapy, but they uniformly decrease to near normal by 6 to 12 months. High levels of CIC detected by Raji cell assay early in the course of systemic lupus erythematosus and before steroid therapy appear to be predictive of the development of chronic lupus nephritis (P < 0.005).
Band Keratopathy in MRL/1 and MRL/n Mice
Authors: Hoffman Robert W. , Kell H. Yang , Waggie Kim S. , Durham James B. , Burge Julia R. , Walker Sara Ellen
Published: February 2007

To define ocular abnormalities in mice with autoimmune disease, we performed biomicroscopic examinations and examined ocular tissue in MRL/1, MRL/n, NZB, NZB/NZW, and Palmerston North mice. Results were compared with MRL/Mp‐Ipr/lpr, C57BL/6J‐lpr/lpr, and normal control strains. Eighty‐seven percent of MRL/1 and MRL/n mice had typical band keratopathy; this was confirmed by histologic examination. Posterior uveitis was found in 35% of adult MRL/l mice. MRL substrains are potentially important models of ocular disease.
Glycosaminoglycan Content in Skin of the Tight‐Skin Mouse
Authors: Ross Stephen C. , Osborn Thomas G. , Dorner Robert W. , Zuckner Jack
Published: February 2007

The tight‐skin (TSK) mouse has cutaneous changes similar to those found in the skin of patients with progressive systemic sclerosis (PSS). Previous studies have shown that both have common abnormalities in skin thickness, dry weight, and hydroxyproline content. In this study, glycosaminoglycans (GAGs), major components of the ground substance, were quantitated in skins from TSK mice and compared with age‐matched normal mice. Biochemical studies included determinations of hexosamines, uronic acids, and total GAGs by cetylpyridinium chloride precipitation. Dry weights and water‐fat content of skin biopsy specimens from TSK mice were also compared with those of normal mice. Hexosamine, uronic acid, total GAGs, and dry weight were increased in TSK mouse skin when compared with normal mouse skin. The water‐fat content did not differ significantly. These findings were similar to those known to occur in PSS skin, further suggesting that the TSK mouse might serve as an animal model for the skin changes found in PSS patients.
Nifedipine as a therapeutic modality for raynaud's phenomenon
Authors: Winston Eileen L. , Pariser Kenneth M. , Miller Kenneth B. , Salem Deeb N. , Creager Mark A.
Published: February 2007

Eight patients with Raynaud's phenomenon were entered into a double‐blind crossover study of nifedipine versus placebo, with 7 patients undergoing finger pleth‐ysmography before and after sublingual nifedipine ad‐ministration. While receiving nifedipine, all patients reported decreased frequency and severity of attacks, and 4 of 5 had digital ulcer healing. Total finger blood flow increased in 5 of 6 patients after treatment with sublingual nifedipine. This preliminary study indicates that nifedipine may be a useful agent for treatment of digital vasospasm.
Application of monoclonal antibodies to the characterization of cells eluted from human articular cartilage. Expression of Ia Antigens in Certain Diseases and Identification of an 85‐kD Cell Surface Molecule Accumulated in the Pericellular Matrix
Authors: Burmester Gerd R. , Menche David , Merryman Parvin , Klein Michael , Winchester Robert
Published: February 2007

Ia antigens were strongly expressed on a considerable proportion of cells eluted from the damaged articular cartilages of certain individuals with osteoarthritis, rheumatoid arthritis, traumatic arthritis, or from osteochondromas. In contrast, la antigens were found on less than 1% of cells eluted from the more normal appearing cartilage of certain other patients with osteoarthritis, rheumatoid arthritis, osteonecrosis, femoral neck fractures, an enchondroma, and a chondrosarcoma. The cell preparations obtained from these latter individuals consisted of clusters of 1–4 chondrocytes embedded in a surrounding island of the pericellular matrix, whereas as in the samples that yielded lapositive cells, many chondrocytes were isolated as free cells without a surrounding matrix. The la antigens were detected primarily on this latter cell population, suggesting that their expression might indicate a chondrocyte activated to alter its surrounding matrix. The cytologic features of the la‐positive cells were indistin guishable from those of the la‐negative chondrocytes. All eluted cells lacked antigens of the monocyte, B cell, or T cell lineages. An antigen detected by the monoclonal reagent MϕR‐17 was identified on the membrane of large numbers of chondrocytes, both with and without the pericellular matrix, indicating that the matrix in these preparations was permeable to molecules the size of antibodies. A monoclonal antibody 83c2, demonstrated to react with an 85‐kD surface molecule expressed on certain fibroblastoid and other cells, detected an antigen present on the majority of normal chondrocytes and in varying percentages of cartilage cells obtained from patients with osteoarthritis or rheumatoid arthritis. Of special interest, the periphery of the pericellular matrix was stained by this reagent in an irregularly distributed ring‐like zone, suggesting that the 83c2 molecule was shed into the matrix from the cell surface and accumulated in these areas.
Lymphocyte infiltrations of the gastric mucosa in sjöugren's syndrome. An Immunoperoxidase Study Using Monoclonal Antibodies in the Avidin‐Biotin‐Peroxidase Method
Authors: Kilpi Anu , Bergroth Ville , Konttinen Yrjö T. , Maury C. P. J. , Reitamo Sakari , Wegelius Otto
Published: February 2007

Biopsy specimens of the gastric mucosa from 7 patients with primary or secondary Sjögren's syndrome were studied using the immunohistochemical avidin‐biotin‐peroxidase complex (ABC) method. Monoclonal antibodies (OKT series) were used as a primary layer to detect the surface antigens of various lymphocyte subsets in situ. Chronic inflammation with mononuclear cell infiltrates and/or glandular atrophy was seen in all 7 biopsy specimens. Immunohistochemical staining showed that the cell infiltrates consisted mainly of OKT3‐positive T lymphocytes. In 1 patient plasma cells predominated at the sites of inflammation. Most of the T cells were OKT4‐reactive lymphocytes, the OKT4/ OKT8 ratio varying between 3 and 7. These findings from the gastrie mucosa are in agreement with those obtained from the salivary glands of patients with Sjögren's syndrome and confirm the view that Sjögren's syndrome is a systemic disease affecting many organs, rather than a local disease restricted only to some exocrine glands.
Assessment of patient satisfaction in activities of daily living using a modified stanford health assessment questionnaire
Authors: Pincus Theodore , Summey Jane A. , Soraci Salvatore A. , Wallston Kenneth A. , Hummon Norman P.
Published: February 2007

Patient satisfaction in performing activities of daily living (ADL) was assessed by using a self‐administered questionnaire modified from the Stanford Health Assessment Questionnaire (HAQ). The HAQ includes questions to determine a patient's degree of difficulty and need for help and assistive devices in ADL. A modification of the HAQ (MHAQ) was developed to include questions concerning perceived patient satisfaction regarding the same ADL, along with perceived change in degree of difficulty. In order to add additional questions while maintaining the length of the questionnaire in a format suitable in routine care, the number of ADL included in the MHAQ was reduced from 20 to 8. Information regarding degree of difficulty derived from 8 questions in the MHAQ is comparable with that derived from 20 questions in the HAQ. The response of a patient that a specific activity is associated with difficulty in functional capacity was not inevitably associated with the absence of patient satisfaction; 43.7% of patients responding “with some difficulty” and 19.1% of patients responding “with much difficulty” expressed satisfaction with their functional capacity. A major determinant of expression of patient satisfaction was perceived change in difficulty: 81.4% of patients noting that their function was “less difficult now,” in contrast to 16.9% of patients responding “more difficult now,” expressed satisfaction. These studies suggest that data regarding patient satisfaction and perceived change in difficulty can be assessed to more completely characterize patients' functional status in ADL.
On the design of the phase iii drug trial
Authors: Hadler Nortin M. , Gillings Dennis B.
Published: February 2007

No new drug can be marketed in the United States unless a favorable risk–benefit ratio has been demonstrated to the satisfaction of the Food and Drug Administration. Inspection of the fashion in which this is accomplished with reference to rheumatoid arthritis reveals shortcomings in form and substance. Ten issues of design are isolated and options discussed. One design suggested, as a compromise, is 2 20‐center trials to supplant late Phase II and Phase III trials. At each center a single investigator evaluates at least a 10‐subject, highly selected cohort over 6 months in a 2‐drug or a 2‐drug plus placebo double‐blind format. The active drug versus active drug component of the program will be pivotal in analysis. Advantages include expedience, lower cost, effective detection of short‐term stratum‐specific toxicity and of investigator bias, and absence of compromise in beta error. With such data and with emphasis on the beta error, the risk–benefit judgment will be more meaningful. Formalized Phase IV monitoring is proposed to detect infrequent or delayed toxicities.
Mortality from polymyositis and dermatomyositis in the united states, 1968–1978
Authors: Hochberg Marc C. , Lopez‐Acuna Daniel , Gittelsohn Alan M.
Published: February 2007

Mortality from polymyositis and dermatomyositis was estimated between 1968 and 1978. Age‐specific average annual mortality rates showed unimodal distributions for all sex‐race groups. Synergistic interaction was demonstrated between female sex and nonwhite race, greatest mortality being in nonwhite females through age 74. Increases in annual death rates occurred among both white males and white females during the interval. Finally, the increase in mean age at death among all sex groups correlated with improved prognosis as well as decreased mortality among younger persons over time.
Labial salivary gland biopsy in sjögren's syndrome
Authors: Daniels Troy E.
Published: February 2007

Xerostomia is an unsatisfactory diagnostic criterion for the salivary component of Sjögren's syndrome (SS). To determine the diagnostic usefulness of the presence of focal sialadenitis in labial salivary gland (LSG) biopsy specimens, 362 patients suspected of having SS prospectively underwent a unique LSG biopsy procedure. The pattern and severity of LSG inflammation were compared with measurements of parotid flow rate, and the presence or absence of symptomatic xerostomia, major salivary gland enlargement, keratoconjunctivitis sicca (KCS), and other connective tissue diseases (CTD). LSG biopsy focus scores of >1 correlated more closely with the diagnoses of KCS alone and with KCS plus a CTD than did either reduced parotid flow rate or symptoms of xerostomia (P < 0.0005 and P < 0.05, respectively). Focal sialadenitis in an adequate LSG specimen is an objective criterion and a more disease‐specific feature of SS than xerostomia or any other feature of salivary disease. The salivary component of SS should be redefined as the presence of LSG focal sialadenitis.
Scleroderma‐like alterations in collagen metabolism occurring in the TSK (tight skin) mouse
Authors: Jimenez Sergio A. , Millan Alberto , Bashey Reza I.
Published: February 2007

Abnormalities in collagen metabolism similar to those occurring in the skin of patients with scleroderma have been demonstrated in the TSK (tight skin) mouse, a dominant mutant of the inbred B10.D2(58N)/SN mouse strain. These abnormalities, which consist of increased collagen content, accelerated biosynthesis, and increased extractibility of the newly synthesized molecules, suggest that the TSK mouse may be an excellent animal model for the study of the connective tissue alterations characteristic of scleroderma.
Immobilization ameliorates chemically‐induced articular cartilage damage
Authors: Williams James M. , Brandt Kenneth D.
Published: February 2007

We have previously shown that immobilization protects against the development of mechanically‐induced osteoarthritis following anterior cruciate ligament transection in dogs. Herein, we examine the effect of immobilization of the leg on the chemically‐induced degeneration of femoral articular cartilage caused by intraarticular injection of iodoacetate in guinea pigs. One week after the injection, cartilage from animals which were not immobilized exhibited a decrease in Safranin O staining and a 10–20% reduction in the number of chondrocytes. Three weeks after injection, cell death and loss of Safranin O staining had progressed, and surface fibrillation and osteophytes had developed. Articular cartilage from the contralateral (uninjected) knees of guinea pigs which received iodoacetate, and from knees of animals which were immobilized for 1 week but did not receive iodoacetate, was histologically and histochemically normal. However, specimens from 2 of 4 untreated knees which were immobilized for 3 weeks showed a reduction in Safranin O staining. Immobilization of the knee did not alter the loss of Safranin O staining seen after intraarticular iodoacetate injection, but did reduce the depletion of chondrocytes (P < 0.05). Furthermore, neither osteophytes nor fibrillation developed in any of the animals which were constrained after iodoacetate injection. Thus, immobilization was clearly protective in this model of chemically‐induced cartilage injury.
The risk of developing ankylosing spondylitis in HLA‐B27 positive individuals
Authors: Van Linden Sjef M. Der , Valkenburg Hans A. , Jongh Bartelt M. De , Cats Arnold
Published: February 2007

The present study was performed on 61 HLA‐B27 positive first‐degree relatives and 40 HLA‐B27 negative relatives of 20 HLA‐B27 positive probands with ankylosing spondylitis (AS). Of 24 HLA‐B27 positive relatives 45 years or older, 21% had AS and 38% sacroiliitis. The HLA‐B27 negative relatives did not have features of either disease. In the population study of 2,957 individuals 45 years or older, we found 5 cases of HLA‐B27 positive sacroiliitis (according to the New York criteria) and 3 of these fulfilled the New York criteria for diagnosis of AS. In 2 of these 3 individuals, the diagnosis was made on clinical grounds. The pheno‐type frequency of HLA‐B27 in this population is 7.8%, or about 230 HLA‐B27 positive individuals in this population sample. Since AS was found in only 3 individuals, 1.3% of the HLA‐B27 positive individuals in the population at large have AS; therefore, our data show that among individuals 45 years or older, 21% of HLA‐B27 positive relatives of HLA‐B27 positive AS patients have AS as compared with 1.3% of the HLA‐B27 positive individuals in the population at large. Thus, the risk for AS is 16 times greater in the HLA‐B27 positive relatives compared with HLA‐B27 positive individuals in the population at large. The discriminatory value of the New York criterion of history of pain or the presence of pain at the dorsolumbar junction or in the lumbar spine was analyzed in the population and family studies and was found to be too nonspecific.
Dysfunction of the sarcoplasmic reticulum in polymyositis
Authors: Kalovidouris Apostolos E.
Published: February 2007

The transport of calcium in vesicles of sarcoplasmic reticulum isolated from muscle specimens from 6 patients with early, active polymyositis and from 11 controls was examined. The time courses of calcium uptake and calcium‐dependent ATPase activity were measured simultaneously. Calcium uptake by sarcoplasmic reticulum vesicles from patients with polymyositis was 50% less than that by vesicles from controls (P < 0.001). In contrast, no difference in calcium‐dependent ATPase activity was noted between vesicles from patients with polymyositis and controls. The demonstrated defect may be important in the pathogenesis of muscle weakness in polymyositis.
Destructive Spondylarthropathy in Hemodialyzed Patients
Authors: Kuntz Daniel , Naveau Bertrand , Bardin Thomas , Drueke Tilman , Treves Richard , Dryll Antoine
Published: February 2007

Spinal radiologic lesions suggestive of destructive spondylarthropathy were found in 10 patients on long‐term hemodialysis. These lesions were characterized by severe narrowing of the intervertebral disc, associated with erosions and geodes of the adjacent vertebral plates without osteophytosis. In 9 of the 10 patients the lesions were located in the cervical spine, and in 1 patient, in the lumbar spine. Microbial spondylitis, degenerative disc disease, and destructive spondylarthropathy of calcium pyrophosphate dihydrate deposition disease were each, in turn, ruled out. The finding of apatite crystals by transmission electron microscopy in 1 disc specimen suggests that these crystals may be associated with destructive vertebral disc lesions in dialysis patients.
The Clinical Utility of the Lupus Band Test
Authors: Smith C. Douglas , Marino Cathy , Rothfield Naomi F.
Published: February 2007

In order to determine the clinical utility of the lupus band test, the presence of dermal‐epidermal junction (DEJ) deposits of IgG, IgM, IgA, C3, C4, C1q, and properdin were studied in biopsies of clinically normal deltoid area skin from 102 patients with systemic lupus erythematosus (SLE) and 151 patients with other rheumatic diseases. One or more proteins were detected at the DEJ in 72.6% (74 of 102) of patients with SLE and in 36.4% (55 of 151) of the other patients, yielding a specificity of 64% and a predictive value of 57%. The predictive value for the diagnosis of SLE was greatest with C4 (100%), properdin (91.3%), and IgA (86.2%) and lowest with IgM (59%). Specificity and predictive value increased with the number of proteins detected at the DEJ. The results suggest that more rigid criteria are required before diagnostic significance is attached to a positive result on the lupus band test.