bluuebunny/crossref_metadata_2025 huggingface dataset:
Synovial amyloidosis in patients undergoing long‐term hemodialysis
Authors: Bardin Thomas , Kuntz Daniel , Zingraff Johanna , Voisin Marie‐Catherine , Zelmar Alain , Lansaman Juliette
Published: February 2007

Synovial amyloid deposits were found in 18 patients with end‐stage renal failure due to various non‐amyloid nephropathies, who had been treated with long‐term, periodic hemodialysis (mean 116 months). All patients had carpal tunnel syndrome, which was bilateral in 14 of them; 4 patients also had finger flexor tenosynovitis. In 2 patients, destructive arthropathies required surgical replacement of the hip. Amyloid deposits were demonstrated by light microscopy in the synovium of the finger flexor tendon and/or transverse carpal ligament of all patients who had surgery for carpal tunnel syndrome, and in the synovium and capsula of the 2 surgically removed hips. Transmission electron microscopy of synovial samples from 6 patients demonstrated the characteristic fibrillar ultrastructure of amyloid deposits, the biochemical nature of which is still unknown. In addition, 9 patients had cystic radiolucencies of bone, which were interpreted as having resulted from local amyloid deposits, involving carpal bones, humeral heads, femoral heads, acetabula, or tibial plateaus. Our results show that amyloidosis is a frequent histologic finding in dialysis patients receiving surgical management of carpal tunnel syndrome, and that it can also be associated with cystic radiolucencies of bones and with destructive arthropathies.
Elevated C3 anaphylatoxin levels in synovial fluids from patients with rheumatoid arthritis
Authors: Moxley George , Ruddy Shaun
Published: February 2007

Because cleavage products of the third component of complement augment inflammation and may contribute to arthritis, we used a competitive inhibition radioimmunoassay to measure levels of the low molecular weight cleavage products of the third component of complement, C3a and C3adesArg, in 72 synovial fluid samples. Mean levels of C3a/C3adesArg were more than sevenfold higher in 41 patients who had rheumatoid arthritis than in 15 patients who had degenerative joint disease or 5 patients who had traumatic arthritis. Striking elevations were also present in 2 patients who had acute gouty arthritis. A calculation of the fraction of intraarticular C3 cleaved showed that the patients with rheumatoid arthritis had a mean C3 cleavage of 11.6 ± 11.0%, which was significantly higher than values of <1.5% for patients with degenerative joint disease or traumatic arthritis. In rheumatoid arthritis and gouty arthritis, specific immunoassay identified substantial quantities of the initial C3 cleavage fragments.
Ibuprofen disposition in obese individuals
Authors: Abernethy Darrell R. , Greenblatt David J.
Published: February 2007

Eleven obese subjects (weight 114 ± 11 kg, mean ± SE) and 11 age‐matched subjects with normal body weight (61 ± 3 kg) were given 600 mg of ibuprofen orally after an overnight fast. Peak ibuprofen concentration was significantly decreased in obese subjects (P < 0.02), although the time from administration to peak concentration was not different. Ibuprofen volume of distribution was increased in obese subjects, and this increased distribution correlated positively with body weight (r = 0.82; P < 0.001). Volume of distribution corrected for body weight was decreased in obese subjects, and this decrease correlated negatively with body weight. Ibuprofen clearance was also increased in obese subjects; the increase correlated positively with body weight (r = 0.81; P < 0.001). Since the independent variables, volume of distribution and clearance, were increased in parallel in the obese subjects, the dependent variable, elimination half‐life, was unchanged. Using mean values of distribution calculated from the 2 groups, ibuprofen distribution into body weight in excess of ideal body weight was found to be approximately 0.44 times as extensive as the distribution into ideal body weight. Furthermore, ibuprofen clearance increased in parallel with the volume of distribution and total body weight. Clinically, these data indicate that in obese patients, the ibuprofen dose may be increased without changing the dose interval, in order to achieve necessary plasma concentrations.
A quantitative test for xerostomia. The saxon test, an oral equivalent of the schirmer test
Authors: Kohler Peter F. , Winter Margaret E.
Published: February 2007

We describe a simple, reproducible, and low‐cost test for xerostomia, which involves chewing on a folded sterile sponge for 2 minutes. Saliva production is quantitated by weighing the sponge before and after chewing. Normal control subjects produced ≤ 2.75 gm of saliva in 2 minutes. Three of 32 consecutive, unselected outpatients in allergy–immunology clinics and 9 of 38 patients in rheumatology clinics had decreased saliva production, which was significantly different compared with controls (P < 0.01). The presence of sicca symptoms was highly correlated with quantitatively abnormal tear and saliva production, according to the results of the Saxon and Schirmer's tests.
Modulatory effect of sera from scleroderma patients on lymphocyte‐induced angiogenesis
Authors: Majewski Slawomir , Skopinska‐Rozewska Ewa , Jablonska Stefania , Polakowski Igor , Pawinska Maria , Marczak Maria , Szmurlo Andrzej
Published: February 2007

Sera from 22 patients with progressive systemic sclerosis were tested for the ability to modify the angiogeneic capability of normal human mononuclear cells. The sera from patients with acrosclerosis, including the abortive form (CREST syndrome: calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasias), markedly enhanced this capability compared with sera from both healthy donors and patients with severe acrosclerosis and diffuse scleroderma. The enhancing effect of sera from patients with acrosclerosis decreased and/or disappeared in cases where the patient's acrosclerosis was chronic and severe. Thus, this test may be of diagnostic value in distinguishing various subgroups of systemic sclerosis.
Computed tomography of the hindfoot with rheumatoid arthritis
Authors: Seltzer Steven E. , Weissman Barbara N. , Braunstein Ethan M. , Adams Douglass F. , Thomas William H.
Published: February 2007

We are performing direct, coronally oriented computed tomographic (CT) scans of the subtalar joint in patients with rheumatoid arthritis. To date, 16 examinations have been performed in 14 patients, 5 of whom were scheduled to undergo subtalar arthrodesis. Scans were 4–7‐mm thick and spanned the length of the subtalar joint. CT scans depicted and localized subtalar abnormalities clearly and precisely. On CT scans, patients showed a characteristic constellation of abnormalities including: soft tissue swelling, cartilage space narrowing, bony erosions, and a pes planovalgus alignment abnormality (consisting of increased heel valgus angulation, and a tendency toward flattening of the sustentaculum tali, and medial and downward slippage of the talar head). The clarity of the CT images facilitated the surgeon's pre‐ and postoperative evaluation. In particular, it was easy to plan the correction of the valgus deformity, anticipate any special requirements for reinforcing the articular surfaces, and assess the results of surgery. We conclude that coronal CT scans clearly demonstrate abnormalities and help the surgeon evaluate the rheumatoid hindfoot.
Adenosine triphosphate pyrophosphohydrolase and neutral inorganic pyrophosphatase in pathologic joint fluids. Elevated pyrophosphohydrolase in calcium pyrophosphate dihydrate crystal deposition disease
Authors: Rachow John W. , Ryan Lawrence M.
Published: February 2007

Adenosine triphosphate pyrophosphohydrolase (ATPPPH) and neutral inorganic pyrophosphatase activities were assayed in synovial fluids (SF) from 37 patients with a variety of arthropathies. ATPPPH activity was detected in all fluids, but was highest in patients with chronic chondrocalcinosis; its activity in patients with osteoarthritis was higher than that in patients with rheumatoid arthritis, gout, or pseudogout. ATPPPH activity correlated positively with SF pyrophosphate concentration and negatively with SF white blood cell count. Pyrophosphatase activity did not correlate with diagnosis, pyrophosphate level, or white blood cell count.
Temporal shifts from Sm to ribonucleoprotein reactivity in systemic lupus erythematosus
Authors: Fisher David E. , Reeves Westley H. , Wisniewolski Rosanne , Lahita Robert G. , Chiorazzi Nicholas
Published: February 2007

The Sm and RNP autoantibodies, found in the sera of many patients who have connective tissue diseases, recognize determinants on small nuclear ribonucleoprotein particles (snRNP). Numerous techniques have been used to distinguish between the subsets of snRNP proteins recognized by these two antibody systems. Using protein and snRNP immunoprecipitation, as well as a competitive enzyme‐linked immunosorbent assay, antibodies in the sera of Sm patients have been observed to include variable quantities of RNP‐like reactivity. To analyze changes in these autoantibodies, 2 patients with anti‐Sm antibodies were followed temporally. The autoantibodies in the sera of both patients underwent shifts from predominant Sm reactivity to predominant RNP reactivity. In 1 patient the shift occurred gradually over several years, while in the other the shift occurred within 8 weeks.
Detection of antibodies to small nuclear ribonucleoproteins and small cytoplasmic ribonucleoproteins using unlabeled cell extracts
Authors: Forman Mark S. , Nakamura Mary , Mimori Tsuneyo , Gelpi Carmen , Hardin John A.
Published: February 2007

RNA molecules immunoprecipitated with sera from patients who have rheumatic diseases can be readily detected in polyacrylamide gels by using ethidium bromide and silver stains. With these stains, we found that RNA patterns characteristic of a broad range of specific small nuclear ribonucleoproteins and small cytoplasmic ribonucleoproteins were recognizable. The stains correctly identified antibodies to ribonucleoproteins in 33 (92%) of 36 patient sera selected for study because of known antibody specificities. The silver stain method detected antibodies to ribonucleoproteins in 25 (76%) of 33 patients with classic systemic lupus erythematosus, a frequency that approximated the frequency observed in the Lerner‐Steitz assay, which is based on autoradiography. This approach considerably simplifies the latter radioimmunoassay with minimal loss of precision and sensitivity.
Partial characterization of synovial fluid nucleotide pyrophosphohydrolase
Authors: Rachow John W. , Ryan Lawrence M.
Published: February 2007

Synovial fluid adenosine triphosphate pyrophosphohydrolase, an enzyme which manifests increased activity in chondrocalcinosis and osteoarthritis, was partially characterized in synovial fluids from 41 patients who had a variety of arthropathies. Activity was found to be a soluble, heat labile, and divalent cationdependent nonspecific nucleotide pyrophosphohydrolase with pH optimum 9.0–9.5.
Effect of total lymphoid irradiation on levels of serum autoantibodies in systemic lupus erythematosus and in rheumatoid arthritis
Authors: Tanay Amir , Schiffman Gerald , Strober Samuel
Published: February 2007

The effects of total lymphoid irradiation (TLI) on serum levels of autoantibodies, and of antibodies to diphtheria toxoid, tetanus toxoid, and pneumococcal polysaccharide in patients with lupus nephritis were compared with those previously observed in rheumatoid arthritis (RA) patients. Baseline levels of antibodies to diphtheria toxoid and tetanus toxoid decreased significantly after TLI in patients with lupus and RA, but antibody levels to pneumococcal polysaccharide remained unchanged. After TLI, the levels of antinuclear and anti‐DNA antibodies were reduced significantly in lupus, but levels of rheumatoid factor, antinuclear, and antigranulocyte antibodies all tended to increase in RA.
Rheumatic diseases in an inuit population
Authors: Oen Kiem , Postl Brian , Chalmers Ian M. , Ling Norma , Schroeder Maria Louise , Baragar Fletcher D. , Martin Liam , Reed Martin , Major Paul
Published: February 2007

Prevalence and incidence rates for rheumatic diseases were found to be minimal among the Inuit people in the Keewatin District of the Northwest Territories, Canada. Patient identification was achieved by a review of medical records. All identified patients were interviewed and examined by a participating rheumatologist. Among women, the prevalence of rheumatoid arthritis, adjusted for age of the Manitoba population, was 1,822 per 100,000 and was comparable with that observed in other populations; no cases of rheumatoid arthritis in men were confirmed. The age‐adjusted prevalence of osteoarthritis, 1,219 per 100,000 in men and 2,144 per 100,000 in women, was apparently low. A moderately high incidence of Reiter's syndrome, 24.9 per 100,000, was found. The findings in children suggested a high frequency of seronegative spondylarthropathies (yearly incidence 60.1 per 100,000), although the adjusted yearly incidence for juvenile rheumatoid arthritis also appeared to be high, 23.6 per 100,000. The frequencies of HLA antigens in patient groups were compared with those found in 19 patients with musculoskeletal complaints but no rheumatic disease. Both HLA‐B27 and HLA‐DR4 appeared to be common in these controls, 36.8% and 63.2%, respectively. Nevertheless, there was a higher frequency of HLA‐B27 in patients with seronegative spondylarthropathies (87%) than in controls (P = 0.001). Because of the small numbers of patients who had rheumatoid arthritis, no associations with HLA were made for this condition. Although the findings suggest differences in the distribution of rheumatic diseases compared with those found in other populations, more complete studies are required to confirm these observations.
Articular cartilage proteoglycans from normal and osteoarthritic mice
Authors: Rostand Katherine S. , Baker John R. , Caterson Bruce , Christner James E.
Published: February 2007

Articular cartilage proteoglycans from an osteoarthritic mouse strain, STR/IN, were labeled in vivo with 35S‐sulfate and characterized with respect to extractability, ability to aggregate, size of monomer and glycosaminoglycan chains, sulfation of glycosaminoglycans, relative amounts of chondroitin‐4 sulfate and chondroitin‐6 sulfate, and link proteins. The proportion of 35S‐labeled proteoglycans extractable by 0.4M guanidine hydrochloride was the same in control and osteoarthritic animals. However, a greater proportion was extractable by 4M guanidine hydrochloride in the STR/IN animals as compared with the control mice. The ability of the 35S‐proteoglycans to aggregate was comparable in controls and osteoarthritic mice, as judged by their exclusion on Sepharose CL‐2B. Monomers from both controls and osteoarthritic animals eluted from Sepharose CL‐2B with a KAV of 0.47. Glycosaminoglycans from control and osteoarthritic animals eluted from Sepharose CL‐6B with a KAV of 0.63, and no differences in sulfation or chondroitin‐4 sulfate content were found. Aggregates were immunoprecipitated with link protein‐specific antiserum, and only link protein 2 was found in aggregates from control and osteoarthritic animals.
Naturally occurring antibody response to DNA is associated with the response to retroviral gp70 in autoimmune New Zealand mice
Authors: Shirai Toshikazu , Ohta Kiyoe , Kohno Atsushi , Furukawa Fukumi , Yoshida Haruyoshi , Maruyama Naoki , Hirose Sachiko
Published: February 2007

The spontaneous occurrence of retroviral gp70 immune complexes (ICs) in the blood of autoimmune New Zealand Black and New (Zealand black × New Zealand white)F1 ([NZB × NZW]F1) mice is determined by a single dominant locus of the NZB strain (provisionally designated Agp‐1). A combined effect of 2 unlinked dominant NZB loci (Ads‐1 and Ads‐2) is required for the production of anti‐double‐stranded DNA (anti‐dsDNA) antibodies in these mice. The present genetic studies using (NZB × NZW)F1 × NZW backcross mice and their second through fourth generation progeny revealed that Agp‐1 and Ads‐1 exist in common or are closely linked on chromosome 17 of NZB mice and are related to the occurrence of renal disease. The renal disease and the serum level of both anti‐dsDNA antibodies and gp70 ICs were more intense in (NZB × NZW)F1 hybrids than in NZB mice, indicating the contribution of NZW genes. In (NZB × NZW)F1 mice, a single dominant locus from the NZW strain, Agp‐3, intensified the magnitude of gp70 IC formation, and a combined effect of 2 unlinked dominant loci from the NZW strain, Ads‐3 and Ads‐4, acted to increase the serum titers of antidsDNA antibodies. This study clearly indicates that the NZW loci Agp‐3 and Ads‐3 exist in common or are tightly linked on chromosome 17, are closely linked to the H‐2 complex, and are associated with the severity of renal disease in (NZB × NZW)F1 hybrids. Thus, it is likely that the abnormalities involved in the antibody response to gp70 are in part genetically related to the production of autoantibodies to dsDNA as well as to the occurrence of renal disease in NZB and (NZB × NZW)F1 mice.
A study of classification criteria for a diagnosis of juvenile rheumatoid arthritis
Authors: Cassidy J. T. , Levinson J. E. , Bass J. C. , Baum J. , Brewer E. J. , Fink C. W. , Hanson V. , Jacobs J. C. , Masi A. T. , Schaller J. G. , Fries J. F. , McShane D. , Young D.
Published: February 2007

Criteria for the classification of juvenile rheumatoid arthritis were analyzed in a detailed database of 250 children in order to assess the accuracy of diagnosis and validity of onset types and course subtypes. A number of conclusions have been derived from this study: 1) All definitions of the 1973 criteria for classification of juvenile rheumatoid arthritis should be retained. 2) The addition of onset types to the 1976 revision of the criteria has been validated. 3) The course of the disease after the onset period of 6 months is as important to the outcome of a group of children as is the onset type. 4) The current classification should be broadened to include the course subtypes.
Progressive multifocal leukoencephalopathy complicating systemic lupus erythematosus
Authors: Newton P. , Aldridge R. D. , Lessells A. M. , Best P. V.
Published: February 2007

Two patients with systemic lupus erythematosus who developed progressive multifocal leukoencephalopathy (PML) are described. Although the time of PML onset could not be determined with certainty, it probably developed after steroid‐induced immunosuppression. However, high‐dose corticosteroids appeared to produce transient amelioration in the neurologic disorder. We suggest that PML is an important addition to the list of conditions to be considered in the differential diagnosis when a patient with systemic lupus erythematosus develops neurologic abnormalities.
Effect of warfarin sodium therapy on excretion of 4‐carboxy‐L‐glutamic acid in scleroderma, dermatomyositis, and myositis ossificans progressiva
Authors: Moore Sterling E. , Jump Adrien A. , Smiley J. Donald
Published: February 2007

The effect of warfarin sodium on excretion of calcium, phosphorus, and 4‐carboxy‐L‐glutamic acid (Gla) was studied in 5 patients with ectopic calcification (2 with scleroderma, 1 with dermatomyositis, and 2 with myositis ossificans progressiva). Warfarin reduced urinary excretion of Gla in all patients, but no changes in calcium and phosphorus excretion or in objective parameters of calcinosis were observed during 6–36 months of treatment. Two patients experienced hemorrhagic complications during therapy, emphasizing a hazard of long‐term anticoagulation treatment. Since ectopic calcium deposits contain Gla‐rich protein, suppression of Gla synthesis by warfarin sodium over a longer period may prevent deposition and allow removal of existing calcinosis deposits.
Evaluation of arthritis self‐management courses led by laypersons and by professionals
Authors: Cohen Judith L. , van Houten Sauter Suzanne , Devellis Robert F. , DeVellis Brenda Mcevoy
Published: February 2007

We compared the relative effectiveness of 2 arthritis patient education interventions. One intervention was modeled after that developed by Lorig, whereas the other had similar content but used health professionals rather than laypersons as instructors. Both interventions resulted in an increase in patients' knowledge of arthritis and in their use of exercise compared with a control group that received no intervention. However, neither intervention was any more effective than nonintervention in lessening patients' pain, improving their functioning, enhancing social support systems, lessening their depression, or improving their health behaviors beyond that of exercise. No differences in outcome measures were found between groups led by professional instructors and those led by lay instructors.
Stimulation of neovascularization by human rheumatoid synovial tissue macrophages
Authors: Koch Alisa E. , Polverini Peter J. , Leibovich S. Joseph
Published: February 2007

Synovial tissue from patients with rheumatoid arthritis was enzymatically dissociated, and single cell suspensions were fractionated into subpopulations by centrifugation on continuous Percoll gradients. Five fractions (F1–F5) with densities of 0.991–0.998 gm/ml, 0.998–1.042 gm/ml, 1.042–1.062 gm/ml, 1.062–1.082 gm/ml, and 1.082–1.180 gm/ml, respectively, were prepared. F3 consistently contained the highest number of macrophages, while F2 and F4 contained substantially fewer macrophages. Macrophages present in F2, F3, and F4 were enriched by differential adherence to fibronectin‐coated collagen gels. These macrophageenriched cell preparations were found to be Fc and C3 positive, esterase positive, and peroxidase negative, to stain positively with anti‐HLA‐DR, anti‐Leu‐M3, OKM1, and OKM5 monoclonal antibodies, and to show characteristic features of macrophages by electron microscopy. Macrophages from F3 consistently induced neovascularization in rat corneas, while equal numbers of macrophages from F2 and F4 did not. Fibroblastic synovial cells and cells that did not adhere to fibronectin‐coated collagen gels did not induce neovascularization. Within the rheumatoid synovium, there appears to be a major subpopulation of macrophages capable of inducing neovascularization, a process vital to the development of the rheumatoid synovial pannus.
The development of disability in rheumatoid arthritis
Authors: Sherrer Yvonne S. , Bloch Daniel A. , Mitchell Donald M. , Young Donald Y. , Fries James F.
Published: February 2007

Six hundred eighty‐one consecutive patients with rheumatoid arthritis were followed for an average of 11.9 years to identify initial factors that predicted subsequent disability. of 39 potentially predictive variables obtained at study onset and studied by stepwise regression methods, age was found to be the most powerful single predictor of disability, followed by radiologic grade, sex, and initial functional class. The worst prognosis for disability was found in patients who were older women and who showed radiologic worsening and developed functional impairment early in the disease course. Both disability and radiologic progression of disease were found to develop most rapidly during the first years after disease onset and to assume a slow, nearly linear rate of increase after 10 years. Approximately 10% of patients did not develop significant disability. This study suggests that it is possible to identify, early in the disease course, those patients who are likely to develop severe disability, and that “disease‐modifying” therapy might well be initiated earlier in such patients and used consistently throughout the subsequent treatment.
Pulmonary hypertension in the crest syndrome variant of systemic sclerosis
Authors: Stupi Angela M. , Steen Virginia D. , Owens Gregory R. , Barnes E. Leon , Rodnan Gerald P. , Medsger Thomas A.
Published: February 2007

Pulmonary hypertension (PHT) occurred in 59 (9%) of 673 systemic sclerosis patients seen between 1963 and 1983. In 30 patients, all with the CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasias), the pulmonary hypertension was isolated, i.e., independent of other pulmonary or cardiac conditions. In 20 patients, isolated PHT was demonstrated by cardiac catheterization. All had normal or only mildly decreased lung volumes, and mild or no pulmonary interstitial fibrosis on chest roentgenogram. In comparison with 287 CREST syndrome patients without PHT, these 20 patients had markedly reduced diffusing capacity for carbon monoxide (DLco) (mean 39% of predicted normal). In 6 patients, the low DLco antedated clinical evidence of PHT by 1–6 years. At autopsy there was marked intimal fibrosis with hyalinization and smooth muscle hypertrophy in the small‐ and medium‐sized arteries, without significant parenchymal fibrosis or inflammation. Patients with isolated PHT did not respond favorably to vasodilators and had a very poor prognosis, with a 2‐year cumulative survival rate of 40%. A DLco < 45% of predicted in the absence of pulmonary interstitial fibrosis may be an important predictor of the subsequent development of isolated PHT.
Suppressive effect of Escherichia coli on adjuvant‐induced arthritis in germ‐free rats
Authors: Kohashi Osamu , Kohashi Yukiko , Takahashi Tokutaro , Ozawa Atsushi , Shigematsu Nobuaki
Published: February 2007

Our previous finding, that germ‐free F344 rats develop severe adjuvant‐induced arthritis, whereas specific pathogen–free rats develop mild disease, prompted us to investigate the role of bacterial flora in promoting the development of this disease. Germ‐free rats given Escherichia coli experienced disease suppression. Germ‐free rats treated with 3 strains of Lactobacillus experienced an enhancement of the disease, although it was not significant. Germ‐free rats treated with a combination of E coli and lactobacilli had disease suppression similar to that of E coli monoassociated rats. Thus, these findings suggest that E coli may play a dominant role in modulating the development of the disease in this particular strain of F344 rats, possibly through its lipopolysaccharide (as evidenced by positive results on limulus tests). These findings also suggest that microflora play an important role in modifying the development of joint disease.
D‐penicillamine–induced polymyositis in patients with rheumatoid arthritis
Authors: Takahashi Koki , Ogita Tadaatsu , Okudaira Hirokazu , Yoshinoya Sadayoshi , Yoshizawa Hisayoshi , Miyamoto Terumasa
Published: February 2007

Among 329 patients with rheumatoid arthritis treated with D‐penicillamine (DP), we found 4 cases of polymyositis. This incidence was significantly higher than the expected coincidence of rheumatoid arthritis and polymyositis/dermatomyositis or than the presumed frequency of this complication among patients treated with DP. In the present report, we summarize and discuss the clinical findings in DP‐induced polymyositis/dermatomyositis in our patients and others described in the literature.
Sjögren's syndrome. Proposed criteria for classification
Authors: Fox Robert I. , Robinson Charles A. , Curd John G. , Kozin Franklin , Howelly Francis V.
Published: February 2007

The term “Sjögren's syndrome” (SS) is frequently used to describe the occurrence of keratocon‐junctivis sicca and xerostomia in association with an autoimmune disorder. However, well‐defined criteria for the classification of SS have not been established, and this diagnosis is being applied to a wide spectrum of conditions, ranging from clear “autoimmune” disease in some patients, to sicca complaints without evidence of a systemic immune process in elderly patients. Here, we review the clinical and laboratory features of patients referred for evaluation of sicca symptoms. In particular, we emphasize the need for care in choosing the site for minor salivary gland biopsy, and we describe the histologic features that aid in the evaluation of these biopsy specimens. In an attempt to identify a population of patients whose conditions might have a common etiopathogenesis and, thus, a common treatment program, we propose the following criteria for a diagnosis of SS: 1) objective evidence of keratoconjunctivis sicca, as documented by rose bengal or fluorescein dye staining; 2) objective evidence of diminished salivary gland flow; 3) minor salivary gland biopsy, obtained through normal mucosa, with the specimen containing at least 4 evaluable salivary gland lobules, and having an average of at least 2 foci/4 mm2; 4) evidence of a systemic autoimmune process, as manifested by the presence of autoantibodies, such as rheumatoid factor and/or anti‐nuclear antibody. The diagnosis of “definite SS” would be made when all 4 criteria are met; the diagnosis of “possible SS” would be made when 3 criteria are present. Specific exclusions for this diagnosis are preexisting lymphoma, graft‐versus‐host disease, sarcoidosis, and acquired immunodeficiency disease. These proposed criteria are more restrictive than are those currently used for diagnosing SS. Future multicenter trials will be required to determine whether these criteria are generally useful. Finally, we emphasize the rheumatologist's role in selecting safe, inexpensive methods of objectively monitoring sicca complaints (i.e., kerato‐conjunctivitis sicca and parotid salivary flow) and in evaluating the results of minor salivary gland biopsy.
Rehabilitation for rheumatoid arthritis patients. A controlled trial
Authors: Spiegel Jane Sinden , Spiegel Timothy M. , Ward Nancy B. , Paulus Harold E. , Leake Barbara , Kane Robert L.
Published: February 2007

For 1 year we obtained questionnaire and clinical measures from 2 cohorts of rheumatoid arthritis patients: 49 experimental patients who were admitted for an average of 13 days to a university‐based rheumatology rehabilitation unit, and 43 control patients who received care from rheumatologists associated with another teaching hospital. At 1 year, after controlling for group differences, the experimental patients demonstrated significant (P < 0.05) improvement compared with control patients in several functional status, mental health, and disease activity measures.
Effects of lupus‐inducing drugs on the B to Z transition of synthetic DNA
Authors: Thomas T. J. , Messner Ronald P.
Published: February 2007

Five drugs associated with systemic lupus erythematosus were studied for their effect on the salt‐induced right‐handed (B) to left‐handed (Z) transition of poly(dG‐me5dC) · poly(dG‐me5dC). Using circular dichroism spectroscopy, procainamide and hydralazine were found to reduce the midpoint of B to Z transition from 0.8M NaCl to 0.5M NaCl and to increase the rate of this transition at 1M NaCl. Isoniazid and D‐penicillamine had less effect on the midpoint of transition and practically no effect on the kinetics. N‐acetyl procainamide (a structurally related control for procainamide) and L‐canavanine had no effect. Procainamide caused slight reduction in the helix‐coil transition (melting) temperature of calf thymus DNA. At a concentration of 1:1 (DNA phosphate: drug ratio), procainamide and hydralazine also caused the aggregation of calf thymus DNA. Since altered DNA conformations, such as Z‐DNA, are more immunogenic, these results suggest that the induction or stabilization of Z‐DNA by these drugs might be important in the pathogenesis of at least some cases of systemic lupus erythematosus.
Treatment of al amyloidosis with melphalan, prednisone, and colchicine
Authors: Benson Merrill D.
Published: February 2007

Seven patients with immunoglobulin type (AL) amyloidosis were treated with combination chemotherapy, including melphalan, prednisone, and colchicine. Two patients died within 5 months of the beginning of therapy. Both had amyloid cardiomyopathy. Five patients were alive 17–60 months after the start of therapy, and none had shown progression of disease. The most significant findings were the resolution of the nephrotic syndrome in 2 patients, and improvement of liver function, as demonstrated by excretion of indocyanine green, in 2 patients. These results are encouraging and support the need for further studies of this regimen of combination chemotherapy for patients with AL amyloidosis.
Survival, prognosis, and causes of death in rheumatoid arthritis
Authors: Mitchell Donald M. , Spitz Patricia W. , Young Donald Y. , Bloch Daniel A. , McShane Dennis J. , Fries James F.
Published: February 2007

The factors associated with mortality were examined in a prospective longitudinal study, over an average of 12 years, with 94% followup of patients diagnosed as having rheumatoid arthritis. Of 805 patients, 233 died during the period of the study. Survivorship of rheumatoid arthritis patients was approximately 50% less than that of population controls. Survivorship was decreased by the traditional demographic variables of greater age and male sex; however, a significant independent effect of variables reflecting disease severity (American Rheumatism Association functional class, rheumatoid factor titer, number of involved joints) was identified by multivariate analysis. Seventy‐nine excess deaths (i.e., those that would not have been expected in a control population) were due in part to disease‐related causes, to infections, and to gastrointestinal complications of therapy. Treatment with gold or prednisone did not seem to affect survivorship or cause of death, except for the clustering of deaths of patients with vasculitis within the prednisone group. Our findings indicate that rheumatoid arthritis, a chronic disabling disease, is also associated with a major decrease in survivorship.
Proliferative responses of mononuclear cells in Lyme disease. Reactivity to Borrelia burgdorferi antigens is greater in joint fluid than in blood
Authors: Sigal Leonard H. , Steere Allen C. , Freeman Daniel H. , Dwyer John M.
Published: February 2007

In 27 patients with early Lyme disease, the mean response of peripheral blood mononuclear cells (PBMC) to Lyme spirochetal Borrelia burgdorferi antigens (723 counts per minute) was similar to that of control subjects. During convalescence, 2–3 weeks later, the patients' mean response was significantly higher (2,075 cpm, P < 0.008). Compared with those with early disease, the PBMC of 22 patients with Lyme arthritis reacted even more to B burgdorferi (2,923 cpm, P < 0.0004), and, by far, the greatest response was in concomitantly obtained synovial fluid mononuclear cells (15,238 cpm, P < 0.001). The PBMC of patients with early Lyme disease reacted slightly less to phytohemagglutinin and pokeweed mitogen than those of normal control subjects, but patients with arthritis had greater than normal mitogen responses. In contrast, mitogen reactivity among synovial fluid cells was markedly decreased and correlated inversely with the response to antigen. Thus, in patients with Lyme disease, the antigen‐specific responses of mononuclear cells increase as the disease progresses, and in those with arthritis, the greatest reactivity to antigen is found in cells in the inflamed joint.
An animal model of antibody binding in cutaneous lupus
Authors: Lee Lela A. , Weston William L. , Krueger Gerald G. , Emam Mansoor , Reichlin Morris , Stevens James O. , Surbrugg Sandra K. , Vasil Adriana , Norris David A.
Published: February 2007

To study antibody binding in cutaneous lupus, we used human skin grafted onto nude mice. By immunofluorescence examination, mice injected with anti‐Ro (SS‐A) sera from subacute cutaneous or neonatal lupus erythematosus patients showed evidence of human IgG deposited in the skin, while mice injected with anti‐native DNA or normal sera did not. We present evidence that there is specific binding of anti‐Ro (SS‐A) antibodies to Ro (SS‐A) antigen in the skin, and we propose that these antibodies may be directly involved in cutaneous disease.
The relationship between ocular and articular disease activity in children with juvenile rheumatoid arthritis and associated uveitis
Authors: Rosenberg Alan M. , Oen Kiem G.
Published: February 2007

The onset patterns and disease courses of children with juvenile rheumatoid arthritis and uveitis were reviewed to establish correlations. Results indicated that although the disease activity of arthritis and uveitis may at times run a parallel course, it is more common for the activities to be independent.
Methotrexate therapy in juvenile rheumatoid arthritis: A retrospective study
Authors: Truckenbrodt H. , Häfner R.
Published: February 2007

Nineteen selected patients with severe, mostly systemic onset, juvenile rheumatoid arthritis were treated with methotrexate (MTX) for an average of 10.5 months. Twelve patients showed statistically significant improvement, as measured by the number of affected, swollen, tender, and functionally impaired joints, and by decreases in erythrocyte sedimentation rate and C‐reactive protein and an increase in hemoglobin level. Systemic manifestations improved in 6 of 8 patients. In 10 children receiving corticosteroids, the dosage was reduced. Seven patients did not respond to MTX therapy. Six of them showed an unchanged disease course, and 1 had a relapse after 4 months of MTX treatments. Probable side effects included gastrointestinal symptoms, elevated liver enzymes, and herpes zoster infection. MTX treatment should be considered for children with life‐threatening or severe disabling arthritis that is unresponsive to other therapy.
Formation of a synovial‐like membrane at the bone‐cement interface: Its role in bone resorption and implant loosening after total hip replacement
Authors: Goldring Steven R. , Jasty Murali , Roelke Merrilee S. , Rourke Carolyn M. , Bringhurst F. Richard , Harris William H.
Published: February 2007

Histologic evaluation of tissue surrounding the loosened components after joint replacement reveals the presence of a synovial‐like lining adjacent to the polymethyl methacrylate cement. The tissue is heavily infiltrated with particulate cement as well as polyethylene associated with a foreign body‐type giant cell reaction. That this tissue response may be responsible for the bone lysis associated with loosening is suggested by the demonstration of high prostaglandin E2 levels and enhanced bone resorbing activity in the tissue culture medium of fragments from this membrane.
Inhibition of excessive scleroderma fibroblast collagen production by recombinant γ‐interferon: Association with a coordinate decrease in types I and III procollagen messenger RNA levels
Authors: Rosenbloom Joel , Feldman George , Freundlich Bruce , Jimenez Sergio A.
Published: February 2007

The effects of recombinant γ‐interferon (rec γ‐IFN) on collagen production by confluent monolayer cultures of progressive systemic sclerosis (PSS) dermal fibroblasts were studied. Five cell lines obtained from patients with rapidly progressive disease of recent onset were examined. All PSS fibroblast cell lines exhibited increased collagen production when compared with normal skin cell lines. It was found that rec γ‐IFN caused potent inhibition of PSS fibroblast collagen production in a concentration‐dependent manner. Greater than 50% inhibition was observed with as little as 50 antiviral units/ml, and maximal effects were attained at a concentration of 500 units/ml. The rec γ‐IFN caused reproducible inhibition of collagen production by the 5 PSS fibroblast cell lines, ranging from 58.9% to 85.6% of control values. Measurement of type I and type III procollagen messenger RNA (mRNA) levels with specific complementary DNA probes demonstrated a coordinate reduction of >60% in mRNA for both transcripts in rec γ‐IFN‐treated cells, compared with control cells. These findings indicate that rec γ‐IFN can modulate the excessive collagen biosynthesis characteristic of PSS fibroblasts and that this effect can be explained largely by the γ‐IFN‐mediated decrease in specific collagen mRNAs.
Treatment of Felty's syndrome with low‐dose oral methotrexate
Authors: Allen Lisa Sherbin , Groff Gerald
Published: February 2007

Recent studies of methotrexate in the treatment of patients with rheumatoid arthritis have precluded patients with neutropenia. We present a patient with rheumatoid arthritis complicated by severe neutropenia and recurrent infections, who was treated with low‐dose methotrexate, orally, for 30 months. The patient experienced symptomatic improvement and a significant increase in granulocyte count, and the dosage of steroids was reduced. Low‐dose oral methotrexate may be a therapeutic option in select patients with Felty's syndrome.
Adult‐type osteopetrosis presenting as carpal tunnel syndrome
Authors: Rakic Milovan , Elhosseiny Abdelmonen , Ramadan Farouk , Iyer Radha , Howard Rudolph G. , Gross Ludwig
Published: February 2007

We describe a patient with adult‐type osteopetrosis presenting as carpal tunnel syndrome. Radiographs demonstrated sclerosis of the carpal bones, bone biopsy revealed wide bone spicules containing areas of cartilage, and electrophysiologic studies confirmed the diagnosis of median nerve entrapment in the carpal tunnel. Any condition which alters the size or shape of the carpal canal or its contents may result in median nerve compression.
Characterization and functional studies of rheumatoid synovial mast cells: Activation by secretagogues, anti‐IgE, and a histamine‐releasing lymphokine
Authors: Gruber Barry , Poznansky Mark , Boss Eleonore , Partin Jacqueline , Gorevic Peter , Kaplan Allen P.
Published: February 2007

Microscopic analysis of synovial specimens from 35 patients with rheumatoid arthritis (RA) and 7 patients with osteoarthritis revealed mast cell hyperplasia in perivascular regions, in fibrous interstitial areas, and clustered around the periphery of lymphoid aggregates. Metachromatic staining, immunofluorescence studies, and ultrastructural analysis revealed a single population of connective tissue‐type mast cells with surface IgE receptors. Total extractable histamine of synovial tissue was 4.15 ± 2.30 μg/gm (n = 8) for RA synovium and 0.53 ± 0.23 μg/gm (n = 7) for OA synovium. Mast cell secretion was assessed and specific release of histamine from RA synovial mast cells was observed following stimulation with anti‐IgE (32.3%), compound 48/80 (40.1%), calcium ionophore A23187 (25.2%), and a partially purified lymphokine with histamine‐releasing activity (23.9%).
Mast cell numbers and histamine levels in synovial fluids from patients with diverse arthritides
Authors: Malone Daniel G. , Irani Anne‐Marie , Schwartz Lawrence B. , Barrett Kim E. , Metcalfe Dean D.
Published: February 2007

Thirty‐five synovial fluid (SF) specimens were examined for the presence of mast cells and for their histamine content. Mast cells were seen in SF cells from 27 of 35 fluids, and histamine was measurable in 19 of 34. There was a strong correlation between mast cell number and histamine content. No consistent relationship was found between either the mast cell number or histamine level and the patients' diagnoses, except that the 2 patients with systemic mastocytosis had markedly elevated values for both SF mast cell number and histamine content. SF mast cells from one of the mastocytosis patients were studied for histamine release; significant amounts of histamine were released upon exposure to anti‐human IgE, but not compound 48/80. Thus, mast cells similar to those present in connective tissue are frequently present in SF in numbers which correlate with SF histamine levels. These mast cells contain active proteases and are capable of degranulation. Mast cells were consistently present in large numbers in the SF of patients with systemic mastocytosis, but their numbers were highly variable in fluids of patients with other diseases.
Psoriatic arthritis affecting the manubriosternal join
Authors: Becker Nancy J. , De Smet Arthur A. , Cathcart‐Rake William , Stechschulte Daniel J.
Published: February 2007

Manubriosternal synovitis in the absence of peripheral arthritis or spondylitis, in a patient with pustular psoriasis, is described. The radiologic changes noted on lateral chest radiograph consisted of erosions, sclerosis, and joint space widening. This coincidence of manubriosternal joint arthritis and pustular psoriasis is unusual in western populations.
Idiopathic familial acroosteolysis: Histomorphometric study of bone and literature review of the Hajdu‐Cheney syndrome
Authors: Udell James , Schumacher H. Ralph , Kaplan Frederick , Fallon Michael D.
Published: February 2007

We describe a 27‐year‐old man with familial acroosteolysis involving 9 fingers. Bone biopsy of an affected digit showed osteolysis with no tetracycline deposition, rare osteoclasts, increased vascularity, and numerous mast cells. In contrast, the iliac crest bone showed active bone remodeling and normal double‐tetracycline labeling. We believe mast cells deserve further study as possible factors in this form of localized osteolysis.
Development of criteria for the classification and reporting of osteoarthritis: Classification of osteoarthritis of the knee
Authors: Altman R. , Asch E. , Bloch D. , Bole G. , Borenstein D. , Brandt K. , Christy W. , Cooke T. D. , Greenwald R. , Hochberg M. , Howell D. , Kaplan D. , Koopman W. , Longley S. , Mankin H. , McShane D. J. , Medsger T. , Meenan R. , Mikkelsen W. , Moskowitz R. , Murphy W. , Rothschild B. , Segal M. , Sokoloff L. , Wolfe F.
Published: February 2007

For the purposes of classification, it should be specified whether osteoarthritis (OA) of the knee is of unknown origin (idiopathic, primary) or is related to a known medical condition or event (secondary). Clinical criteria for the classification of idiopathic OA of the knee were developed through a multicenter study group. Comparison diagnoses included rheumatoid arthritis and other painful conditions of the knee, exclusive of referred or paraarticular pain. Variables from the medical history, physical examination, laboratory tests, and radiographs were used to develop sets of criteria that serve different investigative purposes. In contrast to prior criteria, these proposed criteria utilize classification trees, or algorithms.
A strong association between null alleles at the C4A locus in the major histocompatibility complex and systemic sclerosis
Authors: Briggs David C. , Welsh Ken , Pereira R. Scott , Black Carol M.
Published: February 2007

Allotyping of the major histocompatibility complex (MHC)‐linked complement component C4 has revealed a strong association of the null allele, C4A*Q0, with systemic sclerosis (SSc). Sixty‐four percent of patients with SSc carried the C4A*Q0 allele, compared with 17% of the control group. Twenty‐five patients and their families were typed for HLA antigens (A, B, Cw, and DR) and the MHC‐linked complement components C4 and factor B to identify haplotypes in the MHC linkage group and C4 null alleles. Strong allelic association of HLA‐B8 and DR3 with C4A*Q0 probably explains the previously reported association of SSc with the extended haplotype carrying HLA‐B8 and DR3. Ninety‐two percent of the patients had either C4A*Q0 or DR5; 31% of the controls had either C4A*Q0 or DR5.
Assessment of the potential pathogenicity of type ii collagen autoantibodies in patients with rheumatoid arthritis. Evidence of restricted IgG3 subclass expression and activation of complement C5 to C5a
Authors: Watson W. C. , Cremer M. A. , Wooley P. H. , Townes A. S.
Published: February 2007

IgG subclass analysis by enzyme‐linked im‐munosorbent assay of the autoantibody to native type II collagen, detected in 9 patients with classic or definite rheumatoid arthritis, demonstrated a predominance of IgG3 autoantibody. Gm allotyping revealed no obvious association with a particular phenotype. In comparative studies, IgG antibodies to the capsular polysaccharides of pneumococci and tetanus toxoid protein in these same patients consisted predominantly of IgG2 and IgG4. Purified type II collagen autoantibody from 3 of these patients activated complement C5 to C5a when bound to human cartilage in vitro, as measured by radioim‐munoassay. These results represent direct evidence of a potential pathogenic role for this autoantibody in rheumatoid arthritis.