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Biomedical subjects

S Longley

Publications and source records attributed to S Longley.

At least 19 recordsLinked to original sources
PubMed1990

Magnetic resonance imaging in patients with inflammatory arthritis of the knee.

Magnetic resonance imaging (MRI) permits visualization of anatomic structures not appreciated by conventional radiographic imaging and may quantify inflammatory disease and its progression with greater sensitivity than available techniques. We therefore compared MRI with clinical evaluation and with radiographic examination of 17 patients with inflammatory arthritis of the knee. We sought to determine anatomic integrity of bone, cartilage, menisci, and ligaments, and to quantify joint effusion and synovial proliferation. Patients studied had rheumatoid arthritis (10 patients), juvenile rheumatoid arthritis (4 patients), ankylosing spondylitis (1 patient), and monoarticular arthritis (2 patients). In all patients MRI revealed clinically important abnormalities not detected by physical or conventional radiographic exams. These included proliferative synovitis (13 patients), cartilage thinning (2 patients), cartilage erosion (8 patients), bone infarction (1 patient), meniscal injury (1 patient), and synovial invagination into bone (1 patient). Also MRI indicated inflammatory disease to be quantitatively greater than had been appreciated on clinical examination or routine X-ray studies--proliferative synovitis (12 patients), erosion (7 patients), effusion (8 patients), cartilage thinning (11 patients), and ligamentous/meniscal damage (1 patient). These findings led to reassessment of anatomic staging and influenced therapeutic decision for these patients. Thus MRI provides clinically important information about joint integrity and inflammatory disease, with a sensitivity and resolution considerably beyond conventional techniques.

Adult↗
PubMed1989

Profile of a meeting: how abstracts are written and reviewed.

We analyzed submissions to a recent scientific program to determine (1) how abstracts were reviewed and (2) what constituted a successful abstract. We found that (1) reviewers' gradings varied from 2-29%, in some instances differing significantly; (2) many (<74%) abstracts had inadequacies in form, title, introduction, aims, methods, results, and conclusions(collectively termed "content") or lacked numerical or statistical data; (3) accepted abstracts had fewer inadequacies and better "content"; and (4) abstract grades correlated closely with "content". The quality of preparation and of individual features of abstracts led to favorable review. This information is of potential value to scientists preparing and reviewing abstracts and planning programs.

Abstracting and Indexing↗
PubMed1988

Vasculitis associated with malignancy. Experience with 13 patients and literature review.

Vasculitis is a syndrome which may complicate certain infectious, rheumatic, and allergic diseases. We identified 13 patients, over the past 17 years, who had both vasculitis and lympho- or myeloproliferative disorders and relate their clinical, laboratory, histologic, and immunologic features, course, therapy, and outcome. Nine patients were male, 4 female; ages ranged from 28 to 82 years. Ten of 13 patients presented with cutaneous vasculitis antedating malignancy by an average of 10 months. Three of 13 developed cutaneous vasculitis after malignancy. A statistically significant association between cutaneous vasculitis and lympho- or myeloproliferative malignancies was noted when compared with all other tumors. Dermatologic manifestations included palpable purpura (5 patients), maculopapular eruptions (4), urticarial and petechial lesions (3), and ulcers (1). Hepatitis B surface antigen, Coombs antibodies, rheumatoid factor and antinuclear antibodies were not found. Serum cryoglobulins were detected in 3 patients; serum C3 and C4 were normal in 8 of 9 patients evaluated. Histologic examinations revealed necrotizing leukocytoclastic vasculitis with disruption of endothelial integrity, destruction of endothelium, and neutrophil infiltration. Occasional perivascular mononuclear cell invasion was also noted in 4 patients. Immunofluorescent staining for IgG, IgA, IgM, C3, and C4 was negative in all patients studied. Symptoms were, in general, poorly responsive to therapy, which included nonsteroidal antiinflammatory drugs, antihistamines, antiserotonin agents, and corticosteroids. Chemotherapy directed at the underlying malignancy was also generally ineffective, although the vasculitis appeared to lessen in severity. Vasculitis appeared to lessen in severity as bone marrow function deteriorated. Ten patients died, all as a direct result of their malignancy. We have described a unique clinical syndrome of lympho- and myeloproliferative disease presenting with small-vessel vasculitis. Recognition that rheumatic symptoms may reflect or antedate malignancy may permit early diagnosis, aggressive treatment, and elucidation of pathogenesis.

Adult↗
PubMed1988

'Rhupus' syndrome.

Occasionally patients with overlapping features of rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE), termed "rhupus," have been encountered. We wanted to ascertain the frequency of such patients and determine whether they represent a unique overlap syndrome. Of approximately 7000 new patients evaluated over 11 years, we identified six patients who had, on the average, 6.7 American Rheumatism Association criteria for RA and 4.2 criteria for SLE. Criteria for RA included chronic symmetric arthritis with morning stiffness (six patients); subcutaneous nodules (two patients); positive rheumatoid factors test (four patients); and radiologic erosions (four patients). The criteria for SLE included malar rash (three patients); discoid lupus erythematosus (two patients); biopsy-proved nephritis (one patient); photosensitivity (one patient); leukopenia/thrombocytopenia (four patients); positive antinuclear antibodies or lupus erythematosus cell test (six patients); hypocomplementemia (two patients); and abnormal results from skin biopsy (two patients). During observations of up to ten years, the conditions of three patients were stable or improved, one died, and two were unavailable for follow-up. Patients usually did not have conditions that evolved to classic rheumatic disease patterns. Rhupus was not common and did not occur more frequently (0.09% prevalence among our patients) than expected from chance concurrence of SLE and RA (calculated at 1.2%). These observations confirm that rhupus indeed exists as a syndrome manifested by patients sharing features of probable coincidental concurrence of RA and SLE, but not as a unique clinical pathologic or immunologic syndrome. Appreciation of these patients with rhupus is important since their therapy and outcome differ from those having RA or SLE alone.

Arthritis, Rheumatoid↗
PubMed1988

Diphosphonate bone scans in patients with polyarthralgias.

Early detection of inflammatory arthropathy is notoriously difficult with standard radiographic techniques. We therefore assessed bone turnover with technetium Tc 99m medronate in 16 patients with persistent polyarthralgias who had no clinical synovitis, normal radiographs, and nondiagnostic results from laboratory evaluations. Abnormal scans were found in 11 of 16; five were unremarkable. Scan abnormality corresponded with symptomatic joints (11 of 11 patients). These 11 patients had normal test results for rheumatoid factor, antinuclear antibody, and HLA-B27. Patients with abnormal scans were treated with nonsteroidal antiinflammatory drugs or analgesics (11 of 11), hydroxychloroquine sulfate (four), or gold salts (one), with improvement (nine of 11); patients with normal scans (five of five) were treated successfully with nonsteroidal antiinflammatory drugs or analgesics and reassurance. One patient with a normal scan developed sarcoidosis; one, hypermobility syndrome; and one, a viral syndrome. Two patients had no diagnosis. Abnormal technetium Tc 99m medronate scans in patients with previously undiagnosed polyarthralgias suggested inflammatory arthropathy and influenced management decisions with favorable therapeutic outcomes.

Adult↗
PubMed1987

Silicone and rheumatic diseases.

Silicone generally has been regarded as a biologically inert material. However, recent reports suggest that inflammatory responses to silicone occur. There is some experimental and clinical evidence of a direct inflammatory response to the presence of liquid or particulate silicone. These include granulomatous skin reaction to injected silicone, synovitis around silicone prosthetic joints, and lymphadenopathy proximal to silicone prostheses. There are case reports of systemic rheumatic disease following silicone prostheses, but no definitive proof of a direct relationship between silicone prostheses and systemic disease. The clinical features of the reported cases following breast augmentation include breast tenderness, axillary adenopathy, sclerodermatous skin changes, arthritis, Raynaud's phenomenon, rheumatoid factors, and ANAs. Prior epidemiologic evidence and the number and consistency of our own and others' clinical findings suggest that silicone may indeed be associated with inflammatory processes and rheumatic diseases.

Adult↗
PubMed1987

Technetium pyrophosphate muscle scans in inflammatory muscle disease.

Technetium-99M pyrophosphate (TcPYP) nuclear scans of extremities were performed on 15 patients at 10 minutes and 2 hours after isotope injection. Scans were carried out both to confirm the diagnosis of myositis and to direct subsequent muscle biopsy. Five of six patients with clinical features strongly suggestive of inflammatory muscle disease had positive scans. All muscle biopsies performed at areas of increased isotope uptake showed inflammatory muscle disease. All nine patients not suspected of active inflammatory muscle disease had negative scans. Two of these underwent muscle biopsy with negative results. Our observations suggest that TcPYP muscle scans may be useful both to confirm the clinical suspicion of inflammatory muscle disease and in directing the choice of site for muscle biopsy.

Biopsy↗
PubMed1987

Familial exercise-induced anaphylaxis.

We identified two siblings with exercise-induced anaphylaxis who share the HLA haplotype A3-B8-DR3 with their atopic father. The index case, a 16-year-old female, noted initial episodes at age 13. Intense pruritus, urticaria, facial edema, choking sensation, nausea, hypothermia, and collapse followed vigorous running but not swimming, cycling, racquetball, solar exposure, or cold exposure. Neither antihistamine, antiserotonin, anticholinergic nor epinephrine therapy was entirely effective or protective; only modification of running prevented episodes. Three similar episodes were noted at age 15 years by a brother who, now age 25, relates a 4-year history of seasonal rhinitis and exercise-related urticaria without anaphylactoid reaction. The remainder of the family (father, 47; mother, 46; brother, 22 years) does not have exercise intolerance. The father has allergic rhinitis; his nephew suffers exercise-induced urticaria without collapse. HLA typing revealed the father to be A1-B8-DR3, A3-B8-DR3; the symptomatic daughter to be A3-B8-DR3, A30-B5-DR8; and the symptomatic son to be A3-B8-DR3, A30-B5-DR8. The asymptomatic mother was A30-B5-DR8, A2-B7-DR5 and the asymptomatic son A1-B8-DR3, A30-B5-DR8. We describe exercise-induced anaphylaxis in a unique familial setting, perhaps linked to the HLA haplotype A3-B8-DR3.

Adolescent↗
PubMed1986

Is running associated with degenerative joint disease?

Little information is available regarding the long-term effects, if any, of running on the musculoskeletal system. We therefore compared the prevalence of degenerative joint disease among 17 male runners (mean age, 56 years; height, 180 cm [5 ft 11 in]; and weight, 73.02 kg [161 lb] with 18 male nonrunners (mean age, 60 years; height, 178 cm [5 ft 10 in]; and weight, 78 kg [171 lb]). Running subjects (53% marathoners) ran a mean of 44.8 km (28 miles)/wk for 12 years. Pain and swelling of hips, knees, ankles, and feet and other musculoskeletal complaints among runners were comparable with those among nonrunners. Radiologic examinations (for osteophytes, cartilage thickness, and grade of degeneration) also were without notable differences among groups. We did not find an increased prevalence of osteoarthritis among the runners. Our observations suggest, within the limits of our study, that long-duration, high-mileage running need not be associated with premature degenerative joint disease in the lower extremities.

Aged↗
PubMed1986

Paraneoplastic vasculitis. Unique syndrome of cutaneous angiitis and arthritis associated with myeloproliferative disorders.

Six patients are described whose myeloproliferative disorders were complicated by inflammation in small, predominantly cutaneous blood vessels. The clinical manifestations of the vasculitis included palpable purpura, urticaria, maculopapular lesions, and erythema multiforme. Vascular inflammation was confirmed by skin biopsy. Two patients experienced fleeting, asymmetrical nondestructive arthritis. Transient proteinuria complicated one case and was the only suggestion of visceral vasculitis. The clinical features of cutaneous vasculitis antedated bone marrow deterioration in four patients and diminished as bone marrow function worsened in all patients. Oral corticosteroids or chemotherapy for the underlying disorder inconsistently affected the clinical course of the cutaneous vasculitis. Myeloproliferative disorders should be considered among disorders that are complicated by inflammation in small blood vessels.

Adult↗
PubMed1985

Unrecognized staphylococcal pyarthrosis with rheumatoid arthritis.

Four patients whose rheumatoid arthritis (RA) was complicated by staphylococcal arthritis were identified. All patients had active, long-standing disease with destructive changes. Affected joints included hip (two patients), knee (one patient), and shoulder (one patient). Pain and loss of motion in the affected joint were prominent, but toxic features of pyogenic infections--hectic fever, chills, sweats, local warmth, or erythema--were conspicuously absent. Two patients had moderate fever and three patients had mild leukocytosis. No patient was leukopenic. When present, fever was attributed to infected decubiti or urinary tract infection and treated with antibiotics. Therapy with corticosteroids and nonsteroidal antiinflammatory drugs (NSAIDs) probably masked symptoms and delayed the correct diagnosis. Purulent synovial effusions were discovered serendipitously--during arthrography (knee), attempted Girdlestone procedure (hip), and aspiration prior to steroid injection (shoulder). Sepsis was included in the preoperative diagnoses only once (hip). Prior instrumentation (aspiration or injection) of the affected joint was not a feature in any patients, although one patient had undergone insertion of a knee prosthesis one year prior to sepsis. Infectious organisms were Staphylococcus aureus in three patients and Staphylococcus epidermidis in one. Severe sequelae ensued in three of four patients: death from recurrent sepsis (one patient), loss of prosthesis leading to knee arthrodesis (one patient), and protracted sepsis with additional pyarthrosis (one patient). The only patient to regain preseptic joint function (shoulder) had not been on long-standing corticosteroids. Pyarthrosis must be considered in RA patients with unusually painful or stiff joints even in the absence of toxic symptoms.

Adult↗
PubMed1985

Rheumatoid vasculitis: experience with 13 patients and review of the literature.

Rheumatoid vasculitis is an uncommon but potentially catastrophic complication of RA. There are few current extensive experiences and no consensus regarding the clinical, laboratory, histologic features, and management or prognosis of rheumatoid vasculitis. We therefore reviewed selected observations in 13 patients followed over the past decade and compared them with patients reported and with results of a survey of North American Rheumatologists. Our patients were seven men and six women (age, 33 to 70 years) who had had active RA for 4 to 36 years. They exhibited sensory neuropathy, mononeuritis multiplex, Felty syndrome, cutaneous lesions, leg ulcers, gangrene, anemia, leukocytosis, eosinophilia, high titers of RF, hypocomplementemia, and CICs or cryoglobulinemia approximately as frequently as other reported patients with rheumatoid vasculitis, but they displayed constitutional symptoms, subcutaneous nodules, ischemic changes, and proteinuria rather less consistently than in other series. These observations were not necessarily as expected by survey respondents. We, as in other series and suggested by survey respondents, tended to select penicillamine or cytotoxic drugs (or plasmapheresis) for patients with mononeuritis, gangrene, or leg ulcers, and nonsteroidal antiinflammatory drugs, antimalarials, gold, or penicillamine for sensory neuropathy or digital lesions. Four patients died, two deteriorated, and seven were stable or improved, a finding that was also similar to the experiences of others. Rheumatoid vasculitis is an uncommon, potentially catastrophic syndrome with varying clinico-pathologic features that have different prognostic implications and should be managed individually.

Adult↗
PubMed1985

Detection and quantitation of circulating immune complexes in arterial blood of patients with rheumatic disease.

We developed antigen-nonspecific enzyme-linked immunoassays (ELISA) to quantitate IgG-C3- and IgM-C3-containing circulating immune complexes (CIC) in venous and arterial blood from rheumatic disease patients. Standards were diethylaminoethyl (DEAE)-purified, heat-aggregated IgG incubated with fresh human serum (for IgG-C3 CIC) and IgM rheumatoid factor-rich serum incubated with reduced, alkylated IgG and then with fresh human serum (for IgM-IgG-C3 CIC). Venous serum and plasma IgG-C3 and IgM-C3 CIC correlated closely (P less than 0.01). Rheumatoid arthritis (RA) and systemic lupus erythematous (SLE) patients had elevated levels of venous IgM-C3 CIC (P less than 0.0001) but not IgG-C3 CIC; patients with vasculitis, inflammatory rheumatic diseases, or noninflammatory rheumatic diseases had mean values similar to normal individuals. Venous IgG-C3 and IgM-C3 CIC did not correlate. Paired venous and arterial samples from 16 rheumatic disease patients averaged comparable amounts of IgG-C3 and IgM-C3 CIC, respectively; venous and arterial IgM-C3 CIC levels in patients significantly exceeded normals (P less than 0.05). Venous and arterial IgG-C3 CIC levels correlated closely (P less than 0.01) as did venous and arterial IgM-C3 levels (P less than 0.05). Thus, arterial CIC offered no advantage over venous determinations for rheumatic disease patients. IgM-C3 CIC were elevated in patients with RA and SLE when IgG-C3 CIC were not. Ig isotype-specific CIC quantitation may be useful for certain rheumatic diseases.

Antigen-Antibody Complex↗
PubMed1985

IgM rheumatoid factor elaboration by blood, bone marrow, and synovial mononuclear cells in patients with rheumatoid arthritis.

IgM rheumatoid factor (RF) elaboration by rheumatoid arthritis (RA) synovial, bone marrow, and blood mononuclear cells (MNC) is reported. IgM RF was prepared from RF-positive sera by sequential euglobulin precipitation, Sephacryl S300 gel filtration, and IgG-Sepharose affinity chromatography. Purified material, which contained no detectable IgG or IgA, was used in an enzyme-linked immunosorbent assay (ELISA) to quantitate cellular elaboration of IgM RF. Excellent standard curves (r2 = 0.98) were obtained without nonspecific binding of samples or antisera to IgG-coated microtiter plates and without cross-reactivity of standards with antisera other than anti-IgM. We found RA blood MNC (11 patients) spontaneously averaged 15 ng/ml IgM RF (6% of total IgM produced), but elaborated 254 ng/ml IgM RF following pokeweed mitogen (PWM) stimulation (22 patients), exceeding that of 13 normal controls. Bone marrow MNC spontaneously (4 patients) produced 71 ng/ml IgM RF and secreted 78 ng/ml IgM RF with PWM stimulation (9 patients). In contrast synovial fluid MNC (5 patients) spontaneously elaborated 6652 ng/ml IgM RF, significantly (P less than 0.05) more than blood or bone marrow MNC; PWM-stimulated synovial fluid MNC (5 patients) produced 5472 ng/ml IgM RF. These observations confirm selective localization of activated, IgM RF-producing cells to the rheumatoid synovial space.

Antibody-Producing Cells↗
PubMed1985

Weber-Christian disease. Analysis of 15 cases and review of the literature.

We report 15 patients encountered over 13 years who presented with inflammation of subcutaneous fat and were given clinical and pathologic diagnoses of Weber--Christian disease (WCD). Prominent clinical features included female predominance, lower extremity nodules, fevers, arthritis/arthralgias, and myalgias. Notable laboratory features were elevated erythrocyte sedimentation rate, anemia, leukopenia, and hypocomplementemia, frequently with circulating 7S IgM or immune complexes at times of active symptoms. Histologic findings were lobular--together with frequent septal--panniculitis, fat-laden macrophages, variable cellular infiltrates, necrosis, and occasional vasculitis. Follow-up revealed the death of 2 patients and disease stabilization or improvement in 13 patients. Six patients developed features of other diseases (factitial disease, erythema nodosum, acute myelogenous leukemia, rheumatoid arthritis, systemic lupus erythematosus, and sarcoid) and a seventh may have had erythema induratum. We suggest that classic WCD, as originally described, reflects an increasingly recognized spectrum of panniculitides. These are syndromes of diverse etiology that share many clinical, inflammatory, and immunologic features.

Adult↗