Search PubMed⌕ Search

Biomedical subjects

M B Urowitz

Publications and source records attributed to M B Urowitz.

At least 109 records · Page 6Linked to original sources
PubMed1986

Discordance between HLA-B27 and ankylosing spondylitis: a family investigation.

Forty-one members in 4 generations of a family were evaluated clinically for ankylosing spondylitis (AS). Thirty-five individuals were HLA typed. The propositus and 4 male siblings demonstrated AS. A sister had sacroiliitis. Three of the affected sibs shared the B27 containing haplotype, but the remaining 3 individuals lacked the B27 but shared the other maternal haplotype. Two other first degree relatives with B27 did not show evidence of either sacroiliitis or AS. Four members of the family had psoriasis, but did not all share a common haplotype. The lack of association of known HLA antigens with disease expression in this family suggests a role for a putative disease susceptibility gene for psoriasis in the development of B27 negative spondylitis and illustrates the difficulty with the diagnosis of B27 negative AS, since when the family was first studied, it presented as a family of "pure" AS and only after several years of followup did the first evidence of psoriasis appear.

Adolescent↗
PubMed1985

Myeloproliferative disorders in patients with rheumatoid arthritis treated with total body irradiation.

Four patients with refractory rheumatoid arthritis were treated with total body irradiation administered in two sittings, 300 to 400 rads to each half of the body. All four patients had taken antimetabolites prior to receiving total body irradiation, and two continued to use them after total body irradiation. Two patients had taken alkylating agents before, and one had used them after total body irradiation. All patients showed clinical improvement. However, in two patients myeloproliferative disorders developed: a myelodysplastic preleukemia at 40 months after total body irradiation in one and acute myelogenous leukemia at 25 months in the other. Total body irradiation differs from total nodal irradiation in the total dose of irradiation (300 to 400 rads versus 2,000 to 3,000), and in the duration of the therapy (two sittings versus treatment over several weeks to months). Furthermore, the patients in the total body irradiation study frequently used cytotoxic drugs before and/or after irradiation, whereas in one total nodal irradiation study, azathioprine (2 mg/kg per day or less) was permitted, but no other cytotoxic agents were allowed. Rheumatologists may therefore face a binding decision when deciding to treat a patient with rheumatoid arthritis with either a cytotoxic drug or irradiation.

Aged↗
PubMed1985

Discordance of skin and muscle involvement in dermatomyositis.

The skin manifestations of dermatomyositis infrequently occur without the myositis. A 24-year-old woman presented with concordance of the skin and muscle components of dermatomyositis, followed by a remission of the myositis with a persistence of significant rash for 17 years, finally presenting with a flare of both skin and muscle components together. Clinicians should be alert to the recurrence of an underlying myositis at any time.

Adult↗
PubMed1985

Mortality in systemic lupus erythematosus: the bimodal pattern revisited.

A review of 51 patients who died while enrolled in a long-term prospective study of systemic lupus erythematosus (SLE) revealed that active SLE may persist or reappear late in the course of the disease. Vascular events, especially atherosclerotic coronary artery disease, occurred frequently. Moderate to severe atherosclerosis was seen in patients who had died of any cause after a prolonged duration of the disease and often contributed significantly to death. Diffuse proliferative glomerulonephritis, CNS lupus and major infections were indications of poor prognosis particularly early in its course.

Adolescent↗
PubMed1985

Phospholipase A2 activity in sera and synovial fluids in rheumatoid arthritis and osteoarthritis. Its possible role as a proinflammatory enzyme.

Phospholipase A2 (PLA2) activity was found in the sera and synovial fluids (SF) in rheumatoid arthritis (RA) and osteoarthritis (OA). PLA2 activity in RA SF was 6158 +/- 549 (SEM) U/ml (n = 48) and in RA sera 554 +/- 175 U/ml (normal sera-115 +/- 12 U/ml). In OA SF PLA2 activity was 5069 +/- 542 U/ml (n = 28), and in OA sera 268 +/- 55 U/ml. There was no significant difference between SF PLA2 activity in RA and OA. PLA2 activity in SF did not correlate with muramidase (lysozyme), beta-glucuronidase, total protein or white cell count, which were all significantly higher in RA SF than OA. A positive correlation between PLA2 in SF and matched sera was found in both RA and OA. It may be concluded that significant elevation of extracellular PLA2 occurs in both RA and OA, especially in the SF. The fact that high PLA2 did not correlate with other enzymes such as lysozyme and beta-glucuronidase, which are usually high in RA and low in OA SF, may mean that the handling of PLA2 in the joint space is different from other enzymes.

Arthritis, Rheumatoid↗
PubMed1984

Systemic lupus erythematosus with paraproteinemia.

Nine patients (2.2%) in a group of 415 who were followed in a longitudinal prospective study of systemic lupus erythematosus (SLE) were found to have various monoclonal (M) proteins in their blood (IgG [6 patients], IgA [2 patients], IgM [1 patient]). No other findings compatible with plasmacytic dyscrasia were found. Bence Jones proteinuria was absent. Bone marrow aspirates and skeletal radiographs did not reveal any associated features of malignancy. Four of the 9 patients were under the age of 50. From the point of view of the M components, 3 groups emerged: transient (2 patients), persistently stable (6 patients), and increasing serum concentrations (1 patient). Using current measures of disease status, no correlation was apparent between the presence, type, and concentration of the M protein and the clinical and laboratory variables of lupus activity. Thus, M proteins were found in 2% of our SLE patients, but their relationship to the polyclonal B cell activation seen in this disorder, or perhaps to therapeutic modalities used in its treatment, remains to be elucidated.

Adult↗
PubMed1984

Selective C4 deficiency, systemic lupus erythematosus, and Whipple's disease.

A 45-year-old female with selective deficiency of C4 and systemic lupus erythematosus developed puzzling gastrointestinal and systemic symptoms in the last 6 months of her life. Extensive investigation of the gastrointestinal tract did not yield any diagnosis, and the patient died shortly afterwards. Autopsy revealed evidence of a typical Whipple's disease of the jejunum and lymph nodes. This association has not been previously described. The disease is reviewed with emphasis on its being an opportunistic infection in an immunosuppressed host with a complement deficiency and SLE.

Complement C4↗
PubMed1984

The liver in systemic lupus erythematosus.

A 12-month prospective study was undertaken to determine prevalence and causes of clinical and subclinical liver disease in 260 SLE patients and 100 controls, and to look for concordance between 'unexplained' enzyme elevations and SLE activity. Hepatic status was assessed clinically and by tests of liver function with additional tests where indicated. In 76 per cent of patients there were no clinical or laboratory abnormalities. Liver enzyme elevations occurred in 23 per cent. In 15 per cent there were identifiable causes, and in 8 per cent the elevations were 'unexplained', compared with none in the controls. Four patients had persistent 'unexplained' mild transaminase elevations. Liver tissue available from 14 patients revealed no serious lesions. Data on 156 patients with more than four assessments were analysed. In 12 of 15 patients with 'unexplained' transaminase elevations, changes in SGPT levels were concordant with SLE activity. This study suggests that subclinical liver disease is a manifestation of SLE.

Adolescent↗
PubMed1984

Acute leukemia in rheumatoid arthritis treated with cytotoxic agents.

Acute leukemia is described in two patients treated with cytotoxic agents for a destructive, seropositive rheumatoid arthritis. Both patients had received longterm azathioprine therapy. In addition, one patient had been treated with cyclophosphamide, the other with melphalan. Chromosomal abnormalities were noted in both patients. Studies in one patient included colony forming units, ferrokinetics, electron microscopy of bone marrow, and autopsy examination. All reports of acute leukemia associated with cytostatic drugs in the literature to date are reviewed and the possible mechanisms discussed. It is suggested that patients with rheumatoid arthritis treated with azathioprine and alkylating agents may have an increased risk of developing a therapy-related acute leukemia.

Acute Disease↗
PubMed1984

The lupus activity criteria count (LACC).

A single clinical and laboratory assessment from each of 50 randomly chosen patients with systemic lupus erythematosus followed in a prospective study were evaluated for disease activity by 3 individual rheumatologists. Of the 50 assessments, 24 were considered to be active, 12 possibly active and 14 inactive. The 38 assessments that were clearly active or inactive were then analyzed. Clusters of variables were chosen for clinical relevance and association with activity, and 7 highly associated variables were combined into the lupus activity criteria count. Analysis of these criteria in the 50 assessments revealed that the presence of any 2 correctly predicted active disease in 100% of cases. This activity criteria count was then validated using a second sample of 50 assessments.

Evaluation Studies as Topic↗
PubMed1983

67Gallium lung scans in progressive systemic sclerosis.

67Gallium lung scans were performed in 19 patients with progressive systemic sclerosis (scleroderma). Results were expressed quantitatively as the 67Gallium Uptake Index. The mean total pulmonary 67Gallium Uptake Index in patients was significantly higher than that in controls (41 versus 25), and 4 patients (21%) fell outside the normal range. There were no clinical or laboratory variables that correlated with the 56gallium uptake. Increased pulmonary 67gallium uptake in scleroderma may prove useful as an index of pulmonary disease activity.

Adolescent↗
PubMed1983

The significance of thrombocytopenia in systemic lupus erythematosus.

The significance of thrombocytopenia in systemic lupus erythematosus (SLE) is unclear. Some researchers have found it associated with severe disease, others with mild disease. Thrombocytopenia (platelets less than 100,000) occurred in 21 patients seen at an SLE clinic over 18 months. Prospective assessment of 19 (non-SLE causes excluded) revealed 2 distinct subgroups. Seven were thrombocytopenic only during severe multisystem flares. Twelve had chronic thrombocytopenia with intermittent mild flares in other systems. Serious bleeding was rare in both subgroups. It was concluded that thrombocytopenia clearly is not a prognostic indicator.

Acute Disease↗
PubMed1983

Systemic lupus erythematosus in males.

A study of 51 males being followed in the Wellesley Hospital Toronto SLE Clinic examined the questions of whether SLE in males was similar to that in females and whether affected males differ from unaffected males with respect to their maleness and sex hormone profile. Fifty of the men were phenotypically males, and one was known to have Klinefelter syndrome (karyotype 47 XXY). All had four or more 1982 revised criteria for SLE. Fifty females matched with respect to age and duration of disease were used as controls. In examining the spectrum of the disease, 21 clinical and laboratory manifestations were assessed. Although neurologic involvement, alopecia, and thrombocytopenia were less common and pleuritis more common in the males, none of these was statistically significant. Comparison of disease severity revealed only one statistically significant difference: the mean duration of corticosteroid usage was longer in the females. There was also a tendency for cytotoxic agents to be used more frequently in the females. It was thus concluded that spectrum and severity of the disease tended to be similar in males and females. The frequency of positive family histories for SLE and other autoimmune diseases was similar in males and females. The age of onset tended to be more evenly distributed in males than in females, with one quarter of the males diagnosed after the age of 50. HLA typing revealed increased frequencies of the B8 and DR3 antigens in the SLE males compared with normal controls, as had previously also been shown for SLE populations with a female preponderance.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1983

Systemic lupus erythematosus and diffuse soft tissue calcifications.

A 26-year-old white woman developed systemic lupus erythematosus (SLE) and extensive soft tissue calcification. Striking features of this patient related to severe complication of corticosteroids, including proximal muscle weakness and multiple areas of avascular necrosis. In addition to localized areas of erythematosus induration, the patient demonstrated yellowish papules on her legs. Biopsies of the former showed thickening of the dermis with extensive collagen deposition and focal deposits of calcium. The latter revealed eosinophilic degeneration of the subcutaneous fat.

Adult↗
PubMed1983

Spinal entheseal new bone formation: the early changes of spinal diffuse idiopathic skeletal hyperostosis.

Diffuse idiopathic skeletal hyperostosis is characterized by new bone growth at the point of insertion of ligaments and tendons to bone. We examined retrospectively the anatomical morphologic changes discernible at the insertion of spinal longitudinal ligamentous fibrous tissue to vertebral bodies. The earliest evidence of bone formation was in the "waist" of the vertebral body away from the intervertebral disc area. New bone arose along the insertion of the fibrous tissue to the anterior cortical surface of the vertebral body and progressed along the fibres at an angle to the cortical surface distinct from it until the advanced stages. With disc degeneration the 2 processes were distinct and separate. Degenerative disc disease occurred at the margin of the endplate of the vertebral body with associated changes in the disc itself. Entheseal ossification occurred remote from the margin of the intervertebral disc and remained distinct from the subjacent vertebral body as it followed the ligamentous tissue; fusion with the cortical surface of the subjacent vertebral body was only seen in the most advanced cases of disseminated idiopathic systemic hyperostosis.

Adult↗
PubMed1983

Effect on the human complement system of the major non-steroidal anti-inflammatory drugs: aspirin, indomethacin, phenylbutazone, oxyphenbutazone and sulindac.

The possibility that the major non-steroidal anti-inflammatory drugs may inhibit the complement system and thus ameliorate the acute pathological changes induced by immune complexes was investigated. Treatment of fresh human serum with indomethacin (IDM), sulindac (Su), phenylbutazone (Ph) and oxyphenbutazone (OPh) inhibited both the classical and alternative complement (C) pathway activities in a dose-dependent fashion with a 50% inhibition dose of 4.65, 1.0, 1.65 and 1.3 mg/ml respectively. Aspirin, on the other hand, had a comparatively weak anti-complementary activity. Su, Ph and OPh were shown to form complexes with C5, thereby inhibiting the interaction between C3b and C5 and the cleavage of the latter into phlogistic fragments.

Anti-Inflammatory Agents↗