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Biomedical subjects

M B Urowitz

Publications and source records attributed to M B Urowitz.

At least 127 records · Page 7Linked to original sources

Haemostatic abnormalities in systemic lupus erythematosus.

Coagulation studies were performed in 112 consecutive patients with systemic lupus erythematosus (SLE). Abnormalities of haemostatic function occurred frequently and 96 abnormalities occurred in 64 of 112 (57 per cent) patients. Eighteen patients (16 per cent) had thrombocytopenia, 19 (16.9 per cent) had circulating anticoagulants and 24 had decreased antithrombin III levels. Abnormalities of fibrinogen were found in 28 patients (23 per cent), and abnormalities of platelet factor 3 and 4, indicating in vivo platelet activation occurred in seven patients. In 25 patients two or more abnormalities were detected simultaneously. No haemostatic abnormalities were detected in any of the 50 healthy volunteers who served as controls. Only one patient with thrombocytopenia had petechiae. None of the other patients, even those with multiple defects bled significantly, but several patients had vasculitis and/or phlebitis. There was no correlation between disease activity of SLE and the presence of haemostatic abnormalities, nor was there an association between these abnormalities and specific clinical haematologic manifestations.

Adult↗

Shrinking lung syndrome in SLE--a clinical pathologic study.

A patient with systemic lupus erythematosus (SLE) who developed shrinking lung syndrome during the course of her disease is described. Postmortem findings of diffuse diaphragmatic fibrosis support the hypothesis of an extrapulmonary restrictive etiology in this unusual condition.

Female↗

Liver disease in Felty's syndrome.

Eighteen patients with Felty's syndrome were examined prospectively for the presence of hepatic abnormalities. Twelve patients had abnormal liver histologic features: five with nodular regenerative hyperplasia and seven with portal fibrosis or abnormal lobular architecture. Only seven of the 12 had abnormal liver chemistry results. Four of the 12 had portal hypertension, and three bled from esophageal varices compared with one of six with normal histologic features. When patients with normal and abnormal liver histologic findings were compared, there was no difference in clinical, serologic, or extra-articular manifestations between the two groups, although there was a tendency for the patients with abnormal findings to have a higher incidence of vasculopathy. All patients with Felty's syndrome should be screened for hepatic abnormalities and portal hypertension as they have an increased likelihood of bleeding from esophageal varices.

Adult↗

Prolonged complete remission in previously severe SLE.

Of 160 patients with systemic lupus erythematosus followed up in a long-term prospective study 4 with previously severe disease are in complete remission and have required no therapy for a median time of 75 months. The 4 females all presented with systemic features and a typical butterfly rash prompting early diagnosis and treatment. All patients have had complete remission of clinical and laboratory features of disease, without maintenance suppressive therapy.

Adolescent↗

Long-term outcome in Felty's syndrome.

Felty's syndrome has again been shown to be a severe form of systemic rheumatoid disease characterised by severe joint involvement, many extra-articular features, and a high incidence of infection. In addition we have shown that splenectomy was not protective for infections and in fact may on occasion contributed to infection. Furthermore, although most patients had an increase in white blood cell count after splenectomy, 50% of patients without splenectomy showed a similar increase in white blood cell counts at follow-up. Mortality in Felty's syndrome was high, with infection being the main cause of death.

Aged↗

Long-term effects of azathioprine in rheumatoid arthritis.

Efficacy and safety of azathioprine in 'high' and 'low' dose regimens in rheumatoid arthritis (RA), both in short-term studies and in follow-up over 40 months, have previously been shown. In the present report, 36 patients with RA treated with azathioprine (group I) and 49 age-matched patients with RA (group II), were studied to detect potential early markers of malignancy. Chest x-rays were similar to both groups. One patient in group I had a positive PAP smear and was subsequently found to have uterine carcinoma. Alpha-fetoprotein was positive in one patient in group I and none in group II. CEA was negative in all patients in group I, but positive in seven in group II. On chromosomal analysis group I showed a greater frequency of breakage. Group I showed lower serum folates and a highly significant number of megaloblastic features in marrow aspirates. In group I seven tumours, three being malignant, occurred while taking azathioprine, and in group II six tumours, one malignant, were identified (p = 0.17). The apparent increased risk of malignancy previously suggested by others warrants further studies with larger populations and over a continuous longer period.

Adenocarcinoma↗

Central retinal vein occlusion and scleroderma: implications for sclerodermatous vascular disease.

A patient with scleroderma (progressive systemic sclerosis) who developed central retinal vein occlusion is described. The aetiology of this condition is discussed and the role of possible sclerodermatous vascular disease is highlighted. It is suggested that studies of fundal vasculature could be useful in the clinical assessment of sclerodermatous vascular disease.

Constriction, Pathologic↗

Radiographic features of the hand in diffuse idiopathic skeletal hyperostosis (DISH): comparison with normal subjects and acromegalic patients.

Radiographs of the hands of 25 men and 9 women with diffuse idiopathic skeletal hyperostosis (DISH) were compared with age- and sex-matched controls and acromegalic patients. Characteristics of DISH included broadening and "arrowheading" of the distal phalangeal tufts, enlarged sesamoid bones, increased cortical width of tubular bones, prominent enthesopathy in the proximal phalanges, exostoses (particularly at the metacarpophalangeal heads), and new bone in the joint capsule. There was no consistent change in soft-tissue measurements or cartilage width between DISH and control patients. Acromegaly could be differentiated from DISH by the relative lack of enthesopathy, marked soft-tissue changes, and increased cartilage width. Any metabolic factor responsible for the changes in DISH apparently acts primarily on the enthesis and bone but has little effect on soft-tissue or cartilage growth.

Acromegaly↗

Selective deficiency of the fourth component of complement in a patient with systemic lupus erythematosus (SLE): immunochemical and biological studies.

A 45-year-old female (J.B.) with clinically inactive systemic lupus erythematosus was devoid of haemolytic serum complement activity. Functional and immunoprecipitin assays showed that the complement defect was due to lack of the fourth component of complement (C4). The patient's serum and red cells were Chido-negative, Rodgers-negative. In J.B.'s serum there was marked impairment of opsonization of Staphylococcus aureus and of the formation of chemotactic activity in the presence of zymosan. The chemotactic defect was not due to the presence of inhibitory factors: the activity was increased by the addition of C4. These findings suggest that an intact classical pathway is necessary for the optimal generation of serum chemotactic factors by zymosan via the alternative pathway of the complement system.

Adolescent↗

Venous syndromes and pulmonary embolism in systemic lupus erythematosus.

Deep vein thrombophlebitis (DVT) and pulmonary emboli (PE) have been uncommonly reported manifestations of systemic lupus erythematosus (SLE). This may be partly due to their being masked by other more familiar lesions of the lungs and extremities in SLE. We have identified 17 patients with SLE from a population of 180 being followed up prospectively who had 21 attacks of DVT and/or PE. Of the total of 21 episodes the SLE was considered to be active in 14, inactive in 6, and variable in a patient with recurring phlebitis. The incidence of hyperlipidaemia, smoking history, and use of birth control medication or corticosteroids was not higher in these patients. These clinical findings thus constitute additional features of SLE occurring in about 9% of patients and may be significance for morbidity and mortality.

Adult↗

Serologically active clinically quiescent systemic lupus erythematosus: a discordance between clinical and serologic features.

The significance of abnormal serologic tests in systemic lupus erythematosus (SLE) in the absence of active clinical disease is unclear. In this report we describe a group of 14 patients with SLE in whom a discordance between clinical and serologic features was apparent. These patients had persistently positive lupus erythematosus preparations and antinuclear antibody tests, low serum complement levels and high levels of DNA binding. Their lymphocyte response to concanavalin A (Con A) mitogen was suppressed. They have been asymptomatic and have remained untreated for a mean of four and a quarter years.

Adult↗

Leucapheresis in severe rheumatoid arthritis.

Two patients with severe seropositive rheumatoid arthritis previously unresponsive to conventional therapy have been treated with leucapheresis. This technique involves continuous cell separation daily to remove primarily lymphocytes. Clinical improvement was recorded with the use of standard rheumatological measures of inflammation. It is concluded that leucapheresis may help in the management of severely active rheumatoid arthritis when conventional therapy has been unsuccessful.

Adult↗

Postdiarrheal arthropathy of Yersinia pseudotuberculosis.

Two patients with acute gastroenteritis in whom polyarthritis subsequently developed were found to have positive serologic results for Yersinia pseudotuberculosis. With resolution of the arthropathy the antibody titres decreased. While the patient without the histocompatibility antigen HLA-B27 had an acute, self-limited arthritis, the patient with this antigen had a more chronic arthritis. Serologic typing and stool culture for Y. pseudotuberculosis should be done in cases of postdysenteric arthritis.

Acute Disease↗