Murray B. Urowitz

Felty's syndrome. Clinical and serological analysis of 34 cases.

Review of 34 cases of Feltys syndrome showed this to be a form of super rheumatoid disease because of the severity of joint disease, the prominence of extra-articular features and the remarkable incidence of infection. The response to splenectomy in these 34 patients was shown by a return towards normal of peripheral blood abnormalities and a decrease in bone marrow granulopoiesis. Although some patients remained free of infection after splenectomy, others have continued to have infections despite the return of white blood cell counts to normal levels. Although splenectomy and subsequent increase in white blood cell levels may be beneficial, our experience suggests that other factors are important in the susceptibility to infection of Feltys syndrome patients. Moreover, we think that splenectomy may have been instrumental in the fatal infection of one of our patients.

Peripheral vascular disease in patients with systemic lupus erythematosus.

Patients with systemic lupus erythematosus may develop premature atherosclerosis, notably coronary artery disease. A group of 10 patients with peripheral vascular disease presenting with intermittent claudication or gangrene were studied from a group of 563 patients followed prospectively at the Wellesley Hospital Lupus Clinic. These 10 patients were compared with the next lupus clinic patient matched for age and sex, with respect to demographic characteristics and risk factors. The patients and controls did not differ significantly in lupus activity criteria count, partial thromboplastin time, the number with antibody to cardiolipin, number receiving steroids or mean steroid dose, family history of atherosclerosis, hyperlipidaemia, smoking, hypertension or use of oral contraceptives. The risk factors for developing peripheral vascular disease were a longer duration of systemic lupus erythematosus and a longer duration of use of steroids. Eight of the 10 patients had coexistent coronary artery disease or transient ischaemic attack.

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.

Autoantibodies and neuropsychiatric events at the time of systemic lupus erythematosus diagnosis

OBJECTIVEnTo examine, in an inception cohort of systemic lupus erythematosus (SLE) patients, the association between neuropsychiatric (NP) events and anti-ribosomal P (anti-P), antiphospholipid (lupus anticoagulant [LAC], anticardiolipin), anti-beta2-glycoprotein I, and anti-NR2 glutamate receptor antibodies.nnnMETHODSnNP events were identified using the American College of Rheumatology case definitions and clustered into central/peripheral and diffuse/focal events. Attribution of NP events to SLE was determined using decision rules of differing stringency. Autoantibodies were measured without knowledge of NP events or their attribution.nnnRESULTSnFour hundred twelve patients were studied (87.4% female; mean +/- SD age 34.9 +/- 13.5 years, mean +/- SD disease duration 5.0 +/- 4.2 months). There were 214 NP events in 133 patients (32.3%). The proportion of NP events attributed to SLE varied from 15% to 36%. There was no association between autoantibodies and NP events overall. However, the frequency of anti-P antibodies in patients with central NP events attributed to SLE was 4 of 20 (20%), versus 3 of 107 (2.8%) in patients with other NP events and 24 of 279 (8.6%) in those with no NP events (P = 0.04). Among patients with diffuse NP events, 3 of 11 had anti-P antibodies (27%), compared with 4 of 111 patients with other NP events (3.6%) and 24 of 279 of those with no NP events (8.6%) (P = 0.02). Specific clinical-serologic associations were found between anti-P and psychosis attributed to SLE (P = 0.02) and between LAC and cerebrovascular disease attributed to SLE (P = 0.038). There was no significant association between other autoantibodies and NP events.nnnCONCLUSIONnClinically distinct NP events attributed to SLE and occurring around the time of diagnosis were found to be associated with anti-P antibodies and LAC. This suggests that there are different autoimmune pathogenetic mechanisms, although low sensitivity limits the clinical application of testing for these antibodies.

Atherosclerosis in Systemic Lupus Erythematosus â Epidemiology, Risk Factors, Subclinical Assessment and Future Study

Over the last 40 years, it has become established that patients with Systemic Lupus Erythematosus (SLE) have a greater risk of atherosclerosis and related events compared with the general population of similar age and sex. While the precise mechanisms that cause accelerated atherosclerosis in SLE remain undetermined, much evidence from inceptional and prospective longitudinal studies suggests a role for traditional cardiovascular disease and lupusspecific risk factors, systemic inflammation and lupus-directed therapies. This review will summarize much of the important knowledge available in the medical literature on this topic.

Autoantibodies and Neuropsychiatric events at diagnosis of SLE: Results from an international inception cohort study

OBJECTIVEnTo examine, in an inception cohort of systemic lupus erythematosus (SLE) patients, the association between neuropsychiatric (NP) events and anti-ribosomal P (anti-P), antiphospholipid (lupus anticoagulant [LAC], anticardiolipin), anti-beta2-glycoprotein I, and anti-NR2 glutamate receptor antibodies.nnnMETHODSnNP events were identified using the American College of Rheumatology case definitions and clustered into central/peripheral and diffuse/focal events. Attribution of NP events to SLE was determined using decision rules of differing stringency. Autoantibodies were measured without knowledge of NP events or their attribution.nnnRESULTSnFour hundred twelve patients were studied (87.4% female; mean +/- SD age 34.9 +/- 13.5 years, mean +/- SD disease duration 5.0 +/- 4.2 months). There were 214 NP events in 133 patients (32.3%). The proportion of NP events attributed to SLE varied from 15% to 36%. There was no association between autoantibodies and NP events overall. However, the frequency of anti-P antibodies in patients with central NP events attributed to SLE was 4 of 20 (20%), versus 3 of 107 (2.8%) in patients with other NP events and 24 of 279 (8.6%) in those with no NP events (P = 0.04). Among patients with diffuse NP events, 3 of 11 had anti-P antibodies (27%), compared with 4 of 111 patients with other NP events (3.6%) and 24 of 279 of those with no NP events (8.6%) (P = 0.02). Specific clinical-serologic associations were found between anti-P and psychosis attributed to SLE (P = 0.02) and between LAC and cerebrovascular disease attributed to SLE (P = 0.038). There was no significant association between other autoantibodies and NP events.nnnCONCLUSIONnClinically distinct NP events attributed to SLE and occurring around the time of diagnosis were found to be associated with anti-P antibodies and LAC. This suggests that there are different autoimmune pathogenetic mechanisms, although low sensitivity limits the clinical application of testing for these antibodies.

So You Suspect Systemic Lupus Erythematosus: Which Test to Perform?

Although most physicians today would suspect a diagnosis of systemic lupus erythematosus (SLE) after a careful clinical evaluation, they will invariably depend on the laboratory for confirmation of the diagnosis. The most helpful tests used to confirm the diagnosis of systemic lupus erythematosus fall into three main categories: the hemogram, the acute phase reactants, and the immune factors (Table 1),