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Biomedical subjects

E L Alexander

Publications and source records attributed to E L Alexander.

At least 37 records · Page 2Linked to original sources
PubMed1986

Primary Sjögren's syndrome with central nervous system disease mimicking multiple sclerosis.

Central nervous system involvement has occurred in approximately 20% of patients with primary Sjögren's syndrome evaluated at our institution. Characteristically, the neurologic dysfunction is multifocal, involving both the brain and spinal cord, and is recurrent over time. We present the features of 20 patients with primary Sjögren's syndrome and central nervous system involvement whose neurologic findings, evoked potential abnormalities, and cerebrospinal fluid profiles (elevated IgG indices, oligoclonal bands on agarose gel electrophoresis, and mild pleocytosis with reactive lymphoid cells) closely resembled those of multiple sclerosis. In fact, multiple sclerosis was considered the most likely diagnosis in each of these patients before diagnosis of Sjögren's syndrome, and each patient met criteria for definite multiple sclerosis. The clinical effects of corticosteroid treatment during episodes of acute neurologic dysfunction appeared to be beneficial in these patients.

Adult↗
PubMed1986

Reversible cold-induced abnormalities in myocardial perfusion and function in systemic sclerosis.

The effects of peripheral cold exposure on myocardial perfusion and function were studied in 13 patients with scleroderma without clinically evident myocardial disease. Ten patients had at least one transient, cold-induced, myocardial perfusion defect visualized by thallium-201 scintigraphy, and 12 had reversible, cold-induced, segmental left ventricular hypokinesis by two-dimensional echocardiography. The 10 patients with transient perfusion defects all had anatomically corresponding ventricular wall motion abnormalities. No one in either of two control groups (9 normal volunteers and 7 patients with chest pain and normal coronary arteriograms) had cold-induced abnormalities. This study is the first to show the simultaneous occurrence of cold-induced abnormalities in myocardial perfusion and function in patients with scleroderma. The results suggest that cold exposure in such patients may elicit transient reflex coronary vasoconstriction resulting in reversible myocardial ischemia and dysfunction. Chronic recurrent episodes of coronary spasm may lead to focal myocardial fibrosis.

Adult↗
PubMed1986

Central nervous system (CNS) manifestations of primary Sjögren's syndrome: an overview.

Central nervous system (CNS) complications occur in approximately 25% of patients with primary Sjögren's syndrome (CNS-SS) seen at our institution. We describe herein the clinical, neurodiagnostic, and immunopathologic features of CNS-SS. The neurologic manifestations can be protean and affect the entire neuroaxis. Characteristically, CNS-SS is multifocal, recurrent, and progressive resulting in cumulative neurologic impairment over time. In addition to specific neurologic deficits, more subtle abnormalities of personality and mentation may occur. Whereas, computed axial tomography of the head and cerebral angiography are relatively insensitive techniques in detecting abnormalities, brain magnetic resonance imaging shows multiple small areas of increased intensity in two thirds of CNS-SS patients. Electrophysiologic studies or cerebrospinal fluid analyses are abnormal in two thirds of CNS-SS patients. Preliminary histopathologic studies show several types of CNS inflammatory involvement suggesting an immunologically mediated autoimmune disorder.

Central Nervous System Diseases↗
PubMed1986

Immunopathologic mechanisms of inflammatory vascular disease in primary Sjögren's syndrome--a model.

Our laboratory has been studying the immunopathogenesis of inflammatory vascular disease (IVD) in patients with Sjögren's syndrome (SS). In parallel, we have developed the autoimmune strain of MRL/Mp mice as a model of IVD. Sjögren's syndrome patients and MRL/Mp mice both develop two main histopathologic types of IVD (i.e. mononuclear and neutrophilic) which have differential serologic associations with respect to serum autoantibodies and complement levels. We present evidence that mononuclear (lymphocytic) vasculitis may exist as a distinct form of vascular inflammation and also may transition into neutrophilic vasculitis. On the basis of these studies we propose a new model of lymphocyte initiated vascular inflammation as an alternative to classical immune complex mediated vasculitis.

Animals↗
PubMed1985

Current status of surgical therapy for deep vein thrombosis.

There is renewed interest in the use of operative thrombectomy in the management of acute DVT using the adjuncts of heparin infusion through an indwelling catheter and pneumatic segmental compression of the leg. In the absence of phlegmasia cerulea dolens, however, the indication for thrombectomy remains unresolved. For the postthrombotic syndrome, the most widely accepted procedure is the Palma femorofemoral cross-over bypass, which is indicated for relief of persistent unilateral iliac venous obstruction. Venous valvular incompetence is a much more challenging problem for which some limited success has been achieved by direct valvuloplasty, venous transposition, and autologous vein valve transplantation. For the most serious complication of venous thrombosis, pulmonary embolism, the evolution of mechanical devices has eliminated the need for direct approaches to the vena cava, and the long-term results with the Greenfield filter allow it to be placed in either an infrarenal or suprarenal position with assurance of long-term patency.

Acute Disease↗
PubMed1985

Two histopathologic types of inflammatory vascular disease in MRL/Mp autoimmune mice. Model for human vasculitis in connective tissue disease.

We have recently described 2 histopathologic types of inflammatory vascular disease (IVD) in patients with Sjögren's syndrome (SS): neutrophilic IVD (NIVD) and mononuclear IVD (MIVD). Autoimmune MRL/Mp mice, which have many features of SS, spontaneously develop IVD which is histopathologically indistinguishable from that observed in human SS patients. Both MRL/Mp-+/+ and MRL/Mp-lpr/lpr mice develop MIVD which evolves into NIVD and results in decreased survival; the transition to NIVD is accelerated by the lpr gene. The presence of the lpr gene on other genetic backgrounds does not result in a similar acceleration of IVD and associated decreased survival. Thus, the spontaneous autosomal recessive mutation lpr appears to modulate the development of IVD in a strain of mice with an underlying propensity for vasculitis. Based on our observations on IVD in SS patients and MRL/Mp mice, we propose a new model which may enhance our understanding of the immunopathogenesis of IVD in connective tissue disease.

Animals↗
PubMed1985

Two types of inflammatory vascular disease in Sjögren's syndrome. Differential association with seroreactivity to rheumatoid factor and antibodies to Ro (SS-A) and with hypocomplementemia.

Two types of inflammatory vascular disease (IVD) occur in Sjögren's syndrome: neutrophilic IVD (NIVD) and mononuclear IVD (MIVD). In 45 patients with Sjögren's syndrome, we examined the 2 types of IVD with respect to serologic associations. NIVD, unlike MIVD, was significantly associated with seroreactivity reflected by hyperglobulinemia (P = 0.01), rheumatoid factor (P = 0.002), antinuclear antibodies (P = 0.02), and antibodies to Ro (SS-A) (P = 0.00006), and with hypocomplementemia (P = 0.03). The differential association of the 2 types of IVD with serologic reactivity and hypocomplementemia suggests that there may be basic differences in the immunopathogenesis of these 2 forms of IVD in Sjögren's syndrome.

Adolescent↗
PubMed1985

Peripheral inflammatory vascular disease in Sjögren's syndrome. Association with nervous system complications.

Two histopathologic types of inflammatory vascular disease (IVD) occur in Sjögren's syndrome (SS): mononuclear IVD (MIVD) and neutrophilic IVD (NIVD). We describe 50 SS patients with IVD (30 with NIVD and 20 with MIVD). Thirty-three (66%) of the SS patients with biopsy-documented IVD had nervous system disease unattributable to other causes. Nineteen patients (58%) had involvement of both the central and peripheral nervous systems, while 9 had peripheral and 5 had central nervous system dysfunction alone. Patients with both histopathologic types of IVD were at risk for the development of nervous system abnormalities (57% of NIVD patients and 80% of MIVD patients). Indirect evidence is presented which suggests that IVD may play a role in the immunopathogenesis of nervous system disease, at least in a subset of SS patients.

Adolescent↗
PubMed1985

Posttraumatic thoracic aortic aneurysm associated with a diaphragmatic hernia.

The case of a patient with a chronic, posttraumatic descending thoracic aortic aneurysm and a large diaphragmatic hernia is presented. We repaired the diaphragm with Prolene mesh and externally grafted the aneurysm. To our knowledge, simultaneous repair of these two chronic injuries has not been reported. The merits of external grafting of aortic aneurysms are discussed.

Accidents, Traffic↗
PubMed1985

Neuropsychiatric dysfunction in primary Sjögren's syndrome.

Neuropsychiatric complications developed in 40 patients with primary Sjögren's syndrome, none of whom met American Rheumatologic Association criteria for systemic lupus erythematosus. Twenty-five patients had psychiatric abnormalities, the commonest of which were affective disturbances. Of 30 patients tested, 23 had an abnormal pattern in the Minnesota Multiphasic Personality Inventory, the commonest pattern being a "conversion V." In general, patients presented with hysteroid dysphoric features. Of 16 patients undergoing cognitive function testing, 7 showed mild memory impairment with attention and concentration deficits. On clinical evaluation, 27 patients had neurologic abnormalities unattributable to other causes (central and peripheral nervous system in 16 and 19 patients respectively). There was a significant correlation between psychiatric disturbances and neurologic dysfunction, suggesting a possible organic basis for psychiatric dysfunction. The diagnosis of primary Sjögren's syndrome should be considered in patients with unexplained neuropsychiatric illness.

Adolescent↗
PubMed1985

Ocular inflammation in autoimmune MRL/Mp mice.

Congenic mice of the MRL/Mp strain spontaneously develop an autoimmune connective tissue disease that shares immunologic and histopathologic features with the human disorders systemic lupus erythematosus, rheumatoid arthritis, and systemic vasculitis. The autoimmune disorder in these mice is markedly accelerated by the recessive gene lpr. Older MRL/Mp-lpr/lpr mice develop significant inflammatory ocular disease, including choroiditis, scleritis, and orbital vasculitis. Animals of both the MRL/Mp-+/+ and MRL/Mp-lpr/lpr substrains develop lacrimal gland inflammatory infiltrates. The MRL/Mp mouse provides a potential model for ocular inflammatory disease and for Sjögren's syndrome.

Animals↗
PubMed1984

Sjögren's syndrome. Influence of multiple HLA-D region alloantigens on clinical and serologic expression.

The relationships of HLA-DR and the newer DS (second D locus) B cell alloantigens (MB and MT) to the clinical and serologic expression of primary and secondary forms of Sjögren's syndrome (SS) were examined in 102 patients (86 whites and 16 blacks). Although HLA-DR3 was significantly increased in whites (25 of 50, 50%) and blacks (4 of 5, 80%) with primary SS compared with race-matched normal controls, it was not appreciably elevated in those with systemic lupus erythematosus (SLE)-SS, rheumatoid arthritis (RA)-SS, or connective tissue disease-SS. The MT2 specificity, however, was more strongly associated with primary SS (86% of whites and 100% of blacks) and also with SLE-SS and RA-SS compared with race-matched normal controls. Furthermore, MT2 was significantly increased in SLE-SS and RA-SS when compared with non-sicca SLE and RA controls. Although primary and secondary SS were most strongly associated with this DS specificity (MT2), the anti-Ro (SS-A) and anti-La (SS-B) antibody responses were more closely allied to DR antigens. HLA-DR3 was increased in anti-Ro positive patients, both whites and blacks, with primary SS (74%) and in total anti-Ro positive subjects (54%) compared with their anti-Ro negative counterparts (38% and 31%, respectively). Among DR3 negative patients, HLA-DR2 correlated with anti-Ro in both primary SS (83%) and in the total SS group (58%). Thus, 96% of Ro antibody positive patients with primary SS had DR3 and/or DR2, as did 80% of anti-Ro positive subjects in all categories.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1983

Congenic autoimmune murine models of central nervous system disease in connective tissue disorders.

Congenic mice of the MRL/Mp strain spontaneously develop an autoimmune connective tissue disease that shares immunological and histopathological features with systemic lupus erythematosus, rheumatoid arthritis, and Sjögren's syndrome. The autoimmune disorder in these mice is accelerated markedly by the recessive gene lpr. By 6 months of age, MRL/Mp-lpr/lpr mice developed prominent mononuclear cell infiltrates restricted to the choroid plexus and meninges, whereas congeneric MRL/Mp- +/+ mice (which lack the lpr gene) showed delayed but widespread inflammatory infiltrates involving cerebral vessels and meninges, with sparing of the choroid plexus. These distinctive patterns of cerebral inflammation, which are comparable in many respects to those seen in human connective tissue disease, provide some of the first animal models of relevant central nervous system histopathological processes associated with underlying connective tissue disease.

Animals↗
PubMed1983

Cutaneous manifestations of primary Sjögren's syndrome: a reflection of vasculitis and association with anti-Ro(SSA) antibodies.

Twenty-two symptomatic primary Sjögren's syndrome patients with clinical and histologic evidence of skin disease were studied. Purpura and urticaria were the most frequent clinical cutaneous manifestations. Most of these lesions were associated with a leukocytoclastic angiitis and, less commonly, a mononuclear (lymphocytic) vasculitis. Two main cutaneous syndromes were identified whose clinical, serologic, and histopathologic features were indistinguishable from Waldenström's benign hyperglobulinemic purpura and hypocomplementemic urticaria-like vasculitis. Eighty-four percent of primary Sjögren's syndrome patients with vasculitis demonstrated anti-Ro(SSA) antibodies. Thus, purpura and urticaria may reflect cutaneous vasculitis occurring in the clinical setting of Sjögren's syndrome.

Adolescent↗
PubMed1983

Aseptic meningoencephalitis in primary Sjögren's syndrome.

The clinical features and CSF characteristics of five patients with primary Sjögren's syndrome (SS) and associated aseptic meningoencephalitis (AME) are described. Episodes of AME were recurrent in four patients. Viral, fungal, and bacterial cultures were uniformly negative. Plasma cells were observed in the CSF but not in the blood of three patients. The CSF IgG:albumin index was elevated, suggesting intrathecal synthesis of IgG in each of the four patients tested; each patient had either one or two broad bands with the mobility of IgG on CSF agarose electrophoresis. These observations are consistent with current understanding of SS as a polyclonal gammopathy associated with the multifocal proliferation of B lymphocytes and plasma cells.

Adult↗
PubMed1983

Sjögren's syndrome: association of anti-Ro(SS-A) antibodies with vasculitis, hematologic abnormalities, and serologic hyperreactivity.

The clinical significance of autoantibodies to Ro(SS-A) and La(SS-B) in Sjögren's syndrome was examined in a retrospective evaluation of 75 patients with symptoms of the sicca complex who had either primary Sjögren's syndrome or Sjögren's syndrome associated with another connective tissue disease. The clinical, hematologic, and serologic features associated with autoantibodies to the small molecular weight ribonucleoproteins Ro(SS-A) and La(SS-B) were ascertained. A striking clinical association of anti-Ro(SS-A) antibodies (found in 33 patients) with extraglandular disease (vasculitis, purpura, and lymphadenopathy) was seen. Hematologic abnormalities (anemia, leukopenia, and thrombocytopenia) were also associated with the presence of anti-Ro(SS-A) antibodies. Furthermore, anti-Ro(SS-A) antibody was associated with hyperglobulinemia, increased serologic reactivity in terms of rheumatoid and antinuclear factors, cryoglobulinemia, and hypocomplementemia. The presence of anti-Ro(SS-A) antibodies defines a subset of patients with Sjögren's syndrome who have systemic clinical manifestations including vasculitis, hematologic abnormalities, and serologic hyperreactivity.

Antibodies, Antinuclear↗
PubMed1982

Necrotizing arteritis and spinal subarachnoid hemorrhage in Sjögren syndrome.

A 37-year-old woman with primary Sjögren syndrome developed mixed cryoglobulinemia and systemic vasculitis. Subarachnoid hemorrhage occurred as a result of necrotizing anterior spinal arteritis. Although rarely seen in mixed cryoglobulinemia, central nervous system complications have recently been documented in Sjögren syndrome. The patient's serum contained antibodies to the Ro(SSA) cytoplasmic antigen, and these antibodies were concentrated in the cryoglobulin fraction. Anti-Ro(SSA) antibodies are associated with the occurrence of vasculitis in patients with Sjögren syndrome, which suggests that the spinal arteritis and subarachnoid hemorrhage in this patient may have been directly related to the underlying connective tissue disorder.

Adult↗