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L D Kaufman

Publications and source records attributed to L D Kaufman.

53 records · Page 3Linked to original sources

L-tryptophan-associated eosinophilic perimyositis, neuritis, and fasciitis. A clinicopathologic and laboratory study of 25 patients.

We have described the spectrum and prevalence of the clinical and laboratory manifestations of a multisystem disorder associated with the ingestion of L-tryptophan. At least 3 subsets of clinical disease have been identified: 1) a neuromuscular disorder which may present with myalgias and mild weakness and then progress to quadriparesis related to an axonal neuropathy and interstitial myositis (perimyositis), 2) a syndrome of eosinophilic fasciitis with characteristic cutaneous induration, and 3) the Löffler syndrome consisting of pulmonary infiltrates with eosinophilia. Corticosteroids may be useful for patients with the Löffler syndrome and offer only a modest benefit in the majority of patients with neuromuscular disease. The clinical course appears to be chronic, and the long-term sequelae of this disorder are unknown. The etiologic agent remains undetermined; however, studies are in progress to examine the mechanism of eosinophilia, appropriate therapeutic intervention, and the long-term outcome of the affected individuals.

Adult↗

Eosinophilic neuritis, perimyositis, and vasculitis associated with ingestion of L-tryptophan.

Four cases are described of a clinical syndrome which developed in the setting of L-tryptophan ingestion. The major manifestations consisted of myalgias, neuropathy, weakness, and profound eosinophilia. Pathologically a vasculitis involving predominantly small veins was observed along with a mixed cellular infiltrate in the perimysium and epineurium. Clusters of eosinophils were characteristically noted in the tissue specimens. The clinical course appears to be chronic although further longterm followup will be required. One patient pursued a relentless downhill course with progressive neurologic impairment and death. Although the mechanism of tissue injury in these individuals is speculative, the possible association of this widely used nonprescription medication with this syndrome should be recognized.

Administration, Oral↗

Male lupus: retrospective analysis of the clinical and laboratory features of 52 patients, with a review of the literature.

In conclusion, in a review of 52 male lupus patients we found an increased incidence of thrombocytopenia and renal disease without any other notable differences in clinical, laboratory, or serological parameters. Of further interest was the finding of an early peak in the age of onset of disease resembling that found among female SLE patients, and a generally poor prognosis as has been reported by others.

Adolescent↗

Anti-IgE autoantibodies in systemic sclerosis (scleroderma).

An enzyme immunoassay was used to determine the prevalence of anti-IgE auto-antibodies in 66 patients with systemic sclerosis (scleroderma) stratified according to extent and duration of disease. Serum IgG anti-IgE antibodies were detected in 14 (21%) patients and IgM anti-IgE antibodies in nine (14%) patients. The overall prevalence of IgG or IgM isotypes was 21/66, (32%). Anti-IgE autoantibodies were not found in six patients with undifferentiated connective tissue disease or two patients with eosinophilic fasciitis. Attempts to demonstrate histamine release from basophils in vitro by using serum samples containing high titre anti-IgE antibody were unsuccessful. By multivariate analysis the presence of anti-IgE antibody was not associated with duration of systemic sclerosis; extent of scleroderma; specific visceral features, including heart, lung, renal, and gastrointestinal involvement; or mortality.

Autoantibodies↗

Anti-IgE autoantibodies in systemic lupus erythematosus. Prevalence and biologic activity.

IgG and IgM autoantibodies directed against IgE were determined in 95 serum samples from 67 patients with systemic lupus erythematosus, by an enzyme-linked immunosorbent assay. IgM anti-IgE autoantibodies were found in 18 patients (27%) and IgG anti-IgE autoantibodies were detected in 23 patients (34%). The specificity of the immunoassay was confirmed by the ability to inhibit binding with IgE myeloma, but not other immunoglobulin isotypes and the demonstration that the reactivity was directed to the Fc epsilon fragment of IgE. The IgG fraction of certain sera with anti-IgE activity was capable of inducing histamine release from control basophils and cutaneous mast cells. Clinical associations with the presence of anti-IgE activity were sought by retrospective chart analysis of 61 patients. Significant correlation was found with articular involvement, lymphadenopathy, and anti-DNA antibodies (P less than 0.05). Anti-IgE autoantibodies are observed in a significant number of patients with systemic lupus erythematosus and may contribute to the pathogenesis of the vascular and articular lesions characteristic of this disease.

Autoantibodies↗

Candida pericarditis and tamponade in a patient with systemic lupus erythematosus.

Candida pericarditis and tamponade developed in a patient with sterile purulent pericarditis secondary to systemic lupus erythematosus. Therapy with amphotericin B and properly timed surgical intervention led to a clinical and microbiological cure. This article emphasizes the importance of differentiating an infected pericardial effusion from the sterile pericarditis of systemic lupus erythematosus and provides suggested guidelines for the management of that complication.

Adolescent↗

Association between lupus psychosis and anti-ribosomal P protein antibodies.

In 18 of 20 patients with psychosis secondary to systemic lupus erythematosus (SLE), autoantibodies to ribosomal P proteins were detected by immunoblotting and measured with a new radioimmunoassay using a synthetic peptide as antigen. The frequency of anti-P was not increased in patients with other central nervous system manifestations of SLE (3 of 20, by radioimmunoassay), in patients with transient behavioral abnormalities due to SLE (none of 8), in patients with psychosis who did not have SLE (none of 13), or in normal controls (none of 20). In four of five paired serum samples, anti-P-peptide antibody levels increased 5-fold to 30-fold during the active phase of lupus psychosis. Longitudinal studies of anti-P activity in two patients with psychosis revealed that anti-P levels increased before and during the active phases of psychosis but not during sepsis or other exacerbations of SLE, and that the elevations were selective for anti-P antibodies, as opposed to anti-DNA antibodies. Longitudinal studies of anti-P activity in two patients with anti-P but without psychosis showed less than threefold changes in anti-P levels despite exacerbations of disease. We conclude that anti-P is associated with lupus psychosis and that synthetic peptide antigens may be useful for the detection and measurement of autoantibodies to intracellular proteins.

Adolescent↗

Preliminary observations on the role of magnetic resonance imaging for polymyositis and dermatomyositis.

The potential use of magnetic resonance imaging (MRI) for inflammatory muscle disorders was evaluated in 13 patients with polymyositis and dermatomyositis. Abnormalities in signal intensity (p = 0.0076) and fat replacement (p = 0.0177) were identified and correlated significantly with clinical disease activity. In addition, MRI was useful in directing muscle biopsy of selected abnormal areas.

Adolescent↗

Septic arthritis involving the manubriosternal joint.

Infection of the manubriosternal joint is an exceedingly rare event. We report a case of a 38-year-old patient with systemic lupus erythematosus who developed staphylococcal septic arthritis of this joint after bacteremia. Pathogenic factors are discussed. Physicians should be aware of this unusual complication of staphylococcal bacteremia.

Adult↗

The eosinophilia-myalgia syndrome: current concepts and future directions.

The eosinophilia-myalgia syndrome (EMS) is a recently described systemic disorder distinguished by the development of characteristic muscle spasm, myalgia, neuropathy, and scleroderma-like cutaneous induration. Life-threatening manifestations have included cardiopulmonary and acute ascending neuropathic syndromes. Current evidence indicates that this is a severe illness with the potential for long-term disability. Careful follow-up studies will help to better define new features of this disease, such as the recent awareness of severe neurocognitive abnormalities in some patients. The association of EMS with the ingestion of L-tryptophan may provide important clues toward the understanding of idiopathic fibrosing syndromes, as well as toxin-induced autoimmune phenomena. Rational therapy will be dependent upon a more complete understanding of the pathogenesis of this and related diseases.

Eosinophilia-Myalgia Syndrome↗

Male lupus: prevalence of IgA deficiency, 7S IgM and abnormalities of reticuloendothelial system Fc-receptor function.

Serological studies conducted on sera from a group of 31 male SLE patients revealed a 32% prevalence of 7S IgM and a 9.7% prevalence of IgA deficiency. Previous reports using similar methods indicated a higher (43-50%) prevalence of 7S IgM than was found in studies involving predominantly females with SLE (15-18%), and an overall prevalence of IgA deficiency ranging from 0.95% to 4.6%. Males resembled females in the prevalence of Fc-receptor specific reticuloendothelial system dysfunction, correlating with levels of circulating immune complex-like material and disease activity.

Antibodies, Antinuclear↗