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Biomedical subjects

L E Shulman

Publications and source records attributed to L E Shulman.

At least 19 recordsLinked to original sources
PubMed1991

The eosinophilia-myalgia syndrome: the Los Alamos Conference.

On June 12 and 13, 1990 the Los Alamos National Laboratory in cooperation with the New Mexico Department of Health and Environment, the Centers for Disease Control (CDC), the Food and Drug Administration (FDA), and the National Institute of Arthritis and Musculoskeletal and Skin Diseases of the National Institutes of Health (NIH) hosted a conference on the eosinophilia-myalgia syndrome. Fifty presentations covered a variety of important issues which are summarized herein.

Centers for Disease Control and Prevention, U.S.↗
PubMed1989

Estimates of the prevalence of selected arthritic and musculoskeletal diseases in the United States.

The National Arthritis Data Workgroup focused its studies primarily on joint disease with the addition of osteoporosis which is a frequently occurring musculoskeletal condition and public health problem. We used national data sets as well as published studies to estimate prevalence in the United States. We report on prevalence rather than incidence because of the difficulty in defining the point of onset and because primary national data sources currently available are concerned with disease prevalence.

Adolescent↗
PubMed1984

Diffuse fasciitis with hypergammaglobulinemia and eosinophilia: a new syndrome?

Two male patients (53 and 19 years old) have been seen with a diffuse scleroderma-like illness with firm taut skin bound down to underlying structures (sparing the face in both patients and the hands and feet in one). Flexion contractures of elbows and knees and limitation of abduction at the shoulders developed in a few weeks after onset. Raynaud's phenomenon was absent and thorough evaluation failed to reveal any evidence of myositis or the visceral manifestations of systemic sclerosis. There was no loss of skin appendages. Both had circulating eosinophils (12% and 37%) without drug ingestion or parasitic infestation. Both had elevated sedimentation rates and hypergammaglobulinemia (in one, IgG = 4.1 g.%). Serologic tests for syphilis, rheumatoid factor, LE cells, antinuclear antibodies, complement and cryoglobulins were negative or normal in both patients. Bone marrow examination revealed plasmacytosis and eosinophilia. Biopsies revealed striking thickening of the fascia between the subcutis and muscle. Within the thickened connective tissue there was intense infiltration with plasma cells and lymphocytes, at times in follicles, both about and separate from vessels; eosinophils were absent. Skin biopsies revealed no changes of scleroderma; and muscle biopsies, no evidence of myositis. Prednisone therapy over 15 months in decreasing dosage, using laboratory parameters, induced in the first patient a full remission which has persisted for 5 years without therapy. In the second (more severely involved) patient prednisone therapy has reversed laboratory abnormalities; but clinical improvement to date has been minimal. The pathogenesis of this diffuse fasciitis is obscure, although unusual physical exertion antedated the onset of illness in each case.

Adolescent↗
PubMed1982

Papular mucinosis. A clinicopathologic study of four patients.

Four patients with papular mucinosis were studied. Each patient had clinically characteristic cutaneous lesions, increased dermal deposition of mucin, and a serum monoclonal lgG paraprotein. One patient had sclerodermoid features consistent with the scleromyxedema variant of papular mucinosis. Associated findings in the patients included pachydermoperiostosis (one case), adenocarcinoma of the stomach (one case), carcinoma of the pancreas (one case), bizarre neurologic symptoms (one case), and emphysema (two cases). Autopsies were performed in two cases and no increased mucin deposition was observed in internal organs. Immunofluorescence microscopic study of involved skin showed no immunoreactant deposition in the three patients studied. Our observations support the hypothesis that papular mucinosis is a disorder of skin fibroblasts without internal organ involvement.

Adenocarcinoma↗
PubMed1980

Diffuse fasciitis with eosinophilia. A clinicopathologic study.

Three cases of diffuse fasciitis with eosinophilia are presented, and their clinical, laboratory and histologic follow-up are outlined. Furthermore histologic comparison with scleroderma is attempted and the literature is reviewed. From our findings, we conclude that diffuse fasciitis with eosinophilia is a distinct clinicopathologic entity.

Adult↗
PubMed1980

Cranial nerve involvement in systemic sclerosis (scleroderma): a report of 10 cases.

Ten patients with the diagnosis of systemic sclerosis developed cranial nerve involvement. A trigeminal sensory neuropathy evolved insidiously in all patients and in five of these it was a presenting complaint. The glossopharyngeal nerve was involved in one patient. Taste was impaired in one patient and a unilateral loss of taste with fasciculations of the tongue were noted in another. Tinnitus was a complaint in three patients, two of whom had bilateral impairment of hearing. Facial weakness was noted in five patients. In three, this weakness was bilateral, while in the others the weakness was unilateral, and a past history of acute onset was obtained. The microangiopathy of systemic sclerosis is felt to be primarily responsible for these neurological deficits. The deposition of fibrous tissue may be a secondary phenomenon and contribute to the process by compression of nerves.

Adult↗
PubMed1980

Hematological abnormalities in scleroderma. A study of 180 cases.

Hematological abnormalities in scleroderma indicate a specific complication of the disease itself, or an associated illness. Among 180 patients with scleroderma, anemia was detected in 25% and was attributed to chronic inflammatory disease (usually an overlap syndrome), bleeding mucosal telangiectases as part of the CREST syndrome, intestinal malabsorption, and microangiopathic hemolysis. Leukocytosis, present in 14%, was correlated with active myopathy and/or advanced visceral involvement while leukopenia was suggestive of a crossover with systemic lupus erythematosus. Thrombocytopenia was often a manifestation of microangiopathy, and thrombocytosis was associated with an arteritis or a tumor syndrome.

Anemia↗
PubMed1979

The 'CREST' syndrome. Comparison with systemic sclerosis (scleroderma).

The CREST syndrome refers to a disorder comprising the manifestations of calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia. Thirteen CREST patients (two with CRST) were compared with 26 patients with systemic sclerosis but without the full manifestations of the CRST syndrome. No significant difference was found between the groups in the age of onset of Raynaud's phenomenon, degree of multiphasic digital color changes, ulcerations of fingers, sclerodactyly, or in the frequency of abnormal esophageal peristalsis or dysphagia. Laboratory results were similar, including the frequency of an elevated ESR. However, the CREST patients had a significantly lower frequency of arthralgia (54%) and arthritis (15%) than did those with scleroderma (88% and 65%, respectively). All but one of the CREST patients were women, which was a greater proportion than found among scleroderma cases (69%), and all were white (P less than .05). Most patients with the CREST syndrome had rather severe acrosclerosis. At last evaluation, four patients were chronically ill and three had died. The CREST and CRST syndromes are closely related disorders that seem to be part of the spectrum of systemic sclerosis.

Adult↗
PubMed1978

Acute leukemia following cyclophosphamide therapy for Sjögren's syndrome.

A fatal myeloproliferative syndrome diagnosed as subacute myelomonocytic leukemia developed in a patient with Sjögren's syndrome following treatment with cyclophosphamide. Although patients with Sjögren's syndrome have a recognized increased risk of developing lymphoproliferative disorders, particularly reticulum cell sarcoma, there is no evidence to support a similar predisposition to leukemia. As immunosuppressive therapy is becoming more common in the treatment of patients with rheumatoid arthritis, there have been recent reports of patients developing fatal myeloproliferative disorders after receiving immunosuppressive therapy. Review of these cases shows similar duration of therapy, and cyclophosphamide, among others, has been implicated as the leukemogenic agent in other patients. We feel that great caution should be exercised in the use of immunosuppressive drugs in patients with chronic, nonfatal disorders sich as rheumatoid arthritis and Sjögren's syndrome.

Acute Disease↗
PubMed1976

Studies in familial systemic lupus erythematosus.

Although many examples of familial SLE have been reported, the relative importance of genetic and environmental factors remains unclear. In an effort to better understand these factors, eight such families were studied. These families, plus one deceased pair and 31 described in the literature consisted of 8 pairs of identical twins, one pair of non-identical twins, and 16 sibling and 15 parent-offspring combinations. Fifty-three cases in 25 families provided sufficient documentation for detailed analysis. Each familial case was compared to his affected relative utilizing 23 clinical and laboratory features of SLE. A control group of non-related SLE patients, each matched to a familial case for age, sex, race and disease duration, was similarly analyzed. Impressive concordance for disease expression was found between pairs of identical twins and between parent and offspring. No such concordance was found between siblings and controls. These findings support genetic influences in the expression of SLE in identical twins and parents and offspring pairs. Comparison of the frequencies of clinical and laboratory attributes in the familial group as opposed to non-familial groups showed no real differences. Thus, familial SLE is probably not a different disease entity from non-familial SLE. The finding of four father-offspring pairs lessens the possibility that SLE is transmitted during the perinatal period. The onset of SLE in each of identical twins occurred within an average of 2 years. In siblings, however, while the average difference in age at onset was 9 years, the average difference in time of onset (actual date) was only 3 years. Comparable figures for parents and offspring were 20 and 8 years, respectively. These data suggest environmental influences in the initiation of SLE, especially in siblings. Studies of 27 unaffected first-degree relatives in our eight families revealed two sisters with thyroid disease, persistent leukopenia and sedimentation rate elevation. They were daughters of a patient with SLE and Hashimoto's thyroiditis and sisters of a patient with SLE. The remaining 25 relatives were clinically, hematologically, biochemically, and serologically normal. While these studies suggest both genetic and environmental influences in the pathogenesis of SLE, further studies are necessary to elucidate the mechanisms.

Animals↗
PubMed1975

Colonic perforations in systemic lupus erythematosus.

Five patients with systemic lupus erythematosus (SLE), four of whom died with colonic perforations, are reported. Perforation of the colon constituted the most frequent cause of death among 107 patients with SLE admitted to the Rheumatic Disease Unit during a three year period. All five patients with colonic perforation had clinical and laboratory manifestations of active SLE in addition to the abdominal syndrome. Most striking was evidence of active arteritis in all patients with either central nervous system involvement and/or peripheral arteritis, in addition to that found in the gastrointestinal tract. Hyperglobulinemia and rheumatoid factor as well as antinuclear antibodies were present at some time in all patients. The abdominal syndrome was characterized by the insidious onset of lower quadrant pain which was intermittent and colicky. Although direct abdominal tenderness was eventually present in all patients, rebound tenderness and hypoactive bowel sounds were variable and abdominal rigidity occurred only in one patient and late in the course. The differential diagnosis of abdominal pain in SLE is reviewed and possible mechanisms for the production of colonic perforations are discussed. It is suggested that the presence of rheumatoid factors in conjunction with circulating immune complexes may be the pathogenetic mechanism via the production of a mesenteric arteritis.

Adrenal Cortex Hormones↗