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Biomedical subjects

L E Shulman

Publications and source records attributed to L E Shulman.

7 recordsLinked to original sources
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↗
PubMedDate not supplied

Controlled trial of cyclophosphamide in rheumatoid arthritis.

Twenty-four patients with severe progressive rheumatoid arthritis were randomly assigned to cyclophosphamide or placebo in a double-blind crossover trial. Eleven patients who completed 9 months on cyclophosphamide (average dose: 1.8 mg/kg/day) demonstrated significant decrease in painful joints, swollen joints, and morning stiffness and increase in grip strength when compared to 11 patients on placebo. After crossover, significant improvement was observed in patients switched to cyclophosphamide, and deterioration within 2 months was observed in most patients changed from drug to placebo. Serum immunoglobulins and rheumatoid factor titers decreased with cyclophosphamide but antibody response to Vi antigen was unaffected. Primary delayed immune response to 2,4-dinitrochlorobenzene was markedly depressed. Adverse effects were troublesome--hemorrhagic cystitis affected 4 patients and amenorrhea occurred in 3. Despite striking beneficial effect, cyclophosphamide should be prescribed cautiously and only in severe resistant cases of rheumatoid arthritis.

Adult↗