Search PubMed⌕ Search

Biomedical subjects

G P Rodnan

Publications and source records attributed to G P Rodnan.

At least 55 records · Page 3Linked to original sources
PubMed1982

Reduced T lymphoid colony growth in patients with progressive systemic sclerosis and rheumatoid arthritis.

T cell colony growth in semisolid medium, a sensitive indicator of disease-associated lymphoycte defects, was found to be depressed in patients with PSS and RA. In both disorders, more pronounced abnormalities were observed after incubation with suboptimal concentrations of the polyclonal mitogen PHA. Depletion of monocytes by adherence to plastic surfaces of addition of the prostaglandin synthetase inhibitor indomethacin did not correct the growth abnormality observed in PSS. Reduced colony-forming activity could not be attributed to serum inhibitory factors. The cellular abnormalities detected in patients with these connective tissue diseases were less pronounced than those found in SLE patients.

Adult↗
PubMed1981

Thyroid disease in progressive systemic sclerosis: increased frequency of glandular fibrosis and hypothyroidism.

A series of patients with fatal progressive systemic sclerosis was reviewed with regard to pathologic, clinical, and serologic evidence of thyroid disease. Histologic evidence of severe fibrosis of the thyroid gland was found significantly more frequently in 56 progressive systemic sclerosis cases (14%) compared to an age and sex matched control autopsy series (2%) from the same institution. Based on determination of serum free thyroxine, free triiodothyroxine (T3), thyroid stimulating hormone, and reverse T3, 27 patients were classified as euthyroid (11), euthyroid sick (9), and hypothyroid (7). Patients with hypothyroidism more frequently had subcutaneous calcinosis. Raynaud's phenomenon, esophageal hypomotility, sclerodactyly, and multiple telangiectasias (the CREST syndrome variant of progressive systemic sclerosis); all thyroid glands from the hypothyroid patients had fibrosis, but lymphocytic infiltration was an infrequent finding. Six hypothyroid patients had high titers of serum antithyroid antibodies, suggesting autoimmune thyroid disease. Thyroid gland fibrosis and hypothyroidism, possibly of autoimmune pathogenesis, are thus frequent and often unsuspected findings in progressive systemic sclerosis.

Adult↗
PubMed1981

Abnormalities of T lymphocyte subsets in patients with progressive systemic sclerosis (PSS, scleroderma).

Thirty-one patients with PSS (scleroderma) were examined for evidence of abnormalities in T lymphocyte subsets. TG and TM subpopulations of peripheral blood T lymphocytes were enumerated by rosetting techniques. The helper-suppressor functions of these lymphocytes were studied in the PWM-activated in vitro assay. The absolute numbers of TG cells in patients with DS as well as those with the CREST syndrome variant of PSS were decreased in comparison with those in age-matched healthy controls (p < 0.05). The numbers of TM and T "null" cells were not significantly altered (p > 0.05). The reduction in the number of TG cells was not caused by lymphocytotoxic antibodies. The mean helper-suppressor scores for patients with PSS and DS (1.25) and those with CREST syndrome (1.36) were higher (p < 0.05) than the mean score for healthy individuals (0.80). No relationship between the immunoregulatory abnormality and the presence or titer of CICs, ANAs, hypergammaglobulinemia, or various clinical manifestations of PSS could be demonstrated in individual patients. The finding of abnormalities in T lymphocyte subpopulations and the demonstration of their altered functional expression in patients with PSS indicate that immunoregulatory mechanisms may play an important role in this connective tissue disease.

Adolescent↗
PubMed1980

Diagnostic potential of in vivo capillary microscopy in scleroderma and related disorders.

The prevalence of scleroderma-type capillary abnormalities, as observed by in vivo microscopy, was determined in 173 patients from three rheumatic disease centers. The patients had a variety of connective tissue diseases: scleroderma (systemic sclerosis) 50; systemic lupus erythematosus 60; mixed connective disease 26; Raynaud's disease 11; other rheumatic disorders 26. Enlarged and deformed capillary loops surrounded by relatively avascular areas, most prominently in the nail-folds, were found in 82% of patients with scleroderma and in 54% with mixed connective tissue disease. The rarity of these abnormalities in systemic lupus erythematosus (2%) despite the presence of Raynaud's phenomenon suggests that they are not an expression of the Raynaud's phenomenon frequently associated with scleroderma and mixed connective tissue disease. The single patient with Raynaud's disease and sclerodermatype capillary changes subsequently developed scleroderma.

Adolescent↗
PubMed1980

Collagen types synthesized in dermal fibroblast cultures from patients with early progressive systemic sclerosis.

Fibroblast cultures were established by explant culture from upper and lower dermis of 7 normal donors and 10 patients with early progressive systemic sclerosis (PSS). Antibodies against collagens containing the A and B chains, collagens which occur largely in vascular structures, were isolated and used for immunofluorescence studies. Cultures from lower PSS dermis accumulated significantly more of these collagens than fibroblast cultures from upper PSS dermis or cultures from upper or lower dermis of normal skin. These data indicate that the collagens containing the A and B chains in early PSS lesions are produced by cells of vascular origin and support the concept that alterations in vascular tissue play a prominent role in the pathogenesis of PSS.

Adult↗
PubMed1980

Diversity of antinuclear antibodies in progressive systemic sclerosis. Anti-centromere antibody and its relationship to CREST syndrome.

Antinuclear antibodies were demonstrated in the sera of 43 of 45 (96%) patients with progressive systemic sclerosis (22 of 24 with diffuse scleroderma and 21 of 21 with the CREST syndrome variant). This high percentage of positive reactions resulted from the use of a tissue culture substrate (HEp-2) to detect antinuclear antibodies by the indirect immunofluorescent method. Patterns of nuclear staining included diffuse fine speckles, large coarse speckles, nucleolar and centromere staining. When organ sections such as mouse kidney were used as substrate for the detection of antinuclear antibodies, nucleolar staining and centromere staining were the two patterns most frequently overlooked. Three types of antibodies appeared to be highly specific for scleroderma: antibody to Scl-70 antigen, antibody to centromere, and antinucleolar antibody. The anti-centromere antibody appeared to be highly selective for the CREST variant of progressive systemic sclerosis.

Antibodies, Antinuclear↗
PubMed1979

Skin thickness and collagen content in progressive systemic sclerosis and localized scleroderma.

Skin biopsies of uniform location and surface area (7 mm diameter) were obtained from the extensor aspect of the forearm of 147 patients with progressive systemic sclerosis (PSS) (107 with diffuse scleroderma, 40 with the CREST syndrome variant) and 58 individuals with normal skin. After careful removal of all subcutaneous fatty tissue, the skin cores were weighed and their water and hydroxyproline content determined. Despite recent claims to the contrary, it was found that there is a marked and highly significant increase in the thickness of the skin during the indurative phase of PSS, and that this is associated with a proportionate increase in total dermal collagen content. A similar degree of thickening was found in the skin of patients with eosinophilic fasciitis and acromegaly. A close correlation was observed between clinical estimation of the degree of skin thickening and the weight of the skin biopsy cores. Change in the weight of skin cores was observed during the course of illness of the patients with PSS and may serve as a useful measurement of alteration in the degree of skin thickening.

Acromegaly↗
PubMed1979

Eosinophilic fasciitis. A pathologic study of twenty cases.

This report presents a detailed light-microscopic evaluation of biopsies obtained from 20 patients with eosinophilic fasciitis, a newly recognized disorder characterized by inflammation and thickening of the deep fascia, hypergammaglobulinemia, and peripheral and tissue eosinophilia. Early in the course of the disease, the deep fascia and lower subcutis are edematous and infiltrated with lymphocytes, plasma cells, histiocytes, and eosinophils; these features are associated with impressive peripheral eosinophilia. As the illness progresses, these structures and eventually the dermis become collagenized, thickened, and sclerotic. Tissue eosinophilia may be focal or diffuse and is usually observed in the fascia and/or lower subcutis. Extracutaneous involvement has been limited to a chronic synovitis and tenosynovitis, the latter frequently associated with the carpal tunnel syndrome. Deposits of immunoglobulin and/or complement were found in five of eight biopsies studied by direct immunofluorescence, which suggests that an immunologic stimulus may be responsible for initiating this syndrome. Differential diagnoses are discussed.

Adult↗
PubMed1978

Immunopathology of the renal vascular lesion of progressive systemic sclerosis (scleroderma).

Patients with progressive systemic sclerosis (PSS, scleroderma) exhibit a variety of immunologic abnormalities. To verify whether the renal vascular lesions of such patients might be mediated by an immunologic mechanism, kidney tissues of 16 patients with PSS were investigated by means of fluorescence, light, and electron microscopy; elution of tissue-bound antibody; and fixation of heterologous (guinea pig) complement. Controls consisted of 12 nonsclerodermatous patients with similar levels of hypertension with no evidence of associated immunologic abnormalities. Diffuse vascular deposits of immunoglobulins (predominantly IgM) and/or complement (predominantly Clq) were found in all 16 patients with PSS. These deposits were bound to the intima of intralobular and arcuate arteries which, by light microscopy, often exhibited typical fibromucinous alterations. Elution of antibody and heterologous complement fixation studies suggested that such reactants may represent the interaction of complement-fixing antibody and antigen. Electron microscopies studies demonstrated abundant fibrillar and ground substance material in the arterial intima but features of deposited (circulating) immune complexes were not found. By contrast, in the hypertensive (control) group, deposits of immunoglobulin (s) and/or complement were rare and, when present, were mostly confined to the arterioles. As judged by the results of elution and heterologous complement fixation, these arteriolar deposits appeared to represent trapped rather than specifically bound serum proteins. The possible signficance of these findings are discussed in relation to immunologic mechanisms which might be implicated in the pathogenesis of the renal vascular disease of PSS.

Adult↗
PubMed1977

Pulmonary hypertension in the CREST syndrome variant of progressive systemic sclerosis (scleroderma).

Severe pulmonary hypertension without pulmonary fibrosis occurred in 10 patients with the CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, telangiectasia), reputedly a benign variant of progressive systemic sclerosis. Time from the initial symptom, Raynaud's phenomenon, to the recognition of pulmonary hypertension was as long as 40 years. Pulmonary hypertension and increased pulmonary vascular resistance was shown in all patients. Autopsy examination in three of six deaths attributable to pulmonary hypertension showed intimal proliferation with myxomatous change in the small- and medium-sized pulmonary arteries similar to changes in the digital arteries of patients with scleroderma and Raynaud's phenomenon, and interlobular renal arteries of those with "scleroderma kidney." It is concluded that the CREST syndrome is not entirely benign but may be complicated, after a long clinical course, by progressive pulmonary vascular obliteration, pulmonary hypertension, and death in the absence of significant pulmonary fibrosis.

Adult↗