THE PATHOPHYSIOLOGY OF SCLERODERMA

Systemic scleroderma is a disease of the connective tissue, known to medicine for more than 200 years. The organs most frequently involved are the skin, lungs, gastrointestinal tract and kidneys. The disease may be fatal and the main causes of death are pulmonary fibrosis with right heart decompensation and kidney failure. The involvement in localized scleroderma appears to be restricted to the skin and 3 clinical forms have been recognized: morphea, generalized morphea and linear scleroderma. The pathology of scleroderma is characterized by 2 outstanding features: marked deposition of collagen and vascular changes involving capillaries, arterioles and small-size arteries. Three schools of thought have prevailed in the pathogenesis of scleroderma: (1) those who believe that the primary event is the fibrosis, (2) those who advocate that the primary lesion is in the blood vessel, and (3) those who regard this disease as an autoimmune process. In this paper 1 shall attempt to review some recent work performed in various

[1]  R. Fleischmajer,et al.  [3H]Thymidine labeling of dermal endothelial cells in scleroderma. , 1977, The Journal of investigative dermatology.

[2]  J. R. Reeves,et al.  Cellular infiltrates in scleroderma skin. , 1977, Arthritis and rheumatism.

[3]  K. Shaw,et al.  Skin capillary changes in early systemic scleroderma. Electron microscopy and "in vitro" autoradiography with tritiated thymidine. , 1976, Archives of dermatology.

[4]  R. Bashey,et al.  Connective tissue synthesis by cultured scleroderma fibroblasts. I. In vitro collagen synthesis by normal and scleroderma dermal fibroblasts. , 1976, Arthritis and rheumatism.

[5]  H. Seigler,et al.  Apparent recurrence of progressive systemic sclerosis in a renal allograft. , 1976, JAMA.

[6]  R. Johnson,et al.  Lymphokine stimulation of collagen accumulation. , 1976, The Journal of clinical investigation.

[7]  D. Scott,et al.  Immunohistological studies, with anti‐connective tissue and anti‐immunoglobulin antisera, of the skin in lupus erythematosus and scleroderma , 1975, The British journal of dermatology.

[8]  E. Tan,et al.  Profiles of antinuclear antibodies in systemic rheumatic diseases. , 1975, Annals of internal medicine.

[9]  D. Alarcón-Segovia,et al.  Immunochemical characterization of the anti-RNA antibodies found in scleroderma and systemic lupus erythematosus. I. Differences in reactivity with Poly (U) and Poly-(A) Poly (U). , 1975, Journal of immunology.

[10]  R. Auerbach,et al.  Lymphocyte-induced angiogenesis: a quantitative and sensitive assay of the graft-vs.-host reaction , 1975, The Journal of experimental medicine.

[11]  C. Kovacs,et al.  Properties of scleroderma fibroblasts in culture. , 1974, The Journal of investigative dermatology.

[12]  M. Jayson,et al.  Biosynthesis and maturation of skin collagen in scleroderma, and effect of D-penicillamine. , 1974, Lancet.

[13]  E. Tan,et al.  Antinucleolar antibodies in human sera. , 1973, Journal of immunology.

[14]  R. Fleischmajer,et al.  Generalized morphea. I. Histology of the dermis and subcutaneous tissue. , 1972, Archives of dermatology.

[15]  M. Prunieras,et al.  Generalized morphea. II. Electron microscopy of collagen, cells, and the subcutaneous tissue. , 1972, Archives of dermatology.

[16]  E. Leroy.  CONNECTIVE TISSUE SYNTHESIS BY SCLERODERMA SKIN FIBROBLASTS IN CELL CULTURE , 1972, The Journal of experimental medicine.

[17]  R. Fleischmajer,et al.  Glycosaminoglycans in scleroderma and scleredema. , 1972, The Journal of investigative dermatology.

[18]  H. D. Stein,et al.  Increased protocollagen proline hydroxylase activity in sclerodermatous skin. , 1971, Archives of dermatology.

[19]  R. Fleischmajer,et al.  Scleroderma and the Subcutaneous Tissue , 1971, Science.

[20]  R. P. Thompson,et al.  Scleroderma and Primary Biliary Cirrhosis , 1970, British medical journal.

[21]  Norton Wl.  Comparison of the microangiopathy of systemic lupus erythematosus, dermatomyositis, scleroderma, and diabetes mellitus. , 1970 .

[22]  G. Rodnan,et al.  Serum antinuclear antibodies in progressive systemic sclerosis (scleroderma). , 1968, Arthritis and rheumatism.

[23]  J. Moossy,et al.  Skeletal muscle involvement in progressive systemic sclerosis (scleroderma). , 1968, Arthritis and rheumatism.

[24]  T. Medsger,et al.  The rheumatic manifestaions of progressive systemic sclerosis (scleroderma). , 1968, Clinical orthopaedics and related research.

[25]  S. Krol,et al.  Chemical Analysis of the Dermis in Scleroderma.∗ , 1967 .

[26]  L. Gottlieb,et al.  Functional and morphologic alterations of the gastrointestinal tract in progressive systemic sclerosis (scleroderma). , 1962, The American journal of medicine.

[27]  S. Y. Han,et al.  Intestinal atony in progressive systemic sclerosis (Scleroderma). , 1979, Southern medical journal.

[28]  R. Winkelmann,et al.  Peripheral blood lymphocyte distribution in scleroderma. , 1975, Dermatologica.

[29]  R. Clancy,et al.  Circulating T and B lymphocytes in progressive systemic sclerosis. , 1975, The Journal of rheumatology.

[30]  A. Eisen,et al.  A sensitive microassay for prolyl hydroxylase: activity in normal and psoriatic skin. , 1973, The Journal of investigative dermatology.

[31]  R. Fleischmajer,et al.  Alteration of subcutaneous tissue in systemic scleroderma. , 1972, Archives of dermatology.

[32]  R. Winkelmann,et al.  Immunoglobulins in systemic scleroderma. , 1971, Mayo Clinic proceedings.

[33]  A. Barnett,et al.  RENAL IMMUNE DEPOSITS IN SCLERODERMA , 1971, Pathology.

[34]  W. Norton.  Comparison of the microangiopathy of systemic lupus erythematosus, dermatomyositis, scleroderma, and diabetes mellitus. , 1970, Laboratory investigation; a journal of technical methods and pathology.

[35]  J. Uitto,et al.  Protocollagen proline hydroxylase activity in the skin of normal human subjects and of patients with scleroderma. , 1969, Scandinavian journal of clinical and laboratory investigation.

[36]  E. CARwni LEROYwith,et al.  Increased Collagen Synthesis by Scleroderma Skin Fibroblasts In Vitro A POSSIBLE DEFECT IN THE REGULATION OR ACTIVATION OF THE SCLERODERMA FIBROBLAST , 2022 .