0
December 1983, Vol 119, No. 12 >
Article | December 1983

Scleroderma: Title and subTitle BreakA Model for Fibrosis

Raul Fleischmajer, MD; Jerome S. Perlish, PhD; Matthew Duncan, PhD
[+] Author Affiliations

Department of Dermatology Mount Sinai School of Medicine 1 Gustave L. Levy PI New York, NY 10029


Arch Dermatol. 1983;119(12):957-962. doi:10.1001/archderm.1983.01650360003004
Text Size: A A A
Published online
Article
References
Comments

Systemic scleroderma or progressive systemic sclerosis is a connective-tissue disease characterized by vascular alterations and severe fibrosis. The disease has a predilection for female patients and affects all races and ages, although it is more common during the third and fourth decades of life. The sites most frequently involved are the skin, esophagus, lung, and kidney. Patients with CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasias) seem to have a more benign clinical course than those with the diffuse form of the disease, which has a more serious prognosis owing to the high incidence of severe pulmonary fibrosis and kidney insufficiency.

The pathophysiology of scleroderma remains poorly understood. Two schools of thought prevail: some believe that the primary event is vascular damage, and others propose a derangement in collagen metabolism as the initial event.1 More recently, emphasis has been placed on the presence of mononuclear cell infiltrates

REFERENCES

1 +
Fleischmajer R:  The pathophysiology of scleroderma . Int J Dermatol 1977;;16:310-318.
2 +
Fleischmajer R, Gay S, Meigel WN, et al:  Collagen in the cellular and fibrotic stages of scleroderma . Arthritis Rheum 1978;;21:418-428.
3 +
Wahl SM, Wahl LM, McCarthy JB:  Lymphocyte-mediated activation of fibroblast proliferation and collagen production . J Immunol 1978;;121:942-946.
4 +
Wahl SM, Wahl LM:  Modulation of fibroblast growth and function by monokines and lymphokines . Lymphokines 1981;;2:179-201.
5 +
Maricq HR, LeRoy EC:  Patterns of finger capillary abnormalities in connective tissue disease by `wide-field' microscopy . Arthritis Rheum 1973;;16:619-628.
6 +
Fleischmajer R, Dessau W, Timpl R, et al:  Immunofluorescence analysis of collagen, fibronectin, and basement protein in scleroderma skin . J Invest Dermatol 1980;;75:270-275.
7 +
Norton WL:  Comparison of the microangiopathy of systemic lupus erythematosus, dermatomyositis, scleroderma, and diabetes mellitus . Lab Invest 1970;;22:301-308.
8 +
Farkas TG, Sylvester V, Archer D:  The choroidopathy of progressive systemic sclerosis (scleroderma) . Am J Ophthalmol 1972;;74:875-886.
9 +
Fleischmajer R, Perlish JS:  Capillary alterations in scleroderma . J Am Acad Dermatol 1980;;2:161-170.
10 +
Fleischmajer R, Perlish JS, West WP:  Ultrastructure of cutaneous cellular infiltrates in scleroderma . Arch Dermatol 1977;; 113:1661-1666.
11 +
Kahaleh MB, Sherer GK, LeRoy EC:  Endothelial injury in scleroderma . J Exp Med 1979;;149:1326-1335.
12 +
Fleischmajer R, Perlish JS:  3H-Thymidine labeling of dermal endothelial cells in scleroderma . J Invest Dermatol 1977;; 69:379-382.
13 +
Mackel AM, DeLustro F, Harper FE, et al:  Antibodies to collagen in scleroderma . Arthritis Rheum 1982;;25:522-531.
14 +
Woodhall PB, McCoy RC, Gunnells JC, et al:  Apparent recurrence of progressive systemic sclerosis in a renal allograft . JAMA 1976;;236:1032-1034.
15 +
Lapenas D, Rodnan GP, Cavallo T:  Immunopathology of the renal vascular lesion of progressive systemic sclerosis (scleroderma) . Am J Pathol 1978;;91:243-258.
16 +
Kahaleh MB, Osborn I, LeRoy EC:  Increased factor VIII/von Willebrand factor antigen and von Willebrand factor activity in scleroderma and in Raynaud's phenomenon . Ann Intern Med 1981;;94:482-484.
17 +
Kahaleh MB, Osborn I, LeRoy EC:  Elevated levels of circulating platelet aggregates and beta thromboglobulin in scleroderma . Ann Intern Med 1982;;96:610-613.
18 +
D'Angelo WA, Fries JF, Mast AJ, et al:  Pathologic observations in systemic sclerosis (scleroderma) . Am J Med 1969;;46:428-440.
19 +
Sackner MA: Scleroderma . New York, Grune & Stratton Inc, 1966;, pp 77-81.
20 +
 Case 2-1972, Case records of the Massachusetts General Hospital: Weekly clinicopathological exercises . N Engl J Med 1972;;286:91-99.
21 +
Kovacs CV, Fleischmajer R:  Properties of scleroderma fibroblasts in culture . J Invest Dermatol 1974;;63:456-460.
22 +
LeRoy EC:  Connective tissue synthesis by scleroderma skin fibroblasts in cell culture . J Exp Med 1972;;135:1351-1362.
23 +
Ninomiya Y, Hota RI, Nagai Y, et al:  Glycosaminoglycan metabolism by scleroderma fibroblasts in culture . Biomed Res 1982;;3:70-72.
24 +
Fleischmajer R, Perlish JS:  Glycosaminoglycans in scleroderma and scleredema . J Invest Dermatol 1972;;58:129-132.
25 +
Uitto J, Helin G, Helin P, et al:  Connective tissue in scleroderma . Acta Derm Venereol 1971;;51:401-406.
26 +
Bashey RI, Perlish JS, Nochumson S, et al:  Connective tissue synthesis by cultured scleroderma fibroblasts: II. Incorporation of 3H-glucosamine and synthesis of glycosaminoglycans . Arthritis Rheum 1977;;20:879-885.
27 +
LeRoy EC:  Increased collagen synthesis by sckeriderna skin fibroblasts in vitro: A possible defect in the regulation or activation of the scleroderma fibroblast . J Clin Invest 1974;;54:880-889.
28 +
Perlish JS, Bashey RI, Stephens RE, et al:  Connective tissue synthesis by cultured scleroderma fibroblasts: I. In vitro collagen synthesis by normal and scleroderma dermal fibroblasts . Arthritis Rheum 1976;;19:891-901.
29 +
Buckingham RB, Prince RK, Rodnan GP, et al:  Increased collagen accumulation in dermal fibroblast cultures from patients with progressive systemic sclerosis (scleroderma) . J Lab Clin Med 1978;;92:5-21.
30 +
Tajima S, Pinnell S:  Collagen synthesis by human skin fibroblasts in culture: Studies of fibroblasts explanted from papillary and reticular dermis . J Invest Dermatol 1981;;77:410-412.
31 +
Fleischmajer R, Perlish JS, Krieg T, et al:  Variability in collagen and fibronectin synthesis by scleroderma fibroblasts in primary culture . J Invest Dermatol 1981;;76:400-403.
32 +
Botstein GR, Sherer GK, LeRoy EC:  Fibroblast selection in scleroderma . Arthritis Rheum 1982;;25:189-195.
33 +
Graves PN, Weiss IK, Perlish JS, et al:  Increased procollagen mRNA levels in scleroderma skin fibroblasts . J Invest Dermatol 1983;;80:130-132.
34 +
Vaheri A, Mosher DF:  High molecular weights, cell surfaceassociated glycoprotein (fibronectin) lost in malignant transformation . Biochim Biophys Acta 1978;;516:1-25.
35 +
Yamada KM, Olden K:  Fibronectin-adhesive glycoproteins of cell surface and blood . Nature 1978;;275:179-184.
36 +
Foidart JM, Berman JJ, Paglia L, et al:  Synthesis of fibronectin, laminin, and several collagens by a liver-derived epithelial line . Lab Invest 1980;;42:525-532.
37 +
Perlish JS, Carter V, Fleischmajer R:  Scleroderma mononuclear cell factors and collagen synthesis , abstracted. J Invest Dermatol 1982;;78:352.
38 +
Wiestner M, Krieg T, Horlein D, et al:  Inhibiting effect of procollagen peptides on collagen biosynthesis in fibroblast cultures . J Biol Chem 1979;;254:7016-7023.
39 +
Wiestner M, Rohde H, Helle O, et al:  Low rate of procollagen conversion in dermatosparactic sheep fibroblasts is paralleled by increased synthesis of type I and type III collagens . EMBO J 1982;;1:513-516.
40 +
Krieg T, Horlein D, Wiestner M, et al:  Aminoterminal extension peptides from type I procollagen normalize excessive collagen synthesis of scleroderma fibroblasts . Arch Dermatol Res 1978;;263:171-180.
41 +
Perlish JS, Fleischmajer R, Timpl R, et al:  Effect of Col 1 (I) peptide on the synthesis of collagen by scleroderma skin fibroblasts in culture . Arthritis Rheum , in press.
42 +
LeRoy EC:  Pathogenesis of scleroderma (systemic sclerosis) . J Invest Dermatol 1982;;79 ( (suppl 1) ):S87-S84.
43 +
Postlethwaite AE, Snyderman R, Kang AH:  The chemotactic attraction of human fibroblasts to a lymphocyte-derived factor . J Exp Med 1976;;114:1188-1203.
44 +
Tsukamoto Y, Helsel WE, Wahl SM:  Macrophage production of fibronectin: A chemoattractant for fibroblasts . J Immunol 1981;; 127:673-678.
45 +
Rola-Pleszczynski M, Lieu MH, Hamel J, et al:  Stimulated human lymphocytes produce a soluble factor which inhibits fibroblast migration . Cell Immunol 1982;;74:104-110.
46 +
Wahl SM, Gately CL:  Modulation of fibroblast growth by a lymphokine of human T cell and continuous T cell line origin . J Immunol 1982;;130:1226-1230.
47 +
Postlethwaite AE, Kang AH:  Characterization of fibroblast proliferation factors elaborated by antigen- and mitogen-stimulated guinea pig lymph node cells . Cell Immunol 1982;;73:169-178.
48 +
DeLustro F, Sherer GK, LeRoy EC:  Human monocyte stimulation of fibroblast growth by a soluble mediator(s) . J Reticuloendothel Soc 1980;;28:519-632.
49 +
Schmidt JA, Mizel SB, Cohen D, et al:  Interleukin 1: A potential regulator of fibroblast proliferation . J Immunol 1982;; 128:2177-2182.
50 +
Korotzer TI, Page RC, Granger GA, et al:  Regulation of growth of human diploid fibroblasts by factors elaborated by activated lymphoid cells . J Cell Physiol 1982;;111:247-254.
51 +
Korn JH, Halushka PV, LeRoy EC:  Mononuclear cell modulation of connective tissue function . J Clin Invest 1980;;65:543-554.
52 +
Johnson RL, Ziff M:  Lymphokine stimulation of collagen accumulation . J Clin Invest 1976;;58:240-252.
53 +
Postlethwaite AE, Smith GN, Mainardi CL, et al:  Characterization of a human lymphokine that stimulates fibroblasts to produce collagen , abstracted. Arthritis Rheum 1981;; 24 ( (suppl) ):S20.
54 +
Wahl SM, Wahl LM, McCarthy JB, et al:  Macrophage activation by mycobacterial water soluble compounds and synthetic muramyl dipeptide . J Immunol 1979;;122:2226-2231.
55 +
Anastassiades TP, Wood A:  Effect of soluble products from lectin-stimulated lymphocytes on the growth, adhesiveness, and glycosaminoglycan synthesis of cultured synovial fibroblastic cells . J Clin Invest 1981;;68:792-802.
56 +
McArthur W, Derr K, Dixon M, et al:  Immune modulation of connective tissue function: Studies on the production of collagen synthesis inhibitory factor by populations of human peripheral blood mononuclear cells . Cell Immunol 1982;;74:126-139.
57 +
Clark JG, Marino BA, Kostal KM:  Regulation of fibroblast proliferation and collagen synthesis by bronchoalveolar macrophages in bleomycin-induced pulmonary fibrosis in hamsters . Program and abstracts of the joint meeting of the regional connective tissue societies, St Louis, June 9-12, 1982 .
58 +
Schmidt JA, Mizel SB, Cohen D, et al:  Interleukin I: A potential regulator of fibroblast proliferation . J Immunol 1982;; 128:2177-2182.
59 +
LeRoy EC, Mercurio SM, Kahaleh MB:  A fibroblast mitogen with protease activity in scleroderma serum , abstracted. Arthritis Rheum 1982;;25( (suppl) ):S38.
60 +
Bashey RI, Jinenez SA:  Stimulation of fibroblast collagen synthesis by serum from patients with progressive systemic sclerosis (PSS) , abstracted. Arthritis Rheum 1982;:25( (suppl) ):S13.
61 +
Hooks JJ, Moutsopoulos HM, Geis SA, et al:  Immune interferon in the circulation of patients with autoimmune diseases . N Engl J Med 1979;;301:5-8.
62 +
Wahl SM:  Immunologically induced fibrosis , in Fleischmajer R (ed): Diseases of the Skin . New York, Grune & Stratton Inc, 1983;, vol 2.
63 +
Bitterman PB, Crystal RG:  Alveolar macrophages (AM) in idiopathic pulmonary fibrosis (IPF) and sarcoidosis are spontaneously secreting a growth factor causing human lung fibroblasts to replicate . Clin Res 1981;;49:443A.
64 +
Mayes M, Cathcart M, Dustoor M, et al:  The role of immunoregulatory abnormalities in the immunopathogenesis of progressive systemic sclerosis . Proceedings of the Second International Conference on Progressive Systemic Sclerosis, Austin, Oct 20, 1982 .
65 +
Perlish JS, Fleischmajer R:  Mononuclear cell-fibroblast interaction and collagen synthesis, abstracted . Arthritis Rheum 1982;;25( (suppl) ):S14.
66 +
Cathcart MK, Krakauer RS:  Immunological enhancement of collagen accumulation in progressive systemic sclerosis (PSS) . Clin Immunol Immunopathol 1981;;21:128-133.
67 +
Whiteside T, Prince R, Buckingham R, et al:  The effect of lymphokines from normal and PSS lymphocytes on connective tissue synthesis in vitro, abstracted . Arthritis Rheum 1982;; 25( (suppl) ):S14.
68 +
Krakauer RS, Sundeen J, Sauder DN, et al:  Abnormalities of immunoregulation in progressive systemic sclerosis: Evidence for excess helper-cell function and altered B-cell function . Arch Dermatol 1981;;117:80-82.
69 +
Alarcon-Segovia D, Palacios R, DeKasep GI:  Human postthymic precursor cells in health and disease: Immunoregulatory circuits of the peripheral blood mononuclear cell from patients with progressive systemic sclerosis . J Clin Lab Immunol 1981;; 5:143-148.
70 +
Inoshita T, Whiteside TL, Rodnan GP, et al:  Abnormalities of T lymphocyte subsets in patients with progressive systemic sclerosis . J Lab Clin Med 1981;;97:264-277.
71 +
Salem NP, Morse JH:  Lymphocyte responses to mitogens in progressive systemic sclerosis . Arthritis Rheum 1976;;19:875-882.
72 +
Horwitz DA, Garrett MA:  Lymphocyte reactivity to mitogens in subjects with systemic lupus erythematosus, rheumatoid arthritis and scleroderma . Clin Exp Immunol 1977;;27:92-95.
73 +
Morse JH, Budi BS:  Autologous and allogeneic mixed lymphocyte reactions in progressive systemic sclerosis . Arthritis Rheum 1982;;25:390-395.
74 +
Louie JS, Kallen PS, Loper IH, et al:  Decreased autologous mixed lymphocyte reaction (AMLR) in progressive systemic sclerosis (PSS) , abstracted. Arthritis Rheum 1981;;24:( (suppl) ):S91.
75 +
Winkelstein A, Bernstein ML, Stevens MA, et al:  Reduced T lymphoid colony growth in patients with progressive systemic sclerosis and rheumatoid arthritis . J Lab Clin Med 1982;;100:240-247.
76 +
Wright JK, Hughes P, Rowell NR:  Spontaneous lymphocytemediated (NK cell) cytotoxicity in systemic sclerosis: A comparison with antibody-dependent lymphocyte (K cell) cytotoxicity . Ann Rheum Dis 1982;;41:409-413.
77 +
Segond P, Salliere D, Galanaud P, et al:  Impaired primary in vitro antibody response in progressive systemic sclerosis patients: Role of suppressor monocytes . Clin Exp Immunol 1982;;47:147-154.
78 +
Baron M, Keystone EC, Gladman DD, et al:  Lymphocyte subpopulations and reactivity to mitogens in patients with scleroderma . Clin Exp Immunol 1981;;46:70-76.
79 +
Krawitt EL, Holdstock G, Bland JH, et al:  Suppressor cell activity in progressive systemic sclerosis . J Rheumatol 1982;;9:263-267.
80 +
Gupta S, Malaviya AN, Rajagopalan P, et al:  Subsets of human T lymphocytes: Imbalance of T cell populations in patients with progressive systemic sclerosis . Clin Exp Immunol 1979;; 38:342-347.
81 +
Ehresmann GR, Bakke AC, Horwitz DA:  T cell subsets in progressive systemic sclerosis , abstracted. Arthritis Rheum 1982;; 25( (suppl) )S45.
82 +
Keystone EC, Lau C, Gladman DD, et al:  Immunoregulatory T cell subpopulations in patients with scleroderma using monoclonal antibodies . Clin Exp Immunol 1982;;48:443-448.

First Page Preview

First page PDF preview

Figures

Tables

Interactive Graphics

Video

Country-Specific Mortality and Growth Failure in Infancy and Yound Children and Association With Material Stature

Use interactive graphics and maps to view and sort country-specific infant and early dhildhood mortality and growth failure data and their association with maternal

1 +
Fleischmajer R:  The pathophysiology of scleroderma . Int J Dermatol 1977;;16:310-318.
2 +
Fleischmajer R, Gay S, Meigel WN, et al:  Collagen in the cellular and fibrotic stages of scleroderma . Arthritis Rheum 1978;;21:418-428.
3 +
Wahl SM, Wahl LM, McCarthy JB:  Lymphocyte-mediated activation of fibroblast proliferation and collagen production . J Immunol 1978;;121:942-946.
4 +
Wahl SM, Wahl LM:  Modulation of fibroblast growth and function by monokines and lymphokines . Lymphokines 1981;;2:179-201.
5 +
Maricq HR, LeRoy EC:  Patterns of finger capillary abnormalities in connective tissue disease by `wide-field' microscopy . Arthritis Rheum 1973;;16:619-628.
6 +
Fleischmajer R, Dessau W, Timpl R, et al:  Immunofluorescence analysis of collagen, fibronectin, and basement protein in scleroderma skin . J Invest Dermatol 1980;;75:270-275.
7 +
Norton WL:  Comparison of the microangiopathy of systemic lupus erythematosus, dermatomyositis, scleroderma, and diabetes mellitus . Lab Invest 1970;;22:301-308.
8 +
Farkas TG, Sylvester V, Archer D:  The choroidopathy of progressive systemic sclerosis (scleroderma) . Am J Ophthalmol 1972;;74:875-886.
9 +
Fleischmajer R, Perlish JS:  Capillary alterations in scleroderma . J Am Acad Dermatol 1980;;2:161-170.
10 +
Fleischmajer R, Perlish JS, West WP:  Ultrastructure of cutaneous cellular infiltrates in scleroderma . Arch Dermatol 1977;; 113:1661-1666.
11 +
Kahaleh MB, Sherer GK, LeRoy EC:  Endothelial injury in scleroderma . J Exp Med 1979;;149:1326-1335.
12 +
Fleischmajer R, Perlish JS:  3H-Thymidine labeling of dermal endothelial cells in scleroderma . J Invest Dermatol 1977;; 69:379-382.
13 +
Mackel AM, DeLustro F, Harper FE, et al:  Antibodies to collagen in scleroderma . Arthritis Rheum 1982;;25:522-531.
14 +
Woodhall PB, McCoy RC, Gunnells JC, et al:  Apparent recurrence of progressive systemic sclerosis in a renal allograft . JAMA 1976;;236:1032-1034.
15 +
Lapenas D, Rodnan GP, Cavallo T:  Immunopathology of the renal vascular lesion of progressive systemic sclerosis (scleroderma) . Am J Pathol 1978;;91:243-258.
16 +
Kahaleh MB, Osborn I, LeRoy EC:  Increased factor VIII/von Willebrand factor antigen and von Willebrand factor activity in scleroderma and in Raynaud's phenomenon . Ann Intern Med 1981;;94:482-484.
17 +
Kahaleh MB, Osborn I, LeRoy EC:  Elevated levels of circulating platelet aggregates and beta thromboglobulin in scleroderma . Ann Intern Med 1982;;96:610-613.
18 +
D'Angelo WA, Fries JF, Mast AJ, et al:  Pathologic observations in systemic sclerosis (scleroderma) . Am J Med 1969;;46:428-440.
19 +
Sackner MA: Scleroderma . New York, Grune & Stratton Inc, 1966;, pp 77-81.
20 +
 Case 2-1972, Case records of the Massachusetts General Hospital: Weekly clinicopathological exercises . N Engl J Med 1972;;286:91-99.
21 +
Kovacs CV, Fleischmajer R:  Properties of scleroderma fibroblasts in culture . J Invest Dermatol 1974;;63:456-460.
22 +
LeRoy EC:  Connective tissue synthesis by scleroderma skin fibroblasts in cell culture . J Exp Med 1972;;135:1351-1362.
23 +
Ninomiya Y, Hota RI, Nagai Y, et al:  Glycosaminoglycan metabolism by scleroderma fibroblasts in culture . Biomed Res 1982;;3:70-72.
24 +
Fleischmajer R, Perlish JS:  Glycosaminoglycans in scleroderma and scleredema . J Invest Dermatol 1972;;58:129-132.
25 +
Uitto J, Helin G, Helin P, et al:  Connective tissue in scleroderma . Acta Derm Venereol 1971;;51:401-406.
26 +
Bashey RI, Perlish JS, Nochumson S, et al:  Connective tissue synthesis by cultured scleroderma fibroblasts: II. Incorporation of 3H-glucosamine and synthesis of glycosaminoglycans . Arthritis Rheum 1977;;20:879-885.
27 +
LeRoy EC:  Increased collagen synthesis by sckeriderna skin fibroblasts in vitro: A possible defect in the regulation or activation of the scleroderma fibroblast . J Clin Invest 1974;;54:880-889.
28 +
Perlish JS, Bashey RI, Stephens RE, et al:  Connective tissue synthesis by cultured scleroderma fibroblasts: I. In vitro collagen synthesis by normal and scleroderma dermal fibroblasts . Arthritis Rheum 1976;;19:891-901.
29 +
Buckingham RB, Prince RK, Rodnan GP, et al:  Increased collagen accumulation in dermal fibroblast cultures from patients with progressive systemic sclerosis (scleroderma) . J Lab Clin Med 1978;;92:5-21.
30 +
Tajima S, Pinnell S:  Collagen synthesis by human skin fibroblasts in culture: Studies of fibroblasts explanted from papillary and reticular dermis . J Invest Dermatol 1981;;77:410-412.
31 +
Fleischmajer R, Perlish JS, Krieg T, et al:  Variability in collagen and fibronectin synthesis by scleroderma fibroblasts in primary culture . J Invest Dermatol 1981;;76:400-403.
32 +
Botstein GR, Sherer GK, LeRoy EC:  Fibroblast selection in scleroderma . Arthritis Rheum 1982;;25:189-195.
33 +
Graves PN, Weiss IK, Perlish JS, et al:  Increased procollagen mRNA levels in scleroderma skin fibroblasts . J Invest Dermatol 1983;;80:130-132.
34 +
Vaheri A, Mosher DF:  High molecular weights, cell surfaceassociated glycoprotein (fibronectin) lost in malignant transformation . Biochim Biophys Acta 1978;;516:1-25.
35 +
Yamada KM, Olden K:  Fibronectin-adhesive glycoproteins of cell surface and blood . Nature 1978;;275:179-184.
36 +
Foidart JM, Berman JJ, Paglia L, et al:  Synthesis of fibronectin, laminin, and several collagens by a liver-derived epithelial line . Lab Invest 1980;;42:525-532.
37 +
Perlish JS, Carter V, Fleischmajer R:  Scleroderma mononuclear cell factors and collagen synthesis , abstracted. J Invest Dermatol 1982;;78:352.
38 +
Wiestner M, Krieg T, Horlein D, et al:  Inhibiting effect of procollagen peptides on collagen biosynthesis in fibroblast cultures . J Biol Chem 1979;;254:7016-7023.
39 +
Wiestner M, Rohde H, Helle O, et al:  Low rate of procollagen conversion in dermatosparactic sheep fibroblasts is paralleled by increased synthesis of type I and type III collagens . EMBO J 1982;;1:513-516.
40 +
Krieg T, Horlein D, Wiestner M, et al:  Aminoterminal extension peptides from type I procollagen normalize excessive collagen synthesis of scleroderma fibroblasts . Arch Dermatol Res 1978;;263:171-180.
41 +
Perlish JS, Fleischmajer R, Timpl R, et al:  Effect of Col 1 (I) peptide on the synthesis of collagen by scleroderma skin fibroblasts in culture . Arthritis Rheum , in press.
42 +
LeRoy EC:  Pathogenesis of scleroderma (systemic sclerosis) . J Invest Dermatol 1982;;79 ( (suppl 1) ):S87-S84.
43 +
Postlethwaite AE, Snyderman R, Kang AH:  The chemotactic attraction of human fibroblasts to a lymphocyte-derived factor . J Exp Med 1976;;114:1188-1203.
44 +
Tsukamoto Y, Helsel WE, Wahl SM:  Macrophage production of fibronectin: A chemoattractant for fibroblasts . J Immunol 1981;; 127:673-678.
45 +
Rola-Pleszczynski M, Lieu MH, Hamel J, et al:  Stimulated human lymphocytes produce a soluble factor which inhibits fibroblast migration . Cell Immunol 1982;;74:104-110.
46 +
Wahl SM, Gately CL:  Modulation of fibroblast growth by a lymphokine of human T cell and continuous T cell line origin . J Immunol 1982;;130:1226-1230.
47 +
Postlethwaite AE, Kang AH:  Characterization of fibroblast proliferation factors elaborated by antigen- and mitogen-stimulated guinea pig lymph node cells . Cell Immunol 1982;;73:169-178.
48 +
DeLustro F, Sherer GK, LeRoy EC:  Human monocyte stimulation of fibroblast growth by a soluble mediator(s) . J Reticuloendothel Soc 1980;;28:519-632.
49 +
Schmidt JA, Mizel SB, Cohen D, et al:  Interleukin 1: A potential regulator of fibroblast proliferation . J Immunol 1982;; 128:2177-2182.
50 +
Korotzer TI, Page RC, Granger GA, et al:  Regulation of growth of human diploid fibroblasts by factors elaborated by activated lymphoid cells . J Cell Physiol 1982;;111:247-254.
51 +
Korn JH, Halushka PV, LeRoy EC:  Mononuclear cell modulation of connective tissue function . J Clin Invest 1980;;65:543-554.
52 +
Johnson RL, Ziff M:  Lymphokine stimulation of collagen accumulation . J Clin Invest 1976;;58:240-252.
53 +
Postlethwaite AE, Smith GN, Mainardi CL, et al:  Characterization of a human lymphokine that stimulates fibroblasts to produce collagen , abstracted. Arthritis Rheum 1981;; 24 ( (suppl) ):S20.
54 +
Wahl SM, Wahl LM, McCarthy JB, et al:  Macrophage activation by mycobacterial water soluble compounds and synthetic muramyl dipeptide . J Immunol 1979;;122:2226-2231.
55 +
Anastassiades TP, Wood A:  Effect of soluble products from lectin-stimulated lymphocytes on the growth, adhesiveness, and glycosaminoglycan synthesis of cultured synovial fibroblastic cells . J Clin Invest 1981;;68:792-802.
56 +
McArthur W, Derr K, Dixon M, et al:  Immune modulation of connective tissue function: Studies on the production of collagen synthesis inhibitory factor by populations of human peripheral blood mononuclear cells . Cell Immunol 1982;;74:126-139.
57 +
Clark JG, Marino BA, Kostal KM:  Regulation of fibroblast proliferation and collagen synthesis by bronchoalveolar macrophages in bleomycin-induced pulmonary fibrosis in hamsters . Program and abstracts of the joint meeting of the regional connective tissue societies, St Louis, June 9-12, 1982 .
58 +
Schmidt JA, Mizel SB, Cohen D, et al:  Interleukin I: A potential regulator of fibroblast proliferation . J Immunol 1982;; 128:2177-2182.
59 +
LeRoy EC, Mercurio SM, Kahaleh MB:  A fibroblast mitogen with protease activity in scleroderma serum , abstracted. Arthritis Rheum 1982;;25( (suppl) ):S38.
60 +
Bashey RI, Jinenez SA:  Stimulation of fibroblast collagen synthesis by serum from patients with progressive systemic sclerosis (PSS) , abstracted. Arthritis Rheum 1982;:25( (suppl) ):S13.
61 +
Hooks JJ, Moutsopoulos HM, Geis SA, et al:  Immune interferon in the circulation of patients with autoimmune diseases . N Engl J Med 1979;;301:5-8.
62 +
Wahl SM:  Immunologically induced fibrosis , in Fleischmajer R (ed): Diseases of the Skin . New York, Grune & Stratton Inc, 1983;, vol 2.
63 +
Bitterman PB, Crystal RG:  Alveolar macrophages (AM) in idiopathic pulmonary fibrosis (IPF) and sarcoidosis are spontaneously secreting a growth factor causing human lung fibroblasts to replicate . Clin Res 1981;;49:443A.
64 +
Mayes M, Cathcart M, Dustoor M, et al:  The role of immunoregulatory abnormalities in the immunopathogenesis of progressive systemic sclerosis . Proceedings of the Second International Conference on Progressive Systemic Sclerosis, Austin, Oct 20, 1982 .
65 +
Perlish JS, Fleischmajer R:  Mononuclear cell-fibroblast interaction and collagen synthesis, abstracted . Arthritis Rheum 1982;;25( (suppl) ):S14.
66 +
Cathcart MK, Krakauer RS:  Immunological enhancement of collagen accumulation in progressive systemic sclerosis (PSS) . Clin Immunol Immunopathol 1981;;21:128-133.
67 +
Whiteside T, Prince R, Buckingham R, et al:  The effect of lymphokines from normal and PSS lymphocytes on connective tissue synthesis in vitro, abstracted . Arthritis Rheum 1982;; 25( (suppl) ):S14.
68 +
Krakauer RS, Sundeen J, Sauder DN, et al:  Abnormalities of immunoregulation in progressive systemic sclerosis: Evidence for excess helper-cell function and altered B-cell function . Arch Dermatol 1981;;117:80-82.
69 +
Alarcon-Segovia D, Palacios R, DeKasep GI:  Human postthymic precursor cells in health and disease: Immunoregulatory circuits of the peripheral blood mononuclear cell from patients with progressive systemic sclerosis . J Clin Lab Immunol 1981;; 5:143-148.
70 +
Inoshita T, Whiteside TL, Rodnan GP, et al:  Abnormalities of T lymphocyte subsets in patients with progressive systemic sclerosis . J Lab Clin Med 1981;;97:264-277.
71 +
Salem NP, Morse JH:  Lymphocyte responses to mitogens in progressive systemic sclerosis . Arthritis Rheum 1976;;19:875-882.
72 +
Horwitz DA, Garrett MA:  Lymphocyte reactivity to mitogens in subjects with systemic lupus erythematosus, rheumatoid arthritis and scleroderma . Clin Exp Immunol 1977;;27:92-95.
73 +
Morse JH, Budi BS:  Autologous and allogeneic mixed lymphocyte reactions in progressive systemic sclerosis . Arthritis Rheum 1982;;25:390-395.
74 +
Louie JS, Kallen PS, Loper IH, et al:  Decreased autologous mixed lymphocyte reaction (AMLR) in progressive systemic sclerosis (PSS) , abstracted. Arthritis Rheum 1981;;24:( (suppl) ):S91.
75 +
Winkelstein A, Bernstein ML, Stevens MA, et al:  Reduced T lymphoid colony growth in patients with progressive systemic sclerosis and rheumatoid arthritis . J Lab Clin Med 1982;;100:240-247.
76 +
Wright JK, Hughes P, Rowell NR:  Spontaneous lymphocytemediated (NK cell) cytotoxicity in systemic sclerosis: A comparison with antibody-dependent lymphocyte (K cell) cytotoxicity . Ann Rheum Dis 1982;;41:409-413.
77 +
Segond P, Salliere D, Galanaud P, et al:  Impaired primary in vitro antibody response in progressive systemic sclerosis patients: Role of suppressor monocytes . Clin Exp Immunol 1982;;47:147-154.
78 +
Baron M, Keystone EC, Gladman DD, et al:  Lymphocyte subpopulations and reactivity to mitogens in patients with scleroderma . Clin Exp Immunol 1981;;46:70-76.
79 +
Krawitt EL, Holdstock G, Bland JH, et al:  Suppressor cell activity in progressive systemic sclerosis . J Rheumatol 1982;;9:263-267.
80 +
Gupta S, Malaviya AN, Rajagopalan P, et al:  Subsets of human T lymphocytes: Imbalance of T cell populations in patients with progressive systemic sclerosis . Clin Exp Immunol 1979;; 38:342-347.
81 +
Ehresmann GR, Bakke AC, Horwitz DA:  T cell subsets in progressive systemic sclerosis , abstracted. Arthritis Rheum 1982;; 25( (suppl) )S45.
82 +
Keystone EC, Lau C, Gladman DD, et al:  Immunoregulatory T cell subpopulations in patients with scleroderma using monoclonal antibodies . Clin Exp Immunol 1982;;48:443-448.

Correspondence

Submit a Letter
CME Course for:


You need to register in order to view this quiz.


To understand the clinical management of acute heart failure syndromes.
Accreditation Information The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians.
The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity.
Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
Note: You must get at least of the answers correct to pass this quiz.
Note: You must get at least of the answers correct to pass this quiz.
You have not filled in all the answers to complete this quiz
The following questions were not answered:
Sorry, you have unsuccessfully completed this CME quiz with a score of
The following questions were not answered correctly:
For CME Course: A Proposed Model for Initial Assessment and Management of Acute Heart Failure Syndromes
Indicate what changes(s) you will implement in your practice, if any, based on this CME course.
To view and print your certificate and access a summary of your CME courses go to My CME.
NOTE:
Citing articles are presented as examples only. In non-demo SCM6 implementation, integration with CrossRef’s “Cited By” API will populate this tab (http://www.crossref.org/citedby.html).
Compression-Only CPR: Pushing the Science Forward
Cone
AAM 2010;304:1493-1495.
All you need to read in the other general journals
BMJ 2010;341:c5893-c1494.
Submit a Comment

Some tools below are only available to our subscribers or users with an online account.

Related Content

Customize your page view by dragging & repositioning the boxes below.

© 2012 American Medical Association. All Rights Reserved.
Powered bySilverchair Information Systems